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117 Cards in this Set
- Front
- Back
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DDx of a cystic mass in the pediatric lung?
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CCAM
sequestration bronchogenic cyst |
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What babies does meconium aspiration occur?
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Almost always term infants - look at humeral epiphyses
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Most common posterior mediastinal mass in children?
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Neuroblastoma - other considerations: ganglioneuroblastoma, ganglioneuroma, NF
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Most common place for neurobalstoma mets?
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bone and liver
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Why is round PNA more common in children?
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immature canals of Lambert and pores of Kuhn
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Miliary pattern in child
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Tb
hypersensitivity pneumonitis cocci/histo |
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What is the risk of malignant transformation in a CCAM?
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very small
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three types of CCAM?
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1 - large cysts > 2cm
2 - small cysts but macroscopic 3 - appears solid - microscopic cysts |
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Common complication of a CCAM?
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infection
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Which side does congenital absence of the PA typically occur on?
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opposite of the arch
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Absence of L PA associated with?
Absence of R PA associated with? |
R - TOF
L - R arch but no heart disease |
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How do you differentiate congenital absence of PA from Swyer James?
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no air trapping
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DDx of a hypoplastic lung in a child?
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Swyer-James
Scimitar absence of PA |
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How do you distinguish Swyer James from congenital lobar emphysema?
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The emphysema, the larger lung will be the hyperlucent one.
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Three common causes of viral bronchiolitis?
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rhinovirus, RSV, parainfluenza
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What causes the hyperlucent appearance in Swyer James?
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Decreased vascularity
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Describe fluoroscopic appearance of Swyer-James...
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during expiration, mediastinum moves toward normal side, diaphragm doesn't move well on abnormal side because of air trapping
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What is the most common symptomatic vascular ring?
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Double aortic arch
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What should you do with a newborn on the ventilator that has PIE?
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Switch to high frequency ventilation
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DDx of PIE?
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treated surfactant deficiency
developing BPD |
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What is pathognomonic for a pulmoary sling?
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anterior indentation of the esophagus
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Figure of three
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coarctation
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In addition to aortic findings, what may contribute to HTN in a patient of coarctation?
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decreased renal perfusion through renin-angiotensin system
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Assoications with coarc?
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bicuspid valve, VSD, PDA, mitral stenois, aortic stenosis
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Two associations with Ebstein?
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ASD, reentrant dysrhythmias
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What maternal factor can cause Ebstein?
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Lithium
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What appears similar to Ebstein on CXR? (large heart, diminished vascularity)
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pulmonary atresia with intact septum
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Most common bacteria in Lemierre syndrome?
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Fusobacterium necrophorum
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What space is infected in Lemierre?
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Infection of lateral pharyngeal space that extends into carotid sheath
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Location/imaging differences between:
bronchgenic cyst esophageal dupliction cyst neurenteric cyst |
bronchogenic - usually middle mediastinal
esophageal - usually posterior mediastinal neurenteric - posterior mediastinal, often with a vertebral anomaly |
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Which lobes are more often affected in pulmonary pappilomatosis?
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posterior lower lobes
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DDx of fatty replacement of the pancreas?
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CF, Schwachman-Diamond, Cushing's, obesity, steroid use
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Why do GI duplication cysts enlarge over time?
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mucus secretion
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Describe the US appearance of 'gut signature.'
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hyperechoic mucosa, hypoechoic muscularis propria, echogenic serosa
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What is a characteristic finding of hemagioendotheliomatosis?
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Change in caliber of aorta below celiac axis
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Demographic of a patient with hemangioendothelialmatosis?
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<6 mos, F>M (2:1)
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Typical findings of hemgioendotheliomatosis?
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Hypervascular liver masses with consumptive coagulopathy, may have skin lesions, kasabach-merritt, high-output heart failure
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DDx of failure to pass meconium
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meconium ileus
meconium plug Hirschsrung |
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What part of the GI tract is always aganglionic in Hirschsprung?
