What is the treatment for congenital malrotation?

Treatment for Congenital Intestinal Malrotation

Surgical intervention with the Ladd procedure is the standard of care for symptomatic intestinal malrotation, as it prevents life-threatening complications including midgut volvulus and intestinal necrosis. 1

Diagnosis

Before treatment can begin, proper diagnosis is essential:

• Primary diagnostic tool: Upper GI Series (UGI) is the gold standard with 96% sensitivity 1, 2
• Secondary diagnostic option: Ultrasonography to evaluate:
  • Position of superior mesenteric vein (SMV) in relation to superior mesenteric artery (SMA)
  • "Whirlpool sign" which is specific for volvulus 1
• Clinical presentation: Bilious vomiting is the most common and concerning symptom in neonates and infants 1
• Limitations: Contrast enema has a false negative rate of approximately 20% and false positive rate of up to 15% 1

Treatment Algorithm

1. Symptomatic Patients

• Immediate surgical intervention is required for:
  • Patients with bilious vomiting
  • Evidence of midgut volvulus
  • Signs of intestinal obstruction or ischemia 1

2. Surgical Approach: The Ladd Procedure

The Ladd procedure is the definitive treatment and includes:

• Evisceration and inspection of the mesenteric root
• Counterclockwise rotation of any midgut volvulus
• Lysis of Ladd's bands (abnormal peritoneal attachments)
• Placement of small bowel on the right and colon on the left side of the abdomen
• Appendectomy (to prevent future diagnostic confusion) 1

3. Asymptomatic Patients

• While guidelines focus on symptomatic cases, incidental findings of malrotation in asymptomatic patients often warrant prophylactic surgical correction to prevent future volvulus 1

Special Considerations

Complications and Outcomes

• Postoperative complications occur in approximately 20% of patients 1
• Higher risk groups include:
  • Extremely preterm infants
  • Patients with severely affected intestinal circulation due to volvulus 1
• Long-term complications include:
  • Adhesive bowel obstruction (11%)
  • Recurrent midgut volvulus (rare)
  • Intestinal failure due to midgut loss 1

Adult Presentation

• Adults with malrotation may present with chronic, non-specific gastrointestinal symptoms 3
• Surgical correction can alleviate these symptoms even in adults 4, 5
• Diagnosis is often incidental during laparotomy or diagnostic imaging for other conditions 4

Pitfalls and Caveats

1. Diagnostic challenges:

   • Jejunal position can lead to inaccurate UGI series interpretation 2
   • Ultrasonography may be limited by bowel gas obscuration in up to 17% of cases 1

2. Surgical approach:

   • While laparoscopic Ladd's procedure is accepted in children, it has not been well-studied in adults 5
   • Initial laparoscopic intervention may be complicated by enterotomy, requiring conversion to open surgery 4

3. Delayed diagnosis:

   • Malrotation should be considered in patients with recurrent vague abdominal symptoms 4
   • When the appendix is found outside of normal positioning during appendectomy, evaluation for malrotation is crucial 4

Prompt diagnosis and appropriate surgical therapy are crucial for favorable outcomes, especially in patients with associated anomalies such as situs problems, anomalies of the inferior vena cava, poliesplenia, or preduodenal portal vein 1.