Jejuno-ileal atresia

Question 1

What is the cause of jejuno-ileal atresia?

Answer 1

A mesenteric vascular insult during intrauterine life disrupts the focal development of a section of the intestinal wall, with or without mesenteric involvement.

Question 2

Is jejuno-ileal atresia typically associated with other congenital anomalies?

Answer 2

Jejuno-ileal atresia is usually an isolated defect with minimal associated congenital anomalies, although multiple atresias (type IV) can occur.

Question 3

What are the possible causes of the vascular insult in jejuno-ileal atresia?

Answer 3

The vascular insult may result from micro-emboli, antenatal volvulus (twisting of the bowel), or bowel infarction due to conditions like gastroschisis.

Question 4

What could be the consequence of the vascular insult in jejuno-ileal atresia?

Answer 4

The vascular compromise leads to proximal and distal blind-ending bowel lumens on either side of the reabsorbed necrotic portion of the bowel.

Question 5

What is the potential outcome of bowel infarction in jejuno-ileal atresia?

Answer 5

Bowel infarction can result in a significant loss of bowel length, leading to short bowel syndrome.

Question 6

What does the classification of jejuno-ileal atresia indicate about bowel length?

Answer 6

In types I and II (stenosis), there is no loss of bowel length. In types III and IV, there is a loss of bowel length, which can be substantial and lead to short bowel syndrome.

Question 7

What is short bowel syndrome in the context of jejuno-ileal atresia?

Answer 7

Short bowel syndrome occurs when the remaining length of small bowel is insufficient to sustain normal growth and development, requiring significant parenteral nutritional support.

Question 8

What is unique about type IIIb of jejuno-ileal atresia?

Answer 8

In type IIIb, the distal bowel is spiralled around an abnormal marginal mesenteric blood supply in an “apple peel” configuration, and it is likely to volvulate and become necrotic.

Question 9

What is the classification system for jejuno-ileal atresia based on?

Answer 9

The classification of jejuno-ileal atresia is based on the anatomical type of the atresia and the involvement of bowel length.

Question 10

What characterizes type I jejuno-ileal atresia?

Answer 10

Type I is characterized by stenosis, where there is no loss of bowel length, and the bowel remains intact but narrowed.

Question 11

What defines type II jejuno-ileal atresia?

Answer 11

Type II is also a form of stenosis with no loss of bowel length, but the atresia involves a more pronounced narrowing of the bowel lumen compared to type I.

Question 12

What is the key feature of type III jejuno-ileal atresia?

Answer 12

Type III is characterized by a loss of bowel length, with a necrotic segment of bowel, often leading to short bowel syndrome.

Question 13

What distinguishes type IIIb jejuno-ileal atresia?

Answer 13

In type IIIb, the distal bowel is spiralled around an abnormal marginal mesenteric blood supply in an “apple peel” configuration and is prone to volvulus and necrosis.

Question 14

What is the clinical consequence of types III and IV jejuno-ileal atresia?

Answer 14

Types III and IV are associated with significant loss of bowel length, which may result in short bowel syndrome, where the remaining bowel is insufficient to support normal growth and development.

Question 15

How does short bowel syndrome affect patients with jejuno-ileal atresia?

Answer 15

Short bowel syndrome leads to the need for significant parenteral nutritional support to maintain growth and development due to the insufficient length of the remaining small bowel.

Question 16

What is a common antenatal finding associated with jejuno-ileal atresia?

Answer 16

Polyhydramnios is a common antenatal finding in cases of jejuno-ileal atresia.

Question 17

How can antenatal ultrasound or MRI help in diagnosing jejuno-ileal atresia?

Answer 17

Antenatal ultrasound or MRI can reveal dilated bowel loops, which is indicative of jejuno-ileal atresia.

Question 18

What is the benefit of antenatal detection of jejuno-ileal atresia?

Answer 18

Antenatal detection allows for planned in-utero transfer and delivery at a tertiary unit with a neonatal ICU and pediatric surgical expertise, ensuring timely care after birth.

Question 19

What is a common postnatal symptom of jejuno-ileal atresia?

Answer 19

Vomiting is a common symptom, initially of milk feeds but soon becoming bile-stained. If the diagnosis is delayed, it may even become faeculent.

Question 20

How does the location of the atresia affect abdominal distension in jejuno-ileal atresia?

Answer 20

• Upper gastrointestinal tract obstruction (proximal jejunal atresia) presents with epigastric distension.
• Lower gastrointestinal tract obstruction (ileal atresia) presents with more significant abdominal distension.

Question 21

How can progressive abdominal distension affect neonates with jejuno-ileal atresia?

