What is your diagnosis?

what is your diagnosis ?

CASE HISTORY 1:
Six day old baby girl born vaginally after 30 weeks gestation with persistently enlarged huge solitary gastric bubble, no distal air in the KUB (see adjacent figure). She passed some meconium on the first day of life. After 24 hrs of life she developed hypochloremia. The UGIS shows a dilated stomach, a normal antrum with a cap and faint insinuation of a pyloric canal.	KUB
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CASE HISTORY 2:
14 year old black male with progressive dysphagia, regurgitation, weight loss, and nocturnal cough. Barium swallow shows esophageal dilatation, motility alteration and a small caliber cardio-esophageal junction (see adjacent figure).	Barium Swallow
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CASE HISTORY 3:

4 month female child with a right upper quadrant abdominal mass and jaundice.

Ultrasound (US) and nuclear scintigram (HIDA) are included.

HIDA

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CASE HISTORY 4:
Baby girl born prematurely with the adjacent abdominal wall defect, interrupted dilated small bowel with associated micro-ileum and micro-colon.
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CASE HISTORY 5:
Baby boy born at term with the adjacent abdominal wall defect.
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CASE HISTORY 6:
6 year-old-female with two month history of left upper quadrant abdominal pain, early satiety and non-bilious vomiting. Normal chemistry and amylase/lipase. CT Scan shows a cystic mass behind the stomach.
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CASE HISTORY 7:
2 weeks-old female patient born to a diabetic mother develops abdominal distension. Barium enema (see figure) shows a thin left colon with a transitional zone at the splenic flexure. Ganglion cells are present on suction rectal biopsy.
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CASE HISTORY 8:

A 13 year-old female presented with primary amenorrhea, low abdominal pain and a palpable movable pelvic mass. CT shows multiple cystic cavities in the pelvis.

Physical Exam

Pelvic CT Scan

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CASE HISTORY 9:

48-hours-old term female develops abdominal distension, obstipation and bilious vomiting. Rectal exam reveals a WHITE meconium plug. Simple abdominal films demonstrate multiple dilated small bowel loops. At surgery there is a transitional zone 23 cm proximal to ileocecal valve with the proximal dilated ileum filled with tenacious neconium and distal meconium pellets.

KUB

Operative findings

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CASE HISTORY 10:
3 months-old-male infant with persistent bilious vomiting. Simple abdominal film is included.	KUB
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CASE HISTORY 11:
3 months prematurely born (33 weeks gestational age) male child with history of hyaline membrane disease, pneumothorax, mechanical ventilation. convulsions, prolonged TPN and medically-managed NEC. While at NICU he develops insidious cholestatic jaundice (total bile fluctuating between 5 and 9 mg% with 70-80% conjugated portion). Elevated GGT and LFT's, normal alpha-1-antitrypsin levels and normal TORCH. HIDA scan under phenobarbital induction shows adequate liver uptake with no hepato-biliary excretion in 24 hrs films. Mini-laparotomy reveals a cholestatic liver, cholangiogram shows patent gallbladder, cystic duct and common bile duct with diminutive intrahepatic bile ducts (see figure).
	*What is your diagnosis ?*

CASE HISTORY 12:
Newborn female born with the congenital anomaly depicted in the adjacent picture.
	*What is your diagnosis?*

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Created February 1, 1998

Last updated: November 2024