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310 Cards in this Set
- Front
- Back
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What is the Caudal Regression Syndrome? |
abnormal fetal development of the lower spine which can lead to a variety of problems ranging from partial absence of the tail bone regions of the spine to more severe cases involving major malformation of the lower vertebrae, pelvis and spine. Minor cases may be more of less without symptom, whereas severe cases may come with major birth defects, neurological impairment and incontinence.
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What is Sirenomelia?
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rare congential deformity, legs are fused together, giving the appearance of a mermaid. This condition is found in approximately one out of every 70,000 live births[1] (about as rare as conjoined twins) and is usually fatal within a day or two of birth because of complications associated with abnormal kidney and bladder development and function. It results from a failure of normal vascular supply from the lower aorta in utero. Sirenomelia is associated with maternal diabetes
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What are Tamm-Horsfall Proteinuria?
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also known as stasis nephropathy
perinatal renal insult of less severe degree than acute cortical necrosis prolonged nephograms on IV urograms,renal ultrasound: increased echogenicity in central portions of pyramids, preservation of hypoechoic peripheral medulla due to deposition of Tamm-Horsfall protein in the tubules,Benign course, usually resolves completely |
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What is Wilms Tumor?
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nephroblastoma,
3rd most common kiddie tumor (most common abd tumor) arises from embryonal renal tissue (nephroblastomatosis) large only 10% are calcified 4-10% bilateral mets --> lungs, para-aortic nodes associated with: aniridia Beckwith-Wiedemann syndrome hemihypertrophy Drash syndrome |
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What is Aniridia?
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rare congenital condition characterized by the underdevelopment of the eye's iris. This usually occurs in both eyes. It is associated with poor development of the retina at the back of the eye resulting in visual loss.
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What is Beckwith-Wiedemann syndrome?
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big tongue, organomegaly (liver, kidneys, pancreas, heart)
omphalocele, umbilical hernia or diastasis recti |
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What is the Drash Syndrome?
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male pseudohermaphroditism
progressive nephritis |
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What is Nephroblastomatosis?
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persistent primitive renal tissue (normally present up to 36 weeks)
potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor associated with: hemihypertrophy sporadic aniridia pseudohermaphroditism Klippel-Trenaunay syndrome Beckwith-Wiedemann syndrome splenic agenesis with hepatic malformation findings: kidneys may be enlarged deformity of the pelvocalyceal system US: subtle subcapsular nodules (hypo-/iso-/hyperechoic) CT: hypodense subcapsular nodules after contrast enhancement |
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What is HSP?
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acute, self-limited arteritis
often follows Strep infection symptoms: purpura nephritis abdominal pain joint pain thick SB folds thumbprinting in SB or colon |
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What is CCAM?
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Congenital cystic adenomatoid malformation is a congenital disorder similar to bronchopulmonary sequestration. In CCAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue.
In most cases the outcome of a fetus with CCAM is very good. However in a rare few cases the cystic mass grows so large as to limit the growth of the surrounding lung and cause pressure against the heart. In these situations, the CCAM can be life-threatening for the fetus. |
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What is Klippel-Feil Syndrome and it's associations?
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fused vertebrae
esp. cervical spine (C3-C4) elevation of scapula (Sprengel deformity) omocervical bones GU abnormalities (66%) renal agenesis (33%) deafness (33%) |
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What is Sprengel Deformity?
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congenital elevation of scapula
rotated --> inferior edge points medially,uni- or bilateral almost always a/w Klippel-Feil syndrome, +/- omocervical (or "omovertebral") bones: joins scapula to C5 or C6 |
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What is the Omovertebral bone?
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bony connection between the elevated scapula and one of the cervical vertebrae, usually C5 or C6
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What is MURCS Syndrome?
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Mullerian aplasia, renal aplasia and cervico-thoracic somite dysplasia
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What is Mayer-Rokitansky-Kuster-Hauser syndrome or simply Rokitansky syndrome?
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Agenesis of vagina and uterus is also called Mullerian aplasia
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Transient Tachypnea of the Newborn
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Transient tachypnea of the newborn (TTN) is a self-limited disease common in infants throughout the world and is encountered by all physicians who care for newborn infants. Infants with TTN present within the first few hours of life with tachypnea, increased oxygen requirement, and arterial blood gases that do not reflect carbon dioxide retention. When managing TTN, observing for signs of clinical deterioration that may suggest other diagnoses and for the development of respiratory fatigue is important
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Meconium Aspiration
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intra-uterine fetal distress ==> defecation of meconium
* term + post-term neonates o NOT preemies o NOT kids of diabetic moms * diffuse HYPERaeration o pulm interstitial emphysema (PIE) o pnuemomediastinum + non-tension ptx in 33% * patchy atelectasis + consolidation * lung dz usually resolves spontaneously * morbidity d/t intrauterine cerebral anoxia |
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Meconium Ileus
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obstructed bowel due to impacted, tenacious meconium
* no air-fluid levels (probably d/t adherent bowel contents) DDx: * ileal atresia * Hirschsprung dz * anal atresia a/w cystic fibrosis (CF) |
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Meconium Plug
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functional colonic inertia"
* Dx/Tx: Gastrografin enema (DDx: Hirschsprung dz, meconium ileus) * F/U with another enema for confirmation * a/w maternal diabetes |
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copper beaten skull
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c/w chronic hydrocephalus
elevated icp |
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What are germinal matrix hemorrhages associated with
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<34 weeks
<1500g |
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Periventricular leukomalacia
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Periventricular leukomalacia (PVL) is the most common ischemic brain injury in premature infants. The ischemia occurs in the border zone at the end of arterial vascular distributions. The ischemia of PVL occurs in the white matter adjacent to the lateral ventricles. The diagnostic hallmarks of PVL are periventricular echodensities or cysts detected by cranial ultrasonography. Diagnosing PVL is important because a significant percentage of surviving premature infants with PVL develop cerebral palsy (CP), intellectual impairment, or visual disturbances
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rickets
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relative or absolute deficiency of vitamin D
* presents by 3-6 months, almost always < 2 yrs xray: * loss of zone of provisional calcification * wide physis (> 1 mm)(increased osteoid) * cupping, fraying + irregularity of metaphyses * bowing of long bones * decreased bone density * rachitic rosary * periosteal reaction |
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Tibial Bowing
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* anterior bowing
o OI o hypophosphatasia o thanatophoric dwarfism o neurofibromatosis o --> if fractured, doesn't heal normally * posterior bowing o often bilateral o d/t fetal malposition o fracture will heal normally |
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Osteogenesis Imperfecta
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brittle bone disease": abnormalities of collagen/collagen production
Type I autosomal dominant age at presentation: 2-6 years Type II (congenital lethal OI) autosomal recessive pre or perinatal death (pulmonary hypoplasia) Type III (severe prograssive OI) autosomal dominant marked progressive limb and spine deformity Type IV autosomal dominant most mild form * demineralization, cortical thinning * multiple fractures with pseudoarthrosis * exuberant callus formation * blue sclerae * presenile deafness * dentinogenisis imperfecta * wide sutures + Wormian bones |
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Wormian Bones
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small ossicles within cranial suture lines
* osteogenesis imperfecta * cleidocranial dysostosis * cretinism * idiopathic * Down syndrome * hypophosphatasia * Menkes kinky-hair syndrome * progeria * pyknodysostosis |
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Cleidocranial Dysostosis
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disorder of ossification/development of midline bones
autosomal dominant, 33% sporadic * skull o cranial dysplasia o Wormian bones o basilar invagination * clavicles o aplasia / hypoplasia, usually lateral portion * other skeletal abnormalities o small, high scapula o wide symphysis pubis o acro-osteolysis |
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basilar invagination
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* dens projects into base of skull
o McGregor's line > 4.5 mm o Chamberlain's line > 3 mm * may --> obstructive hydrocephalus etiology: * 1' anomalies of occiput, atlas, axis * softened bone: rickets / osteomalacia, Paget's dz * fibrous dysplasia * dysostoses: OI, achondroplasia, cleidocranial dysostosis Cf: platybasia |
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Aplasia of Distal Clavicle
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absent distal clavicle
* cleidocranial dysostosis * rheumatoid arthritis * hyperparathyroidism |
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acro-osteolysis
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loss of terminal tufts of digits
* scleroderma / connective-tissue disease * psoriatic arthritis * Reiter disease * frostbite(thumbs spared)/burns * leprosy * polyvinylchloride exposure * hyperparathyroidism * Lesch-Nyhan syndrome (they bite 'em off) * cleidocranial dysostosis * progeria * pyknodysostosis (aplasia/hypoplasia of distal digits) * sarcoidosis * epidermolysis bullosa |
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pyknodysostosis
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* autosomal recessive
* dense, sclerotic bones [Cf: osteopetrosis (sporadic, not inherited)] Features: * open cranial sutures + fontanelles * Wormian bones * dolichocephaly * sclerotic vertebrae * fractured long bones * short, stubby hands * partial agenesis/aplasia of terminal phalanges [simulates acro-osteolysis] |
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toddler's fracture
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non-displaced spiral fx of tibia
* no h/o trauma * tender swelling of tibia * +/- periosteal new bone * main problem: make correct dx DDx: * infection * tumor * non-accidental trauma (abuse) |
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slipped capital femoral epiphysis (SCFE)
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* boys > girls
* age 10 - 15 yrs * blacks > whites (unlike CHD + Legg-Perthes disease) * related to height, weight, athletic activity * if BILATERAL ==> renal osteodystrophy * femoral epiphysis slips POSTERIORLY and MEDIALLY * use frog-leg + lateral projections * complications: o AVN o acute cartilage necrosis (chondrolysis) o varus deformity is a salter 1 |
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developmental dysplasia of the hip
aka: congenital hip dislocation (CDH or CHD) |
* females (80-90%), 2/3 are firstborn
* white > blacks * associated: o breech delivery (6x cephalic),C-section, torticolis, family history * due to ligamentous laxity from increased circulating maternal estrogen, not acetabular dysplasia * unilateral LEFT (70%); bilateral in only 5% * Putti's triad 1. supero-lateral migration of femoral head 2. increased acetabular angle 3. small capital femoral epiphysis |
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Salter-Harris fractures
classification of epiphyseal fractures |
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pure epiphyseal separation if non-displaced, jt effusion may be only sign II metaphyseal fracture + epiphyseal separation III epiphyseal fracture IV vertically oriented fx thru epiphysis + metaphysis V crush injury of epiphysis (not detected acutely) * most common: type II * type I (best) --> type V (worst) * seen in kiddies before the physes close |
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Tillaux fracture
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fracture of LATERAL aspect of distal tibia
* analog of Salter III fx of distal tibia in kids: o distal tibial epiphysis closes in 12-18 months o MEDIAL portion closes first |
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Little Leaguer's elbow
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avulsion fx of MEDIAL epicondyle
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congenital infection: ToRCHS syndrome
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T
Toxoplasma R Rubella C Cytomegalic inclusion disease (CID, CMV) H Herpes S Syphilis * transplacentally acquired * congenital infection * celery-stalk metaphyses, esp. long bones * intracranial calcification * decreased growth * vascular stenosis (aorta, pulm artery) |
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DDX Neonatal Respiratory distress
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surfactant deficiency
TTN Meconium Aspiration Neonatal Pneumonia |
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intussusception
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* 75% in kids <2 y/o
o ileocolic (75%), ileo-ileocolic (15%) o 90% idiopathic o leading point: lymphoid tissue (possibly increased 2' to enteritis) * in older kids + adults (ileo- or colocolic) o Meckel diverticulum o Peyer's patches o lymphoma o large mesenteric nodes o duplications o polyps * "currant jelly" stools * "coiled spring" on BE * * ** transient intussusception: a/w sprue |
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Meckel diverticulum
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* rule of 2's
o 2 cm wide o 2 feet from I-C valve o males 2:1 o 2% of population * antimesenteric (unlike duplication cyst) * +/- ectopic gastric/pancreatic tissue |
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Meckel scan
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* agent: Tc-99m pertechnetate
* prep: o pentagastrin -- increase acid production o cimetidine -- block secretion of acid o glucagon -- decrease bowel activity |
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what are the u/s findings in intussusception?