Answer 21

Progressive abdominal distension can lead to respiratory distress due to diaphragmatic splinting, as neonates are obligate diaphragmatic breathers.

Question 22

What is a common sign of jejuno-ileal atresia related to meconium passage?

Answer 22

Babies with atresia usually fail to pass meconium, or pass only a small amount, which may be white meconium.

Question 23

What biochemical abnormalities may occur in neonates with jejuno-ileal atresia due to vomiting?

Answer 23

• Vomiting of upper gastrointestinal contents leads to hypochloraemic hyponatraemic metabolic alkalosis.
• Vomiting of lower gastrointestinal contents leads to progressive metabolic acidosis.

Question 24

Is jaundice common in cases of jejuno-ileal atresia?

Answer 24

Yes, jaundice is common, especially in duodenal atresia, occurring in about 40% of cases due to disrupted enterohepatic circulation and sepsis.

Question 25

What is the role of abdominal x-ray in diagnosing jejuno-ileal atresia?

Answer 25

Abdominal x-ray should be performed in all cases after 18-24 hours post-birth, as it can take 12-18 hours for swallowed air to reach the rectum, and an x-ray taken earlier may falsely show no gas in the rectum.

Question 26

Is the abdominal x-ray the only diagnostic investigation needed for jejuno-ileal atresia?

Answer 26

Yes, abdominal x-ray is usually the only diagnostic investigation required for jejuno-ileal atresia.

Question 27

When is an upper gastrointestinal contrast used in diagnosing jejuno-ileal atresia?

Answer 27

Upper gastrointestinal contrast is sometimes performed when the diagnosis is unclear, though it is not routinely used.

Question 28

What alternative method is recommended for diagnosis instead of contrast in jejuno-ileal atresia?

Answer 28

The installation of air via the nasogastric tube (NGT) is recommended over contrast due to the risks of vomiting and aspiration associated with contrast.

Question 29

What finding on upper gastrointestinal contrast is diagnostic for jejuno-ileal atresia?

Answer 29

Contrast hold-up in the proximal dilated loops with no passage through the rest of the bowel is diagnostic for jejuno-ileal atresia.

Question 30

What is the purpose of a contrast enema in diagnosing jejuno-ileal atresia?

Answer 30

A contrast enema is performed to differentiate mechanical causes of luminal obstruction, like atresia, from functional bowel obstructions such as Hirschsprung’s disease or meconium ileus, in cases where the anus is normal.

Question 31

How does the contrast enema help differentiate jejuno-ileal atresia from Hirschsprung’s disease?

Answer 31

If a normal rectosigmoid ratio (where the rectum is wider than the sigmoid) is present, Hirschsprung’s disease is unlikely, suggesting the need for surgery to check for atresia.

Question 32

When might a contrast enema be performed in cases of proximal atresia?

Answer 32

A contrast enema may be performed in proximal atresias to rule out other distal atresias, especially if a minimal access approach to surgery (e.g., peri-umbilical incision) is planned.

Question 33

What finding on contrast enema is typically seen in intestinal atresias?

Answer 33

A “micro-colon of disuse” is typically seen on contrast enema in cases of intestinal atresia.

Question 34

What are common comorbidities associated with jejuno-ileal atresia?

Answer 34

Common comorbidities include prematurity, cystic fibrosis, and gastroschisis.

Question 35

How can cystic fibrosis contribute to jejuno-ileal atresia?

Answer 35

Cystic fibrosis causes heavy inspissated meconium, which can lead to antenatal volvulus, especially in type III jejunal/ileal atresia. It can be excluded with a sweat test or genetic investigation.

Question 36

What percentage of patients with gastroschisis have intestinal atresia?

Answer 36

Approximately 15% of patients with gastroschisis have one or more intestinal atresias.

Question 37

How can gastro-oesophageal reflux be differentiated from jejuno-ileal atresia?

Answer 37

Gastro-oesophageal reflux typically causes persistent milk vomiting but does not lead to biochemical abnormalities and never results in faeculent vomitus.

Question 38

How can lower gastrointestinal tract obstructions be differentiated from distal atresia?

Answer 38

Lower GIT obstructions like Hirschsprung’s disease, neonatal small left colon syndrome, meconium plug syndrome, and meconium ileus can be differentiated from distal atresia via contrast enema. An anorectal malformation can be identified on perineal inspection.

Question 39

What is the initial preparation for surgery in cases of jejuno-ileal atresia?

Answer 39

Initial preparation includes placing a nasogastric tube and keeping the patient nil by mouth.

Question 40

What intravenous fluids are needed in preparation for surgery in jejuno-ileal atresia?

Answer 40

Intravenous fluids for resuscitation, ongoing maintenance fluid, and replacement of ongoing fluid losses are required.

