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183 Cards in this Set

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Which of the following structures is most echogenic on a neonatal cranial ultrasound?

A. Caudate nucleus

B. Thalamus

C. Cerebellar vermis

D. Corpus callosum

E. Cingulate gyrus
C. Cerebellar vermis
Neonate 3 weeks old, ultrasound scanning of brain reveals small cystic lesions immediately lateral to the thalamus. Likely causes
include:

a. intraventricular hemorrhage

b. intraparenchymal hemorrhage

c. cerebral vasculitis

d. infection

e. anoxia/ischemia
b. intraparenchymal hemorrhage
Premature infants, born less than 34 weeks gestational age or with birth weight less than 1500 grams, are extremely susceptible to ischemic brain injury. Subependymal hemorrage in the residual germinal matrix and
are the two most common forms of hypoxic brain injury in premature infants. The germinal matrix hemorrhage (GMH) also called subependymal or intraventricular hemorrhage, occurs in the residula germinal matrix over the frontal hron and body of the lateral ventricles.
The germinal matrix is a fragile gelatinous mass of tissue found in the fetal brain between the ependyma lining the ventricles and the caudate nucleus. The germinal matrix is highly vascular and is a major source of hemorrhage when it becomes ischemic. The germinal matrix is the source of neuroblasts and spongioblasts which migrate to the brain surface to form the glial cells of the cortex. The germinal matrix involutes by 32 weeks of gestational age, so only premature infants are susceptible to germinal matrix hemorrhage.
Most hemorrhages originate in the region of the caudothalamic groove. Some hemorrhages rupture into the ventricles or they can cause obstruction of the adjacent medullary veins. This obstruction causes infarction along the course of the medullary veins which extend along the floor of the frontal horn of the lateral ventricles and lateral to the thalamus and head of the caudate. The infarction leads to the development of .
A previously normal 2 years old patient presents with dehydration and unresponsiveness. An MRI demonstrates increased T2 weighted intensity in a lesion with mass effect in the bilateral thalami. The most likely diagnosis is:

a. Non-accidental trauma

g. Basilar artery thrombosis

h. Thrombosis of the deep sinus

i. Hemorrhagic teratoma

j. Glioma
h. Thrombosis of the deep sinus
Neonates and infants suffering from dehydration may develop cerebral venous thrombosis. Cerebral venous thrombosis often presents with hemorrhagic infarction in areas atypical for arterial vascular distribution. Venous infarctions frequently are hemorrhagic and commonly occur within the white matter or at the gray-white matter junction. Involvement of the deep cerebral veins (eg, basal vein of Rosenthal) can progress
to bilateral thrombosis of the internal cerebral veins with thalamic hemorrhagic infarction. (emedicine:topic105)
Basilar artery thrombosis would lead to posterior-circulation symptoms; the terminal branch of the basilar artery is the PCA, which supplies the midbrain, thalamus, medial aspect of the temporal lobe, and occipital lobe. Prior to the terminal branch, the superior cerebellar artery branches off and supplies the lateral pons and midbrain and superior surface of the cerebellum. Prognosis is poor. (emedicine:topic407
Atlanto-axial subluxation is seen in all of the following except

A. Down syndrome

b. hypothyroidism

c. trauma

d. juvenile RA

e. Morquio's
b. hypothyroidism
The differential for atlantoaxial subluxation is: (Kirks) Congential:
o o o
 Down syndrome (trisomy 21)  Morquio syndrome = dwarfism w/ LIG laxity  Dysplasia (spondylometaphyseal dysplasia)
Infection:  Retropharyngeal abcess  Rhinopharyngitis
Trauma:  Fracture
 Ligamentous Injury Other
 Rheumatoid Arthritis  Ankylosing spondylitis  SLE  Psoriatic Arthritis
Davidoff-Dyke-Mason Syndrome is characterized by all of the following except:

A. ipsilateral calvarial thickening

B. mastoid air cell hyperpneumatization

C. ipsilateral paranasal sinus hypoplasia

D. ipsilateral enlarged lateral ventricle
ipsilateral paranasal sinus hypoplasia
In Dyke-Davidoff-Masson syndrome, (It may be associated with Sturge-Weber. ) there is hemiatrophy of one hemisphere, with ipsilateral calvarial thickening. The petrous ridge and sphenoid wing may be elevated, and the frontal sinus grossly enlarged ipsilaterally. Cerebral
atrophy causes apparent ipsilateral ventricular enlargement. This denotes a in utero cerebral injury, usually middle cerebral vascular- ischemic cause.
A newborn is noted to have a unilateral small orbit, homogeneous high density globe on CT, and leukokoria. What is the most likely diagnosis?

A) Retinoblastoma

B) Persistent primary vitreous hyperplasia

C) Coat’s disease

D) Retrolental fibroplasia
Persistent primary vitreous hyperplasia
Persistant hyperplastic primary vitreous is a rare condition with persistance and hyperplasia of the vascular system
embryonic hyaloid of primary vitreous. Friable vessels may lead to intravitreal hemorrhage. It presents with unilateral leukokoria. On CT there is micropthalmia, small optic nerve, deformity of the globe and lens, dense vitreous with fluid-fluid levels (breakdown of recurrent hemorrhage), enhancing cone-shaped central retrolental density extending from lens through vitreous body to back of orbit just lateral to optic nerve. No calcifications. (Dahnert)

Retrolental fibroplasia = retinopathy of prematurity = premature infants with respiratory distress syndrome requiring prolonged oxygen therapy. There is leukokoria in severe cases (traction retinal detachment, usually bilateral), dense vitreous, and calcification of the choroid and lens. Would be bilateral

Retinoblastoma is a primary intraocular malignant tumor of childhood arising from photoreceptor cells (neuroectoderm) of the retina. It is the most common malignant tumor of the eye in infants <3 years. Usual age at presentation is 18 months. It presents with leukokoria (whitish mass behind the lens) and is most common in the posterolateral wall of the globe. Note: may be multicentric within the eye (25%) or involve both eyes (25%). On CT there is a solid, smoothly marginated klobulated retrolental hyperdense mass, there may be partial or complete calcification (50-90%) and may have extraocular extension (optic nerve enlargement, abnormal ST in the orbit, intracranial extension. The eye will be normal-sized or enlarged (no micropthalmos)

Coat’s disease (Retinal Telangiectasis, Exudative Retinitis)=pseudoglioma = primary retinal vascular anomaly with telangectasia and detachment of the retina secondary to the accumulation of lipoproteinaceous exudate in the retina and
subretinal space. Presents at 6-8 years withleukocoria (if retina is massively detached) and is seen as unilateral dense vitreous without focal mass or calcification.
Which of the following does not present with leukocoria

a. Coats disease

b. retinoblastoma

c. Persitent primary vitreous hyperplasia

d. Congenital cataracts

e. Optic glioma
e. Optic glioma
Leukokoria DDx: retinoblastoma, retinal detatchment, persistent hyperplastic primary vitreous, Toxocariasis, cataract, retrolental fibroplasia, Coat’s disease, trauma (Primer 602, baby Nelson 330).
MR findings in a 12 year old reveal a prominent optic chiasm and a focus of increased T2 signal within the basal ganglia and cerebellum. This most likely represents?

A. Neurofibromatosis 1

B. Neurofibromatosis 2

C. Tuberous Sclerosis

D. Sturge Weber

E. V on Hippel Lindau
A. Neurofibromatosis 1
1. Six or more cafe café au lait macules greater than 5 mm in diameter in a prepubertal child, and six or more cafe café au lait macules greater than 15 mm in diameter in postpubertal individuals

2. Two or more neurofibromas (any type) or one plexiform neurofibroma

3. Axillary or inguinal freckling

4. Optic pathway glioma

5. Two or more Lisch nodules (multiple pigmented hamartomas of the iris)

6. A distinctive osseous lesion (e.g., sphenoid dysplasia, cortical thinning of a long bone with or without pseudoarthrosis)

7. First-degree relative with NF-I by the above criteria
True regarding myelinization?

a. dorsal to ventral

b. rostral to caudal

c. peripheral to central

d. sensory to motor
a. dorsal to ventral
Myelination usually proceeds from dorsal to ventral, from caudad to cephalad, and from central to peripheral
Although they can be isolated entities, which chromosomal abnormality has been associated with choroid plexus cysts?

a. Trisomy 21
b. Trisomy 13
c. Trisomy 18
c. Trisomy 18
Choroid plexus cysts are seen in ~30% pts with trisomy 18 (Edward syndrome). Answer: C. nomal second-trimester fetus (mainly between 16-21 weeks, and should regress by the 23 week, and are very unsual after 26 weeks). They are typically 0.5 - 2cm and are often multilocular. There is no way to distinguish which patients are normal or have Trisomy 18 based on the appearance of the choroid plexus cyst. If you see a cyst, look for other common abnormalities seen with Trisomy 18 (congenital heart disease, diaphragmatic hernia, omphalocele, esophageal atresia or tracheoesphageal fistula, hydronephrosis, horsehoe kidney, clubfoot deformity, arthrogryposis, clenched hands, large cisterna magna, micrognathia, dolichocephaly, prominent occiput, strawberry-shaped skull).
However, they are seen in 1-4% of
7 year old female presents with precocious puberty. The likely cause for a sellar/suprasellar mass is:

k. prolactinoma

l. hypothalamic glioma

m. hamartoma of the tuber cinereum

n. craniopharyngioma
m. hamartoma of the tuber cinereum
Hypothalamic hamartomas are located in the region between the mamillary bodies an dthe uber cinereum of the hypothalamus. The most common presenting symptom is precocious puberty. Patients with prolactinoma result in delayed menarche. Patients with craniopharyngiomas present with headaches, visual field defects (optic chiasm compression) and anterior pituitary dysfunction (due to compression, and usually manifested as delayed growth). Compression of the hypothalamus results in diabetes insipidus. Patients with hypothalamic gliomas present with diminished visual acuity and short stature, secondary to decreased growth hormone. (barkovich)
A child has unusual seizures. There is a structure which is iso to brain and demonstrates similar enhancement which is anterior to the
mamillary bodies and posterior to the infundibulum?

a. hamartoma of tuber cinereum

b. pituitary adenocarcinoma

c. optic chiasm glioma
a. hamartoma of tuber cinereum
Hypothalamic hamartomas are located in the region between the mamillary bodies an dthe uber cinereum of the hypothalamus. The most common presenting symptom is precocious puberty. Patients with prolactinoma result in delayed menarche. Patients with craniopharyngiomas present with headaches, visual field defects (optic chiasm compression) and anterior pituitary dysfunction (due to compression, and usually manifested as delayed growth). Compression of the hypothalamus results in diabetes insipidus. Patients with hypothalamic gliomas present with diminished visual acuity and short stature, secondary to decreased growth hormone. (barkovich)
A child is found to have a cystic cerebellar lesion with an enhancing mural nodule. This most likely represents?

Astrocytoma

Ependymoma

Medulloblastoma

Brainstem glioma Choroid plexus papilloma
Astrocytoma
Answer: A. is the second most common tumor of the posterior fossa in children 10-20%. Classically a cystic lesion with a tumor nodule (mural nodule) in the cyst wall (50%), solid mass with cystic center 40-45%. Calcifications in 20%.
Ependymoma is 15% of pediatric posterior fossa tumors. Majority are benign, slow-growing tumors of mature well-differentiated ependymal cells. Location: floor of fourth ventricle (70%) of all intracranial ependymomas. CT: sharply marginated multilobulated iso- slightly hyperdense 4th ventricular mass with heterogeneous to moderately uniform enhancement of the solid portions (80%).
Medulloblastoma is the most common neoplasm of the posterior fossa in children 30-40%. Classic features in 53%: 70% hyperdense, rapid intense homogeneous enhancement 97%. Atypical features: cystic/necrotic areas 10-16%, calcifications 13%. May metastasize to subarachnoid with drop mets.
Brainstem glioma is 20-30% of all infratentorial brain tumors, 12-15% of all pediatric brain tumors. Usually an anaplastic astrocytoma / GBM. Location: pons > midbrain > medulla. CT: iso / hyopdense mass with indistinct margins, absent / minimal / patchy contrast enhancement (50%), ring enhancement in necrotic / aggressive tumor, engulfment of basilar artery.
Choroid plexus papilloma 2-5% of all brain tumors in children. Location: glomus of choroids plexus in trigone of lateral ventricles L > R (in children). Large mass with smooth lobulated border and small foci of calcifications. Distinctive feature: engulfment of the glomus of the choroids plexus. Ventricular dilatation secondary to overproduction / decreased resorption of CSF. CT: iso- to mildly hyperdense with intense homogeneous enhancement with contrast.
Cerebellar astrocytoma
Child has an enhancing mass arising from vermis and bulging into 4th ventricle No hemorrhage is seen. Dx?

a. Astrocytoma

b. Brainstem glioma

c. Ependymoma

d. Hemangioblastoma

e. Medulloblastoma
e. Medulloblastoma
A PNET is a malignant, embryonal childhood tumor arising in the cerebellum composed of “small round blue cells”. A PNET usually arises in the cerebellum and is centered in the midline. The mass is homogeneous and spherical in shape, growing as if a solid expanding ball. Hemorrhage, cysts, and calcifications occur in approximately 10% of cases. The residual lumen of the 4th ventricle may be seen as a crescent of CSF density or intensity along the anterior surface of the mass.
On CT, the tumor frequently appears of homogeneous hyperattenuation relative to gray matter. On MR, the mass is homogeneously hypointense on T1- and usually hypo- or isointense on T2-weighted images relative to gray matter. By
either CT or MR, the PNET homogeneously enhances following intravenous contrast injection. Treatment is complete surgical resection followed by aggressive chemotherapy and/or radiation therapy.
A complete metastatic work-up is needed as up to 30% of patients with PNETs have leptomeningeal spread of tumor at the time of diagnosis. Older children at presentation typically have much better 5-year survival rates.
Low lying cerebellar tonsils (Chiari I malformation) is most closely associated with?

A. Meningomyelocele

B. Prominent massa intermedia

C. Small posterior fossa

D. Beaking of the tectum

E. Syringomyelia
E. Syringomyelia
The Chiari I malformation consists of protrusion of the cerebellar tonsils downward through the foramen magnum; obliteration of the cisterna magna; and variable, usually very mild, elongation and caudal displacement of the fourth ventricle, brain stem, and cerebellum. Chiari I malformation is associated with hydrosyringomyelia (60%-70% of patients), hydrocephalus (20%- 25%), and segmentation anomalies of the craniovertebral junction including basilar impression (25%), assimilation of C1 to the occiput (10%), Klippel-Feil (10%), and incomplete ossification of the C1 ring (5%). Chiari I is not associated with myelomeningocele.
The Chiari II malformation is the most common serious malformation of the posterior fossa. It is a complex deformity of the calvarium, dura, and hindbrain, which nearly always is associated with spina bifida and (meningo) myelocele. In aggregate, the changes create a bony and dural posterior fossa that is too small for the brain stem and cerebellum. Then, as the brain grows, it erodes the walls of the posterior fossa and extrudes into the spinal canal below and the supratentorial space above. In this process, the posterior fossa contents mold and become molded by the structures upon which they impinge. The changes induced in the very fluid immature brain become set by the process of myelination and appear to persist relatively unchanged despite later alterations in the bone. The midbrain is abnormal in nearly every case. Characteristically, the four colliculi are at least partially fused into a conical mass designated the "beak". Since the beaking affects the inferior colliculi more than the superior colliculi, beaking is seen best on low CT sections caudal to the expected position of midbrain. The upper midbrain may, in fact, appear deceptively normal.
Many patients with Dandy-Walker malformation show persistent fetal position of tentorium and sinuses, so the tentorium lies in a nearly vertical position along the parietal bones and the torcular lies far above the lambda (torcular-lambdoid inversion). The posterior tentorium may be dehiscent with terminal duplication of the straight sinus. The incisura in the tentorium is inclined nearly vertically with the tentorial leaves and is often wider than normal. In axial CT sections, the tentorial leaves and the incisura usually form an inverted-V
Leukenshadel is most commonly associated with?