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# Transverse sonography of the mass reveals a swirled pattern of alternating hyperechogenicity and hypoechogenicity representing the alternating layers of mucosa, muscularis, and serosa. target or donut sign.
Longitudinal sonography reveals a sandwichlike appearance of the alternating loops of bowel with a loop-within-loop appearance. |
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Rule of 3's with Intussusception
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when reducing intussusception 3 ft high, 3 min per try, 3 tries
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Henoch-Schoenlein syndrome
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acute, self-limited arteritis
* often follows Strep infection * symptoms: o purpura o nephritis o abdominal pain o joint pain * thick SB folds * thumbprinting in SB or colon |
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tracheo-esophageal fistula
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Type Frequency Description
A 10% esoph atresia w/o fistula B 1% esoph atresia w/ upper fistula C 80% esoph atresia w/ LOWER fistula D 2% "K" type: atresia w/ upper & lower fistulae E 7% "H" type VATER syndrome |
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VATER syndrome
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V
vertebral anomalies vascular anomalies A anal atresia T tracheo-esophageal fistula E esophageal atresia R renal anomalies radial dysplasia |
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Klippel-Feil syndrome
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fused vertebrae
esp. cervical spine (C3-C4) elevation of scapula (Sprengel deformity) omocervical bones GU abnormalities (66%) renal agenesis (33%) deafness (33%) |
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cervical spine fusion (ankylosis)
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posterior elements
JRA vertebral bodies block vertebrae Klippel-Feil |
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Sprengel deformity
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congenital elevation of scapula
rotated --> inferior edge points medially uni- or bilateral almost always a/w Klippel-Feil syndrome +/- omocervical (or "omovertebral") bones: joins scapula to C5 or C6 |
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hemihypertrophy
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unilateral enlargement (one digit to entire 1/2 of body)
associated with benign + malignant neoplasms: kidney nephromegaly medullary sponge kidney polycystic kidney disease Wilms tumor / nephroblastomatosis adrenal cortical hyperplasia / adenoma / adenoCa pheochromocytoma liver focal nodular hyperplasia hepatoblastoma hemangioendothelioma gonads phakomatoses |
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nephroblastomatosis
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persistent primitive renal tissue (normally present up to 36 weeks)
potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor associated with: hemihypertrophy sporadic aniridia pseudohermaphroditism Klippel-Trenaunay syndrome Beckwith-Wiedemann syndrome splenic agenesis with hepatic malformation findings: kidneys may be enlarged deformity of the pelvocalyceal system US: subtle subcapsular nodules (hypo-/iso-/hyperechoic) CT: hypodense subcapsular nodules after contrast enhancement |
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Pulmonary lymphangiectasia
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rare
dilated lymphatic channels secondary to either abnormal embryonic development of the lymphatic system or obstruction of lymphatic drainage. The dilated lymphatics cause a coarsely nodular or reticular pattern in the lungs, usually developing early in infancy |
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Pulmonary hemangiomatosis
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rare
Recurrent hemorrhage into the lungs in patients with idiopathic pulmonary hemosiderosis eventually leads to chronic diffuse haziness or reticula in the lungs, representing pulmonary fibrosis |
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histiocytosis X
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aka: Langerhans cell histiocytosis LCH
intense proliferation of reticulohistiocytic cells * eosinophilic granuloma (EG) o 60-80% of histiocytosis X o age 5 - 10 yrs most common o best prognosis * Hand-Schuller-Christian disease o age 1 - 3 yrs * Letterer-Siwe disease o age 0 - 1 yr o worst prognosis |
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eosinophilic granuloma
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* 60-80% of histiocytosis X
* age 5 - 10 yrs * presentation: bone pain, local swelling, irritability * bones o 50 - 75% solitary / monostotic o skull/mandible (50%): "punched-out" lucencies, "hole within a hole", "button sequestrum", "floating teeth" o spine/pelvis (25%): vertebra plana (most common pediatric cause) o long bones (15%): medullary lucency +/- thin sclerotic rim * lungs o involved in <10%, signals worse prognosis o apical reticulonodular infiltrates o honeycomb lung |
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Hand-Schueller-Christian disease
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chronic diseminated form of histiocytosis X
* age 1 - 3 yrs most common * skull and mandible lesions similar to EG, but more numerous * "geographic skull", "floating teeth" * adenopathy * enlarged liver and spleen * skin lesions * diabetes insipidus * exophthalmos * lung disease |
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Letterer-Siwe disease
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Acute diseminated form of histiocytosis X
* age 0 - 1 yr * worst prognosis (70% mortality) * visceral involvement: hepatosplenomegaly * purpura, anemia, lymphadenopathy * bone: extensive lytic skull lesions, "raindrop" skull |
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3 subtypes of Histiocytosis X
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Hand-Schuller-Christian disease
Eosinophilic Granuloma Letterer-Siwe disease |
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Causes of Miliary Nodules
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Infection
Tuberculosis Histoplasmosis Viral Idiopathic pulmonary hemosiderosis Metastatic disease |
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hypoplastic left heart syndrome
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underdevelopment of the left side of the heart characterized by:
* aortic valve atresia * hypoplastic ascending aorta * hypoplastic/atretic mitral valve * endocardial fibroelastosis most common cause of CHF in neonate * 25% of cardiac deaths in 1st week of life * prognosis if untreated: 100% fatal by 6 weeks hemodynamics: * pulmonary venous return is diverted from LA to RA through ASD * RV supplies pulmonary artery, ductus arteriosus, descending aorta (antegrade flow), aortic arch, ascending aorta, coronary circulation (retrograde flow) * leads to RV work overload and CHF Rx: * Norwood procedure (palliative) - three stage surgery that results in the RA feeding the pulmonary circulation and the RV feeding the systemic circulation. * transplant |
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Congenital Pulmonary Hypoplasia
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hypoplasia or absence of the ipsilateral pulmonary artery
a/w TOF, TA |
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tetralogy of Fallot
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underdevelopment of pulmonary infundibulum
* 8% of all congenital heart disease * most common CHD with cyanosis after 1 year of life TETRAD: 1. obstruction of RV outflow tract; usually at the pulmonary infundibulum, occasionally at pulmonary valve 2. VSD 3. RV hypertrophy 4. aorta overriding the interventricular septum associated with: * bicuspid pulmonary valve (40%) * stenosis of left pulmonary artery (40%) * right-sided aortic arch (25%) * TE fistula * Down syndrome * anomalies of coronary arteries (10%) * forked ribs, scoliosis |
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pulmonary venolobar (scimitar) syndrome
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* always involves aplasia of one or more lobes of right lung
* variably involves: o partial anomalous pulmonary venous return (scimitar-shaped vein) + usually drains to IVC; but portal vein, hepatic vein, RA possible o absent or small pulmonary artery perfusing abnormal lung segment + arterial supply from thoracic/abdominal aorta or celiac axis o anomalies of hemidiaphragm on affected side o anomalies of bony thorax or thoracic soft tissues |
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What causes pulmonary agenesis
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insult during 4th week of fetal life
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diastrophic dwarfism
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short-limbed dwarfism ("diastrophic" means twisted, thus scoliosis)
x-ray features: * short flared metaphyses with flattened epiphyses in tubular bones of extremities * equinovarus deformity of feet -- major joints subluxed * spine: kyphoscoliosis with gradual decrease in interpediculate distance toward caudal end of L-spine * hands o "hitch-hiker's thumb" - widely abducted o hypoplastic first metacarpal o other metacarpals too broad, esp. at growing end o carpal bones with too many centers ossified |
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Ellis-van Creveld syndrome
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chondro-ectodermal dysplasia
* hereditary (esp. seen in Amish of Pennsylvania) * polydactyly (100%) * congenital heart dz (60%) * abnormalities of cutaneous appendages (skin, hair, nails) |
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Potter syndrome
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autosomal recessive ("infantile") polycystic disease * "microscopic" (1-mm) cysts * US: +/- increased echo, no gross cysts II multicystic dysplastic kidney ("renal dysplasia") * US: multiple cysts, usually unilateral III autosomal dominant ("adult") polycystic disease * usually not present until adult life * US: multiple cysts renal agenesis associated with: * oligohydramnios * abnormal facies * hypoplastic lungs |
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prune belly (Eagle-Barrett) syndrome
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* absent abdominal musculature
* undescended testes * dilated ureters & calyces * clubbed feet * heart & lung abnormalities * ** predominantly in males |
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Swyer-James syndrome
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"unilateral hyperlucent lung", Macleod syndrome
* may be bilateral * hyperlucency + air-trapping * decreased pulmonary arteries * paucity of bronchial subdivisions +/- proximal bronchiectasis * h/o early + recurrent lower respiratory tract infections * possibly 2' to obliterative bronchitis/bronchiolitis (adenovirus?) Air trapping results in a lung that changes very little in size between inspiration and expiration. This important feature helps to distinguish the hypoplastic Swyer-James lung from the congenitally hypoplastic lung |
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cystic fibrosis
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* probably autosomal recessive
* common in whites, 1:2000 * clinically: resp. disease, GI symptoms complications: * bronchiectasis * pneumothorax * hemoptysis * lobar atelectasis * pulmonary HTN * cor pulmonale {normal-size heart may represent CMG} * large, opacified sinuses DDx: Riley-Day syndrome |
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Riley-Day syndrome
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familial dysautonomia
* autosomal recessive * seen in Jewish infants * malfunction of autonomic nervous system * possibly associated with catecholamine release + beta-NGF * hypersecretion of mucous glands * XR resembles cystic fibrosis |
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A right-sided aortic arch is the key radiographic clue to the presence of ?