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anus
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Meconium plug?
Meconium ileus? Hirschsprung? |
plug - rectum:sigmoid ratio >1, small left colon, multiple meconium filling defects
ileus - rectum:sigmoid ratio >1, microcolon Hirsch - retum:sigmoid ratio <1 |
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Why is anaglionic colon resected in hirscrpung?
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to avoid toxic megacolon
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near _______% of meconium ileus patients have CF
About _____% CF patients have meconium ileus |
100%
15% |
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treatment for meconium ileus
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hyperosmotic contrast enema
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DDx of hypertrophic pyloric stenosis?
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annular pancreas
duodenal web |
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signs of hypertrophic pyloric stenosis
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mushroom sign, caterpillar sign, string sign
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age for hypertrophic pyloric stenosis>
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2-12 weeks
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GU rhabdomyosarcoma is more common in males...why?
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typically arises from prostate
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Findings in ileal atresia?
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microcolon, no reflux in TI, multiple air-fluid levels in proximal SB
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What is pathognomonic for ileal atresia?
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meconium peritonitis with small bowel obstruction
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Metabolic effects of hypertrophic pyloric stenosis?
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hypochloremic metabolic alkalosis
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Differentiaton of MCKD from MLCN?
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MLCN will have surrounding frunctional renal tissue
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DDx of nephroblastomatosis?
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Wilms tumor - more heterogenous
lymphoma - usually infiltrative rather than mass-like |
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assoications with nephroblastomatosis?
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beckwith weidman, sporadic aniridia, 3x18
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What is the rcommendation for nephroblastomatosis? What usually happens? What can happen?
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U/S screening until age 7. Most spontaneously regress, 1/3 will get wilm's tumor
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What is found as a precurosr in nearly 100% of bilateral wilm's tumor
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nephroblastomastosis
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Age group for intussusception?
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80% < 2 years, rare < 3 mos and > 6 years
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Cure rate of wilm's
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90%
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median age for wilm's
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3 years
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DDx for wilm's
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NB - displaces rather than replaces kidney, usually calcification
mesoblastic nephroma - neonates |
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Hepatoblastoma?
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large at presentation, typically about one year old, hypervascular, hypdense on CT, elevated afp
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Is fibromatosis colli tender?
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No. If it is consider infection.
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three associations with a nasal hemangioma?
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PHACES
Dandy-Walker Kasabach-Meritt |
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What is PHACES?
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Posterior fossa malformation, hemangiomas, arterial abnormalities, coarctation, eye abnormalities
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What neonatal disease can mimic surfactant deficiency?
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strep B PNA
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Stage I Hodkin lymphoma?
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one lymph node region
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Stage II Hodkin lymphoma?
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two nodes, same side
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Stage III Hodgkin lymphoma?
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both sides of diaphragm
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Stage IV Hodgkin lymphoma?
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spread outside nodes
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What usually causes Menetrier disease in children?
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CMV or campylobacter
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Tumor associated with horseshoe kidney in children?
In adults? |
Wilm's
adenocarcinoma and TCC |
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Associations with horseshoe kidney?
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imperforate anus, 3x18
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Next step in neurogenic bladder?
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urodynamic study
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Most common type of neurogenic bladder?
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Spastic bladder - overactive detrusor and dyssynergic sphincter
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Less common type of neurogenic bladder?
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flaccid bladder - normal detrusor, inactive sphincter
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When do you examine a baby for DDH?
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2-4 weeks
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What should the alpha and beta angles be in a hip exam?
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alpha - 60 degress
beta - 70 degrees |
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What is Jaffe Campanacci?
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Multiple NOFs, cafe au lait, retardation, hypogonadism, ocular abnormality
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What helps distinguish osteoid osteoma from osteoblastoma?
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size 1.5-2.0 cm
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What is the classification system of elbow fxs?