A. Hydrocephalus

B. Craniosynostosis

C. Dandy Walker

D. Myelomenigocele

E. Small foramen magnum
D. Myelomenigocele
Nearly all patients with Chiari II malformation (thus presenting with myelomeningocele) manifest a dysplasia
of the membranous bones of calvarium called Lückenschädel.
This appears as clusters of thinnings, pits, and fenestrae that are most prominent near the vertex or the torcula. Lückenschädel may be observed in a fetus in utero as early as at 8 months of gestation. It typically persists until 1 to 3 months of age and then disappears after approximately age 6 months, whether or not there is progressive hydrocephalus.
Picture of a line graft that shows progressive increase in size of ventricles in head of fetus with increasing gestational age, from 15mm to 20mm, with preserved normal thickness of mantle and head circumferance. What is the most likely etiology?

a. Hydranencephaly

b. Normal

c. Absence of the corpus callosum

d. Holoprosencephaly

e. Myelomeningocele
e. Myelomeningocele
Normal ventricular size is less than 1 cm. 30% of fetal ventriculomegaly cases are associated with Chiari / myelomeningocele
On fetal US, a myelomeningocoele is a/w?

Obliterated cisterna magna
Obliterated cisterna magna
A 7 year old patient with scoliosis and bilateral lower extremity weakness and leg length discrepancy (left greater than right). The patient also has a sacral dimple with a hairy patch. This is most consistent with?

A. Tethered cord

B. Chiari II with lipomeningocele

C. Astrocytoma

D. Ependymoma of the conus medullaris

E. Meningocele
A. Tethered cord
Tethered cord The most common signs of tethered cord syndrome include loss of function in the legs and/or loss of bowel or bladder control. Other symptoms and signs include:
Decrease in strength of legs or feet, deformity of the legs or hips, loss of reflexes and sensation in the legs Stumbling or walking changes Leg or back pain Curvature of the spine Skin abnormalities directly overlying the spinal cord – midline dimples - sinuses or tracts leading from the skin toward the spinal cord, birthmarks, hairy patches, skin discoloration, skin tags or fatty lumps..
Which of the following is commonly seen in a fetus of a diabetic mother?

a. Sacral teratoma

b. Myelomeningocele

c. Caudal regression syndrome

d. Syrulgohydromyelia
c. Caudal regression syndrome
20% of children with sacral agenesis have mom's with DM.
Which is true about branchial cleft cysts?

a. Usually type I

b. Usually muliseptated

c. Present at angle of mandible

d. Presents in adolescence
c. Present at angle of mandible
Branchial cleft cysts are congenital epithelial cysts derived from the 1st or 2nd (most common) cervical pouch and are the most common congenital cause of a neck mass. An estimated 2-3% of cases are bilateral. Many branchial cleft cysts are asymptomatic.
Imaging: water density (<20 HU) unless infected (debris); intense rim enhancement in infected cysts.

First branchial cleft cyst (5-8%) = Parotid lymphoepithelial cyst
Residual embryonic tract begins near submandibular triangle and ascends through the parotid gland, terminates at junction of cartilaginous and bony external auditory canal Age: middle aged women
*enlarging mass near lower pole of parotid gland *recurrent parotid abscesses *+/- facial nerve palsy *otorrhea (if cyst drains into EAC)

Second branchial cleft cyst (95%)
Incomplete obliteration of 2nd branchial cleft tract (cervical sinus of His) Age: 10-40 years; M=F Bailey classification: Type 1: along anterior surface of SCM, just deep to platysma
Type 2: along anterior surface of SCM, lateral to carotid space, posterior to submandibular gland adhering to the great vessels (most common) Type 3: at bifurcation of external and internal carotid arteries Type 4: within pharyngeal mucosal space
*oval/round cyst near mandibular angle CT/MR: “beak sign” = curved rim of tissue pointing medially between internal and external carotid arteries (pathognomonic)

Third branchial cleft fistula/cyst (extremely rare)
Above superior laryngeal nerve Unilocular cystic mass within posterior cervical space
Fourth branchial fistula (extremely rare, R>L)
Below superior laryngeal nerve Recurrent episodes of “suppurative thyroiditis”/ neck abscesses
Which is true about developmental venous anomaly?

a. Usually not seen at angio

b. Usually not seen on MRI

c. Associated with cavernous malformations
c. Associated with cavernous malformations
Venous vascular malformations, also known as venous angiomas or, more properly, developmental venous anomalies (DVAs), represent congenital anatomically variant pathways in the normal venous drainage of an area of the brain. DVAs are associated with other CNS vascular malformations (eg, arteriovenous malformation [AVM], cavernous malformation, capillary telangiectasia) in approximately 15-30% of patients, although it most frequently occurs in conjunction with cavernous angiomas. MRI findings are diagnostic in almost all instances. However, in cases with questionable findings, MR venography usually suggests the diagnosis.
MRI Findings: On contrast-enhanced MRI, the cluster of veins in DVAs has a spoke-wheel appearance; the veins are small at the periphery and gradually enlarge as they approach a central draining vein. This appearance has also been referred to as caput medusa, or the head of Medusa, because of the serpentine appearance of the curvilinear peripheral draining veins. The intervening brain parenchyma is normal; this is a distinguishing characteristic of a DVA. Angiogram Findings: DVAs found on angiograms are almost invariably incidental findings, as they are with newer MRIs, and the diagnosis can be made in almost every instance. When observed, the DVA appears as a blush of contrast enhancement during the venous phase of the study and drains into a large anatomically anomalous vein. In its most frequent location (adjacent to the lateral ventricles), the vein usually drains into a subependymal vein, although superficial drainage also occurs.
What is true regarding venous malformations?

a. grows rapidly during infancy

b. involutes during childhood

c. is not readily apparent at birth

d. growth proportional to age
d. growth proportional to age
Picture of a line graft that shows progressive increase in size of ventricles in head of fetus with increasing gestational age, from 15mm to 20mm, with preserved normal thickness of mantle and head circumferance. What is the most likely etiology?

a. Hydranencephaly

b. Normal

c. Absence of the corpus callosum

d. Holoprosencephaly

e. Myelomeningocele
e. Myelomeningocele
Normal ventricular size is less than 1 cm. 30% of fetal ventriculomegaly cases are associated with Chiari / myelomeningocele
On fetal US, a myelomeningocoele is a/w?

Obliterated cisterna magna
Obliterated cisterna magna
Sturge-Weber is associated with the following except:

a. pial angiomatosis

b. unilateral choiroid plexus hypertrophy

c. strawberry angioma in the ophthalmic division of CN V

d. dilated ependymal veins
c. strawberry angioma in the ophthalmic division of CN V
Sturge-weber is associated with in the ophthalmic division of CN V. It is also known as encephalotirgeminal angiomatosis.

Dilated ependymal veins (and their flow voids) as well as hypertrophy of the choroids plexus is seen on the ipsilateral side as the nevus. Pial angiomatosis is demonstrated with gadolinium and leads to age-dependant calcifications which are seen as gyral cortical calcifications on CT. Pial angiomatosis also causes chronic ischemia of the gray matter which results in gyral atrophy and gliosis. Gradient recalled echo MRI is used to demonstrate the dysplasia
Which is most common presentation of a tethered cord?

A. Chronic Diarrhea

B. Back Pain

C. Scoliosis

D. Recurrent UTI

E. Lower Extremity Paralysis

F. GU anomaly
B. Back Pain
Scoliosis 20%. pain, parasthesias, nerogenic bladder, spasticity. Danhert 223. Chronic diarrhea most common in older children.
Which of the following is commonly seen in a fetus of a diabetic mother?

a. Sacral teratoma

b. Myelomeningocele

c. Caudal regression syndrome

d. Syrulgohydromyelia
c. Caudal regression syndrome
20% of children with sacral agenesis have mom's with DM.
Which is true about branchial cleft cysts?

a. Usually type I

b. Usually muliseptated

c. Present at angle of mandible

d. Presents in adolescence
c. Present at angle of mandible
Branchial cleft cysts are congenital epithelial cysts derived from the 1st or 2nd (most common) cervical pouch and are the most common congenital cause of a neck mass. An estimated 2-3% of cases are bilateral. Many branchial cleft cysts are asymptomatic.
Imaging: water density (<20 HU) unless infected (debris); intense rim enhancement in infected cysts.

First branchial cleft cyst (5-8%) = Parotid lymphoepithelial cyst
Residual embryonic tract begins near submandibular triangle and ascends through the parotid gland, terminates at junction of cartilaginous and bony external auditory canal Age: middle aged women
*enlarging mass near lower pole of parotid gland *recurrent parotid abscesses *+/- facial nerve palsy *otorrhea (if cyst drains into EAC)

Second branchial cleft cyst (95%)
Incomplete obliteration of 2nd branchial cleft tract (cervical sinus of His) Age: 10-40 years; M=F Bailey classification: Type 1: along anterior surface of SCM, just deep to platysma
Type 2: along anterior surface of SCM, lateral to carotid space, posterior to submandibular gland adhering to the great vessels (most common) Type 3: at bifurcation of external and internal carotid arteries Type 4: within pharyngeal mucosal space
*oval/round cyst near mandibular angle CT/MR: “beak sign” = curved rim of tissue pointing medially between internal and external carotid arteries (pathognomonic)

Third branchial cleft fistula/cyst (extremely rare)
Above superior laryngeal nerve Unilocular cystic mass within posterior cervical space
Fourth branchial fistula (extremely rare, R>L)
Below superior laryngeal nerve Recurrent episodes of “suppurative thyroiditis”/ neck abscesses
Which is true about developmental venous anomaly?

a. Usually not seen at angio

b. Usually not seen on MRI

c. Associated with cavernous malformations
c. Associated with cavernous malformations
Venous vascular malformations, also known as venous angiomas or, more properly, developmental venous anomalies (DVAs), represent congenital anatomically variant pathways in the normal venous drainage of an area of the brain. DVAs are associated with other CNS vascular malformations (eg, arteriovenous malformation [AVM], cavernous malformation, capillary telangiectasia) in approximately 15-30% of patients, although it most frequently occurs in conjunction with cavernous angiomas. MRI findings are diagnostic in almost all instances. However, in cases with questionable findings, MR venography usually suggests the diagnosis.
MRI Findings: On contrast-enhanced MRI, the cluster of veins in DVAs has a spoke-wheel appearance; the veins are small at the periphery and gradually enlarge as they approach a central draining vein. This appearance has also been referred to as caput medusa, or the head of Medusa, because of the serpentine appearance of the curvilinear peripheral draining veins. The intervening brain parenchyma is normal; this is a distinguishing characteristic of a DVA. Angiogram Findings: DVAs found on angiograms are almost invariably incidental findings, as they are with newer MRIs, and the diagnosis can be made in almost every instance. When observed, the DVA appears as a blush of contrast enhancement during the venous phase of the study and drains into a large anatomically anomalous vein. In its most frequent location (adjacent to the lateral ventricles), the vein usually drains into a subependymal vein, although superficial drainage also occurs.
What is true regarding venous malformations?

a. grows rapidly during infancy

b. involutes during childhood

c. is not readily apparent at birth

d. growth proportional to age
d. growth proportional to age
What type of lesion would you expect in a patient who is initially hypoxic and does not correct with 100% O2 administration?

a. right to left shunt

b. left to right shunt

c. V/Q mismatch
a. right to left shunt
On prenatal ultrasound, outlet view shows two parallel vessels. (or: the pulmonary artery and ascending aorta are seen in parallel).

a. Tetralogy of Fallot

b. Normal

c. Truncus arteriosus

d. Transposition of great vessels

e. Hypoplastic left heart

f. Tricuspid atresia
d. Transposition of great vessels
Which of the following entities requires a PDA for survival?

A) VSD

B) Transposition

C) hypoplastic left heart

D) TAPVR

E) Truncus type 2
C) hypoplastic left heart
Hypoplastic left heart is underdevelopment of the left side of the heart characterized by aortic valve atresia, hypoplastic ascending
aorta, hypoplastic/atretic mitral valve and endocardial fibroelastosis giving rise to small LA, small LV, and small ascending aorta. Pulmonary venous return is diverted from LA to RA via ASD. RV supplies PAPDA which supplies the descending aorta (anterograde) and ascending aorta / arch / coronaries (retrograde). PDA is necessary as there is no functional LV so it’s the only pathway to get blood into the aorta. MC CAUSE OF CARDIAC DEATH IN 1ST WEEK OF LIFE. REQUIRES PDA TO SUPPLY SYSTEMIC BLOOD.
Transposition is characterized by aorta originating from RV and PA originating from LV. Admixture of blood between the 2 circulations is necessary for survival via some combination of PDA, PFO, and VSD, however if you have a PFO and a VSD you don’t necessarily need a PDA, so C is still a better answer.
In TAPVR there is anomalous connection between the pulmonary and systemic veins secondary to embryologic failure of the common pulmonary vein to join the posterior wall of the left atrium. There is a L -> R shunt from pulmonary veins to right atrium. and an ASD is necessary to return oxygenated blood to the left side.
Truncus is characterized by one great artery arising from the heart giving rise to the pulmonary, coronary, and systemic arteries. The classification system is based on the origin of the pulmonary arteries. I = commoin origin from aortic trunk, II = same orifice / III = separate orifices ascending trunk or IV = separately from descending trunk. Since a PDA causes admixture between PA and aorta, clearly that would be redundant.
Note: As a general rule a PDA is nonbeneficial in all conditions resulting in a L -> R shunt since it increases shunt volume and adds to CHF.
What is the appropriate description of congenitally corrected transposition?

a. atrio-ventricular discordance with ventriculo-arterial discordance

b. atrioventricular concordance with ventriculoarterial discordance

c. atrioventricular discordance with ventriculoarterial concordance

d. atrioventricular concordance with ventricularterial concordance
a. atrio-ventricular discordance with ventriculo-arterial discordance
What is associated with sinus venosus ASD?

a. Anomalous pulmonary venous return

b. Interruption of the IVC with azygous continuation
a. Anomalous pulmonary venous return
An atrial septal defect (ASD) is a deficiency of the atrial septum. ASDs account for about 10-15% of all congenital cardiac anomalies and are the most common congenital cardiac lesion presenting in adults. Sinus venosus ASDs account for only 10% of ASDs. The remaining ASDs are ostium secundum type (70%), ostium primum type (20%), and unroofed coronary sinus (coronary sinus septal defects) (<1%). Almost all patients with sinus venosus ASD become symptomatic when they are younger than 40 years, but excellent surgical results with a mortality rate near 0% can be expected.
The more common sinus venosus type defect occurs in the upper atrial septum and is contiguous with the superior vena cava (SVC). The lesion is rostral and posterior to the fossa ovalis (where secundum type defects occur) and is separate from it. . It is almost always associated with anomalous pulmonary venous drainage of the right upper pulmonary vein into the SVC. Less commonly, the defect may occur at the junction of the right atrium and inferior vena cava (IVC) and be associated with anomalous connection of the right lower pulmonary vein to
the IVC. Rarely, sinus venosus defects occur posterior to the fossa ovalis without bordering the SVC or IVC. The predominant hemodynamic consequence is a left-to-right shunt through the defect. Chronic atrial fibrillation is often present in older patients and may trigger decompensation.
A newborn presents with pulmonary edema and a normal sized cardiac sihlouette. This most likely represents:

a. Tetralogy of Fallot

b. Vein of Galen aneurysm

c. Total anomalous pulmonary venous return with obstruction

d. Anomalous left coronary artery
c. Total anomalous pulmonary venous return with obstruction
What is the most likely plain film finding within the tibia of a child undergoing prolonged prostaglandin therapy for congenital heart deisease?

A. Periosteal elevation

B. Sclerosis

C. Osteopenia

D. Osteoporosis

E. Osteomalacia
A. Periosteal elevation
Periosteal reaction in childhood: “DR SPOCK”:, D vitamin, rickets, scurvy, syphilis, sickle cell, physiologic or ,
osteomyelitis, osteosarcoma, child abuse, kinky hair syndrome.
What is associated with juxtaductal aortic coarctation?

a. trisomy 21

b. Turners (XO)

c. Holt-Orham
b. Turners (XO)
What is the most common (? complete)vascular ring?

a. Right aortic arch with aberrant left subclavian artery

b. Left aortic arch with aberrant right subclavian artery
a. Right aortic arch with aberrant left subclavian artery
Differentiate Meconium aspiration from Hyaline Memb Dz on CXR??

