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GI > 26. Congenital defects of the bowel > Flashcards

26. Congenital defects of the bowel Flashcards

1
Q

What is the key presentation that can indicate malrotation (medical emergency)?

A

Bilious emesis (yellow/green)

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2
Q

When in devo does the gut extend out of the abdomen, and how does it rotate?

A

4th-8th weeks

Twists 90 degrees CCW while hernitated, and an additional 180 decrees CCW when back in the abdomen

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3
Q

What fixes the SI at the duodenal jejunum?

A

Ligament of Treitz

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4
Q

What are bands that cross the duodenum and can cause obstruction with midgut volvulus?

A

Ladd’s bands

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5
Q

What will be seen on abdominal x-ray with an NGT in when there is midgut volvulus?

A

Gasless abdomen

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6
Q

Where should the ligament of Treitz be located on abdominal X-ray?

A

To the left of the patient’s spine at the duodenojejunal junction

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7
Q

What is the Ladd’s procedure?

A

Treatment for midgut volvulus

  • untwisting of the bowel, removal of the Ladd’s bands, widening of the mesentery
  • Put the small bowel on the right and colon on the left
  • Appendectomy to prevent future confusion with appendicitis
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8
Q

What is the classic sign for duodenal atresia on a prenatal US?

A

Double bubble sign, polyhydraminos

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9
Q

What is the pathogenesis of duodenal atresia?

A

Failure of recanalization–duodenum passes though solid phase but should have reopening of the lumen between weeks 8 and 10

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10
Q

Embryological insult during the recanalization period (weeks 8-10) can result in: (3)

A
  1. Duodenal stenosis
  2. Duodenal web
  3. Duodenal atresia
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11
Q

What causes the double bubble sign?

A

Dilated stomach and proximal duodenum resulting from obstruction

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12
Q

What are the two things that should indicate duodenal atresia within a few hours of birth?

A

Vomiting that is clear or bilious

Double bubble sign on X-ray

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13
Q

What are the two possibilities for level of obstruction with duodenal atresia and which one is more common?

A

Pre or post-ampullary

80% are post-ampullary, resulting in bilious emesis

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14
Q

What genetic defects/congenital abnormalities is duodenal atresia associated with?

A

Trisomy 21 ***
Cardiac
Genitourinary
Anorectal

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15
Q

What are the steps for tx of duodenal atresia?

A

NG tube
R/o other causes with physical exam and echo
Duodenoduodenostomy: anastomosis of proximal to distal duodenum

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16
Q

What is the pathogenesis of jejunoileal atresia?

A

In utero vascular disruption leads to ischemic necrosis of the fetal intestine
Necrotic intestine is reabsorbed, leaving blind proximal and distal ends, often with a gap in the mesentery

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17
Q

What are risk factors for development of jejunoileal atresia?

A
  • Inherited thrombophilia leading to spontaneous thormbosis
  • Maternal use of vasoconstrictive medications may disrupt the mesenteric blood flow: pseudoephedrine, cocaine, and nicotine
  • Rare familial inheritance
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18
Q

What causes the low birth weight and prematurity in jejunoileal atresia?

A

Polyhydraminos

19
Q

Congenital anomalies associated with jejunoileal atresia?

A

Unlike duodenal atresia, not commonly associated with extraintestinal anomalies

20
Q

Treatment of jejunoileal atresia

A

Surgery: explartory lapartomy, primary anastomosis

**long term issues depending on the intestinal length

21
Q

What can be used for a child that is hard to get an IV in?

A

IO line (intraosseous)

22
Q

What is a congenital disease in intestinal inntervation? More common in what gender?

A

Hirschsprung’s disease

Males

23
Q

Hischsprung’s disease is associated with what congenital disorder?

A

Trisomy 21 (downs syndrome)

24
Q

What is the MC genetic mutation to be associated with Hirschsprung’s disease?

A

RET mutations: RET is a receptor tyrosine kinase that transduces differentiation signals in many developing tissues, including neural crest cells

25
Q

What is the pathogenesis of Hirschsprung’s disease?

A

Normal migration of neural crest cells from the SI to the cecum is disrupted (usually serves to establish the enteric nervous system)
**leads to loss of both the Meissner/submucosal and Auerback/myenteric plexus, or aganglionosis

26
Q

What happens in the aganglionated segment of the bowel in Hirschsrung’s disease?

A

Peristalsis is absent and results in a functional obstruction

27
Q

Where is the most common location to have Hirschprung’s disease?

A

Rectosigmoid

28
Q

What is the clinical presentation of Hirschsrung’s disease in the newborn period?

A
  1. Failure to pass meconium within 24 hr
  2. Functional obstruction and bilious emesis
  3. Enterocolitis
29
Q

What is enterocolitis?

Who is more likely to get enterocolitis?

A

Life threatening complication of bacterial overgrowth, leads to sepsis
More common in patients with Down syndrome

30
Q

What is the tx for Hirschsprung’s disease?

A

Surgical resection of the aganglionated segment

Anastomosis of the normal colon to the rectum

31
Q

Who gets necrotizing enterocolitis

A

Premature infants

  • *occurs in 10% of infants that weigh <1,500 gm at birth
  • *infrequent in term infants
32
Q

What are two things that are protective against necrotizing enterocolitis?

A

Breast milk

Probiotics

33
Q

What characteristics are used for the diagnosis of necrotizing enterocolitis?

A

Abdominal distension
Feeding intolerance and emesis
Rectal bleeding (inflammation of the mucosa)
Abdominal wall erythema
Temperature instability/apnea/bradycardia
Pneumatosis on Xray

34
Q

What is pneumatosis (seen with necrotizing enterocolitis?

A

Gas within the bowel wall

Occurs as a result of gas produced by bacteria within the serosal and muscularis layers of the bowel

35
Q

What are risk factors for necrotizing enterocolitis?

A

Prematurity
Milk feeding
Microbial bowel overgrowth
impaired mucosal defense
Circulatory instability of the intestinal tract
Medications that cause intestinal mucosal injury or enhance microbial overgrowth

36
Q

What are the components of tx of necrotizing enterocolitis?

A

Broad spectrum antibiotics: ampicillin, gentamycin, ceftriaxone, metronidazole
Bowel rest: NPO, TPN
Surgery

37
Q

What are the indications for surgery with necrotizing enterocolitis?

A
  • Pneumoperitoneum–> free air
  • Clinical deterioration
  • Discolored abdomen, fixed loop, and obstruction
38
Q

What will be seen on histology with necrotizing enterocolitis?

A

Epithelial layer is absent, villi are necrotic, hemorrhage and some inflammation

39
Q

What are the two genetic anomalies that anorectal malformations are associated with?

A

Down syndrome

VACTERL complex

40
Q

What is VACTERL complex

A 
Vertebral anomalies
Anorectal anomalies
Cardiac anomalies
TE fistula
Renal anomolies
Limb anomalies (radius)
41
Q

What happens in anorectal malformations?

A

Abnormality of caudal descent of the urorectum: failure of descent of the hindgut to anus or the fusion of urorectal ridges to the walls of the cloaca

42
Q

The antorectal malformations in males are most often ___ with rectovesicular or rectourethral fistula
Females have ___ malformations to the perineum or vestibule

A

High

Low

43
Q

What is important in evaluation of a patient with anorectal malformation?

A

r/o VACTERL

Get ECHO, renal US, do good physical exam

44
Q

What is the tx for anorectal malformations?

A

Colostomy with later “pull through” operation

Occasionally one-stage

GI (28 decks)

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