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an obstructing vascular ring
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pulmonary sling
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aberrant course of LEFT pulmonary artery between trachea + esophagus, compressing the trachea posteriorly
left pulmonary artery comes off the r pulm artery * may cause obstructive emphysema of the right lung if the right main stem bronchus is compressed |
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Lateral radiographs demonstrate increased retrotracheal opacity, tracheal narrowing, and anterior tracheal bowing
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Right ascending aortic arch, Aberrant left subclavian artery that passes posterior to the esophagus\
Ligamentum arteriosum or Persistent ductus arteriosus stretching from the left subclavian artery to the pulmonary artery anterior to the trachea |
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patent ductus arteriosus (PDA)
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* 9% of all CHD; M:F = 1:2
* associated with: prematurity, birth asphyxia, rubella, coarctation, VSD, trisomy 18 + 21 * mostly assymptomatic * CHF (rare) usually by 3 months in large L-R shunts (PDA increases shunt volume) findings (mimicks VSD): * LA enlargement * enlarged PA * increased pulmonary vasculature * enlarged RV + LV * "railroad track": calcified ductus arteriosus PDA beneficial in: * tetralogy of Fallot * Eisenmenger pulmonary hypertension * interrupted aortic arch (supply lower extremity via PDA) |
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congenital infections
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ToRCHS syndrome
T Toxoplasma R Rubella C Cytomegalic inclusion disease (CID, CMV) H Herpes S Syphilis * transplacentally acquired * congenital infection * celery-stalk metaphyses, esp. long bones * intracranial calcification * decreased growth * vascular stenosis (aorta, pulm artery) |
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aortic coarctation
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* most frequent cause of PVH + CHF in 2nd + 3rd weeks of life
associated with * bicuspid aortic valve (50-80%) * calcific aortic stenosis (d/t bicuspid valve) * PDA or VSD (in most) rf:figure 3 sign post rib notching T4-T8 (only occurs when child is 7 yoa |
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neonatal pulmonary venous hypertension
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* with cardiomegaly
o hypoplastic left heart o critical aortic stenosis o cor triatriatum o pulm venous atresia * normal heart size o infradiaphragmatic TAPVR |
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What is the best way to determine abnormal lung?
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inspiratory and expiratory frontal views of the chest or fluoroscopy can be helpful. The lung that changes the least in volume between inspiration and expiration is the abnormal lung
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congenital lobar emphysema
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* caused by bronchial cartilage abnormality
* SOLID mass at birth: dilated alveoli filled w/ fetal lung fluid * usually in UPPER lobes (incl. RML) * Tx: surgical lobectomy Cf: cystic adenomatoid malformatio |
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cystic adenomatoid malformation of lung
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* only true pulmonary cystic dz of newborn
* three xray types: 1. multicystic, walls of varying thickness 2. multicystic, one large dominant thin-walled cyst 3. solid * mediastinal shift common * cysts often contain fetal lung fluid * Tx: surgery |
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Causes of Unilateral Obstructive Emphysema
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Bronchial foreign body
Mucous plug Congenital lobar emphysema Bronchial stenosis/atresia Tuberculosis Vascular anomalies Mediastinal masses |
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congenital absence of pulmonary valve
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* BIG central PAs
* big RV |
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congenital cardiomyopathy
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* endocardial fibroelastosis
* myocarditis * glycogen storage dz (Pompe's) * anomalous origin of left coronary artery from pulmonary artery |
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endocardial fibroelastosis
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* unknown etiology
* diffuse thickening of endocardium * few live > 2 yrs * limited to LEFT chambers in 80% * causes restrictive cardiomyopathy |
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congenital heart disease
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* INCREASED PULMONARY BLOOD FLOW
o Acyanotic + L -> R shunts o Cyanotic + admixture ("T") lesions * NORMAL / DECREASED PULMONARY BLOOD FLOW o Acyanotic + normal + aortic coarctation + cor triatriatum + cardiomyopathy + congenital mitral stenosis + hypoplastic left heart o Cyanotic + tetralogy of Fallot + pulmonary atresia + TAPVR III + Ebstein anomaly |
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total anomalous pulmonary venous return (TAPVR)
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admixture lesion: cyanosis, increased pulmonary blood flow
* incidence 2% * must have R->L shunt for survival (eg, ASD) * Darling's classification o Type I = supracardiac (55%) - "Snowman heart" o Type II = cardiac (30%) o Type III = infracardiac (12%) associated with * ASD (needed for survival) * asplenia - 3/4 of cases have TAPVR see congenital heart disease |
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admixture lesion
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cyanosis + increased pulmonary blood flow
* transposition (TGV) * truncus arteriosis * TAPVR * tricuspid atresia (sort of "intracardiac TAPVR") * single ventricle * common atrium * hypoplastic left heart * DORV (double outlet right ventricle) |
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asplenia / polysplenia syndromes
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probably different degrees of the same disorder
* asplenia (Ivemark syndrome, bilateral right sidedness) o more severe o right lung lobar anatomy bilaterally, bilateral eparterial bronchi o associated with cardiac, GI/GU and situs anomalies + TAPVR(~100%),ECD(85%)and many others o Howell-Jolly bodies - RBC inclusions * polysplenia (bilateral left-sidedness) o left lung lobar anatomy bilaterally, bilateral hyparterial bronchi o multiple spleens/splenules o bilateral SVCs, azygos continuation of IVC o associated with cardiac(less common than in asplenia), GI/GU and situs anomalies |
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hypoplastic left heart syndrome
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underdevelopment of the left side of the heart characterized by:
* aortic valve atresia * hypoplastic ascending aorta * hypoplastic/atretic mitral valve * endocardial fibroelastosis most common cause of CHF in neonate * 25% of cardiac deaths in 1st week of life * prognosis if untreated: 100% fatal by 6 weeks hemodynamics: * pulmonary venous return is diverted from LA to RA through ASD * RV supplies pulmonary artery, ductus arteriosus, descending aorta (antegrade flow), aortic arch, ascending aorta, coronary circulation (retrograde flow) * leads to RV work overload and CHF Rx: * Norwood procedure (palliative) - three stage surgery that results in the RA feeding the pulmonary circulation and the RV feeding the systemic circulation. * transplant |
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congenital insensitivity to pain
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* autosomal recessive
* neuropathic joints * micro- and macrofractures * epiphyseal separation * osteomyelitis (mandible, fingers, toes) DDx: * congenital insensitivity to pain with anhidrosis (aut. rec.) * hereditary sensory radicular neuropathy (aut. rec.) * congenital sensory neuropathy (aut. dom.) * familial dysautonomia (aut. rec.) * Lesch-Nyhan syndrome (X rec.) |
|
|
congenital renal cysts
|
* congenital solitary cyst
* multilocular cyst * multicystic disease (renal dysplasia) * polycystic disease o autosomal-recessive ("childhood") form o autosomal-dominant ("adult") form * medullary sponge kidney * medulary cystic disease see also: Potter syndrome |
|
|
multicystic dysplastic kidney disease
|
* most severe form of renal dysplasia
* most common cause of palpaple abdominal mass in infants * unilateral (90%; fatal if bilateral) * ureter/renal artery absent or hypoplastic * NOT associated with other cysts or with periportal fibrosis * Potter type II |
|
|
congenital renal osteodystrophy
|
tubular form of renal osteodystrophy
* vitamin D-resistant rickets * Fanconi syndrome * renal tubular acidosis |
|
|
vitamin D-resistant rickets
|
* X-linked dominant defect in renal tubular resorption of phosphate
* presents in 1st yr * progressive limb deformities xray: * less severe changes than other rickets * presents later DDx features: * family hx * normal serum calcium * marked hypophosphatemia (decreased PO4) * no secondary hyperPTH |
|
|
congenital sinus tumor
|
* midline depression or tract
* lined with stratified squamous epithelium * most common sites: o lumbosacral area o occiput * epidermoid or dermoid cyst anywhere along tract |
|
|
congenital syphilis
|
* Wimberger sign
* periostitis * part of ToRCHS complex |
|
|
congenitally short esophagus
|
very rare
findings: * nonreducible intrathoracic gastric segment * short straight esophagus * circular narrowing at GE junction frequently with ulcer * GE reflux see also: hiatal hernia |
|
|
Which organism is associated with pneumatocele formation
|
Staph
|
|
|
An important clue to the correct diagnosis of diaphragmatic hernia?