A. pneumomediastinum

B. hyperinflation

C. Air Bronchograms

D. Interstital emphysema
B. hyperinflation
Diagnosis is suspected when a neonate demonstrates respiratory distress in the setting of meconium-tinged amniotic fluid and is confirmed by chest x-ray demonstrating hyperinflation with variable areas of atelectasis and flattening of the diaphragm. Fluid may be seen in the lung fissures or pleural spaces, and air may be seen in the soft tissues or mediastinum.
Resp dist in full term infant—inhalation of meconium which causes chem. Pneumonitis of medium and small airways...10% deliveries have mec stained amn fluid, 1% have resp distress.. CXR: Bil diffuse gross patchy opacities, Hyperinflation with areas of emphysema, No air Bronchograms!!!, spont PTX and Pneumomediastinum (20%) that require no therapy..
HMD—Da 520—Decreased surfactant=Inc surface tension-Inc risk-premature, diabetic moms, c section.. slight male predominance..

Meconium Aspiration
Bil diffuse gross patchy opacities, Hyperinflation with areas of emphysema, No air Bronchograms!!!, spont PTX and Pneumomediastinum (20%) that require no therapy..

HMD
Low lung vols, bilateral and symmetric, diffuse granularity(vis dist term bronchioles and alv ducts against a background of alv atelectasis), prominent air bronchograms... Tx Surfactant
What finding on chest x-ray can best differentiate untreated hyaline membrane disease from fetal wet lung syndrome?

a. Pleural effusion

b. Resolution within 24 hours

c. Low lung volumes

d. Kerley B lines
c. Low lung volumes
Which of the following regarding Swyer-James is FALSE?

a. Hyperlucent lung

b. Air trapping

c. Result of prior viral infection

d. hyperinflation
d. hyperinflation
Severe pulmonary hypoplasia is seen in which of the following?

a. Diaphragmatic hernia

b. Intralobar sequestration

c. Congenital emphysema

d. Extralobar sequestration
a. Diaphragmatic hernia
Which Foregut malformation is most likely to communicate with the bronchopulmonary tree?

a) Mediastinal bronchogenic cyst

b) Intrapulmonary bronchogenic cyst

c) CCAM

d) Intralobar sequestration

e) Extralobar sequestration
c) CCAM
19 y/o with hyperlucent left upper lobe. Branching mass in left Hilum. Diagnosis?

a) congenital lobar overinflation

b) CCAM

c) Segmental bronchial atresia

d) Sequestration

e) Pulmonary AVM
c) Segmental bronchial atresia
CLO—progressive overdist of one/multiple lobes. Males 3:1, associated with CHD 15%(PDA, VSD) ; LUL>RML>RUL Present:1 6 months of life. Compress collapse adj lobes. May be caused by endobronchial obstruction. Tx:surgery (478 Da,478 BH)
Segmental bronchial atresia
st
Bronch-Atresia---Apperaant later in life/early adulthood. LUL>>>RUL>RML. Overexpanded/lucent segment. Mucus Plug of AtreticBronchus Tx: None(asymptomatic) 464Da, 478BH
CCAM-25%cong lung. TYPE 1-3. 1 single lg cyst to type 3 mass like secondary to innumerable small cysts. Clin: Neonate-resp distress and cyanosis. Loc:equal freq all lobes. CXR:Unilat exp mass with well def margins 80%. 480Da, 475 BH
Sequestration—Non fx lung segment. Intralobar (~80%) Clin:adults with recur pneumonias. L>R(3:2) 1lg artery—numerous pulm veins. Extralobar Clin:Neonate variable present. L>>R(4:1) variable #(mc systemic a)-systemic veins. Inc Cong anomalies---diap hernia (20- 30%) Da 472, BH 478 Pulm AVM- simple 80% vs complex 20% --1 vs mult feed arteries. Loc:lower lobes 70%. Asympto:56%. Tx: Emboize coils/detach balloons... Da 514
Which of the following mediastinal masses is least likely to contain calcifications without previous treatment?

a. Lymphoma

b. Teratoma

c. Some variety of thymic mass...
a. Lymphoma
Most ASSOCIATED with necrotizing/cavitary pneumonia in a child?

a. staph aureus

b. pneumococcus

c. mycoplasma

d. TB
b. pneumococcus
What is the most likely etiology of round pneumonia in the pediatric population?

a. Strep pneumoniae.

b. Histoplasmosis.

c. Staph aureus

d. Mycoplasma.

e. Pseudomonas.

f. H. influenzae.
a. Strep pneumoniae.
2 yo w/ SOB runny nose & CXR showing hyperinflation, peribrochial cuffing, & interstitial prominence. Most likely dx?
Viral pneumonia
Male child status post bone marrow transplant presents with fever and cough and a 3 cm nodule in the right upper lung:

A) Invasive aspergillosis

B) Candida

C) E.Coli infection

D) MAI

E) TB

F) PCP
A) Invasive aspergillosis
19y/o man has history of cough and chest pain. He presents with a palpable mass on his left back , and a chest x-ray reveals left lower lobe opacity. Which of the following is most likely?

A. TB

B. actinomyces

C. nocardia

D. streptococcus
B. actinomyces
Asymptomatic child with HIV and diffuse pulmonary nodularity likely represents:

a. Pneumocystis carinii

b. LIP

c. Lymphoma
b. LIP
PCP-Early interstitial disease progressing to airspace disease. Early symptoms may rapidly progress to respiratory failure. kirks. LIP-
LIP
Indicative of AIDS in children under 13. Diffuse reticulonodular pattern. Usually stable disease, progressive in 33% of pts.
Most common medistinal mass in a child:

x. neuroblastoma

y. lymphoma

z. teratoma

aa. bronchogenic cyst

bb. germ cell tumor
y. lymphoma
Lymphoma presents as an anterior or middle mediastinal mass. After leukemia and CNS tumors, lymphoma is the 3rd most common neoplasm in children <16 years old. 60% of childhood lymphoma is non-Hodgkins type with peak age between 7-11 years old. Neuroblastoma is the most common mediastinal mass for children < 2 yrs old, and presents as a posterior mediastinal mass. Bronchogenic cysts are middle-mediastinal masses, or pulmonary parenchymal masses. These are the most common intrathoracic foregut cysts. Mediastinal and lung neurofibromas, and intrathoracic or lateral thoracic meningoceles are associated with neurofibromatosis type I. Paraspinal neurofibromas are associated with neurofibromatosis types 1 and 2. These are posterior mediastinal masses, and are less common than other mediastinal masses. NF-I is the most common of the phakomatoses. Germ cell tumors do not present until late (20-40 years). (Kirks)
Eight year old, previously well child presents with bulky mediastinal adenopathy and SVC obstruction.

cc. lymphoma

dd. fibrosing mediastinitis

ee. histoplasmosis

ff. TB
cc. lymphoma
Most common pediatric chest WALL mass?
Ewings
Child with SVC syndrome and diffuse mediastinal calcifications is most likely the result of:

A) TB

B) Histoplasmosis

C) Lymphoma

D) Teratoma
B) Histoplasmosis
Afebrile neonate presents with stridor. On plain radiographs, there is eccentric narrowing of the subglottic trachea. Most likely cause?

a. Croup

b. Hemangioma

c. Epidermoid

d. Papilloma
b. Hemangioma
Causes of subglottic narrowing:
CONGENITAL
SUBGLOTTIC STENOSIS: cricoid malformation in utero; circumferential narrowing of subglottic trachea during inspiration, no change during expiration.

INFLAMMATORY
CROUP: symmetric funnel-shaped narrowing below lower margin of pyriform sinuses on AP radiograph, accentuated on expiration, with paradoxic inspiratory collapse. Age range is 6 months to 3 years (after maternal antibodies ebb)

NEOPLASTIC
SUBGLOTTIC HEMANGIOMA: the most common soft tissue mass causing respiratory obstruction in neonates . Croup-like symptoms. 50% have hemangiomas elsewhere. Eccentric subglottic trachea. Arises from posterior wall below true cords (lateral view).

PAPILLOMA: sqamous papilloma is the most common benign tumor of the larynx, caused by HPV type 6. Can occur in < 10 years (diffusely / papillomatosis) or typically singular in 21-50 y age range.

TRAUMATIC ACQUIRED STENOSIS: granuloma as result of prolonged endotracheal intubation (5%).

IDIOPATHIC
MUCOCELE: mucous retention cyst can be a rare complication of prolonged intubation.
Membranous croup is caused by?

a. H. influenza type B

b. Staph aureus

c. E. coli

d. parainfluenza virus
b. Staph aureus
(VIRAL) croup is caused by parainfluenza.

Membranous croup is most commonly caused by Staphylococcus aureus.
What is the most common cause of calcified tracheal rings?

a. right aortic arch with aberrant left subclavian artery

b. left aortic arch with aberrant right subclavian artery

c. pulmonary sling
c. pulmonary sling
Simple pulmonary AVM in child. How do you embolize?

a. Coils

b. Gelfoam

c. EtOH
a. Coils
The most common pattern of esophageal atresia is:

A. EA with proximal TEF

B. EA without TEF

C. EA with proximal and distal TEF

D. EA with distal TEF

E. EA with H-type TEF
D. EA with distal TEF
The most common pattern of esophageal atresia is a fistula between the distal esophageal segment and the trachea (85%, aka “N” fistula or isolated esophageal atresia). The next most common is no fistula (10%). Other types are H-fistula (4%, aka isolated TEF), both proximal and distal fistula (1%), and proximal fistula (1%). (emedicine)
Gastric Duplication cyst, most common location???

A. cardia

B. posterior fundus

C. mid greater curvature

D. distal greater curvature

E. pylorus
D. distal greater curvature
Atresia of which of these segments is associated with other congenital anomalies:

a. Duodenum

b. Jejunum

c. Ilium

d. Right colon

e. Left colon
a. Duodenum
Duodenal obstruction is usually caused by duodenal , annular pancreas, and midgut volvulus. Less frequently, it may be secondary to duodenal web, Ladd bands, or preduodenal portal vein. Duodenal is the most important cause of complete duodenal obstruction. The cause of this condition is thought to be failure of recanalization of the duodenum between approximately the 9th and 11th weeks of gestation. Unlike jejunal and ileal , this condition does not appear to be related to intrauterine vascular accidents. Major associated anomalies are present in about 50% of cases. Approximately 30% of patients have Down syndrome. Other anomalies include malrotation of the small , esophageal , congenital heart disease, imperforate anus, small , biliary , annular pancreas, and renal anomalies
Meckel’s diverticulum is a remnant of which of the following?

A) Urachus

B) Omphalomesenteric duct

C) Ligamentum teres
B) Omphalomesenteric duct
A Meckel's diverticulum is a persistance of the omphalomesenteric duct (vitelline duct) which usually obliterates by the 5th embryonic week. It is the most common anomaly of the GI tract. Remember the rule of 2s: 2% of population, symptomatic before age 2, located within 2 feet of the iliocecal valve (on antimesenteric border), 2 inches long. May contain heterotpoic tissue in 12-16% (gastric/pancreatic/colonic). Can cause GI bleeding (secondary to ulceration), acute diverticulitis, intestinal obstruction (secondary to intussusception/fibrous bands/volvulus), malignant tumor (carcinoma/sarcoma/carcinoid), and chronic abdominal pain. NUCS scan (Tc-99 pertechnetate, 5-20 mCi) >85% sensitive, >95% specific. (Dahnert)
The most reliable sign to diagnose malrotation is:

a. cecum is in the right upper quadrant

b. jejunum is to the right of the spine

c. duodeno-jejunal junction is
inferomedially positioned

d. proximal jejunal loops to the right of the spine
c. duodeno-jejunal junction is
inferomedially positioned
Which is the most specific finding for malrotation?

a. Proximal jejunum to the right of the spine

b. Inferior medial displacement of the duodenal jejunal junction

c. High and medial location of the cecum

d. Sigilloid volvulus
b. Inferior medial displacement of the duodenal jejunal junction
A child with known aortic coarctation develops pneumotosis intestinalis on flat plate of the abdomen. What is the most appropriate imaging study to do next?

A) CT with oral and IV contrast

B) Barium enema

C) Cross-table lateral of the abdomen

D) Ultrasound of the aorta to rule out clot

E) Mesenteric angiogram
C) Cross-table lateral of the abdomen
Because the radiographic appearance of pneumatosis in a child with necrotizing enterocolitis is pathognomonic, especially if it is associated with pneumoperitoneum or portal vein gas, a barium enema examination is rarely needed for diagnosis, and considered hazardous. Necrotizing enterocolitis is a complication of neonatal stress particularly common in the premature infant, usually developing 1 to 2 weeks after birth. The earliest radiographic finding is an alteration of the normal intestinal gas pattern (typically decreased but occasionally increased). The identification of gas in the bowel wall or in the portal circulation is diagnostic. The complication of perforation indicated by free air in the peritoneal cavity is an indication for surgical intervention. A complication of those infants who recover from necrotizing or ischemic bowel disease is the development of strictures, usually 30 to 40 days (1 month) after recovery. An obstructive pattern develops and may be confirmed by using either nonionic contrast media or barium. Pneumatosis, portal venous gas and the “persistent loop” sign are not completely specific for identification of perforation or impending perforation and none are very sensitive, occurring in a minority of patients. The only universally agreed on indication for surgery is pneumoperitoneum. Unfortunately, only 50-75% (less than 3⁄4) of patients with perforation have detectable free air. Small amounts of air may be detectable on supine cross-table lateral films, where it may be seen as triangular lucencies that collect between loops of bowel below the anterior abdominal wall, or anterior to the liver.
The first radiographic sign of necrotizing enterocolitis:

a. portal venous air

b. pneumatosis

c. wall thickening

d. dilated bowel
d. dilated bowel
The first sign of NEC is dilated loops of small bowel and colon. Edema occurs early, and may manifest as wall thickening. Pneumatosis intestinalis (80%) is the second most common finding. Free air occurs in 20%, and is an indication for surgery. The order is thus A, D, C, B.
The most common site affected is terminal ileum and ascending colon. The bowel mucosa is destroyed and bacteria produce gas which passes into the bowel wall creating pneumatosis cystoids intestinalis and sometimes gas into the portal vein. Portal venous air can be seen on liver US before XR. Treatment includes with holding feedings, antibiotics, and blood transfusions, also surgery in case of perforation or peritonitis. Perforation may show free air or ascites. Post NEC strictures are most common in the colon (80%), specifically the splenic flexure, and occur in 30% of cases.
Which of the following is NOT associated with necrotizing enterocolitis?

a. Hypoxemia-associated

b. Bacterial overgrowth

c. Worsened/initiated by feeding

d. Most commonly in TI or right colon

e. Late stricture usually on right
e. Late stricture usually on right
The first sign of NEC is dilated loops of small bowel and colon. Edema occurs early, and may manifest as wall thickening. Pneumatosis intestinalis (80%) is the second most common finding. Free air occurs in 20%, and is an indication for surgery. The order is thus A, D, C, B.
The most common site affected is terminal ileum and ascending colon. The bowel mucosa is destroyed and bacteria produce gas which passes into the bowel wall creating pneumatosis cystoids intestinalis and sometimes gas into the portal vein. Portal venous air can be seen on liver US before XR. Treatment includes with holding feedings, antibiotics, and blood transfusions, also surgery in case of perforation or peritonitis. Perforation may show free air or ascites. Post NEC strictures are most common in the colon (80%), specifically the splenic flexure, and occur in 30% of cases.
Which is not associated with NEC (necrotizing enterocolitis):

A) hyperosmolar feedings

B) caesarian section

C) premature

D) bacterial overgrowth

E) bowel obstruction
B) caesarian section
NEC is the most common GI emergency in premature infants. It commonly develops within 2-6 days after birth (85%). Clinical presentation includes bloody diarrhea or abdominal distention, possibly signs of sepsis, vomiting, apnea, or lethargy. The occurrence of NEC has been associated with hypoxia, stress, low blood pressure, and infection. PROM, preeclampsia, DM, multiparity, early feedings with high osmolar formula, Hirschsprung’s
disease, bowel obstruction, and placement of umbilical artery and venous catheters have all been implicated. Location is most common in TI, also cecum/right colon (rarely stomach / upper bowel). Radiographic findings
include distention of small and large bowel (esp. right lower quadrant), thumbprinting, pneumatosis, PV gas, pneumoperitoneum, ascites. Barium enema is contraindicated! (Dahnert, Peds Req)
Reduction of intestinal intussusception with enema is contraindicated in:

A. age less than 3 months

B. age older than 3 years

C. peritonitis

D. symptoms present for more than 24 hours
C. peritonitis
Intussusception is most common in children aged 6 months to 2 years. In those older than 2 years, there is usually an associated bowel abnormality (lead point). Only 5% have a lead point identified overall. Reduction may be accomplished with air or fluid. Contraindications to reduction are perforation, peritonitis, and Henoch-Schonlein purpura. Air reduction is preferred, and pressure should not exceed 110 mmHg. Reduction is less successful when intussusception has been present more than 24-48 hours.
Which of the following is not true in regard to CT imaging in a child in shock?

a. small IVC

b. hyperenhancing kidneys

c. decreased enhancement of bowel mucosa
c. decreased enhancement of bowel mucosa
Pediatric shock bowel (hypoperfusion complex) is seen with all except:

a. Bowel wall hyperenhancement

b. Adrenal hyperenhancement

c. Pancreas hyperenhancement

d. IVC hyperenhancement

e. Liver hyperenhancement

f. Dense nephrogram

g. Peritoneal fluid
d. IVC hyperenhancement
e. Liver hyperenhancement
Microcolon is most commonly seen in:

a. Meconium ileus

b. Meconium plug

c. Hirschprungs

d. Ileal atresia
a. Meconium ileus
Microcolon can be caused (mnemonic: “MI MCA”) by meconium ileus, meconium peritonitis (CF), Ileal/jejunal atresia, megacystisti-microcolon-hypoperistalsis syndrome, colonic atresia, or aganglionosis (Hisrchprungs).
Meconium Ileus: obstruction of the distal ileum by inspissated meconium; a microcolon is always present. 99% of cases have cystic fibrosis, and 20% of CF kids have meconium ileus at birth. Presents as N/V, dilated loops of bowel on AXR. Water soluble contrast enema is therapeutic.
Meconium plug: (1 in 500-1000) is also referred to as functional immaturity of the colon or small left colon syndrome and is most frequently encountered in neonates who fail to pass meconium. It is a temporary phenonmenon, and will resolve. There is an increased incidence in diabetic mothers, MgSO4 for eclampsia. Rectal biopsy is not unreasonable; it can mimic Hirschprung’s, though the rectum tends to have a normal diameter. The ascending/transverse colon may be more dilated than the descending colon, but, there is no microcolon. Enema is therapeutic.
Meconium peritonitis (1 in 35,000) occurs antenatally when bowel performation causes a sterile chemical peritonitis; this may be due to small bowel or colonic atresia or web, or due to meconium ileus (bowel obstruction), or viral infection. It is associated with microcolon.
Meconium ileus equivalent happens only in older children with cystic fibrosis; improper (too few or too many) digestive enzymes causes inspissated stool to build up and impact within small bowel. This presents as SBO (small bowel obstruction).
Hirschprungs (aganglionosis) (1 in 5000) is associated with trisomy 21 (Down’s) and more common in boys. It most often presents as failure to pass meconium within 48 hours, and involves a transition zone from abnormally wide (proximal) to abnormally small (distal), though it can involve multiple segments. Long-segment involvement looks small but it is not a microcolon. Note that in a normal patient, the rectum has the largest luminal diameter of the left-sided colon. Thus if the rectum is smaller than the sigmoid, this is an abnormal rectosigmoid ratio, and is consistent with Hirschprungs.
Which is true regarding Hirsprung’s dz?
Increased incidence of Trisomy 21
Which of the following is false regarding Hirschsprung's?

a. Aganglionic segment is proximal to ganglionic segment

b. Dilation occurs proximal to the aganglionic segment

c. May present with mucus plug syndrome

d. ganglionic segment is proximal to the aganglionic segment

e. assoc with downs.
a. Aganglionic segment is proximal to ganglionic segment
Regarding Hirschsprung’s, which of the following is false?

A) Aganglionic cells are located proximal to the dilated colon

B) Distal colon is most often affected

C) Abnormal retention of barium

D) Associated with Down's

E) Associated with infants of diabetic mothers
A) Aganglionic cells are located proximal to the dilated colon
Aganglionic cells in Hirschsprung’s disease are distal to the dilated colon, and characterized by the absence of
parasympathetic ganglia in muscle and submucosal layers (Meissner and Auerbach plexi, formed at 12th gestational week) leading to relaxation failure. It occurs in full term infants and presents during the first 6 weeks of life with a male:female of 6:1. It has a 2% association with Down's. Transition zone usually occurs in the rectosigmoid (80%) and usually involves a short segment (80%). Total colonic aganglionosis occurs in only 5%. The transition zone (aganglionic segment) is normal in caliber with dilitation of large and small bowel proximal to this point. On BE there is marked retention of barium on delayed films after 24 hours. Presenting symptoms include failure to pass meconium in the first 24 hours of life, with intermittent constipation and paradoxical diarrhea. It is diagnosed by rectal biopsy and treated with colostomy. Complications include NEC, cecal perforation (secondary to stasis, distension, ischemia) and obstructive uropathy. Remember that infants of diabetic mothers can have meconium plug syndrome due to a hypotonic colon, which can mimic Hirschprung’s
A 1 y/o boy presents with abdominal/pancreatic pseudocyst. What is the etiology?
Child abuse
A neonate with hyperbilirubinemia demonstrates no bowel activity after 4 hours on a hepatobiliary scan. What should be done next?

a. Administer phenobarbital and repeat the examination

b. Obtain delayed images at 24 hours

c. Administer CCK and repeat the examination

d. Administer morphine
b. Obtain delayed images at 24 hours
Plaque-like calcifications are identified within the abdomen of a neonate. This most likely represents?

A. In-utero adrenal hemorrhage

B. Nephroblastoma

C. Teratoma

D. Meconium peritonitis
D. Meconium peritonitis
Meconium peritonitis occurs as a result of bowel perforation in utero with subsequent chemical peritonitis due to the irritative properties of bowel contents. The peritoneal inflammation leads to peritoneal fluid, fibrotic scarring, and often calcification. The perforation may occur proximal to an obstruction caused by atresia, volvulus, intussusception, or meconium ileus (in cases of cystic fibrosis). Several patterns of sonographic findings may occur: a thick-walled cyst, often partially calcified ("meconium cyst"); ascites, usually with foci of peritoneal calcification; and intra-abdominal calcification without ascites or a cyst.
Most common cause of hermaphroditism?
Congenital Adrenal HYPERplasia
Cortical echogenic kidneys are associated with:

a. ATN

b. Nephrocalcinosis

C. RTA

D. Renal vein thrombosis
b. Nephrocalcinosis
Nephrocalcinosis commonly caused by hypercalciuria, hypercalcemia, soft tissue renal damage .

Most common cause of pediatric nephrocalcinonsis is chronic diuretic therapy in infants
Echogenic neonatal kidneys are associated with:

a. Lasix treatment

b. Calciuria

c. Nephrocalcinosis

d. ATN

e. Renal vein thrombosis
c. Nephrocalcinosis
Nephrocalcinosis commonly caused by hypercalciuria, hypercalcemia, soft tissue renal damage .

Most common cause of pediatric nephrocalcinonsis is chronic diuretic therapy in infants
Renal cortical hyperechogenicity is not associated with?

a. hypercalciuria

b. chronic lasix tx

c. medullary cystic disease

d. nephroblastomatosis
d. nephroblastomatosis
Neonate w/↑ renal Pyramid echogenicity – seen in all, except?

A. Prune Belly syndrome

B. ATN/RTA

C. Lasix

D. Hyperparathyroidism

E. Fanconi’s
A. Prune Belly syndrome
Gestational age at which oligohydramnios can be
attributed to renal insufficiency?
16 weeks
Before 16wks, amniotic fluid is from skin.

After 16wks amniotic fluid is from kidneys.
What is the earliest time you can diagnose oligohydramnios from renal cystic disease (? bilateral
renal agenesis)?

A. 8wks

B. 12 wks

C. 16 WKS

D. 18 wks

E. 21 wks

F. 24 wks
C. 16 WKS
Before 16wks, amniotic fluid is from skin.

After 16wks amniotic fluid is from kidneys.
What is associated with autosomal recessive kidney disease (ARKD)?

a. hepatic fibrosis

b. bilaterally enlarged kidneys

c. large renal cysts
a. hepatic fibrosis
ARKD
Infantile polycystic disease Potter type 1

KIDNEYS
enlarged hyperechoic kidneys (hallmark) cysts 1-2 mm are only seen with high-resolution US faint, striated nephrogram due to tubular ectasia

OTHER FINDINGS
pulmonary hypolasia, pneumothorax, pneumomediastinum
hepatic fibrosis, hepatosplenomegaly,
portal venous hypertension pancreatic fibrosis
What ultrasound findings are expected in a neonate with ARPKD:

a. Multiple bilateral renal cysts

b. Bilateral large echogenic kindeys

c. Polyhydramnios on prenatal US
b. Bilateral large echogenic kindeys
Autosomal recessive PKD (also called infantile PKD) manifests as progressively enlarging, hyperechoic kidneys. Most findings, including oligohydramnios, occur in the 3rd trimester of pregnancy. Pathology is actually tubular ectasia, not cysts as the name implies. Renal cysts are seen in autosomal dominant PKD
In utero US shows bilateral renal cysts and profound oligohydramnios:

A. Multicystic dysplastic kidney

B. Autosomal recessive PCKD (ARPCKD)

C. Autosomal dominant PCKD (ADPCKD)

D. Medullary cystic disease
A. Multicystic dysplastic kidney
MCDK is cystic dysplasia secondary to in utero obstruction. Usually unilateral ; bilateral is incompatible with life. US features: paraspinal mass with numerous macroscopic cysts; cysts are noncommunicating (thus able to differentiate from hydro); ureter and renal pelvis are atretic and not visualized; oligohydramnios if bilateral; contralateral renal anomalies, 40% = UPJ obstruction common, agenesis, MCDK. On ultrasound, this can be detected at about 12 weeks of gestational age.

Autosomal recessive PCKD has US features including: bilaterally enlarged hypoechoic kidneys, enhanced thru transmission, individual cysts are not seen by US, liver fibrosis and potter sequence (absent bladder, oligohydramnios, small thorax).

Medullary cystic disease and autosomal dominant PCKD are renal abnormalities in the adult and should be excluded.
Unilateral MCDK is a/w?
Contralateral UPJ obstruction
The most common cause of a unilateral cystic kidney in a child is:

A. MCDK

B. ARPCKD

C. ADPCKD

D. Wilm's tumor
A. MCDK
MCDK (multicystic dysplastic kidney) has multiple macroscopic cysts throughout or replacing the renal parenchyma. It is most often unilateral (85%), but contralateral ureteropelvic junction obstruction is frequently
present. On ultrasonography, multiple cysts of various sizes are seen in the expected location of the kidney . Results from occlusion (severe UPJ) of the ureter in the fetus before 10 weeks gestation. Absent or atretic renal vessels and collecting systems. Hypertrophy of the contralateral kidney.
MCDK is the second most common cause of an abdominal mass in the neonate after hydronephrosis. MCDK is the most common form of cystic disease in infants. ARPCKD and ADPCKD are usually bilateral.
Other causes of renal cysts include obstruction (UPJ, posterior valves, MCDK), tuberous sclerosis, trisomy (13, 18, 21), beckwith-weidemann, and von hippel-lindau.
A 3 month old presents with a renal mass? What is the most likely diagnosis?

a. Wilms’ tumor

b. rhabdoid tumor of the kidney

c. neuroblastoma

d. mesoblastic nephroma
d. mesoblastic nephroma
Benign fetal mesoblastic nephroma (hamartoma, mesenchymoma, or fibroma) is also called Bolandes tumor.

Most common renal tumor of the neonate. It is solid and echogenic on US and may have cystic components. It may bleed. A variation of this tumor contains dense calcifications and is known as the ‘ossifying renal tumor of infancy.’
Congenital mesoblastic nephroma can produce rennin, leading to hypertension, and can metastasize. Swischuk 636-7

Wilms’ Tumor is the most common renal neoplasm in childhood. It is rare in neonates. The rhabdoid form of Wilms’ tumor is highly malignant. Associations include aniridia, hemihypertrophy, Beckwith-Wiedemann syndrome and Drash
syndrome ( male pseudohermaphrodism, nephritis, and Wilms’ tumor.) Also, there is an increased incidence in horseshoe kidneys and crossed renal ectopy. Bilateral Wilms’ occurs in 10%. Metastatic disease most commonly occurs to the lungs. Swischuk 630-2

In the fetus or neonate, neuroblastoma is more likely to present with a large liver resulting from metastatic disease. In the older infant and child, the most common presentation of neuroblastoma is that of an adrenal mass (50-60%.) They can also arise from the sympathetic ganglia in the lower abdomen, the Presacral region from the gland of Zukerkandl, the chest, the gonads and even the neck and nasopharynx. Swischuk 707
Which of the following is the most common abdominal mass in a 2-month old?

A Rhabdomyosarcoma

B Wilms tumor

C Mesoblastic nephroma

D Neuroblastoma

E Medullary cystic disease
C Mesoblastic nephroma
The most common causes of a neonatal abdominal mass are (1) hydronephrosis and (2) MCDK (multicystic
dysplastic kidney). would be good choices. Wilms is the most common abdominal malignancy of childhood.
A. Wilm's tumor B. Mesoblastic nephroma C. Duplication of collecting system with dilated upper pole calyx (anechoic) D. Unilateral autosomal recessive polycystic kidney disease E. Multicystic dysplastic kidney F. Cyst
Answer: B?
In peds reqs, echoic (pg 168), , although not restricted to the upper pole (pg 172). MCDK is cystic as are dilated collecting systems and cysts (pg 160).
However, if medullary cystic disease was not an option, neuroblastoma and mesoblastic nephroma
Fetal ultrasound shows a cystic structure in the superior pole of one kidney. What is this most likely to represent?

a. Duplicated collecting system (with obstructed upper pole moiety)

b. MCDK

c. Wilm’s

d. Simple cyst

e. Autosomal recessive polycystic kidney disease
a. Duplicated collecting system (with obstructed upper pole moiety)
Most common renal neoplasm in a child?
Wilm's tumor
Wilm's tumor is not associated with which of the following?

a) Beckwith Weidman

b) Hemihypertrophy

c) Horseshoe kidney

d) Hereditary aniridia

e) Gonadal abnormalities
All a/w Wilms tumor
Statdx Wilms a/w:

Genitourinary anomalies
Overgrowth syndromes (Beckwith-Wiedemann and isolated hemihypertrophy)
Sporadic aniridia
Trisomy 18
Sotos syndrome
Bloom syndrome
Denys-Drash syndrome
WAGR syndrome: Wilms tumor, aniridia, genitourinary anomalies, and mental retardation
Contiguous gene deletion syndrome involving the Wilms tumor 1 gene (WT1), the paired box gene 6 (PAX6), and possibly other genes on chromosome 11p13

In children, Wilms tumor 2-8x more common in patients with horseshoe kidney
Wilms tumor is associated with all of the following except?

A) Horseshoe kidney

B) Beckwith-Wiedemann

C) Hemihypertrophy

D) Cryptorchidism

E) Meckel-Gruber
E) Meckel-Gruber
Statdx Wilms a/w:

Genitourinary anomalies
Overgrowth syndromes (Beckwith-Wiedemann and isolated hemihypertrophy)
Sporadic aniridia
Trisomy 18
Sotos syndrome
Bloom syndrome
Denys-Drash syndrome
WAGR syndrome: Wilms tumor, aniridia, genitourinary anomalies, and mental retardation
Contiguous gene deletion syndrome involving the Wilms tumor 1 gene (WT1), the paired box gene 6 (PAX6), and possibly other genes on chromosome 11p13

In children, Wilms tumor 2-8x more common in patients with horseshoe kidney

Meckel-Gruber Syndrome is autosomal recessive and is more common among Yemenite Jews. It ischaracterized by large polycystic kidneys, encephalocele, polydactyly, microcephaly, cleft lip and palate and oligohydramnios. It is invariably fatal at birth due to pulmonary hypoplasia and renal failure.
What is not associated with von-hippel lindau?

a. angiomyolipoma

b. renal cysts

c. pheochromocytoma

d. Islet cell tumor

e. renal cell ca.
a. angiomyolipoma
Von Hippel-Lindau syndrome is an inherited multi-system disorder characterized by abnormal growth of blood vessels. The gene for Von-Hippel Lindau disease (VHL) is found on chromosome 3, and is inherited in a dominant fashion. Findings can include:
- CNS/retinal hemangioblastoma,
- pheochromocytoma,
- renal cysts,
- RCC, and
- epididymal cysts,
- epididymal csystadenomas,
- pancreatic cysts,
- and rarely malignant islet cell tumors.

