GI Development Clinical Cases Flashcards
-3 wk old male
-Projectile vomiting
-Vomits immediately after eating, looks like breast milk
-Stools decreasing in frequency
-Few wet diapers
-Moderately jaundiced
-Fontanelle slightly sunken
-Abdomen flat, soft with active bowel sounds
-Peristaltic wave in LUQ
-Bicarb 33
-Bilirubin 14, Direct bilirubin 0.8
Diagnosis?
Congenital hypertrophic pyloric stenosis (key pts: projectile, non-bilious, 3-6 weeks, first born male child, erythromycin use - exposed to chlamydia at birth)
What is the etiology of Congenital hypertrophic pyloric stenosis?
Hypertrophy of the muscles at the distal end of the stomach.
What is the treatment for Congenital hypertrophic pyloric stenosis?
Normalize electrolytes then call a surgeon to perform the Ramstedt procedure (incising pyloric muscle)
Why is a baby with pyloric stenosis jaundiced?
Starvation increases the enter-hepatic recirculation of bilirubin. (deconjugates and gets reabsorbed)
-17 day old male
-Bilious vomiting and lethargy
-Breast feeding well and growing
-Difficulty passing stools
-Decreased interesting eating
-Vomiting 12 hrs ago, hasn’t eaten since
-Lethargic male
-high temp
-Abdomen distended and no bowel sounds
-Mass felt in lower abdomen
-Abdomen tender to palpation
-Barium enema performed
-Distended colon seen on imaging
What is the diagnosis?
Hirschsprung disease (think about this with chronic constipation in an older child)
What is the embryology of Hirschsprung disease
Absence of autonomic ganglion cells in the distal myenteric plexus. (always present at end of GI tract - important question is where it starts because this demarcates where the ganglion cells are missing - myenteric plexus allows bowel to relax)
How do you initially manage Hirschsprung disease?
Stop feeds, decompress the stomach, start broad spectrum antibiotics (need antibiotics to ensure baby does not develop toxic megacolon - gram negatives like Klebsiella, E. coli)
What is the definitive therapy for Hirschsprung disease?
Surgical exploration and repair (do a “pull through procedure” to bring healthy bowel down to the rectal area)
-8 month old male
-Lethargy, vomiting, irritability
-2 days of intermittent vomiting and pain
-Pulls legs up to chest and cries
-Spasms last between 5 and 10 minutes
-Most recent attack was noted to include bile in vomitus
-Lethargic
-Abdomen moderately distended and tender to palpation
-“Current jelly” stool
What is the diagnosis?
Intussusception (telescoping part can resolve and then telescope again - leading to intermittent pain)
How do you treat Intussusception?
Barium enema and Surgical consult (want to put barium or air behind the telescoping part to push it out)
What associated anomaly might be present with Intussusception?
Meckel diverticulum
What is the embryology of Meckel diverticulum?
An ileal outpouching that often contains gastric or pancreatic tissue.
-35 week gestation male
-C section to 23 yr old primigravida woman
-Size greater than dates
-Large quantity of amniotic fluid was expelled
-Infant cried but hypotonic
-Required oxygen stimulation and frequent suctioning of pharynx
-Developed resp. distress
-Flat occiput, extra nuchal skin, almond shaped eyes with Brushfield spots, distended epigastrium, decreased bowel sounds, low bowel tone
-Xray shows air in stomach and proximal duodenum = double bubble sign
What is the diagnosis?
Duodenal atresia
What is the embryology of duodenal atresia?
Failure of re-formation of the duodenal lumen
What global diagnosis fits this patient with duodenal atresia?
Down syndrome