|
is the absence or paucity of gas-filled bowel loops within the abdomen
|
|
|
Congenital diaphragmatic hernias most often occur through?
|
foramen of Bochdalek, which lies posteriorly and medially in each hemidiaphragm
|
|
|
Where do the foramen of Morgagni and Bochdalek lie?
|
Morgagni: anterior
Bochdalek: Post/Medial |
|
|
Which cells produce surfactant
|
Type 2 Alveolar
surfactant decreases surface tension of the alveoli |
|
|
What causes BPD
|
positive pressure ventilation
high O2 concentration which damages structually immature lung low birth weight/prematurity are predisposing factors |
|
|
What are the 2 phases of BPD
|
Edematous Phase
Bubbly Phase |
|
|
Retained fetal lung fluid
|
result of delayed clearance of the fluid normally present in the fetal lung.
common in infants delivered by cesarean section, presumably caused by the lack of squeezing of the chest as it passes through the vaginal canal |
|
|
Pulmonary lymphangiectasia
|
associated with congenital heart disease or generalized lymphangiectasia
|
|
|
ECMO is used for which conditions
|
congenital diaphragmatic hernia meconium aspiration syndrome neonatal sepsis
pneumonia |
|
|
What are 2 clues to a subpulmonic effusion
|
Flattening of the diaphragm
Lateral displacement of the dome |
|
|
Unilateral Pleural effusions likely represent?
|
pneumonia
|
|
|
Sources of Diffusely Hazy or Reticular Lungs in the Neonate
|
Decreased lung volumes
Poor inspiration Hyaline membrane disease Normal to increased lung volumes Retained fluid Aspiration (amniotic fluid/meconium) Pneumonia Pulmonary edema Pulmonary lymphangiectasia |
|
|
Possible Causes of Pleural Effusions
|
Unilateral
Pneumonia/empyema Chylothorax Iatrogenic Trauma Intra-abdominal inflammation Intrathoracic neoplasm Ruptured aneurysm of ductus arteriosus Bilateral Renal disease Lymphoma Neuroblastoma Congestive heart failure Collagen vascular diseases Fluid overload |
|
|
bronchogenic cyst
|
abnormality of primitive foregut (ventral segment = tracheobronchial tree; dorsal segment = esophagus)
* lined with columnar respiratory epithelium * CT: water density (50%); higher density (50%); may have air/fluid level # mediastinal brochogenic cyst (86%) * associated with spinal abnormalities * M:F = 1:1 * stridor, dysphagia; usually assymptomatic * may communicate with tracheal lumen * location: posterior mediastinum (50%), pericarinal (35%), superior mediastinum (14%); usually on right # intrapulmonary bronchogenic cyst (14%) * M>F; LL:UL = 2:1 (usually medial third) * infection (75%), dyspnea, hemoptysis * 36% will eventually contain air |
|
|
broncho-pulmonary sequestration
|
* congenital lung malformation
* portion of lung receives blood supply from SYSTEMIC artery * types o intralobar o extralobar * usually LEFT lower lobe |
|
|
intralobar sequestration
|
nonfunctioning portion of lung WITHIN visceral pleura of a pulmonary lobe
* LEFT lower lobe (67%); rarely in upper lobes * arterial supply: SYSTEMIC, usually aorta * venous drainage: usually via pulm. vv. (occ. systemic) * usually does NOT communicate with bronchial tree * cystic, may become infected * not a/w other abnormalities see: sequestration Cf: extralobar sequestration |
|
|
extralobar sequestration
|
"accessory lung": lung tissue in its own pleura
* LEFT lower lobe (90%) * systemic arterial AND venous connection (unlike intralobar) results in L->R shunt * a/w congenital anomalies of diaphragm: o eventration o paralysis o ipsilateral hernia * XR: homogeneous ST mass, rarely infected see also: sequestration Cf: intralobar sequestration |
|
|
A mass connected to an unusually large vessel is likely
|
pulmonary arteriovenous malformation
|
|
|
central, oval-shaped nodule associated with overaeration of the involved lobe suggests
|
mucocele in a patient with bronchial atresia
|
|
|
Pulmonary hamartoma
|
benign congenital tumor flocculent calcifications
|
|
|
Ewing sarcoma
|
small, round-cell sarcoma of mesenchyme of medullary bone
* age 5 - 14 yrs * most lethal of all 1' bone tumors * any bone o < 20 y/o: long bones o > 20 y/o: flat bones (where there's still red marrow) * purely lytic (62%), purely sclerotic (15%) * periosteal reaction (onion-skin or perpendicular) * similar lesion at different age o < 5 y/o -- neuroblastoma o > 30 y/o -- mets, reticulum cell sarcoma |
|
|
DiGeorge syndrome
|
* absent thymus(aplasia) + parathyroids
o (branchial cleft anomaly) * can't diagnose from CXR o (stress or infection --> involution of thymus) caused by faulty development of the third and fourth pharyngeal pouches |
|
|
Cystic hygroma
|
congenital malformation of lymphatic origin that commonly arises in the neck. Cystic hygroma tends to be locally invasive and often extends into the mediastinum
|
|
|
dermoid / epidermoid tumor
|
"intracranial pearly tumor"
* congenital ectodermal tumor * stratified squamous capsule secretes cholestrine + desquamated cells * site: o petrous apex / CPA (most common) o suprasellar cistern (parasellar mass) o cerebral hemisphere o cerebellum / 4th ventricle * CT: o low density (d/t fat content); occ. high density o no enhancement o extra-axial |
|
|
What causes GI duplication cysts
|
abnormal development of the posterior division of the primitive foregut.
they generally do not communicate with the esophagus |
|
|
Aortic aneurysms in children can be seen in pts with
|
Trauma
Ehler-Danlos Marfans |
|
|
Ehlers-Danlos syndrome
|
* autosomal dominant
* connective-tissue disorder: o hyperelasticity of skin, poor wound healing o hyperextensibility of joints o soft-tissue calcifications (spheroids) * vascular lesions: o aortic dissection o aneurysms o rarely: tortuous arch, ectatic pulmonary aa. o tissue fragility ==> hematomas, aortic rupture after angio * clinical types: o gravis, mitis, benign hypermobile, ecchymotic, X-linked * a/w ** medullary sponge kidney |
|
|
Marfan syndrome
|
connective-tissue disorder: autosomal dominant, 15% sporadic
* cardiovascular system (prob. cause of death in 93%) o aortic aneurysm (mostly ascending) o dilatation of aortic sinuses o aortic dissection o coarctation * musculoskeletal system o tall stature, long limbs o arachnodactyly o lax joints * spine o scoliosis (60%) / kyphosis / pectus o dural ectasia * eye o lens subluxation Cf: homocystinuria |
|
|
Coronary Artery Aneurysms are commomly seen in pts with
|
Polyarteritis Nodosa
Mucocutaneous Lymph node Syndrome |
|
|
polyarteritis nodosa
|
necrotizing vasculitis of medium-sized arteries
* usually in male adults * associated with hepatitis B antigen * kidney: most frquently involved organ (85%) o multiple small intrarenal aneurysms o aneurysms may disappear (thrombosis) or appear in new locations o arterial narrowing and thrombosis (chronic/healing stage) o multiple small cortical infarcts o associated with hypertension and renal failure * chest involvement (70%) o cardiomegaly/pericardial effusion (14%) o wedge shaped/round peripheral infiltrates simulating PE (14%) o interstitial lower lung field pneumonitis * also may involve liver (66%), mesenteric vessels (50%), skeletal muscle (39%), skin (20%) |
|
|
neuroblastoma
|
* most common solid abdominal mass of infancy (12% of all perinatal neoplasms)
* 3rd most common malignancy in infancy (after leukemia and CNS tumors) * 2nd most common tumor in children (Wilms more common in older kids) location: anywhere within sympathetic neural chain * adrenal gland (36%): bilateral in 7-10% * extraadrenal in sympathetic chain (18%) * thorax and posterior mediastinum (14%) * neck (5%); pelvis (5%) * skull, olfactory bulb, cerebellum, cerebrum: (2%) * other sites (10%); unknown primary (10%) metastasis: bone (60%); regional nodes (42%); liver (15%); intracranial (14%); lung (10%) see also: Hutchinson, Pepper, and blueberry muffin syndromes |
|
|
Hutchinson syndrome
|
1. primary adrenal neuroblastoma
2. extensive skeletal metastases, particularly skull 3. proptosis (metastases to orbit) 4. bone pain |
|
|
Pepper syndrome
|
* primary adrenal neuroblastoma
* massive hepatomegaly from metastases |
|
|
blueberry muffin sign
|
* raised, purple skin lesions
* dermal metastases of neuroblastoma (stage IV-S) |
|
|
Normal vascularity is seen in which cases of cong heart disease
|
uncomplicated valvular disease
coarctation of the aorta mild forms of cardiomyopathy |
|
|
Asymmetry of pulmonary blood flow is most commonly seen in which cases of congenital heart disease
|
tetralogy of Fallot
persistent truncus arteriosus valvular pulmonic stenosis |
|
|
An enlarged PA may represent?