I did not find any association with angiomyolipoma. (Emedicine)
The most common location of bilateral collecting system obstruction in a male infant is:

a. UPJ

b UVJ

a. prostatic urethra

b. bulbous urethra

c. posterior urethral valves
c. posterior urethral valves
Posterior urethral valves (statdx)

General path comments: Related to abnormal thickening and/or fusion of normal circular mucosal folds in the urethra
Epidemiology
Seen only in males
Incidence between 1 in 8,000 to 25,000 births
Associated abnormalities: 80% have associated vesicoureteral reflux

UPJ (statdx)
General Features

Genetics
Sporadic
Isolated UPJ obstruction not associated with aneuploidy
Etiology
Abnormal interwoven muscularis layer of wall
Impairs distensibility
2° to crossing vessel
1/3 of UPJ obstructions have an accessory crossing vessel
Vessel lies anterior to UPJ
Perhaps leaves fibrous scar
Abnormal neural innervation at UPJ
"Hirschsprung equivalent"
Epidemiology
1:2,000 live births
M > F
Associated abnormalities
25% with contralateral renal anomaly
10% with bilateral UPJ obstruction
10% with non-genitourinary anomalies
What is the most common location of urethral obstruction in newborn males?

A) Penile urethra

B) Bulbous urethra

C) Prostatic urethra

D) Trigone

E) Internal urethral sphincter
C) Prostatic urethra
The most common cause of urethral obstruction (35%) in boys is posterior urethral valves (PUV), which is
characterize by congenital thick folds of mucous membrane located in the posterior urethra (prostatic and
membranous) distal to verumontanum. On VGUC there is reflux, mainly on the left, fusiform distension and elongation of proximal posterior urethra, transverse/curvilinear filling defect in posterior urethra, dimunition of
urethral caliber distal to severe obstruction, hypertrophy of bladder neck, trabeculation and sacculation of bladder wall, and large post-void residual.
What is a good prognostic sign in a patient with posterior urethral valves?

a. Diagnosis before 24 weeks

b. Echogenic kidneys with cysts

c. Urinary ascites
c. Urinary ascites
the underlying renal dysplasia, highgrade bilateral reflux, and the noncompliant bladder are signs that will have a negative impact on therapy. On the same scale, a low nadir creatinine or glomerular filtration rate (estimated creatinine clearance), ascites, diverticulum, and unilateral reflux are good prognostic indicators

Two US renal characteristics have been found useful in predicting dysplasia (irrev. renal damage) when in utero renal obstruction is present:

a. Renal cortical echo greater than liver and identification of cortical/subcortical cysts in the remaining parenchyma.

b. When one or both are present, its indicative of bad prognosis.

c. Lack of ability to demonstrate these findings doesn’t necessarily mean dysplasia is
not present.
5yo w/ h/o enuresis since birth. Best initial work-up?
US
A young girl has her first urinary tract infection. Why do you image?

a. check for congenital anomaly b. check for cortical scarring c. check renal function
a. check for congenital anomaly
Urinary tract infection. Obtain a renal and bladder ultrasound and a VCUG. The ultrasound reveals any upper tract abnormalities (hydronephrosis, dilated ureter) that would predispose a patient to persistence of bacteriuria. The VCUG is used to evaluate the presence of reflux, a risk factor for upper tract scarring when coexistent infection is present. In patients with pyelonephritis, generally wait until patients are afebrile and clinically improved before obtaining the VCUG. The study is normally obtained near the end of the hospital stay.
8 yo girl with UTI, fever, leukocytosis, and flank pain. Initial imaging study should be:

A. US

B. CT

C. MRI

D. VP

E. VCUG
A. US
Why is renal scan done in acute pyelonephritis in kids?
-Assess fibrosis/scarring
Neonate with enlarged scrotum and scrotal calcifications:

a. Hemangioma

b. TB peritonitis

c. Meconium peritonitis

d. teratoma
c. Meconium peritonitis

(patent process vaginalis)
A child presents with chronic testicular torsion. All of the following are findings on U/S except:

a. Heterogenous appearance to the testicle.

b. Increased peripheral flow around the testicle.

c. Hydrocele

d. Microlithiasis
d. Microlithiasis
Child with nontender ovarian mass and precocious puberty is likely secondary to:

a. Granulosa tumor

b. Krukenberg tumor.

c. Brennan tumor
Granulosa tumor-
Granulosa tumor-most common estrogen secreting tumor. associated with endometrial thickening and hemorrhage.

Krukenberg tumor-drop mets. colon:stomach 2:1.

Brennan tumor-benign, associated with older woman. may secrete estrogen.
Newborn female has a mass between the rectum and the bladder. This most likely represents:

a. Hydrometrocolpos

b. NeUroblastoma Chordoma

c. Sacrococcygeal teratoma.

d. Rectal duplication

e. Rhabdomyosarcoma
a. Hydrometrocolpos
Hydrometrocolpos and ovarian cysts are the two genital masses in newborn girls. kirks 837.
Which secondary ossification center of the elbow is the first to ossify?
Capitellum
A fracture at which site is more likely to be a Salter-Harris 4 rather than a Salter-Harris 2?

A. Base of the 1st metacarpal

B. Lateral humeral epicondyle

C. Lateral femoral condyle

D. Distal radius

E. Medial clavicle
B. Lateral humeral epicondyle
15% of elbow fractures (distal humerus) in children are Salter 4 involving the capitellum. Fractures of the distal forearm (radius) are the most common of all skeletal injuries, and Salter 2 is the most common especially with gymnasts. Clavicles fracture in middle third 80%, distal third 15%, no Salter. Salter classification is not really used for the tibia, although a Tillaux fracture is considered a Salter 3.
A juvenile Tillaux fracture of the distal tibia involves the?

A. Medial physis and epiphysis

B. Lateral physis and epiphysis

C. Medial physis and metaphysis

D. Lateral physis and metaphysis
B. Lateral physis and epiphysis
Tillaux fracture: avulsion of the lateral tibial epiphysis (Salter-Harris III)
Wormian bones are associated with all of these EXCEPT:

a. Chiari II

b. Normal variant

c. Pyknodysostosis

d. Osteogenesis imperfecta

e. Cleidocranial dysostosis
a. Chiari II.
Wormian bones are intrasutural bones most commonly located in the lambdoid suture. These can be a normal finding until 1 year of age. Other common associations are osteogenesis imperfecta, cleidocranial dysplasia, and hypothyroidism.

Mnemonic is PORKCHOPS:
- pyknodysostosis
- osteogenesis imperfecta
- rickets in healing
- kinky hair syndrome
- cleidocranial dysplasia
- hypothyroidism
- otopalatodigital syndrome
- pachydermoperiostosis
- syndrome of Down
Dislocated hip with coxa valga?
cerebral palsy
Most common LETHAL dwarfism?

d. Thanatophoric dwarf

e. Osteogenesis imperfecta type 2

f. Homozygous achondroplasia

g. Camptomelic

h. Hypophosphatasia
d. Thanatophoric dwarf
With developmental hip dysplasia, what maneuver is most likely to cause dislocation?

a. hips in neutral position and flexion

b. neutral position with internal rotation

c. abduction with external rotation

d. extension of the hips.

e. Adduction and internal rotation
e. Adduction and internal rotation
Barlow dislocation maneuver: apply posterior pressure to hips, with hips in adducted position. The Barlow maneuver most likely causes dislocation. A ‘clunk’ indicates subluxation in either maneuver. These must be
performed before 12 weeks.

The classic Ortolani reduction maneuver: gently abduct the hip while gently pushing the greater trochanter.

The Galeazzi sign of unilateral hip dislocation: patient is supine, with hips and knees flexed; any limb-length discrepancy is suspicious for DDH (or another cause); there may also be decreased abduction on the affected side. (emedicine)
Regarding achondroplasia, which of the following are FALSE?

a. Trident hands

b. Autosomal dominant

c. The shortest components of the limbs are the hands and feet

d. Narrowed interpedicular distance
c. The shortest components of the limbs are the hands and feet
Achondroplasia is a rhizomelic type of dwarfism short limbs with proximal segments of limbs shorter than distal (thus femura and humeri are shorter). Achondroplastics have trident hands (separation of the 2nd and 3rd fingers and an inability to approximate the 3rd and 4th fingers). Autosomal dominant inheritance. Other manifestations of achondroplasia include: macrocrania, lumbar kyphosis in infancy progressing to lordosis, prominent buttocks and abdomen, neurologic symptoms due to relative spinal stenosis. Achondroplasia also has decreasing interpedicular distance caudally, and a “ping-pong” paddle pelvis.
Clubfoot?

1. hindfoot varus
1. hindfoot varus
Different forms of clubfoot may include talipes equinovarus in which the foot is turned inward and downward; calcaneal
valgus in which the foot is angled at the heel with the toes pointing upward and outward; and metatarsus varus in which the front of the foot is turned inward.
Patient with club foot. What is commonly associated with what hind foot deformity:

a. Coxa valga

b. Coxa vara

c. Pes planus

d. Increased talo-calcaneal angle

e. Increased tibiocalcaneal angle
e. Increased tibiocalcaneal angle
Increased tibio-calcaneal angle (hindfoot equinus)

DECREASED talo-calc angle (near 0 degress)

Finding in Clubfoot are hindfoot equinus (calcaneotibial angle > 90deg), hindfoot varus (decreased talocalcaneal angle), and fore foot varus(

Hind foot varus: Club foot
Hind foot valgus: Congenital vertical talus, pes planovalgus)
Forefoot varus: Club foot, and metatarsus adductus. Forefoot valgus: Congenital vertical talus and pes planovalgus
Where is the first place you would expect to see fatty change within the bone marrow of a child?

a. epiphysis

b. diaphysis

c. metaphysic

d. metadiaphysis
Epiphysis
At what age can physiologic periostitis be seen?

a. 1 month

b. 3 months

c. 12 months

d. 15 months
b. 3 months
1-6 months
What don’t you see with physiologic periostitis?

a. Predilection for humerus, femur, tibia

b. Multilaminated appearance

c. Interrupted appearance

d. Symmetric

e. Not present during first 2 weeks of life
c. Interrupted appearance
is a benign periosteal reaction which is symmetric and typically involves the diaphyses (of the lower extremities and humerus) in the first 1 to 6 months of life.
Which pediatric tumor does not metastasize to bone:

a. Neuroblastoma

b. Rhabdomyosarcoma

c. Wilm’s tumor
c. Wilm’s tumor
Most cases of metastatic disease are from small round blue cell tumors. Most common primary neoplasms to metastasize to
bone are neuroblastoma and leukemia or lymphoma (Donnelly p. 187).

Rhabdo. is 3rd most common extracranial solid tumor of childhoon after Wilms and Neuroblastoma. Consists of small round blue cells. Lung is most frequent site mets, followed by bone, bone marrow and lymph nodes. (Rhabdomyosarcoma,
an overview. The Oncologist, Vol. 4, No. 1, 34-44, February 1999)

Wilms tumor metsastasizes to liver and lung, involves lymph nodes (Donnelly p. 159, did not mention bone mets).
A 12-year-old presents with enlarged 2nd and 3rd digits with x-rays demonstrating increased skin thickness and bony
sclerosis:

A) Klipel-Trennaunay-Webber

B) Macrodystrophia lipomatosis

C) Mafucci’s syndrome

D) Neurofibromatosis type 1

E) Ollier’s disease
B) Macrodystrophia lipomatosis
Macrodystrophia lipomatosa (neuronal fibrolipoma with macrodactyly) is a rare nonhereditary congenital form of localized gigantism. It may be associated with syn-, clino-, or polydactyly and is painless. Usually the 2nd or 3rd digit of hand/foot and unilateral. Lucent areas of fat seen with long and broad splayed
phalanges with endosteal and periosteal bone deposition and overgrowth of the soft tissues is diagnostic.. May be associated with accelerated maturation; growth stops at puberty. Differential diagnosis: fibrolipomatous hamartoma associated with macrodystrophia lipomatos, Klippel-Trenaunay-Weber syndrome, lymphangiomatosis, hemagniomatosis, nuerofibromatosis, chronic vascular stimulation, Proteus syndrome.
Which is most typical of Ewing’s sarcoma in an adolescent?

Soft tissue mass
Soft tissue mass
A young patient presents with pain around the knee. MRI reveals increased signal within the patella and the
patellar tendon. What is the most likely diagnosis?

a. Osgood-schlatter

b. jumper’s knee
b. jumper’s knee
Patellar injuries can result from chronic overuse injuries or as a result of acute trauma. Patellar tendonosis, or jumper's knee, results from chronic overload on the patellar tendon because of strain from the quadriceps muscle group, as described by Khan. Chronic overload results in microtears and focal degeneration, which is characterized histologically by pseudocyst formation, increased fibrocartilage, and myxomatous and hyaline metaplasia. Because few inflammatory cells are identified in tendons of patients with chronic patellar tendinopathy, the term patellar tendonitis should be reserved for acute tendinopathy. Patellar tendonosis frequently is seen in participants in sports associated with jumping and is associated with malalignment syndromes as described by Allen and coworkers. MRI: MRI findings consist of focal thickening of the proximal patellar tendon, with increased signal on T1-weighted and T2-weighted images (see Image 8). It is important to recognize that intermediate signal on T1-weighted images is observed in the posterior margin of the proximal patellar tendon and near the tibial tuberosity in normal tendons, as described. Edema in the paratenon, infrapatellar fat pad, and subcutaneous adipose tissue is associated with acute patellar tendonitis, which is defined as symptoms of less than 2-week duration and patellar tendon tears. Acute patellar tendonitis is associated with increased intrasubstance signal on T1- weighted and T2-weighted images without tendon thickening. emedicine
What is associated with pectus excavatum?
prematurity

homocystinuria

Marfans

Noonan syndrome

fetal ETOH syndrome
Unilateral sclerotic pedicle in a kid?

a. contralateral pars defect
a. contralateral pars defect
Per dahnert, the most common causes of a sclerotic pedicle are osteoid osteoma
, unilateral spondylolysis (ipsa or
contralateral), or contralateral congenitally absent or hypoplastic pedicle or posterior elements. Many google links for contralateral pars defect. Osteoblastic mets also like the pedicles, but would be uncommon in kids. Osteoblastoma would
show lucency w/ sclerotic margin rather than a purely sclerotic pedicle. A is probably correct if the congenital absence was not included.
Which fracture is most suggestive of nonaccidental trauma?
Scapula
Regarding plain film technique for the evaluation of scoliosis in an adolescent female, which of the following is true?

A. PA to decrease does to the ovaries

B. AP to decrease dose to the spinal cord

C. PA to decrease dose to the breast tissue

D. AP to decrease dose to the thymus
C. PA to decrease dose to the breast tissue
Adolescent females comprise roughly two thirds of the scoliosis population requiring treatment and follow-up. The risk of breast cancer is much higher when the radiation exposure occurs during puberty. Compared to the AP projection, the PA projection reduces radiation to the breast. Absorption in the bone marrow is increased, but the marrow is six times less radiosensitive for the induction of malignant disease than is the breast. Other exposure-reduction methods include the use of (1) very fast or rare-earth intensifying screens, (2) compensating or wedge-filters, (3) breast and gonad shields, (4) appropriate collimation, and (5) lower grid ratios and higher kV. Lateral examinations should be limited to absolute need. Bending films, although needed initially, are not required in the management of most curves.
Which of the following places a pediatric patient at high risk for latex hypersensitivty?

A. Tetralogy of Fallot

B. Meningomyelocele

C. Unilateral renal agenesis

D. Allergy to penicillin
B. Meningomyelocele
Children with myelodysplasia, including meningomyelocele, develop latex hypersensitivity due to the multiple surgical procedures that they undergo at an early age. (J Allergy Clin Immunol 1994;93:181)
What occurs more commonly in kids as compared to adults after abdominal radiation?

a. Osteonecrosis

b. Radiation induced sarcoma
b. Radiation induced sarcoma
Umbilical cord contains?

a. one artery, two veins
b. two arteries, one vein
c. two veins, two arteries
d. one vein, one artery
b. two arteries, one vein
Which of the following is not seen with histiocytosis X?

