Intestinal malrotation

Congenital anomaly of intestinal rotation and fixation resulting in abnormal bowel positioning; predisposes to life‑threatening midgut volvulus and duodenal obstruction by Ladd's bands.

• Even if rare, malrotation can present with catastrophic midgut volvulus in infancy – a surgical emergency that can lead to bowel necrosis and shock if not promptly treated. It's a classic neonatal scenario (bilious vomiting) that requires rapid recognition. Untreated malrotation may also cause chronic GI issues or intermittent obstruction, impacting feeding and growth.

• Neonate (acute volvulus) – Typically a newborn with bilious (green) vomiting, abdominal distension, and pain. May rapidly progress to ischemia (signs include bloody stools, peritonitis, shock) if midgut volvulus occurs.
• Older infants/children – Sometimes malrotation isn't obvious at birth. Children can have recurrent episodes of abdominal pain and vomiting, failure to thrive, or malabsorption due to intermittent obstruction (Ladd's bands or partial volvulus). Symptoms may be chronic and vague (e.g. "abdominal migraines").
• Adults – Rarely, malrotation presents in adulthood (often <1% of cases). Adult patients might have chronic digestive complaints or an acute bowel obstruction/ischemia. Some people remain asymptomatic and malrotation is discovered incidentally.

1. Always suspect malrotation in any neonate with bilious emesis – consider it midgut volvulus until proven otherwise (don't wait for the infant to become critically ill).
2. Initial management: stabilize the patient. Start IV fluids and electrolyte resuscitation, and place a nasogastric (NG) tube to decompress the stomach. Simultaneously, call for an urgent surgical evaluation.
3. Diagnostic imaging (if stable): an upper GI series (contrast study) is the gold standard for malrotation. It will show an abnormal position of the duodenojejunal junction (Ligament of Treitz) – often misplaced to the right side. In volvulus, the upper GI may show a characteristic "corkscrew" twisted appearance of the duodenum/jejunum. An abdominal X-ray can suggest proximal obstruction (e.g., stomach and duodenal dilation with little distal gas) but is not definitive. Ultrasound can demonstrate the "whirlpool sign" (twisting of the SMV around the SMA) or an inverted SMA/SMV relationship. A contrast enema (lower GI series) may show the cecum in an abnormal location (not in the RLQ). In older patients, abdominal CT or MRI can also help confirm malrotation anatomy.
4. Do not delay surgery for imaging if volvulus is highly suspected or if the infant is unstable. Malrotation with volvulus is a surgical emergency – the definitive treatment is prompt operative intervention (Ladd's procedure).

1. Emergent surgical correction (Ladd's procedure). If volvulus is present, first untwist the bowel (usually rotating counterclockwise). Then divide the Ladd's bands that are crossing and obstructing the duodenum, and broaden the mesenteric base by arranging small intestine on the right and large intestine on the left.
2. Perform an appendectomy during the Ladd's procedure (the cecum/appendix will be in an atypical location, so removing it prevents future diagnostic confusion). Any necrotic bowel found during surgery must be resected.
3. Pre-operative care: aggressive fluid resuscitation, gastric decompression, and IV broad-spectrum antibiotics (especially if bowel perforation or peritonitis is suspected). Post-operatively, monitor for short bowel syndrome if significant resections were done, and for potential lingering GI dysmotility issues.

• Neonate + green vomit = malrotation with midgut volvulus until proven otherwise. Always treat bilious emesis in infancy as an emergency workup.
• Remember the Ladd's procedure steps: untwist the bowel (counterclockwise detorsion), cut the obstructing Ladd's bands, broaden the mesentery (place small bowel on the right, colon on the left), and remove the appendix (which will be in the wrong place).

• Any bilious (green) vomit in a neonate should be treated as a surgical emergency until malrotation with volvulus is excluded.
• Signs of intestinal ischemia in an infant (e.g. bloody stools, abdominal wall discoloration, peritonitis, shock) → suggest midgut necrosis from volvulus; requires immediate surgery.

1. Neonate with bilious emesis → NPO (nothing by mouth), NG tube for decompression, IV fluids; urgent surgical consult.
2. If stable, perform an upper GI series to confirm malrotation (abnormal position of duodenojejunal junction or corkscrew sign). If unstable or clinical suspicion is high, go straight to surgery.
3. Positive malrotation (or high suspicion) → prompt Ladd's procedure (detorsion of bowel, cut Ladd's bands, position bowel, appendectomy).
4. Post-op: monitor for complications (short gut if any bowel removed, or ongoing motility problems). Even after a successful Ladd's procedure, some patients have residual feeding intolerance or bowel dysmotility that needs supportive care.

• Newborn with bilious vomiting, abdominal distension, and hemodynamic instability (shock) → Intestinal malrotation with midgut volvulus (surgical emergency).
• Upper GI contrast study showing an abnormal duodenum (e.g., not crossing midline or a "corkscrew" configuration) in an infant with vomiting → malrotation of the midgut (predisposing to volvulus).