What is the management approach for intestinal malformation and malrotation?

Management of Intestinal Malrotation and Malformation

The management of intestinal malrotation requires prompt diagnosis with upper GI series as the gold standard test, followed by urgent surgical intervention with a Ladd procedure to prevent life-threatening midgut volvulus. 1

Diagnosis

Clinical Presentation

• Infants: Bilious vomiting is the most common and concerning symptom in the first month of life 2, 3
• Children 1-5 years: Vomiting remains the predominant symptom 4
• Children 6-15 years and adults: Abdominal pain becomes the predominant symptom 4, 5
• Other symptoms may include:
  • Abdominal distention
  • Failure to thrive
  • Recurrent episodes of partial bowel obstruction
  • Chronic abdominal pain

Diagnostic Imaging

1. Upper GI Series (UGI):

   • Gold standard for diagnosis with 96% sensitivity 1
   • Evaluates position of duodenal-jejunal junction (ligament of Treitz)
   • Can identify midgut volvulus requiring urgent surgery
   • Meticulous technique is essential as redundant duodenum, bowel distension, and jejunal position can lead to false interpretations (10-15% false-positive rate) 1

2. Abdominal Ultrasound:

   • Limited accuracy as primary diagnostic tool
   • Can evaluate position of superior mesenteric vein (SMV) in relation to superior mesenteric artery (SMA)
   • "Whirlpool sign" is specific for midgut volvulus 1
   • Normal SMV/SMA relationship does not exclude malrotation (2-3% false-negative rate) 1

3. Abdominal Radiographs:

   • Limited diagnostic value but may guide further imaging
   • Normal radiographs do not exclude malrotation 1

4. Contrast Enema:

   • Not recommended as initial imaging study for suspected malrotation
   • May be used if UGI is equivocal 1

Surgical Management

Acute Presentation with Suspected Volvulus

• Emergency surgical intervention is mandatory due to risk of intestinal ischemia and necrosis 2
• Midgut volvulus is a life-threatening emergency requiring immediate attention 3

Ladd Procedure

The standard surgical approach includes:

1. Evisceration and inspection of the mesenteric root
2. Counterclockwise derotation of midgut volvulus (if present)
3. Lysis of Ladd's bands with straightening of the duodenum along the right abdominal gutter
4. Appendectomy (due to abnormal position of appendix)
5. Placement of the cecum into the left lower quadrant 2

Surgical Approach

• Open laparotomy: Traditional approach, especially in emergency settings 5
• Laparoscopic approach: Feasible alternative in stable patients 5

Postoperative Care and Complications

Nutritional Support

• If the patient is malnourished or at risk:
  • Try oral supplements/dietary adjustments first 1
  • If oral feeding unsuccessful, consider gastric feeding 1
  • If gastric feeding fails, consider jejunal feeding 1
  • Parenteral nutrition may be needed if enteral feeding fails 1

Complications

• Overall complication rate: approximately 20% 4
• Higher risk in:
  • Extremely preterm patients
  • Patients with severely affected intestinal circulation 4
• Specific complications include:
  • Wound infection
  • Delayed gastric emptying
  • Adhesive bowel obstruction (11% of patients) 4, 5
  • Recurrent volvulus (rare)
  • Intestinal failure due to midgut loss (rare) 4

Long-term Follow-up

• Monitor for recurrent symptoms
• Approximately 11% of patients may develop adhesive bowel obstruction requiring surgical treatment 4
• Mortality is rare but can occur, particularly in extremely preterm infants 4

Special Considerations

Adult Presentation

• Malrotation in adults may present with:
  • Chronic intermittent abdominal pain
  • Acute bowel obstruction
  • Incidental finding during surgery for other conditions 5, 6
• Diagnostic approach and surgical management similar to pediatric cases
• Good outcomes generally reported after surgical correction 5, 6

Management of Ileus

If postoperative ileus develops:

• Discontinue inciting medications, particularly opioids 7
• Implement opioid-sparing analgesia strategies 7
• Avoid routine nasogastric tube placement and excessive fluid administration 7
• Consider early mobilization to stimulate bowel function 7
• Avoid bowel regimens as they may worsen the condition 7

Early recognition of malrotation and prompt surgical intervention are critical to prevent the potentially catastrophic consequences of midgut volvulus, leading to favorable outcomes in most cases.