A. Renal Failure
B. Thymus involvement
C. Lytic Bone Lesions
D. Diabetes Insipidus
\E. Vertebra Plana
A. Renal Failure
Histiocytosis X(Langerhans cell histiocytosis):
Poorly understood proliferation of Langerhans cells(first line immunologic cells of skin). Most present 1-4yrs. Involves bone, lymph nodes, thymus, liver/spleen, GI tract, endocrine system.
EG:
most benign, localized to bone in children, lung in adults. Geographic, permeative pattern, moth eaten bone lesions. Vertebra plane, with preserved disc spaces.
Hand-schuller-christian disease:
Chronic form of LCH. Classic triad-exopthalmos, diabetes insipidus, destructive bone lesions(usually calvarium). Hepatosplenomegaly. Enlarged thymus. Lymphadenopathy.
Letter-Siwe disease:
Acute fulminant form. Hemorrhage, anemia, fever, skin rash, HSM, Adenopathy. Jaundice. Widespread lytic lesions.
Downs syndrome not associated with:

a. Glossoptosis
b. Hypertrophic pyloric stenosis
c. Duodenal atresia
d. VSD
e. Atlantoaxial dislocation
b. Hypertrophic pyloric stenosis
Downs syndrome is associated with congenital heart disease (40%; especially endocardial cushion defects, VSD, TOF), atlantoaxial subluxation (25%), duodenal atresia (8-10%), clinodactyly (50%; widened space between first 2 digits of hands/feet), duodenal stenosis, annular pancreas, and persistent metopic suture (after age 10).
Hypertrophic pyloric stenosis is more common in boys (4:1) and generally at 2-8 week of life. It may be a dominant polygenic trait or acquired. It may be associated with esophageal atresia / TOF / hiatal hernia / renal issues / Tuners / Trisomy 18.

Twenty-seven patients with Down syndrome were identified who had undergone cine MRI because of persistent obstructive sleep apnea after tonsillectomy and adenoidectomy. The mean age of the 27 patients was 9.9 years (range, 4–19 years; 16 male and 11 female).
Diagnoses identified included glossoptosis in 17 patients (63%), hypopharyngeal collapse in six (22%), recurrent and enlarged adenoid tonsils in 17 (63%), enlarged lingual tonsils in eight (30%), and macroglossia in 20 (74%). Multiple patients had more than one diagnosis. Examples of diagnoses are illustrated in Figures 1A, 1B, 1C, 1D, 1E, 2A, 2B, 3A, 3B, 3C, 3D, 4A, 4B, 4C, 4D, 4E, 5A, 5B. When surgically manageable diagnoses (such as recurrent enlarged adenoid tonsils, enlarged lingual tonsils, or glossoptosis) were identified on cine MRI, these conditions were subsequently managed surgically on the basis of the cine MRI results, and in all cases the findings suggested on cine MRI were confirmed.
AJR July 2004 vol. 183 no. 1 175-181
Which is not associated with Down syndrome:

A. (cerebral) ventriculomegaly
B. pyelectasis
C. echogenic bowel
D. nuchal thickening
E. short femurs and humeri
A. (cerebral) ventriculomegaly
Down syndrome is the most common chromosome abnormality, occurring in 1 of 800 births. A variety of US findings serve as markers for the condition. Major structural defects found in Down’s fetuses include congenital heart disease (endocardial cushion defects), duodenal atresia, and cystic hygromas. Nuchal fold thickening greater than 6mm in the second trimester is strongly associated. The skinfold is measured from the occipital bone to the external skin surface on the transcerebellar plane view.
If two or more of the following findings are present, trisomy 21 is likely: short femur, short humerus, echogenic bowel, mild fetal renal pyelectasis, intracardiac echogenic focus, hypoplastic middle phalanx of 5th finger.

Note that altogether, these symptoms are seen in only about 70%. (B&H, emedicine)
All of the following are characteristics of Down’s syndrome except

A) two or more manubrial ossification centers
B) scaphocephaly
C) clinodactyly
D) duodental atresia
E) flattening of the acetabular roof
F) Nuchal thickening
G) Hyperechogenic bowel
B) scaphocephaly
Down’s syndrome (trisomy 21) is associated with all of the above and can have delayed closures of sutures and fontanelles, characterized by brachycephaly (short, wide head; premature closure of coronal and lambdoid sutures), not scaphocephaly (premature sagittal suture, resulting in a long head).
A child about a year old presents with patchy peribronchial opacities, failure to thrive, and diarrhea. What of the following is not associated with this disease process?

a. Meconium ileus equivalent
b. Sterility
c. Intussception
d. Polyposis
e. Bronchiectasis
f. Chronic sinus infections
d. Polyposis
The question is referring to a patient with cystic fibrosis, which is an autosomal recessive multisystem disease characterized by mucous plugging of exocrine glands secondary to dysfunction of exocrine glands forming a thick tenacious material obstructing conducting system and reduced mucociliary transport. Clinical findings include chronic cough, recurrent pulmonary infections, and progressive respiratory insufficiency due to obstructive lung disease.
CXR findings include mucus plugging, subsegmental / segmental / lobar atelectasis with right upper lobe predominance (10%), progressive cylindrical / cystic bronchiectasis, parahilar linear densities + peribronchial cuffing, focal peripheral / generalized hyperinflation (secondary to collateral air drift into blocked airways), hilar adenopathy, large pulmonary arteries (pulmonary arterial hypertension), recurrent local pneumonitis , and allergic bronchopulmonary aspergillosis (with bronchial dilatation + mucoid impaction).
Meconium ileus is the earliest clinical manifestation of CF. Meconium plug syndrome is common in the infant, causing colonic obstruction. Meconium ileus equivalent occurs in older children (related to digestive enzymes). Rectal prolapse can occur (6 mo to 3 year old) in untreated patients. Intusseption can occur.
Sterility occurs due to vas deferens are absent. Undescended testicles or hydrocele can occur.
There is no association of CF with polyposis.
A 3 yo boy presents with a fever, cervical adenopathy and a rash on the palms and soles and trunk. Chest film shows an interstitial pattern in the lungs. Most likely diagnosis:

A. RSV
B. Tetralogy of Fallot
C. Kawasaki’s disease
D. Rheumatic fever
C. Kawasaki’s disease
Kawasaki’s disease or mucocutaneous lymph node syndrome is an idiopathic acute febrile multisystem vasculitis involving large, med and small arteries with a predilection for the coronaries. Its incidence is 1.1:100,000.
It presents <5 yo with fever, mucosal reddening, cervical lymphadenopathy, maculopapular extensor surface rash, erythema with desquamation of the palms and soles.
Primary pulmonary involvement is extremely rare, however, pulmonary edema (interstitial edema) could occur with ischemic heart failure, due to coronary involvement. (Dahnert, emedicine)
RSV has a peak incidence of between 2 and 8 months. Chest radiograph typically reveal hyperinflated lungs with diffusely increased interstitial markings. Rash does not occur. No mention of lymphadenopathy is made. (Dahnert, emedicine)
TOF typically presents with cyanosis at 3-4 mo (previously concealed by PDA), DOE, clubbing of fingers and toes, squatting when fatigued (to increase pulmonary blood flow). Radiographically, there is decreased caliber and number of pulm vessels, asymm pulm vascularity, reticular pattern with a horizontal course (from pleuropulmonary collaterals). (Dahnert).
Rheumatic fever peaks between 5-15 years and is a distinct event following oropharyngeal group A strep infection. The criteria for diagnosis were updated with the 1992 Jones criteria: major: carditis, migratory polyarthritis, Sydenham’s chorea, subq nodules and erythema marginatum (trunk), minor: fever, arthralgia, incr ESR, prolonged PR interval. No pulmonary radiographic findings were discussed.
Which of the following is not seen with Kawasaki’s disease?

A. Cervical lymphadenitis
B. Gallbladder hydrops
C. Renal failure
D. Pericardial effusion
E. Myocarditis
C. Renal failure
Kawasaki findings: fever, mucosal reddening, cervical lymphadenopathy, maculopapular rash on extensor surfaces, bilateral nonpurulent conjunctivitis, palmar erythema, coronary artery aneurysm stenosis and occlusion, myocarditis, pericarditis, valvulitis, AV conduction disturbances, intestinal pseudoobstruction, gallbladder hydrops. (Dahnert).
Diabetic mothers are more prone to children with:

A. Caudal regression
B. Respiratory distress syndrome
C. myelomeningocele
D. intraspinal lymphoma
E. tethered cord
F. renal agenesis
A. Caudal regression

but also

B. Respiratory distress syndrome
Statdx
Epidemiology
Males > females
Most common cause of death in live newborn infants
Occurs in 40,000 infants each year in USA
More common in infants of diabetic mothers

Caudal Regression Syndrome
Pathology
Most severe cases ⇒ sirenomelia ("mermaid")
Most cases sporadic
1/7,500 births (milder forms > severe forms)
15-20% are infants of diabetic mothers; 1% of offspring from diabetic mothers affected
Genitourinary abnormalities (24%)
Which of the following is a true statement regarding early pregnancy?

A) HCG doubles every 24 hours

B) The gestational sac is usually visualized when the HCG is >1500

C) The MSD approximately doubles in size each day

D) MSD increases 3mm / day

E) The HCG and MSD typically increase proportionately until the 5th week
B) The gestational sac is usually visualized when the HCG is >1500
hCG is detectable in the serum in 5% of patients 8 days after conception, and in >98% by 11 days. At 4 weeks gestation, the hCG doubling time is approximately 2.2 days; this falls to 3.5 days by 8-9 weeks gestation. hCG peaks at 10-12 weeks gestation; it then rapidly declines until approximately 22 weeks, where it gradually rises until term. GSD and HCG increase more slowly at 8-9 weeks gestation; GSD increases about 1 mm / day.
For TVUS, most people use a conservative discriminatory level of 1500 (3600 by TAUS) mIU/mL (about 8 mm or larger). Experienced operators should find a gestational sac at levels of 1000. Ultrasound should not be delayed purely based on hCG levels of < 1000, however, because one study showed that 13% of abnormal IUP and 39% of ectopic pregnancies could still be identified by TVUS. The yolk sac is commonly seen at 2500 mIU/mL, the embryonic pole at 5000 mIU/mL, and the fetal heartbeat can be seen in the vast majority by 10,000 mIU/mL. (emedicine)
Serum progresterone levels can be useful in viability determination. Viable intrauterine pregnancies are associated with serum progesterone levels > 25 (ng/mL). Progesterone levels < 5 (ng/mL) are 100% sensitive for diagnosing a nonviable pregnancy. Serum progesterone is produced by the corpus luteum, which is stimulated by a viable pregnancy.
At what gestational age should you check for nuchal translucency?
a. 8-11 weeks
b. 11-14 weeks
c. 12-15 weeks
d. 13-16 weeks
e. anytime after 15 weeks
b. 11-14 weeks
Typically done between 10-14 weeks by measuring the anechoic region between the subcutaneous fascia and skin at the back of the neck. Increased Nuchal Translucency as a Marker for Fetal Chromosomal Defects (Taipale, et al, 1997) concluded that transvaginal ultrasonography to detect increased nuchal translucency and cystic hygroma is a sensitive test for fetal aneuploidy. It can be done earlier in pregnancy than serum screening, and it decreases the subsequent need for chorionic-villus sampling or amniocentesis.
What are the genetic characteristics of partial mole?

a. diploid
b. triploid
c. 45XO
d. trisomy 18
e. trisomy 21
b. triploid
Partial Mole has a triploid karyotype (66% XXY; 33% XXX) due to fertilization of single ovum with 2 sperm.
Classic/Complete Mole = fertilization of ovum by two 23,X sperm after loss of maternal haploid chromosomes (46,XX) or occasionally fertilization of an ‘empty egg’ by 2 different sperm (46, XY.)
45,XO is Turner Syndrome, which is associated with coarctation, aortic stenosis, horseshoe kidney, sexual infantilism, short stature, webbed neck, shield-shaped chest, idiopathic hypertension and more… Dahnert 170-1

PARTIAL MOLE (statdx)
General Features
Genetics
Partial moles have a triploid karyotype in which two of the three chromosomal complements are derived from the father → diandric monogynic (70% → 69, XXY; 27% → 69, XXX; 3% → 69 XYY)
75% of cases → normal ovum (one set of haploid maternal chromosomes) fertilized by two spermatozoa (two sets of haploid paternal chromosomes)
25% of cases → non-disjunction in meiosis I or meiosis II of spermatogenesis results in extra set of paternal chromosomes in one sperm (diandric) which fertilizes a haploid (monogynic) ovum
Not all triploid gestations present as partial moles → 10% occur due to non-disjunction in meiosis I or meiosis II of oogenesis (digynic) and one haploid set of paternal chromosomes (monoandric)
If two of the three chromosomal complements are derived from mother → triploidy will present with intrauterine growth retardation, normal placenta
Rare reported cases of non-triploid partial moles (likely misdiagnosed early complete moles)
Epidemiology
Occurs in 1 of 700 conceptions
Incidence is 2-3 times higher than complete mole
What is present with stuck twin phenomenon?

a. diamniotic, dichorionic
b. monoamniotic, dichorionic
c. diamniotic, monochorionic
d. monoamniotic, monochorionic
c. diamniotic, monochorionic
shared chorion = risk for twin-twin transfusion
shared amnion = risk for cvord accidents
Stuck Twin Phenomenon – One twin with IUGR with an oligo/anhydraminiotic sac of a diamnoitic twin pregnancy. Amnion is invisible due to close proximity with fetal parts. The fetus is fixed relative to uterus without change in postion during shift in maternal position. Diminished/absent fetal movement. Fetal dath in utero. Danhert 1057-58
When measuring abdominal circumference in a fetus, what structure must be seen to get an accurate measurement?

a. left portal vein
b. kidneys
c. umbilical cord
d. stomach
a. left portal vein
The fetal abdomen is measured axially at the level of the fetal liver, just below the heart, using the landmark of the umbilical portion of the left portal vein at its junction with the portal sinus and right portal vein. The abdominal circumference can be determined by tracing the perimeter of the abdomi or by averaging two orthogonal abdominal diameters and calculating the AC from the equation. Should not use abdominal measurement for gestational age. They are used for calculating the estimated fetal weight, and to determine the normal proportionality between the head-to-abdomin circumference. US REQ 314-315
Regarding an IUGR infant, which is true?

a. If starts late in pregnancy, likely a placental cause

b. Normal S/D ratio is less than 1

c. Usually have normal AC
a. If starts late in pregnancy, likely a placental cause
Early Onset IUGR (intrauterine growth retardation): restricted growth resulting in death or delivery before 32 weeks gestational age. Usually incident to chromosomal abnormalities.” SYMMETRIC “- worse px
Late Onset IUGR: occurs after 32 weeks GA. Usually due to uteroplacental dysfunction.
TORCH infections can result in either early or late IUGR.
Parameters for diagnosing IUGR:
• Fetal weight below 10th percentile (640 g) [NPV 99%, Sensitivity 89%, Specificity 88% for IUGR]
• Elevated Head circumference to Abdominal circumference ratio (HC:AC); normally < 1.2;
But >= 1.35 is consistent with IUGR [NPV: 98%, Sensitivity: 89%, specificity: 88%]. AC generally decreases with IUGR.