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Gartland
I - minimal displacement II - intact posterior cortex III - displaced with no cortical contact |
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Derscribe the course of a UVC
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UV -> LPV -> DV -> HVs -> IVC -> RA
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What causes labyrinthitis ossificans?
What structures are involved? |
Meningitis from S. Pneum or H. influenza
Structures of the membranous labyrinth |
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What is Goldenhar-Gorlin syndrome?
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Facioauriclovertebral sequence
a unilateral insult of first and second branchial arch derivatives |
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Hemangioma verus venous malformation versus lymphatic malformation
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hemangioma - arterial flow
venous - no arterial flow lymphatic - no enhancement |
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three types of pleuropulmonary blastoma?
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I - cystic, infants
II - cystic/solid, < 5yo III - solid, better prognosis |
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What is necessary for survival in Tapvr?
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ASD
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What is Denys-Drash syndrome?
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medullary glomerulosclerosis, wilm's tumor, ambiguous genitalia
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Small left colon syndrome is assoicated with maternal____?
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diabetes
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Frasier syndrome?
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chronic renal failure
xy gonadal dysgenesis increased risk of gonadoblastoma |
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WAGR
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Wilm's, aniridia, GU anomlaies, retardation
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Which way does the midgut rotate during deveopment?
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270 degrees counter-clockwise
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What are bladder ears associated with?
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inguinal hernia
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Treatment for urethral fx?
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Placing a catheter and waiting for healing
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What causes nephroblastomatosis?
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persistent metanephric blastema - usually perilobar rather than intralobar
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What is the worst type of OI to have?
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II
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Caffey's disease?
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infantile cortical hyperostosis
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Monoarticular JRA usually affects which joints?
Polyarticular JRA usually affects which joints? |
large
small |
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Osteopetrosis aka
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Albers-Schonberg
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DDx erlenmeyer flask deformity?
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Sickle cell, gaucher, neiman-pick, fibrous dysplasia
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Long-term complication of legg-calve-perthes
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coxa vara deformity and painful OA
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What does LCH look like on a bone scan?
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increased uptake
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Sites of osseous involvement in LCH in decreasing order?
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skull, femur, mandible, pelvis, ribs, spine
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Invisible fetus?
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OI type II
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What syndrome is rhombencephalosynapsis assoicated with?
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Gomez-Lopez-Hernandez
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What is Joubert syndrome?
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aplasia of the vermis, molar tooth brainstem, bat-wing 4th ventricle
absence of pyramidal decussation |
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Hypertrophied rosenthal fibres?
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alexander disease
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Rosai Dorfman?
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sinus histiocytosis and massive painful lymphadenopathy
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Kikuchi disease?
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histiocytic necrotizing lymphadenopathy
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CHARGE?
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Coloboma, heart defeacts, atresia (choanal), retardation, genital anomlaies, ear anomalies
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What is the Haller index neede to call pectus excavatum?
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> 3.25 (width to narrowest AP diameter)
Nuss procedure |
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Waldman disease?
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primary interstital lyphangiectasia - dilated lymph vessels along sb wall. Treat with low-fat diet
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What is Loeys-Dietz syndrome?
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similar to margan but very aggressive and dissection occurs at very early age
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Chondrodysplasia punctata
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rhizomelic limb shortening, stippled epiphyses, die within one year
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What is double-layer patella associated with?
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multiple epiphyseal dysplasia
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DDx of wormian bones?
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PORKCHOPS - pynknodystosis, OI, Rickets, Kinky hair, Cleidocranial dysplasia, Hypothyroidism, oropalatodigital syndrome, pachydermoperistitis, syndrome of down
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Rulke of three's in intussusception
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3 tries, 3 feet high, 3 minutes each
dilute contrast or air contrast - do not exceed 110-129 mm Hg |
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Solid renal mass in a newborn, think...?
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mesonephric blastoma
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how do you tell the difference between choanal atresia and pyriform aperture stenosis?
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anterior narrowing, also look for central megaincisor
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