Congenital GI Diseases Flashcards
How does malrotation develop?
improper rotation drawing the intestines back in to the abdominal cavity
What is the ligament of Treitz?
fixed at duodenal jejunum and colon, keeps intestines in LUQ
should be locates LEFT of spine
What occurs with malrotation?
the narrow mesenteric base permits abnormal mobility of the small bowel, allowing the mesentery to twise–>midgut volvulus (entire midgut can die)
ladd’s bands (cross the duodenum), can lead to obstruction
What are the sx of malrotation?
tachycardic, tachypneic, crying/grunting
lungs clear
abdoment tender
What is done to correct malrotation?
surgery-Ladd’s procedure
What is the key imaging finding of duodenal atresia?
double bubble sign
What causes duodenal atresia?
failure of recanalization
Besides the double bubble sign, what are signs of duodenal atresia?
polyhydramnios from high intestinal obstruction
What are other features of duodenal atresia?
asociated with trisomy 21 (&cardiac, genitourinary & anorectal abnormalities)
How is duodenal atresia treated?
NG tube
operative repair
What are signs of jejunoileal atresia?
doesn’t pass meconium
bilious emesis
What causes jejunoileal atresia?
in utero vascular disruption leading to ischemic necrosis of the fetal intestine
may be linked to inherited thrombophilia & spontaneous thrombosis may play a role in some cases
maternal use of vasoconstrictive medications may contribute to interruption of mesenteric blood flow (pseudophedrine, cocaine, nicotine)
rare familial inheritance
How is jejunoileal atresia treated?
surgery
What is a potential long term issue of jejunoileal atresia?
short gut syndromes, may need SI transplant
What is hirschspurng’s disease?
congenital defect in intestinal innervation (both meissners and auerbachs plexi)
more common in males
associated w/trisomy 21
possible assosciated w/ RET mutation
What does is the lack of peristalsis in hirschspurng’s disease called?
fuctional obstruction
Where is hirschspurngs most commonly located?
rectosigmoid
What are symptoms of hirschspurng’s?
failure to pass meconium
functional obstruction/bilious emesis
enterocolitis
What is the tx ofr Hirschspurng’s?
surgical resectin of aganglionated segment, anastomosis of normal colon to recturm
How is necrotizing enterocolitis?
abdominal distention
feeding intolerance/emesis
rectal bleeding
abdominal wall erythema
temperature instability/apnea/bradycardia
peumatosis (gas within the bowell wall) on XRay
What is protective against necrotizing enterocolitis?
breast milk
probiotics
What causes pneumatosis?
gas produced by bacteria which is seen within serosal and muscularis layers of bowel
What are some potential causes of necrotizing enterocolitis?
prematuritiy
formula feeding
indomethacin (other medications)
How is necrotizing enterocolitis treated?
abx-broad spectrum -ampicillin (gram +) -gentamycin of ceftriaxone (gram -) -metronidazole (anaerobe) bowel rest (NPO, NG tube) TPN Surgery
What are indications for surgery for necrotizing enterocolitis?
pneumoperitoneum free air
clinical deterioration
What is a common imaging finding of anorectal malformation?
no air in rectom
What are anorectal malformations associated with?
trisomy 21
VACTERL complex
How do anorectal malformations occur?
abnormality of caudal descent of urorectum
- failure of descent of hindgut to anus
- fusion of urorectal ridges to walls of cloaca
Which types of anorectal malformations are more common in males? females?
males-“high” rectovesicular or rectourethral fistula
females-“low” malformations to perineum or vestibule
How is anorectal malformation treated?
colostomy with later pullthrough operation