S/D ratio (systolic : diastolic) of > 3 after 30 weeks gestation; flow reversal in ductus venosus suggests severely IUGR with fetal metabolic academia. S/D ratio should decrease throughout pregnancy, though it is normally about 4 early (< 20 weeks).
Not a/w IUGR?

a. Increased HC?AC ratio
b. Decreased flow in umbilical artery
c. Increased flow in carotid artery
d. AFI >20
d. AFI >20
Maternal factors of placental insufficiency
Hypertension
Collagen vascular disease
Diabetes mellitus
Drugs/alcohol/cigarette smoking
Malnutrition
Abnormalities in a number of placental hormonal factors
Vascular endothelial growth factor, leptin, resistin
Failure of spiral artery changes in first trimester
Normal low vascular tone in placenta fails to develop
Reduced placental transfer of amino acids/other metabolic substrates
In severe cases, even glucose transport is impaired
Epidemiology
IUGR occurs in 10% of pregnancies by definition
Some of these infants will be normal, just constitutionally small
Risk of IUGR up to 25% if past history IUGR fetus +/- maternal risk factors

Abnormal biometry
Head circumference (HC) and biparietal diameter are preserved
Abdominal circumference (AC) is small and exhibits poor interval growth
EFW calculations heavily weighted to AC
Poor AC growth ⇒ EFW less than expected for GA

Umbilical artery (UA) Doppler
UA blood flow quantified by systolic-diastolic (SD) ratio = peak-systolic velocity/end-diastolic velocity
UA SD ratio should be < 3 in third trimester
As placental resistance ↑, UA diastolic flow ↓
Absent end diastolic flow (AEDF) ⇒ no flow into placental vascular bed during diastole
Reversed end diastolic flow (REDF) ⇒ vascular resistance so high blood flows away from placenta in diastole
Studies suggest > 70% placental vascular bed obliterated before REDF is seen
↑ Cardiac work required to perfuse abnormally resistive placenta
Eventual cardiac decompensation
↑ Right atrial pressure
Reversal of flow in inferior vena cava
Reversal of "a" wave in ductus venosus
Pulsatile flow in umbilical vein
Middle cerebral artery (MCA) flow also quantified by SD ratio
MCA SD ratio should be > UA SD ratio at all gestational ages
Reversal of this pattern = "head sparing" effect
Pulsatile flow in UV reflects breakdown of fetal circulatory compensation to high placental resistance
Maternal uterine artery (Ut A) Doppler may predict patients at risk for IUGR
All of the following are associated IUGR, except?

A) Increase HC to AC measurement
B) AFI (amniotic fluid index) > 20
C) Weight less than 10% percentile
D) Decreased velocity in the umbilical cord
E) Increased HC to femur length
B) AFI (amniotic fluid index) > 20
Maternal factors of placental insufficiency
Hypertension
Collagen vascular disease
Diabetes mellitus
Drugs/alcohol/cigarette smoking
Malnutrition
Abnormalities in a number of placental hormonal factors
Vascular endothelial growth factor, leptin, resistin
Failure of spiral artery changes in first trimester
Normal low vascular tone in placenta fails to develop
Reduced placental transfer of amino acids/other metabolic substrates
In severe cases, even glucose transport is impaired
Epidemiology
IUGR occurs in 10% of pregnancies by definition
Some of these infants will be normal, just constitutionally small
Risk of IUGR up to 25% if past history IUGR fetus +/- maternal risk factors

Abnormal biometry
Head circumference (HC) and biparietal diameter are preserved
Abdominal circumference (AC) is small and exhibits poor interval growth
EFW calculations heavily weighted to AC
Poor AC growth ⇒ EFW less than expected for GA

Umbilical artery (UA) Doppler
UA blood flow quantified by systolic-diastolic (SD) ratio = peak-systolic velocity/end-diastolic velocity
UA SD ratio should be < 3 in third trimester
As placental resistance ↑, UA diastolic flow ↓
Absent end diastolic flow (AEDF) ⇒ no flow into placental vascular bed during diastole
Reversed end diastolic flow (REDF) ⇒ vascular resistance so high blood flows away from placenta in diastole
Studies suggest > 70% placental vascular bed obliterated before REDF is seen
↑ Cardiac work required to perfuse abnormally resistive placenta
Eventual cardiac decompensation
↑ Right atrial pressure
Reversal of flow in inferior vena cava
Reversal of "a" wave in ductus venosus
Pulsatile flow in umbilical vein
Middle cerebral artery (MCA) flow also quantified by SD ratio
MCA SD ratio should be > UA SD ratio at all gestational ages
Reversal of this pattern = "head sparing" effect
Pulsatile flow in UV reflects breakdown of fetal circulatory compensation to high placental resistance
Maternal uterine artery (Ut A) Doppler may predict patients at risk for IUGR

An AFI > 20 is associated with polyhydramnios. AFI < 5 is associated with oligohydramnios. The assessment of AFV is important in pregnancies complicated by abnormal fetal growth or IUGR. AFV has been shown to be predictive in discriminating normal from decreased growth. Oligohydramnios is a frequent finding in pregnancies involving IUGR and is most likely secondary to decreased fetal blood volume, renal blood flow, and subsequently, fetal urine output. Pregnancies complicated by severe oligohydramnios have been shown to be at increased risk for fetal morbidity
Impaired growth may be caused by factors that are intrinsic to the fetus or related to a hostile fetal environment. Fetuses with intrinsic insults have fixed defects and will not benefit from early delivery. The pattern of growth impairment tends to occur early in the 2nd trimester and tends to be symmetric in that the head, abdomen, and femurs are proportionally small. Fetuses exposed to an extrinsically impaired growth environment will usually benefit from therapies that include early delivery.
The growth impairment occurs in the later second and third trimesters and tends to be asymmetrical in that the fetal abdomen is disproportionally small relative to the head and femur. The AC is small because of decreased glycoogen stores in the fetal liver and decreased subcutaneous fat.
TORCH infections can result in either early or late IUGR.
Parameters for diagnosing IUGR:
• Fetal weight below 10th percentile (640 g) [NPV 99%, Sensitivity 89%, Specificity 88% for IUGR]
• Elevated Head circumference to Abdominal circumference ratio (HC:AC); normally < 1.2; >= 1.35 is consistent with IUGR [NPV: 98%, Sensitivity: 89%, specificity: 88%]. AC generally decreases with IUGR.
S/D ratio (systolic : diastolic) of > 3 after 30 weeks gestation; flow reversal in ductus venosus suggests severely IUGR with fetal metabolic academia. S/D ratio should decrease throughout pregnancy, though it is normally about 4 early (< 20 weeks).
In a patient with asymmetric IUGR, which finding is NOT typically seen?

A. Increased diastolic flow / velocity in the fetal carotid artery
B. Increased HC/AC ratio
C. An AFI (amniotic fluid index) of greater than 20 cm
D. A fetal weight less than the 10th percentile
E. Decreased velocity within the umbilical cord
C. An AFI (amniotic fluid index) of greater than 20 cm
Maternal factors of placental insufficiency
Hypertension
Collagen vascular disease
Diabetes mellitus
Drugs/alcohol/cigarette smoking
Malnutrition
Abnormalities in a number of placental hormonal factors
Vascular endothelial growth factor, leptin, resistin
Failure of spiral artery changes in first trimester
Normal low vascular tone in placenta fails to develop
Reduced placental transfer of amino acids/other metabolic substrates
In severe cases, even glucose transport is impaired
Epidemiology
IUGR occurs in 10% of pregnancies by definition
Some of these infants will be normal, just constitutionally small
Risk of IUGR up to 25% if past history IUGR fetus +/- maternal risk factors

Abnormal biometry
Head circumference (HC) and biparietal diameter are preserved
Abdominal circumference (AC) is small and exhibits poor interval growth
EFW calculations heavily weighted to AC
Poor AC growth ⇒ EFW less than expected for GA

Umbilical artery (UA) Doppler
UA blood flow quantified by systolic-diastolic (SD) ratio = peak-systolic velocity/end-diastolic velocity
UA SD ratio should be < 3 in third trimester
As placental resistance ↑, UA diastolic flow ↓
Absent end diastolic flow (AEDF) ⇒ no flow into placental vascular bed during diastole
Reversed end diastolic flow (REDF) ⇒ vascular resistance so high blood flows away from placenta in diastole
Studies suggest > 70% placental vascular bed obliterated before REDF is seen
↑ Cardiac work required to perfuse abnormally resistive placenta
Eventual cardiac decompensation
↑ Right atrial pressure
Reversal of flow in inferior vena cava
Reversal of "a" wave in ductus venosus
Pulsatile flow in umbilical vein
Middle cerebral artery (MCA) flow also quantified by SD ratio
MCA SD ratio should be > UA SD ratio at all gestational ages
Reversal of this pattern = "head sparing" effect
Pulsatile flow in UV reflects breakdown of fetal circulatory compensation to high placental resistance
Maternal uterine artery (Ut A) Doppler may predict patients at risk for IUGR

An AFI > 20 is associated with polyhydramnios. AFI < 5 is associated with oligohydramnios. The assessment of AFV is important in pregnancies complicated by abnormal fetal growth or IUGR. AFV has been shown to be predictive in discriminating normal from decreased growth. Oligohydramnios is a frequent finding in pregnancies involving IUGR and is most likely secondary to decreased fetal blood volume, renal blood flow, and subsequently, fetal urine output. Pregnancies complicated by severe oligohydramnios have been shown to be at increased risk for fetal morbidity
Impaired growth may be caused by factors that are intrinsic to the fetus or related to a hostile fetal environment. Fetuses with intrinsic insults have fixed defects and will not benefit from early delivery. The pattern of growth impairment tends to occur early in the 2nd trimester and tends to be symmetric in that the head, abdomen, and femurs are proportionally small. Fetuses exposed to an extrinsically impaired growth environment will usually benefit from therapies that include early delivery.
The growth impairment occurs in the later second and third trimesters and tends to be asymmetrical in that the fetal abdomen is disproportionally small relative to the head and femur. The AC is small because of decreased glycoogen stores in the fetal liver and decreased subcutaneous fat.
TORCH infections can result in either early or late IUGR.
Parameters for diagnosing IUGR:
• Fetal weight below 10th percentile (640 g) [NPV 99%, Sensitivity 89%, Specificity 88% for IUGR]
• Elevated Head circumference to Abdominal circumference ratio (HC:AC); normally < 1.2; >= 1.35 is consistent with IUGR [NPV: 98%, Sensitivity: 89%, specificity: 88%]. AC generally decreases with IUGR.
S/D ratio (systolic : diastolic) of > 3 after 30 weeks gestation; flow reversal in ductus venosus suggests severely IUGR with fetal metabolic academia. S/D ratio should decrease throughout pregnancy, though it is normally about 4 early (< 20 weeks).
Ischemic insult in 3rd trimester or in the neonate generally presents as:

a. Polymicrogyria
b. Septo-optic dysplasia
c. Periventricular leukomalacia
d. Watershed distribution
c. Periventricular leukomalacia
Discussion: Ischemic insult in utero commonly results in periventricular leukomalacia. These are white matter insults from watershed infarcts in the perforating arteries. Cystic cavitation and necrotic areas may be striking. The most severe changes are classically seen at the trigone of the lateral ventricle, and are seen as hyperchoic foci on ultrasound. Earlier than 26 weeks, hypoxic injury may result in hydraencephaly, neuronal migration abnormalities, and cortical dysplasias such as polymicrogyria and septo-optic dysplasia.
Term newborns with partial asphyxia may cause watershed infarcts between the ACA, MCA and PCA cerebral artery distributions; this results in parasagittal cortical atrphy termed ulegyria (“mushroom shaped”).
Which of the following is not a cause of nonimmune hydrops?

A) Trisomy 13
B) CMV infection
C) Turner syndrome
D) Erythroblastosis fetalis
E) Placental chorioangioma
F) Complete heart block
D) Erythroblastosis fetalis
FETAL HYDROPS
Terminology
Excessive fetal body fluid
Immune hydrops (10%)
Nonimmune hydrops (90%)
Imaging Findings
Ascites
Bilateral pleural effusions
Skin/subcutaneous edema
Placentomegaly
Polyhydramnios
MCA Doppler screening for fetal anemia
M-mode to rule out tachyarrhythmia

Pathology
Turner syndrome (45,XO)
Trisomies: 21 > 18, 13
Maternal Rh sensitization
Atypical antibodies
Fetal anemia leads to hydrops
Variety of causes for nonimmune hydrops
No underlying cause found in 50-80% of cases
Clinical Issues

Nonimmune hydrops in 1:3,000 births
22% cardiac abnormality
16% aneuploidy
10% alpha thalassemia
6% twin-twin transfusion
5% congenital infection
Associated abnormalities: Cystic hygroma
Placental Choioangioma
Imaging Findings
Most common on fetal side of placenta, near cord insertion
Generally hypoechoic
Well-defined
> 5 cm more likely to have complications
Vascularity may be more important than size for predicting outcome
Polyhydramnios common with large masses
Hydrops from arteriovenous shunting or from fetal anemia secondary to hemolysis
Presentation
Most common signs/symptoms
Incidental finding
Most often diagnosed after 20 weeks
Other signs/symptoms
Large masses
Elevated maternal serum alpha-fetoprotein
Polyhydramnios
Rarely, fetal hydrops
Preterm labor
Likely from polyhydramnios but does not explain all cases
Rarely, preeclampsia reported
Which of the following doesn't have Kerley B lines and pleural effusions

a. Obstructed total anamolous pulmonary venous return
b. Ventricular septal defect
c. Tetralogy of Fallot
d. Transient dypnea
e. Aberrant LAD (L coronary?)
c. Tetralogy of Fallot
Which of the following is most associated with cardiomegaly?

a. VSD
b. Tetralogy of Fallot
c. Ebsteins
d. ASD
c. Ebsteins
Ebsteins Downward displacement of the septal and posterior leaflets of the tricuspid valve leading to atrialization of the inflow portion of the RV Classic plain film appearance: Massive right-sided cardiomegaly ("box-shaped" heart) Category: Cyanotic, (severe) cardiomegaly, normal or decreased pulmonary vascularity Hemodynamics: Determined by severe tricuspid valve regurgitation Volume overload to right heart Right-to-left shunting through patent foramen ovale (PFO) → cyanosis.
One month old who was breach delivery, increased T2 signal and enlarged muscle in sternocleidomastoid, also torticolis?

a. Fibromatosis coli
b. denervation
c. Mysositis
d. Leukemia
e. Rhabdosarcoma
a. Fibromatosis coli
What is the lowest level that the conus is normally seen in a newborn/1yr old?

a. T12
b. L2
c. L4
d. S1
b. L2
(L2-3)
Physiologic bowel herniation is seen at what gestational age?

a. 4-8 weeks
b. 8-12 weeks
c. 12-14 weeks
d. 16-20 weeks
b. 8-12 weeks
Omphalocoele can be diagnosed 15 weeks GA (reference: Diagnostic Imaging, Pediatrics, p. 462)
Midgut grows faster than embryo, and herniates into base of cord (week 6), due to relative shortage of intra-abdominal space.