|
generalized increased pulmonary blood flow poststenotic dilation caused by valvular pulmonic stenosis (pulmonary valve insufficiency caused by increased rv output
ventricular output |
|
|
A small PA may represent?
|
Pulmonary outflow obstruction ie:
Truncus Arteriosis Transposition of Great Vessels |
|
|
A small Aorta may represent?
|
Hypoplastic Left heart
L to R shunts (ASD/VSD) |
|
|
A large Aorta may represent?
|
(MC) post stenotic dilitation from aortic stenosis
Aortic Valve insufficiency L to R shunting at great vessel level PDA/PTA/TOF |
|
|
Coarctation
|
A narrowing of the aorta where the aorta and pulmonary arteries are joined by the ductus arteriosus
|
|
|
PDA beneficial in:
|
* tetralogy of Fallot
* Eisenmenger pulmonary hypertension * interrupted aortic arch (supply lower extremity via PDA) |
|
|
Right Sided Aortic Arch
A/W |
often just isolated anomaly
TA TOF |
|
|
right aortic arch
|
TYPES:
* with aberrant LEFT subclavian artery (5% ==> CHD) o most common type of right arch o second most common cause of vascular ring * with mirror image branching (95% associated with congenital ht dz) o 90% tetralogy of Fallot (TOF) (25%) ** decreased PBF o 2.5% truncus (30-50%) ** increased PBF o 1.5% transposition (TGV) (5%) * with isolated left subclavian artery |
|
|
Causes of Acyanotic HD with increased pulmonary vascularity
|
VSD
ASD PDA |
|
|
Eisenmenger syndrome
|
* pulmonary aterial HTN
o heart size normal (or slightly increased) o big central PAs with abrupt tapering * bidirectional or reversed cardiac shunt |
|
|
Eisenmenger physiology
|
Reversal or balancing of an initial L to R shunt
|
|
|
atrial septal defect (ASD)
|
most common CHD presenting in adults
* 8-14% of CHD * M:F 1:4 * secundum > sinus venosus > primum * average lifespan: 40 yrs CXR: * shunt vascularity with >2:1 shunt * normal aortic knob * enlargment of pulmonary arteries * loss of visualization of SVC due to RVH and clockwise rotation of heart complications: * atrial fibrillation * pulmonary artery HTN (15%) * Eisenmenger syndrome (6-9%, NOT related to ASD size) Lutembacher syndrome: ASD + mitral stenosis |
|
|
Lutembacher syndrome
|
atrial septal defect + mitral stenosis (ASD + MS)
* increased pulm blood flow * cephalization (PVH) |
|
|
Aortopulmonary window
|
failure of complete division of the primitive truncus arteriosus, which leaves a communication between the aorta and the PA just above the valves. similar to a PDA
|
|
|
transposition of the great vessels
|
"TGV" / transposition:
aorta arises from RV pulm artery arises from LV intracardiac shunt increased pulmonary vascularity cyanosis xray: heart size may be normal at birth gradually enlarges: globular or egg-on-its-side appearance |
|
|
cyanosis
|
A bluish cast to the skin resulting from increased deoxyhemoglobin in the blood.
Increased pulmonary blood flow and cyanosis = admixture lesions TGV (transposition) -- most common cause on 1st day of life TAPVR (total anomolous pulmonary venous return) truncus arteriosis tricuspid atresia with TGV (25% of TA cases) single ventricle common atrium hypoplastic left heart DORV (double outlet right ventricle) Decreased pulmonary blood flow and cyanosis- right to left shunts TOF (tetralogy of Fallot) -- most common overall Ebstein's anomaly tricuspid atresia without TGV (75% of TA cases) pulmonic valvular atresia |
|
|
truncus arteriosus
|
right aortic arch (30-50%)
high arch elevaated left PA VSD always immediately beneath common truncal valve "waterfall" or "hilar comma" sign (types II + III) Collet & Edwards classification I short main PA arises from left post-lat aspect II PAs arise separately from left post-lat aspect III each PA arises from lateral aspects IV PAs arise from dorsal aorta ("pseudotruncus") probably tetralogy of Fallot with pulmonary atresia truncus fails to divide into 2 separate structures the aort and the pulm artery |
|
|
What is the most critical component of TOF
|
Pulmonary Stenosis and therefore degree of r to l shunting
|
|
|
Name the 4 components of TOF
|
VAPR
obstructed RV outflow tract VSD RVH Aorta overriding septum |
|
|
causes of right ventricular hypertrophy
|
pulmonary valve stenosis
pulmonary hypertension tetralogy of Fallot VSD |
|
|
Causes of Right Aortic Arch
|
right aortic arch
-------------------------------------------------------------------------------- TYPES: with aberrant LEFT subclavian artery (5% ==> CHD) most common type of right arch second most common cause of vascular ring with mirror image branching (95% associated with congenital ht dz) 90% tetralogy of Fallot (TOF) (25%) ** decreased PBF 2.5% truncus (30-50%) ** increased PBF 1.5% transposition (TGV) (5%) with isolated left subclavian artery |
|
|
What drug is used to help maintain a PDA
|
Prostaglandin E1
|
|
|
Uhl disease
|
"parchment right ventricle"
focal or complete absence of RV myocardium |
|
|
What changes on a cxr result from LVH
|
rounded appearance of left heart border
|
|
|
What changes on a cxr result from RVH
|
retrosternal fullness
upward displacement of cardiac apex |
|
|
Williams elfin-facies syndrome
|
sporadic, congenital disorder
idiopathic hypercalcemia of infancy supravalvular aortic stenosis facial dysmorphism associated with medullary nephrocalcinosis pancreatitis |
|
|
What aare the 2 types of coarctation
|
preductal or infantile
juxtaductal or adult |
|
|
Cor Triatriatum
|
pumonary venous obstruction secondary to an abnl connection of the pulm veins with the LA
|
|
|
Situs Solitus
|
Normal Position of the Viscera
|
|
|
Concordant
|
refers to structures that remain in normal relation to one another
|
|
|
Which skin lesion is associated with esophageal atresia?
|
Epidermolysis Bullosa
|
|
|
What are the 2 classications of gastric volvulus
|
organoaxial
mesoaxial |
|
|
what are the dimensions of the sphincter found in pyloric stenosis
|
thickness > 3 mm
pyloric canal > 14 mm |
|
|
duodenal atresia
|
double bubble sign
polyhydramnios associated with Down syndrome VATER syndrome NB: annular pancreas also may cause duodenal obstruction, and is associated with VATER syndrome |
|
|
Ladd's Bands
|
Peritoneal bands in patients with malrotation
bands extend from cecum to porta hepatis may cause duodenal obstruction |
|
|
Apple peel small bowel
|
familial
diffuse atresia nd stenosis of small bowel |
|
|
meconium ileus
|
obstructed bowel due to impacted, tenacious meconium
no air-fluid levels (probably d/t adherent bowel contents) DDx: ileal atresia Hirschsprung dz anal atresia a/w cystic fibrosis (CF |
|
|
meconium plug
|
"functional colonic inertia"
Dx/Tx: Gastrografin enema (DDx: Hirschsprung dz, meconium ileus) F/U with another enema for confirmation a/w maternal diabetes caused by abnl peristalsis |
|
|
meconium aspiration syndrome
|
intra-uterine fetal distress ==> defecation of meconium
term + post-term neonates NOT preemies NOT kids of diabetic moms diffuse HYPERaeration pulm interstitial emphysema (PIE) pnuemomediastinum + non-tension ptx in 33% patchy atelectasis + consolidation lung dz usually resolves spontaneously morbidity d/t intrauterine cerebral anoxia |
|
|
What is the earliest manifestation of CF
|
Meconium Ileus
|
|
|
How 2 distinguish ileal atresia from meconium ileus?
|
ileal atresia has air/fluid levels.
tx=surgery |
|
|
Hirschsprung disease
|
aganglionosis of myenteric plexus of colon
functional obstruction aganglionic segment of normal caliber continuous involvement, from rectum proximally males (80%) usually presents by 6 wks complications: enterocolitis perforation a/w Down syndrome DDx: ileal atresia meconium ileus (no air-fluid levels) dx made by biopsy look for transition zone |
|
|
Functional megacolon
|
spasm of the puborectalis muscle
|
|
|
hydrometrocolpos
|
accumulation of secretions in the vagina and uterus
dilatation of the vagina proximal to a congenital obstruction (e.g., imperforate hymen) produces a palpable, fixed midline mass may cause hydronephrosis AXR: abdominal mass extending from pelvis US: cystic mass posterior to bladder with scattered echoes due to cellular material and blood Cf: hydrometros = uterine dilatation only (obstruction at cervix) |
|
|
Cremin's M Line
|
Imperforate Anus
The M line is drawn horizontally through the junction of the middle and lower thirds of the ischium (M). The line demarcates the level of the puborectalis sling |
|
|
What is a common cause of gastritis in infants that leads to vomiting and bleeding
|
Milk allergy
|
|
|
Where does regional enteritis commonly occur
|
terminal ileum
|
|
|
necrotizing enterocolitis
|
"NEC"
pneumatosis intestinalis submucosal: initial finding, mult. cystic lucencies; looks like stool (!) subserosal: linear lucencies portal vein: +/- transient; not significant etiology: preemie, perinatal stress, intestinal ischemia survivors may develop intestinal strictures caused by hypoxia/ischemia |
|
|
pneumatosis intestinalis
|
multiple thin-walled, non-communicating, gas-filled cysts located in subserosa +/- submucosa with normal mucosa and muscularis
primary pneumatosis intestinalis (15%) secondary pneumatosis intestinalis (85%) ischemia necrotizing enterocolitis mesenteric vascular disease trauma (sigmoidoscopy, biopsy, BE, postsurgical anastomosis) infection (primary infection, parasites, perforated jejunal tic) inflammation inflammatory bowel disease connective tissue disease (esp. scleroderma) Whipple disease pulmonary dz (COPD, artificial ventilation) colonic obstruction (air dissecting distally) |
|
|
What is the hallmark of NEC
|
pneumatosis intestinalis
always look for air in the portal vein |
|
|
pneumatosis cystoides
|
"pneumatosis cystoides intestinalis"
multiple gas filled cysts of various sizes located in subserosa (sometimes in submucosa) age: adulthood location: predominantly in lower colon X-ray: best demonstrated on CT segmental mucosal nodularity (DDx: polyposis) complication: assymptomatic large pneumoperitoneum (may persist for months/years) |
|
|
appendicitis
|
Alvarado score
M migration of pain = 1 pt. A anorexia/acetone = 1 pt. N nausea/vomiting = 1 pt. T tenderness RLQ = 2 pts. R rebound = 1 pt. E elevated temp = 2 pts. L leukocytosis (>10K) = 1 pt. S shift to left (>75%) = 1 pt. 5-6 pts = compatible 6-7 pts = probable 8-9 pts = very probable |
|
|
Omental infarction
|
mimics appendicitis clinically but can be distinguished from appendicitis on US and CT. The infracted omental segment has the appearance of a heterogeneous mass that is usually located between the anterior abdominal wall and the colon
|
|
|
Mesenteric adenitis
|
is a self-limiting inflammatory condition involving mesenteric lymph nodes and is frequently viral in etiology. US usually demonstrates a cluster of enlarged lymph nodes in the right lower quadrant and a normal appendix.