Question 41

When are broad-spectrum intravenous antibiotics needed in jejuno-ileal atresia?

Answer 41

Broad-spectrum intravenous antibiotics are administered if the neonate is septic or very distended.

Question 42

How should warmth be maintained in neonates with jejuno-ileal atresia before surgery?

Answer 42

Maintaining warmth is essential, often through radiant warmers or other methods to prevent hypothermia.

Question 43

What is crucial to monitor in preparation for surgery in cases of jejuno-ileal atresia?

Answer 43

Serum glucose levels should be maintained and monitored to avoid hypoglycemia or hyperglycemia.

Question 44

What respiratory support may be necessary before surgery?

Answer 44

Oxygen (via nasal cannula or intubation) may be necessary if the neonate is in respiratory distress.

Question 45

What cardiovascular, respiratory, and metabolic parameters should be monitored preoperatively?

Answer 45

Pulse, respiratory rate, blood pressure, serum glucose, urine output, and blood gases (serum base excess, lactate, and pH) should be monitored, especially in delayed presentations, to manage metabolic acidosis or alkalosis.

Question 46

How should metabolic acidosis or alkalosis be corrected in neonates with delayed presentation of jejuno-ileal atresia?

Answer 46

Metabolic acidosis or alkalosis should be corrected through careful intravenous fluid and electrolyte replacement.

Question 47

When should surgery be performed for jejuno-ileal atresia?

Answer 47

Surgery should be performed on the 2nd or 3rd day postnatally once the baby is stable, avoiding undue delays to prevent sepsis and bowel ischemia due to increasing distension.

Question 48

What should be done if there is a delay waiting for surgery in jejuno-ileal atresia?

Answer 48

Intravenous (parenteral) nutrition should be started via a central venous line or peripherally inserted central catheter (PICC) if there is a delay.

Question 49

What surgical approach is used for jejuno-ileal atresia?

Answer 49

The surgical approach is via a transverse laparotomy or peri-umbilical incision.

Question 50

What happens if there is adequate distal bowel length during surgery for jejuno-ileal atresia?

Answer 50

If there is adequate distal bowel length, the dysfunctional proximal dilated bowel is resected. The length of bowel is determined by age-based normograms (150cm at 28 weeks gestation to 250cm at 40 weeks gestation).

Question 51

What is done if short bowel syndrome is a concern during surgery for jejuno-ileal atresia?

Answer 51

If short bowel syndrome is a concern, the dilated proximal bowel is tapered to preserve bowel length, and the distal bowel is cut back about 2cm.

Question 52

What are the final steps in the surgical repair of jejuno-ileal atresia?

Answer 52

The distal bowel is spatulated, an end-to-end anastomosis is performed, and the mesenteric defect is closed.

Question 53

What is the post-operative care for pain management after surgery for jejuno-ileal atresia?

Answer 53

Analgesia and intensive care monitoring are required for effective pain management and close observation.

Question 54

How should nasogastric drainage be managed post-operatively for jejuno-ileal atresia?

Answer 54

Nasogastric drainage should be used with replacement of gastrointestinal fluid losses greater than 10ml/kg/day, until losses decrease and become pale green or non-bilious.

Question 55

How long may parenteral nutritional support be needed post-surgery for jejuno-ileal atresia?

Answer 55

Parenteral nutritional support may be required for several days to weeks after surgery, until at least 75% of nutritional requirements are met orally.

Question 56

What factors determine the advancement of oral feeds post-operatively in jejuno-ileal atresia?

Answer 56

The advancement of feeds depends on the degree of remaining proximal distended bowel (with abnormal motility) and whether short bowel syndrome is present.

Question 57

Why is early initiation of small volumes of oral breast milk encouraged after surgery?

Answer 57

Early initiation of small volumes of oral breast milk helps stimulate the development of a normal swallowing reflex, gastrointestinal motility, and endothelin growth factor.

Question 58

What is a common post-operative issue related to vomiting, and how can it be managed?

Answer 58

Ongoing intermittent vomiting is common due to poor motility of the proximal dilated bowel. Small, frequent feeds and a 30° head-up position can help prevent vomiting and aspiration.

Question 59

What is the post-operative outcome for most patients with jejuno-ileal atresia?

Answer 59

Most patients (around 90%) do well post-operatively.

Question 60

When does short bowel syndrome occur after surgery for jejuno-ileal atresia?

Answer 60

Short bowel syndrome occurs if there have been multiple atresias and the residual bowel length is too short (around 75cm or less) to maintain growth and development.

Question 61

What is a potential complication after surgery for jejuno-ileal atresia, though it is rare?

Answer 61

Anastomotic strictures or breakdown is a possible, though rare, complication after surgery.