Diagnosis

Intestinal malrotation

Etiology

Cause [ebw]

1. Genetic mutations in the gene BCL6 critical for regular intestinal rotation

2. Resulting in:

   • Abnormal location of the cecum

   • Fibrotic band formation from the cecum to the retroperitoneum passing over the duodenum creating a potential point of obstruction

Pathophysiology [ebw]

1. Midgut (duodenum to mid-transverse colon) develops extraperitoneally and migrates intraperitoneally at 12 weeks

2. During this migration, the midgut rotates 270 degree counterclockwise around the superior mesenteric artery (SMA)

3. Intestinal malrotation is a result of an error in the embryonic rotation of the gut

4. Classification of malrotation:

   • Non-rotation

   • Incomplete rotation

   • Reverse rotation

   • Anomalous fixation of the mesentery

5. Clinical presentation:

   • Acute: emesis, abdominal pain/distension, peritonitis, hypovolemia or shock

   • Intermittent: intermittent/chronic abdominal pain/vomiting

   • Asymptomatic

Complications [ebw]

• Volvulus

• Mid-gut ischemia

• Adhesive ileus

• Deep vein thrombosis

• Aspiration pneumonia

Epidemiology

Incidence per 100.000 [o89][vse][o8u][7g2][1vm]

Approach

• Blood tests: complete blood count, hematocrit

• Blood gas: lactate

• Abdominal x-ray: double-bubble sign

• Barium enema: sensitivity 93%, specificity 77%, and accuracy 86% [ebw]

• Abdominal CT

• Abdominal ultrasound

• Abdominal MRI

Treatment

1. Preoperative treatment: [vse]

   • Fluid resuscitation

   • Nasogastric tube for decompression

   • Treatment of septic shock

   • Administration of broad-spectrum antibiotics to cover bowel flora

2. Surgery: [o89][vse]

   • Adhesiolysis

   • Ladds procedure:

     • Division of obstructing Ladd bands

     • Widening the base of the mesentery

     • Placing the bowel in a position of nonrotation

     • Creating adhesions that fixate the intestines in place

     • Incidental appendectomy

   • Small bowel resection

Differential diagnoses

Acute mesenteric ischemia, Acute pancreatitis, Adhesion, Appendicitis, Cholecystitis, Choledocholithiasis, Cholelithiasis, Crohn disease, Gastric outlet obstruction, Gastroenteritis, Gastroesophageal reflux disease, Gastrointestinal obstruction, Hirschsprung's Disease, Hypovolemia, Inguinal hernia, Intestinal atresia, Intussusception, Irritable bowel syndrome, Meckels diverticulum, Meconium ileus, Necrotizing enterocolitis, Ovarian torsion, Pyloric stenosis, Sepsis, Shock, Volvulus