|
|
|
What group of children are predisposed to developing peritonitis
|
Nephrotic syndrome
|
|
|
Causes of cholecystitis in children
|
sickle cell disease, congenital obstructive anomalies of the biliary tract, total parenteral nutrition, furosemide treatment, dehydration, hemolytic anemia, and short gut syndrome
|
|
|
2 causes ofbiliary atresia/hepatitis in the neonatal period
|
hep b
CMV alpha 1 antitrypsin def Byler disease |
|
|
alpha fetoprotein (aFP)
|
ELEVATED in:
wrong dates multiple gestation midline closure (neural-tube) defects encephalocele anencephaly liver tumors hepatoblastoma hemangioendothelioma (occ., mild) hepatocellular carcinoma (HCC, "hepatoma") DECREASED in: wrong dates chromosome abnl (incl. Down syndrome) |
|
|
pancreas divisum
|
unfused ducts of Wirsung and Santorini
incidence: 5-11% 21-45% get pancreatitis, possibly due to stenosis of the minor ampulla |
|
|
Meckel diverticulum
|
rule of 2's
2 cm wide 2 feet from I-C valve males 2:1 2% of population antimesenteric (unlike duplication cyst) +/- ectopic gastric/pancreatic tissue see: Meckel scan |
|
|
What type of ectopic tissue may be found in a Meckel's diveticvulum
|
pancreas
gastric 25% |
|
|
Abdominal calcifications in a neonate may be C/w
|
meconium peritonitis
|
|
|
Hutch bladder diverticulum
|
bladder tic near uretero-vesical junction
weakens detrusor muscle may lead to VU reflux |
|
|
How is VUR graded
|
Reflux into the distal ureter is grade I.
Reflux into the upper collecting system with no dilation of the upper tract is grade II. Grade III reflux shows similar findings, but with mild blunting of the calices. All of these findings are exaggerated in grade IV reflux, with marked hydroureter and caliceal dilatation. When the ureter is massively dilated and tortuous and the upper tract is markedly dilated, grade V reflux is present |
|
|
high-output hydronephrosis is rare but may be seen with
|
Bartter syndrome
diabetes insipidus psychogenic water drinking |
|
|
primary mega-ureter
|
aperistaltic segment of distal ureter
no anatomic obstruction males (80%) left > right 20% bilateral diagnosis of exclusion may be a/w UPJ obstruction, reflux |
|
|
In ureteral duplication which ureter is in an ectopic location
|
superior pole
|
|
|
Ureterocele
|
saccular dilated segment of the distal ureter that invaginates into the bladder lumen and impedes the flow of urine from the ureter into the bladder
|
|
|
Causes of Renal Cysts
|
Single
Simple cyst Caliceal diverticulum Abscess Multilocular cystic nephroma Multiple Multicystic dysplastic kidney Polycystic kidney disease Glomerulocystic disease Medullary cystic disease (juvenile nephronophthisis) Tuberous sclerosis Turner syndrome von Hippel-Lindau disease Zellweger syndrome Beckwith-Wiedemann syndrome Meckel-Gruber syndrome |
|
|
Causes of Echogenic Renal Pyramids (nephrocalcinosis)
|
Normal neonate
Tamm-Horsfall proteinuria Sickle-cell disease Hypercalciuria Renal tubular acidosis Medullary sponge kidney Hyperparathyroidism Drugs (furosemide, steroids, vitamin D) Prolonged immobilization Bartter syndrome Williams syndrome Autosomal recessive polycystic kidney disease Storage diseases Glycogen-storage disease type 1A Hurler mucopolysaccharidosis Lesch-Nyhan syndrome Oxalosis may be normal in newborns at the tips of the medullary pyramids |
|
|
detrusor hyperreflexia
|
Upper motor neuron abnormalities, which occur above the level of the pons, result in loss of voiding control and spastic bladder
|
|
|
detrusor-sphincter dyssynergy
|
abnormal external sphincter contraction occurs during contraction of the detrusor muscle, preventing urination and increasing the pressure within the bladder. This condition, known as
|
|
|
Multiple bladder diverticula may be associated with which syndromes
|
Ehlers-Danlos syndrome, Williams syndrome,
Menkes syndrome prune belly syndrome |
|
|
Megacystitis may be seen in 2 syndromes
|
Prune Belly (Eagle-Barrett)
MMH Megacystis-microcolon-hypoperistalsis (MMH) syndrome |
|
|
What is Megacystis-microcolon-hypoperistalsis (MMH) syndrome
|
disorder of smooth muscle in the urinary and GI tract. exclusively in girls Insufficiency of the abdominal musculature
bladder is very large and dysfunctional. Decreased intestinal peristalsis leads to poor evacuation of the colon in infants who survive. Bladder exstrophy occurs when the bladder is exposed through a large defect in the anterior abdominal wall. Marked widening of the symphysis pubis and splaying of the pelvic bones are noted on radiographs in these infants |
|
|
rhabdomyosarcoma
|
extremely malignant tumor of totipotential mesenchyme
peak age: 3 - 5 yrs, 15 - 18 yrs site: pelvis / GU tract (39%) head / neck (31%) types: embryonal (incl. sarcoma botryoides) alveolar pleomorphic see also: bladder rhabdo sarcoma botryoides |
|
|
bladder rhabdomyosarcoma
|
usually arises at trigone
causes outlet obstruction --> big bladder DDx: hemorrhagic cystitis (small bladder, filling defects) |
|
|
sarcoma botryoides
|
type of embryonal rhabdomyosarcoma
mass resembles "bunch of grapes" originates submucosally, grows into lumen |
|
|
What causes a hydrocele
|
Peritoneal fluid passes into the scrotum through a patent processus vaginalis.