Omphalocoele can be diagnosed 15 weeks GA (reference: Diagnostic Imaging, Pediatrics, p. 462)

Returns to abdomen by 12 weeks.
C - <10-12 weeks, the intestines return to the peritoneal cavity. The final 90 degree counterclockwise rotation of the duodenum, with the duodeno-jejunal flexure lying to the right of the midline. The cecum rotates 180 degrees. The right colon is the last portion of the GI tract to rotate completely, allowing the cecum to descend into the right lower quadrant.
Ewings Sarcoma, which is not true?

a. Older children increased involvement of flat bones
b. Soft tissue mass
c. pain / warmth
d. blastic bone lesion
d. blastic bone lesion
Most common nonimmune hydrops in the United States?

a. Cardiac anomalies
b. Infections
c. Thalasemia
a. Cardiac anomalies
Nonimmune hydrops in 1:3,000 births
22% cardiac abnormality
16% aneuploidy
10% alpha thalassemia
6% twin-twin transfusion
5% congenital infection
Fetal u/s shows a hypoechoic mass in the kidney, most likely diagnosis?

a. RCC
b. Wilms
c. Mesoblastic nephroma
d. Neuroblastoma
c. Mesoblastic nephroma
Baby has increased retropharyngeal thickness on expiratory, flexion lateral neck xray. Next step?

a. U/S
b. CT
c. Fluoro
d. Repeat xray with extention, at inspiration
d. Repeat xray with extention, at inspiration
What is most the most common ASD?

a. primum
b. secundum
c. unroofing of sinus venosus
b. secundum
Incidence is increased in Down syndrome except:

1. duodenal stenosis
2. annular pancreas
3. pancreas divisum
4. Hirschprung’s
3. pancreas divisum
Kid with single renal collecting system (ie not duplicated) what is reason for vesicouretral reflux

a. short intramural segment
b. medial insertion of ureter
c. lateral insertion of ureter
a. short intramural ureteral segment
3-week old neonate with multiple anechoic areas in the deep white matter lateral to the thalamus is likely secondary to:

a. Hypoxia

b. Intraparenchymal hemorrhage

c. Intraventricular hemorrhage

d. cerebritis, hypoglycemia
a. Hypoxia
Prior recalls had it has B. From A+:
Premature infants, born less than 34 weeks gestational age or with birth weight less than 1500 grams, are extremely susceptible to ischemic brain injury.
Subependymal hemorrhage in the residual germinal matrix and periventricular leukomalacia are the two most common forms of hypoxic brain injury in premature infants.
The germinal matrix hemorrhage (GMH) also called subependymal or intraventricular hemorrhage, occurs in the residual germinal matrix over the frontal horn and body of the lateral ventricles.
Down’s syndrome does not get:

a. Hypertrophic pyloric stenosis
b. sleep apnea
c. duodenal atresia
d. atlantooccipital subluxation
e. VSD
a. Hypertrophic pyloric stenosis
HPS is associated with cystic fibrosis and erythromycin exposure prenatally and postnatally via breast milk.
30% with DA have Down syndrome (trisomy 21) (Stat dx).
Most common umbilical cord anomaly?

a. Single artery

b. Knot

c. Cyst

d. Hemangioma
c. Cyst
Statdx
SINGLE UMBILICAL ARTERY

Single Umbilical Artery

Seen best on free loop of cord cross-section
Only 1 artery adjacent to fetal bladder
Single umbilical artery (SUA) is larger than normal UA (i.e., in a 3-vessel cord)
Carries twice the blood volume
15% develop intrauterine growth restriction (IUGR)
Look for additional fetal anomalies
50% risk of aneuploidy if other anomalies in addition to SUA

The presence of a single umbilical artery is recognised as a soft marker for congenital anomalies, aneuploidy, earlier delivery and low birthweight. Most of the available data are derived from case series or highly selected populations and are therefore likely to be unrepresentative. In this retrospective case-comparison study, we firstly aimed to determine the incidence of a single umbilical artery in an unselected population and secondly to examine the clinical significance of this soft marker. Over a 40-month period, 107 cases were identified from a cohort of 35 066 births giving an incidence of 3.1 per 1000 total births and late pregnancy losses. The antenatal detection rate was only 30%. Compared to fetuses with normal cord vasculature, fetuses with a single umbilical artery were more likely to be delivered at an earlier gestation and to weigh less, were 1.7 times more likely to be delivered by a Caesarean section and 19% of the cases had a congenital anomaly. The perinatal mortality was 49.0 per 1000 total births, which was 6 times higher than the background hospital rate. The presence of a single umbilical artery is associated with a poorer perinatal outcome compared to that in fetuses with three vessels in the cord. Unfortunately, the antenatal detection rate is poor. Recognising the importance of this soft marker in counselling and management of pregnancies should provide the stimulus to improve detection rates.
(Prenatal Diagnosis Volume 23, Issue 2, pages 117–123, February 2003)

UMBILICAL CORD CYST
Imaging Findings

Paraxial (60%)
Seen anywhere along length of cord
Thin walled cyst or cysts
Usually anechoic
2% prevalence
Most often transient finding
Most are pseudocysts
Multiple UCC with increased risk of anomalies and aneuploidy
Look at fetal bladder if UCC is near fetal end of umbilical cord
Allantoic cysts can grow

A number of reports described an association between umbilical cord cysts noted initially in the second and third trimesters and trisomy 18 [8-10]. Recently, Ross et al. [5] assessed the prevalence, morphologic characteristics, and natural history of umbilical cord cysts detected by first trimester transvaginal ultrasound. These authors prospectively screened for the presence of umbilical cord cysts in 859 women with singleton live fetuses between 7 and 13 weeks' gestation. Umbilical cord cysts were defined as an echolucent area within the umbilical cord, and the yolk sac was always identified separately from the cyst. Umbilical cord cysts were present in 29 (3.4 percent) of the gestations. Fetal anomalies were noted in seven (26 percent) of cases with ongoing pregnancies. The fetus was more likely to be abnormal if the cyst was located near the placental or fetal extremity of the umbilical cord, (relative risk [RR] 3.3; 95 percent confidence interval [CI] 1.3, 8.5; or paraxially RR 3.8, 95 percent CI 1.2 12.0) or if it persisted beyond 12 weeks' gestation (RR 7.7, CI 3.2, 18.6). Overall, the presence of umbilical cord cysts between 7 and 13 weeks' gestation was 3 percent and in more than 20 percent of cases fetal chromosomal or structural defects were noted.
(Sherer, David M.; Anyaegbunam, Akolisa. Prenatal Ultrasonographic Morphologic Assessment of the Umbilical Cord: A Review. Part I. Obstetrical & Gynecological Survey Issue: Volume 52(8), August 1997, pp 506-514)

Cord cysts can be defined as true or false cysts, and they can occur at any location along the cord. They are irregular in shape and are located between the vessels.

Cysts are found in 0.4% of pregnancies.[42]
(emedicine)

KNOTS
True knots and false knots can form in the umbilical cord. True knots occur in approximately 1% of pregnancies, with the highest rate occurring in monoamnionic twins. False knots (kinks in the umbilical cord vessels) are more common.
(emedicine)

HEMANGIOMAS
Hemangiomas are hyperechogenic masses that are found mainly at the placental end of the cord.

Hemangiomas rarely occur.
What progressively thickens the subendocardium in a kid who eventually dies?

a. endocardial Fibroelastosis

b. Duchene musckuldoystrophy
Neither
Endocardial fibroelastosis is a thickening of the endocardium by layers of collagenous and elastic fibres. Quantitatively, it is a right ventricular endocardium thicker than 10 micrometres or a left ventricular endocardium thicker than 20 micro- metres.1 This endocardial abnormality is unlike endomyocardial fibrosis, where there is a concomi- tant involvement of the sub-endocardial myocar- dium, often in association with hypereosinophilia.2

Lurie. Changing concepts of endocardial fibroelastosis. Cardiology in the Young (2010), 20, 115–123
Diffuse endocardial thickening?

a. Duchennes
b. endocardial fibroelastosis
c. coxsackie b
b. endocardial fibroelastosis
Clinical Diagnosis of Congenital Heart Disease
By M. Satpathy

- endocardial fibroelastosis is thought to be 2/2 to cocksackie B and mumps
Myelination occurs in what direction?
a. dorsal to ventral
b. peripheral to central
c. rostral to caudal
a. dorsal to ventral
What is most common subglottic mass in a child?
a. Hemangioma
b. Mucocele
c. Lymphangioma
d. Rhabdomyosarcoma
e. Papilloma
a. Hemangioma
Hemangioma statdx
Best diagnostic clue

Well-defined mass with diffuse enhancement
High flow vessels in and adjacent to mass during proliferative phase (PP)
Decrease size with fatty replacement during involutional phase (IP)

Location

60% occur in head and neck (any space including parotid glands, orbit, nasal cavity, subglottic airway)
Other locations: Extremities, trunk, rarely cerebellopontine angle

Pediatric Imaging: Rapid-Fire Questions and Answers
By Frank Quattromani, Gilbert A. Handal, Richard Lampe
Isolated short segment small bowel intussception?

a. No additional imaging
b. Meckel’s
c. Air enema
d. Hemangioma
a. No additional imaging
Child with dehydration, AMS, and bilateral thalamic hemorrhage?
a. Nonaccidental trauma
b. deep venous thrombosis
b. deep venous thrombosis
Clinical sign most suggestive of a tethered cord?

Asymmetric gluteal cleft
Lumbosacral dimple
Sacral dimple
Hyperpigmentation of lumbosacral region
Sacral tuft
Sacral tuft
Midline cutaneous stigmata of spinal dysraphism:
Hair > lipoma > hemangioma > hyperpigmentation
Dimples are very common and only worrisome if above gluteal crease or associated with hypertrichosis, hyperpigmentation, etc.
*Child presents with an aggressive anterior mediastinal mass. What is most likely the dx?

Immature teratoma
Neuroblastoma
Ewing’s sarcoma
Immature teratoma
Mature teratoma: 60% of germ cell tumors

Usually asymptomatic; large tumors may present with dyspnea or chest pain
Infants may present with respiratory distress
Gastric/pancreatic juices may cause erosion into tracheobronchial tree, pericardium, pleural space, and lung

Seminoma: 30% of germ cell tumors

Usually symptomatic (70-80%)
Most common malignant germ cell tumor of mediastinum
Symptoms related to tumor size and location, and compression or invasion of adjacent structures
Chest pain, shortness of breath, hoarseness, dysphagia are most common
Less commonly: Fever, weight loss, superior vena cava obstruction (10%), aortic or pulmonary artery compression
Elevated beta-human chorionic gonadotropin (β-HCG) in ≈ 10%; normal alpha fetoprotein (AFP)

Nonseminomatous: 10% of germ cell tumors

Usually symptomatic (90-100%)
Related to invasion or compression of adjacent mediastinal structures; proportional to tumor size
Chest pain, dyspnea, cough, fever, weight loss
↑ AFP in ≈ 80%, ↑ β-HCG in ≈ 60%, ↑ lactose dehydrogenase (LDH) in ≈ 30%

Journal of Pediatric Surgery

Volume 28, Issue 9, September 1993, Pages 1161–1164

Papers Presented at the 24th Meeting of the Canadian Association of Paediatric Surgeons
Cover image
Mediastinalteratomas: Review of 15 pediatric cases ☆

K. Lakhoo,
M. Boyle,
D.P. DrakeCorresponding author contact information

Departments of Paediatric Surgery and Pathology, Hospital for Sick Children, London, England

http://dx.doi.org/10.1016/0022-3468(93)90155-E, How to Cite or Link Using DOI

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Abstract

One hundred fifty-three children with a teratoma presented to one hospital between 1970 and March 1992. The clinical and pathological features of 15 patients with mediastinalteratomas are reviewed; six were newborn and nine aged from infancy to 13 years. Thirteen patients including the six newborns presented with respiratory distress and all 15 patients had a mass on chest radiograph. A definite diagnosis of teratoma was not made preoperatively in any of these patients. At operation, a median sternotomy was used to approach seven anterior tumors and a lateral thoracotomy performed in the other eight patients. Histologically two were mature, 10 had immature elements, and three were malignant teratomas. The patients with malignant tumors were all over 12 years of age and died within 6 months of treatment. All six neonates had immatureteratomas. Raised serum α-fetoprotein levels provided useful markers in two patients with recurrent tumors. Three conclusions can be drawn: (1) mediastinalteratomas are rare in children and frequently are not diagnosed before operation; (2) in newborns these tumors may be immature and present with respiratory distress; and (3) a median sternotomy gives excellent exposure for anterior mediastinal tumors.
Most common cause of a limp in a child?

toxic synovitis
osteomyelitis
Legg-Calve Perthes
septic arthritis
tarsal coalition
toxic synovitis
Toxic synovitis is the most common cause of non-traumatic hip pain in children. TS typically presents in patients between the ages of three and eight years, with a mean age at presentation of six years and a male-to female ratio of slightly more than 2:1.
Don’t forget that if trauma is listed in this question, it is still the most common cause for pain and limp in a child.

References: Up to Date


Transient Synovitis

a.k.a. irritable hip, toxic synovitis
Age: 18 months to 10 years; most common from age 4-7
Typically follows recent upper respiratory infection
Radiographs
Normal
Widening of medial joint space, lateral displacement of femoral head
70% hip effusion on ultrasound
Hip held in flexion, external rotation, and abduction, restricted abduction and internal rotation
± fever (often < 38° C)
May have mildly elevated erythrocyte sedimentation rate and white blood cell
Symptoms improve (usually within 48 hours) in 1-5 weeks
If symptoms persists beyond 1 week, consider another diagnosis
Recur in up to 17%
Legg-Calvé-Perthes develops in 1-3%
Lymphoma in kids where is the most common location?
a. Anterior cervical
b. Mediastinal
a. Anterior cervical
Anterior cervical for Hodgkins


Lymphoma (Hodgkin’s and non-Hodgkin’s) is the third most common childhood malignancy. Non-Hodgkin’s lymphoma (NHL) accounts for approximately 7% of all cancers in children aged younger than 20 years, or about 1000 cases annually in the United States [1]. The overall incidence of NHL in children and adolescents is approxi- mately 10 per 1 million population, but the incidence increases with age. In their Surveillance Epidemiology and End Results (SEER) database, the National Cancer Institute reported incidence per 1 million by age group among pediatric patients from 1975 to 2000. For patients aged younger than 5 years, the incidence was 2.8 (66 cases annually); for those aged from 5 to 9 years, the incidence was 8.8 (147 cases); for those aged from 10 to 14 years, the incidence was 14.3 (290 cases); and for those aged from 15 to 19 years, the incidence was 21.8 (413 cases) [1]. Child- hood NHL differs from adult NHL with respect to disease types, staging systems, biology, treatment, and outcome.
inferior Frontal lobe 4 year old kid with ring enhancing and edema:

a. abscess
b. DNT
c. Ganglioganlioma
a. abscess
DNET (statdx)
Terminology
Dysembryoplastic neuroepithelial tumor (DNET)
Benign mixed glial-neuronal neoplasm
Frequently associated with cortical dysplasia

Imaging
May occur in any region of supratentorial cortex
Mesial temporal lobe most common
Mass frequently "points" towards ventricle
Sharply demarcated, wedge-shaped
Cystic ("bubbly ") intracortical mass
Minimal/no mass effect
NO SURROUNDING EDEMA
Slow growth over years
Usually does not enhance
Faint focal punctate or ring enhancement in 20-30%
Higher rate of recurrence if enhancement

GANGLIOGLIOMA
Terminology
Epilepsy-associated developmental tumor made of admixture of atypical ganglion cells and neoplastic glial cells

Imaging Findings
Best diagnostic clue: Partially cystic enhancing mass involving the cortex in patient with refractory epilepsy
Cortex: Temporal mostly (40%), then parietal (30%), frontal (18%), or occipital
May involve basal ganglia/thalamus, or hypothalamus/optic pathways

MR Findings
T1WI: Hypo- to isointense, sometimes hyperintense to gray matter (Ca++)
T2WI
Solid portion hyperintense at least in part, heterogeneous
Cystic fluid hyperintense
NO SURROUNDING EDEMA
T1WI C+
Variable enhancement, usually moderate but heterogeneous
May be minimal, ring-like, homogeneous
Marked meningeal enhancement in desmoplastic infantile ganglioglioma
MRS: Typical for glial, mostly grade I/JPA tumor: High choline/lactate
2 y/o with scrotal mass and elevated AFP

a. Yolk sac tumor
b. Seminoma
a. Yolk sac tumor
Testicular Tumors:
Only 1-2% of all pediatric tumors

Bimodal age peaks

< 2 years old and young adults

Germ cell variety (60-77%)

Teratomas:
- Benign in pediatric patients (malignant in adults)

Yolk sac tumors:
- Elevated α-fetoprotein

Mixed:
- Variable behavior

Seminomas:
- Rare in children
- Sertoli cell and Leydig cell tumors
- Hormonally active: Gynecomastia, precocious puberty
Juvenile granulosa cell tumors
27% of all neonatal testicular tumors, benign

Gonadoblastoma:
- Intersex disorders

Leukemia/lymphoma secondary involvement, bilateral

Cystic dysplasia: Benign, associated ipsilateral renal agenesis/dysplasia

Extratesticular rhabdomyosarcoma
Highly aggressive malignancy
70% retroperitoneal nodal spread at diagnosis
Lateral epicondylar fracture classification system?
Milch
- based upon fracture plane relation to trochlear ridge of the humerus
- lateral to trochlear ridge = Milch I (stable if nondisplaced)
- medial to trochlear ridge = Milch II (unstable)
Craniosynostoses
Scaphocephaly:
↓ Transverse, ↑ AP
⇒ sagittal synostosis

Trigonocephaly:
"Ax-head"
"pear-shaped" on axial
"quizzical" orbits (elevation of medial orbital rim)
⇒ metopic syonstosis

Plagiocephaly
Asymmetry
⇒ unilateral single or asymmetric multiple synostoses

Brachycephaly
↑ Transverse, ↓ AP
"harlequin" orbits (elevtion of lateral orbital rim)
⇒ bicoronal or bilambdoid

Turricephaly
"Towering skull"
⇒ bicoronal or bilambdoid

Kleebattschadel
Bulging temporal, shallow orbits
⇒ bicoronal and bilambdoid

Craniofacial dysostoses:
"Towering skull"
shallow orbits