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A 3-week-old full-term infant born with hypoplastic left heart syndrome presents with bilious vomiting for 2 days. The patient appears irritable.

Laboratory: HCO3 20; BE 8; hematocrit (Hct) 30

An upper GI contrast study shows duodenojejunal flexure to the right of the midline, jejunal loops on the right side of the abdomen, and a high cecum on delayed film.


Intestinal malrotation is a developmental anomaly that affects the position and peritoneal attachments of the small and large intestines during organogenesis in fetal life. Intestinal malrotation occurs in 1 in 500 live births. Male predominance is present during neonatal presentations, with a male-to-female ratio of 2:1. About 40% of patients with malrotation present within the first week of life, 50% at up to 1 month of age, and 75% by the age of 1 year; the remaining 25% present later, even into adult life.

Malrotation may be associated with other congenital abnormalities, including congenital heart disease, diaphragmatic hernia, esophageal atresia, duodenal or jejunal web or atresia, omphalocele, and gastroschisis.

Patients either are diagnosed as an incidental finding and will present to the operating room for an elective Ladd’s procedure or will present with a tender, distended abdomen and bilious vomiting secondary to bowel obstruction. Acidosis due to dehydration and bowel ischemia is associated with volvulus.

If malrotation is associated with volvulus, it is an absolute surgical emergency.


  • When this is an elective procedure, mask or IV induction is acceptable.

  • Full stomach precautions should be taken during induction if the patient is obstructed, including rapid-sequence IV induction.

  • Consider an arterial line if the patient is obstructed or has a congenital cardiac disease.

  • Aggressive fluid resuscitation with large volumes of colloids and blood and circulatory support with pressors may be needed.

  • Correction of acidosis is done initially with fluid resuscitation followed with bicarbonate, diluted to 0.5 mEq/mL to avoid causing acute hypernatremia, which could increase the risk of intracerebral bleeding in neonates.

  • Opioid-based anesthetics are best tolerated.

  • Patients with associated congenital cardiac disease, especially those with Blalock-Taussig shunts, have to be aggressively fluid resuscitated to avoid clotting of their shunts.


Patients may require admission to the intensive care unit if this condition is associated with other congenital anomalies. Patients may require postoperative fluid resuscitation. Risk of postoperative bowel obstruction does exist after Ladd’s procedure.

DOs and DON’Ts

  • ✓ Do use rapid-sequence induction when patients are obstructed.

  • ✓ Do aggressive fluid resuscitation and replacement of the third space extracellular fluid loss.


The surgical management of malrotation is based on Ladd’s procedure. The base of the mesenteric pedicle is broadened by dividing the peritoneal bands ...

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