most resolve by 2 yos |
|
|
What types of Posterior urethral valves are there
|
1: Abnormal migration and insertion of the urethrovaginal folds result in sail-like flaps of tissue that arise at the base of the prostatic urethra below the verumontanum,
The valves cause obstruction to antegrade flow of urine, leading dilatation of the posterior urethra, bladder wall thickening and trabeculation, and VUR 2: consists of a nonobstructive mucosal fold rather than an obstructing membrane. 3: posterior urethral valves consist of a membrane caused by incomplete canalization in the region of the urogenital diaphragm it is possible to have an anterior urethral valve but they are rare |
|
|
Cryptorchidism
|
undescended testis
4% of term newborn boys The majority of undescended testes will spontaneously descend into the scrotal sac by 1 year of age |
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Gonadoblastoma
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neoplasm that occurs in children with intersex disorders, usually arising in the gonadal streaks or intra-abdominal testes of phenotypic females
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nephroblastomatosis
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persistent primitive renal tissue (normally present up to 36 weeks)
potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor associated with: hemihypertrophy sporadic aniridia pseudohermaphroditism Klippel-Trenaunay syndrome Beckwith-Wiedemann syndrome splenic agenesis with hepatic malformation findings: kidneys may be enlarged deformity of the pelvocalyceal system US: subtle subcapsular nodules (hypo-/iso-/hyperechoic) CT: hypodense subcapsular nodules after contrast enhancement usually spont regress by 4 mos |
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Wilms tumor
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nephroblastoma
3rd most common kiddie tumor (most common abd tumor) arises from embryonal renal tissue (nephroblastomatosis) large only 10% are calcified 4-10% bilateral mets --> lungs, para-aortic nodes associated with: aniridia Beckwith-Wiedemann syndrome hemihypertrophy Drash syndrome Cf: neuroblastoma (more likely calcified) |
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What should you always look for when ruling out torsion
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spiral appearance of the spermatic cord
decreased flow |
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Causes of Renal Enlargement
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Bilateral
Diffuse renal disease (e.g., nephrotic syndrome, glomerulonephritis) Diabetic mother Autosomal recessive or autosomal dominant polycystic kidney disease Leukemia, lymphoma Hemolytic uremic syndrome Henoch-Schönlein purpura Beckwith-Wiedemann syndrome Glycogen-storage disease Tuberous sclerosis Nephroblastomatosis Unilateral Hydronephrosis Duplication anomaly Compensatory hypertrophy Crossed fused ectopia Multicystic dysplastic kidney Renal abscess Renal neoplasm Renal vein thrombosis |
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What is the prognosis of Wlms tumor dependent upon
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histology
resectability |
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It is important to look at what structures when considering a dx of Wilms tumor
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extension into renal vein
IVC RA |
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Claw sign
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renal parenchymal stretched along the margin of a mass
MC= WIlms tumor |
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how does WIlms tumor usually present
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assymptomatic abdominal mass
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name 2 rare renal tumors
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clear cell sarcoma
rhabdoid tumor |
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Similar radiographic entities when considering Wilms tumor
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Renal Cell Carcinoma
Clear Cell Sarcoma (bone mets) Rhabdoid Tumor (brain mets) |
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adrenal hemorrhage
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neonate
* RIGHT more common, 10% bilateral * birth trauma * hypoxia * septicemia * congenital syphilis * hemorrhagic disorders (hemophilia, etc.) adult * septicemia (Waterhouse-Friderichsen syndrome) * tumor * trauma Notes: * usually resolves in 4-6 wks * adrenal insufficiency rare, even if bilat. * may calcify |
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neuroblastoma
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* most common solid abdominal mass of infancy (12% of all perinatal neoplasms)
* 3rd most common malignancy in infancy (after leukemia and CNS tumors) * 2nd most common tumor in children (Wilms more common in older kids) location: anywhere within sympathetic neural chain * adrenal gland (36%): bilateral in 7-10% * extraadrenal in sympathetic chain (18%) * thorax and posterior mediastinum (14%) * neck (5%); pelvis (5%) * skull, olfactory bulb, cerebellum, cerebrum: (2%) * other sites (10%); unknown primary (10%) metastasis: bone (60%); regional nodes (42%); liver (15%); intracranial (14%); lung (10%) contrast to Wilms it is poorly marginated and frequently extends into the chest commonly calcifies, Wilms does not |
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Hutchinson syndrome
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1. primary adrenal neuroblastoma
2. extensive skeletal metastases, particularly skull 3. proptosis (metastases to orbit) 4. bone pain |
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Pepper syndrome
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* primary adrenal neuroblastoma
* massive hepatomegaly from metastases |
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blueberry muffin sign
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* raised, purple skin lesions
* dermal metastases of neuroblastoma (stage IV-S) |
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Iodine-131-meta-iodobenzylguanidine
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is a tracer that resembles norepinephrine and is metabolized by neuroblastoma, pheochromocytoma, and other catecholamine-producing tumors. Octreotide is a ligand for G-protein receptor cell membranes. These two tracers have improved detection of primary tumor and metastases in some cases
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Adrenocortical carcinoma
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highly malignant
CT and US characteristics similar to those of neuroblastoma metastasizes to the lungs, liver, and regional lymph nodes. The tumor frequently causes endocrine symptoms, such as virilization and Cushing syndrome. Hypercortisolism may cause an increase in retroperitoneal fat that is visible on CT and MR and is a clue to the diagnosis. |
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Adrenal elargement
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adrenocortical hyperplasia which causes adrenogenital syndrome
cerebriform looking adrenals infants treated with adrenocorticotropic hormone for spasm |
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Wolman disease
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rare, familial xanthomatosis
* causes death early in infancy * adrenal calcification o diffuse, punctate calcifications throughout o enlarged, normally shaped adrenals * hepatosplenomegaly |
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Gallbladder Hydrops has been associated with what disease
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Mucocutaneous lymph node syndrome
(Kawasaki's Disease) |
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choledochal cyst
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* segmental dilatation of CBD
* F >> M * 60% under 10 y/o * classic clinical triad in < 40% o jaundice, RUQ mass, abd pain * choledochocele (type 3 cyst): bloops into duodenum * 8% a/w Caroli disease |
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Caroli disease
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communicating, cavernous ectasia of the intrahepatic bile ducts
* rare, autosomal recessive * usually detected in childhood or early adulthood * no cirrhosis or portal HTN * predisposed to calculus formation * recurrent cholangitis ==> liver abscesses ==> death * increased risk of cholangiocarcinoma * a/w medullary sponge kidney (renal tubular ectasia) in 80% |
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What usually causes choledocal cysts
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anomalous junction of the common bile duct and pancreatic duct (abnormal pancreatobiliary junction [APBJ]). The APBJ allows pancreatic enzymes to reflux into the common bile duct, which may lead to inflammation and weakening of the bile duct wall
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liver hemangioendothelioma
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* females (67%)
* infants (90% before 6 m/o) * sx: mass (hepatomegaly) * cutaneous hemangiomas (45%) * mildly increased aFP (occasionally) * +/- CHF 2' to shunting (only 4%) * usually diffuse, precludes surgery * most involute spontaneously or with steroids DDx: o mesenchymal hamartoma o cavernous hemangioma o hepatoma o hepatoblastoma (increased aFP) o metastatic neuroblastoma (increased serum VMA) |
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Mesenchymal hamartoma
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uncommon benign tumor seen most often in infants and young children
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Hepatic adenomas are rare in childhood but have been reported in association with
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Fanconi anemia
glycogen-storage disease type 1 Hurler disease severe combined immunodeficiency. |
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hepatoblastoma
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* 3rd most common intra-abd malignancy (after neuroblastoma + Wilms tumor)
* kids: 50% before 18 m/o, almost all < 3 y/o * right lobe (75%), both lobes or multicentric (33%) * mets at dx in 10% ==> local invasion, regional nodes, lungs * increased aFP in 67-90% * liver enzymes usually normal associated with * hemihypertrophy * macroglossia * sexual precocity Assoc with Beckwith-Wiedemann and Familial adenomatous polposis |
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Beckwith-Wiedemann syndrome
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* big tongue
* organomegaly (liver, kidneys, pancreas, heart) * omphalocele, umbilical hernia or diastasis recti a/w Wilms tumor More info: Beckwith-Wiedemann syndrome [OMIM] |
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familial polyposis coli
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* autosomal dominant (33% sporadic)
* adenomas ==> colon Ca o absent at birth o adenomas develop in teenagers * associations: o desmoid tumors o SB adhesions Cf: polyposis syndromes |
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splenomegaly
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* infection
o SBE, TB, mono, brucellosis, syphilis, histoplasmosis, malaria, schistosomiasis * sarcoidosis * connective tissue diseases o SLE, RA, Felty's * lymphoma, leukemia, myelofibrosis, myeloid metaplasia * anemias * congestion o portal HTN, cirrhosis, splenic vein thrombosis * storage diseases o Gaucher, Niemann-Pick, amyloidosis * masses o cyst, abscess, cavernous hemangioma |
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Cystic lymphangiomatosis
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is a benign lymphatic malformation with a characteristic multiloculated cystic appearance
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Hemophagocytic lymphohistiocytosis (HLH)
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rare disease that consists of overactive histocytes and macrophages that phagocytize the normal cellular structures of the blood.
not truly malignant and is probably caused by an inappropriate immune reaction. HLH usually occurs in infants under 1 year of age and is characterized by hepatosplenomegaly, ascites , gallbladder wall thickening, lymphadenopathy, and pleural effusion. |
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Causes of Ascites
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Newborn
Hydrops fetalis Chylous ascites Urinary tract obstruction Iatrogenic (line perforation) Intestinal perforation (necrotizing enterocolitis) Older infants and children Liver disease Nephrotic syndrome Portal vein obstruction Traumatic intestinal injury Peritonitis Hypoproteinemia Pancreatitis Ruptured abdominal cyst Intestinal lymphangiectasia GI ischemia Bile duct perforation |
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Cysts that contain gastric mucosa are detectable by scintigraphy using |
technetium-99m-pertechnate
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True congenital cysts of the pancreas are rare and occur chiefly in association with
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autosomal dominant polycystic kidney disease
von Hippel-Lindau syndrome. |
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von Hippel-Lindau syndrome
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retinocerebellar angiomatosis
* phakomatosis * autosomal dominant (variable penetrance) * hemangioblastoma: most frequent cause of death o cerebellar (most common) o also medullary and spinal * retinal angiomatosis (45%) * renal cell Ca: 2nd most common cause of death * pheochromocytoma (17%) * cortical renal cysts (75%) * cysts in virtually any organ * renal/liver hemangioma/adenoma * pancreatic cystic neoplasms, islet-cell tumors * paraganglioma |
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CSF pseudocyst
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is a complication of the use of a ventriculoperitoneal shunt for the treatment of hydrocephalus
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Solid-cystic papillary tumor of the pancreas
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uncommon tumor that contains variable amounts of cystic and solid tissue
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Tumors of the mesentery and omentum are primarily
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Burkitt lymphoma
metastases |
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sacral agenesis
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# agenesis or hypoplasia of sacrum
* part of caudal-regression syndrome * a/w maternal diabetes * +/- inherited * possible features: o absent lower extremities o bladder/bowel impairment |
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chordoma
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tumor of notochord remnants
* site: o sacrum (45%) o clivus (39%) o spine * destruction, no sclerosis * +/- calcification (30-80%) midline tumor see also: para-sellar mass |
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Anterior sacral meningoceles
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develop when a portion of the thecal sac protrudes anteriorly into the presacral space through a sacral defect
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Currarino triad
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partial sacral agenesis
anorectal stenosis presacral mass |
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juvenile rheumatoid arthritis (JRA
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* 15% seropositive
o F > M o onset > 10 yrs * 85% seronegative (Still disease) * sites: KNEES, ANKLES, hands, wrists, feet findings: * decreased bone density * early epiphyseal closure --> short bones * ankylosis of spinal apophyseal jts * big "squared" epiphyses * wide intercondylar notch * tibio-talar slant |
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Still disease
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seronegative juvenile rheumatoid arthritis
* 85% of kids with RA * uncommon: adult-onset Still dz findings: * fever * rash * adenopathy * splenomegaly |
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cervical spine fusion (ankylosis)
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* posterior elements
o JRA * vertebral bodies o block vertebrae o Klippel-Feil |
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tibio-talar slant
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* juvenile rheumatoid arthritis
* sickle-cell disease * hemophilia |
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MURCS association
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(müllerian duct, renal, and cervical vertebral defects)
Duncan described the MURCS association in 1979. It consists of a nonrandom association of müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. The incidence of cervicothoracic vertebral defects, especially from C5-T1, is 80%. Other abnormalities may include Sprengel deformity, upper limb defects, and moderately frequent rib anomalies |
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rhabdomyosarcoma
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* extremely malignant tumor of totipotential mesenchyme
* peak age: 3 - 5 yrs, 15 - 18 yrs * site: o pelvis / GU tract (39%) o head / neck (31%) * types: o embryonal (incl. sarcoma botryoides) o alveolar o pleomorphic see also: bladder rhabdo sarcoma botryoides |
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bladder rhabdomyosarcoma
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* usually arises at trigone
* causes outlet obstruction --> big bladder o DDx: hemorrhagic cystitis (small bladder, filling defects) |
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cardiac rhabdomyoma
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associated with tuberous sclerosis
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Tydings-McDuffie Act 1934
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Bill to provide independence to the Philippines by 1946. A transition government with consitutional safeguards for the people would be in place in the meantime.
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most common cause of adrenal mass in the neonate
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Adrenal hemorrhage
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what is th etriad that is pathognomonic for adrenal hemorrhage in the neonate
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triad of jaundice, anemia, and an abdominal mass in a neonate is practically pathognomonic
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Predisposing factors for the development of large ovarian cysts are
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maternal diabetes, toxemia of pregnancy, and Rh incompatibility.
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Differential diagnosis for cystic abdominal mass which persists after catheterization should include
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enteric duplication cyst, choledochal cyst, mesenteric cysts, neonatal cyst, and hydrocolpos.
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The differential diagnosis of bilateral hydroureteronephrosis should include
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bladder outlet obstruction (secondary to posterior urethral valves, urethral polyp, tumor, ureterocele), ureteral atony secondary to infection, bilateral vesicoureteral reflux, bilateral ureteral ectopia (either from single systems or from upper poles of duplex kidneys), prune-belly, neuropathic bladder, and obstructive megaureter.
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differential possibilities for diffuse bladder wall thickening,
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one should mainly consider conditions of bladder outlet obstruction or neurogenic bladder dysfunction. Focal bladder wall thickening may be caused by previous surgery, e.g., ureteral reimplantation, and tumors, such as rhabdomyosarcoma, neurofibroma, or pheochromocytoma. Chemical cystitis, e.g., secondary to cyclophosphamide therapy, can cause mucosal edema mimicking any of the above conditions.
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most common malignancy in childhood.
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Leukemia
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differential diagnosis of hypoechoic lesions of the testicles includes
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germ cell tumors; however, given the clinical history, leukemia would be the most likely possibility.
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DDX solid polypoid masses or mass-like lesions that may occur at the base of the bladder in a child include
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focal cystitis, rhabdomyosarcoma, pheochromocytoma. Neurofibromatosis
Hamartomatous Polyp |
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differential diagnosis of an anterior urethral diverticulum is
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Cowper's duct syrinx, which is typically located closer to the bulbar portion of the anterior urethra at the insertion of the Cowper's duct.
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The differential diagnosis of a periumbilical infection should also include
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persistent omphalomesenteric duct and simple omphalitis, urachal abnormality.
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most common type of soft-tissue sarcoma in childhood.
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Rhabdomyosarcoma
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DDX Bladder Masses
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hemangiomas and neurofibromas occur rarely in the bladders of children. It is also important to realize that acute viral cystitis in the child can occasionally appear as a bladder mass and can mimic bladder rhabdomyosarcoma.
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differential diagnoses, in this case, in which there is a cystic mass behind the bladder in association with ipsilateral absence of the right kidney in the right renal fossa, include
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ureterocele (with renal dysplasia), ectopic multicystic dysplastic kidney, and utricular (mullerian duct) cyst. Renal dysplasia and ectopic kidney
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most common presacral mass in a child
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Sacrococcygeal teratoma
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differential diagnosis of a presacral mass in the newborn and child includes
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sacrococcygeal teratoma, meningocele, lipoma, rectal duplication, abscess, and pelvic neuroblastoma
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differential diagnosis of a complex suprarenal mass in the neonate is
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adrenal hemorrhage, neuroblastoma, and (exophytic) hepatoblastoma. These entities can look similar on cross-sectional imaging. Laboratory value assessment and follow-up imaging may be needed to differentiate NBI from adrenal hemorrhage. In the older child, the differential diagnosis may also include Wilms' tumor. On cross-sectional studies, Wilms' tumor should be recognized as arising from the kidney with a tendency to displace vessels, while NBI not only displaces but also encases them.
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most frequent cause of hydronephrosis in childhood, accounting for approximately 50% of cases,
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Ureteropelvic junction (UPJ) obstruction followed in frequency by ureterovesical junction obstruction, ureteroceles, ectopic ureters, posterior urethral valves, prune-belly syndrome, and neuromuscular vesical dysfunction
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most frequent renal anomaly
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Ureteral duplication
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DDX Multilocular Cystic Nephroma
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Cystic wilm's
Cystic RCC |
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most frequent causes of renal parenchymal disease in childhood and adolescence are
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acute pyelonephritis, glomerulonephritis, nephrotic syndrome, and hemolytic uremic syndrome. Less frequent etiologies include sickle cell anemia, glycogen storage disease, polycystic disease, lymphoma, polycythemia, and renal dysplasia.
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differential diagnosis of Wilm's includes
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multilocular cystic nephroma, mesoblastic nephroma, and renal cell carcinoma, lymphoma, clear cell, rhabdoid.
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primary differential diagnoses of nephroblastomatosis are
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lymphoma and leukemia
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DDX Perinephric masses
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be seen in retroperitoneal fibrosis (rare in children) and in renal lymphangiomatosis which produces fluid-filled rather than solid lesions.
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DDX Wilm's
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Clear cell sarcoma, Wilms' tumor, mesoblastic nephroma, and renal cell carcinoma can have appearances similar to that of malignant rhabdoid tumor. The presence of subcapsular or perinephric fluid collections should suggest either malignant rhabdoid tumor or clear cell sarcoma, since similar fluid collections have not been reported in the other tumors.
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DDX Atypical Teratoma
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other cystic ovarian lesions, such as simple cysts, hemorrhagic cysts, and cystadenoma.
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DDX Vaginal obstruction in the neonate
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In the neonate, obstruction may be the result of vaginal atresia or stenosis, a transverse septum, or a cloacal malformation
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differential diagnostic considerations of scrotal swelling in the neonatal period include
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meconium peritonitis or intraperitoneal blood tracking through the patent process vaginalis, hernia, and testicular tumor, especially teratoma
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In prepubertal boys, the most common malignant tumor of the testes
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yolk sac carcinomas (mean patient age, 2 years);
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In prepubertal boys, the most common benign tumor of the testes
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teratoma
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In neonates and infants, causes of nephrocalcinosis include
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furosemide therapy, renal tubular acidosis, ACTH treatment, and Williams' syndrome (idiopathic infantile hypercalcemia)
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In older children and adolescents, the most common causes of nephrocalcinosis are
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renal tubular acidosis and oxaluria. Less frequent causes include hypercalcemic states such as hyperparathyroidism, sarcoidosis, hypervitaminosis D, milk-alkali syndrome, malignancy, Cushing syndrome and hyperthyroidism; parenchymal renal diseases including chronic glomerulonephritis, tuberculosis, mycoses, and medullary sponge kidney; and vascular conditions such as acute cortical or tubular necrosis.
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differential diagnosis of hyperechogenic medullary pyramids includes
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precipitation of Tamm Horsfall proteins, vascular congestion, papillary necrosis, infection of the renal medulla with cytomegalovirus or Candida albicans, medullary fibrosis, and autosomal recessive polycystic disease.
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DDX low T2 signal seminal vessicles
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low T2 signal intensity material is secondary to intracellular methemoglobin from recent biopsy, prior radiation, amyloid, or hormone therapy
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optimal location for an UVC tip
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The optimal location for an UVC tip is in the inferior vena cava (IVC), or at the junction of the IVC and the right atrium and is defined by the catheter tip being located between the eighth and ninth thoracic vertebrae on plain radiography. The majority of UVC tips sited below this level are found to be in vessels in the liver proximal to the ductus venosus.
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DDX infant presenting with a heterogeneous liver mass on ultrasound i
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hepatic hemangioma
mesenchymal hamartoma hepatoblastoma. |
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most common cause for a pancreatic mass in the pediatric population is
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lymphoma
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DDX Double bubble
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Duodenal atresia
Duodenal web Malrotation with midgut volvulus Annular pancreas Malrotation with crossing Ladd bands Normal neonate imaged too early for distal progression of gas |
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Most common cause of macrocephaly in infants?
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Benign enlargement of the subarachnoid spaces in infancy (BESSI) |
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What is the clinical triad of Prune Belly Syndrome? |
Prune belly syndrome classically presents with a clinical triad of: absent/hypoplastic abdominal wall musculature (causing bulging flanks and the wrinkled “prune” appearance of the abdomen), large trabeculated urinary bladder with hydroureteronephrosis and vesicoureteral reflux, and undescended testes (in males). * Include prune belly syndrome in the differential diagnosis of a male neonate with radiographic signs of urinary bladder outlet obstruction.* Rare in Females * aka Eagle-Barrett syndrome |
