Snapshot A 10-day-old baby boy is brought to the emergency room for repeated episodes of vomiting. His mother describes the vomit as green and yellow. On physical exam, the physician has difficulty at first locating the heartbeat, but finally hears a heartbeat on the right side of the chest. Suspicious, he sends the baby for abdominal radiography and upper GI series. Imaging reveals dilated bowel loops and a “birds beak” appearance of bowel. Introduction Malrotation errors during development increases risk of midgut volvulus 1/3 of children with malrotation develop volvulus Midgut volvulus abnormal rotation of bowel due to pathologic adhesions fixating the bowel around itself twists around SMA compromises blood flow to bowel majority in ileum increased risk of bowel necrosis and perforation Cecal volvulus common in young, active patients "coffee bean" sign Associated conditions gastroschisis omphalocele situs inversus cardiovascular defects Hirschsprung disease heterotaxy Malrotation with midgut volvulus is a surgical emergency Epidemiology Most common in newborns Males > females 80% of cases are newborn Elderly population can have malrotation, but volvulus is less often Presentation Symptoms neonates (majority of patients) bilious vomiting within first week of life colicky pain older patients nonbilious vomiting crampy abdominal pain change in bowel pattern nausea Physical exam abdominal distention abdominal tenderness acute abdomen shock Imaging Abdominal radiography multiple air fluid levels dilated loops of bowel with loss of haustra normal radiography does not exclude disease Upper GI imaging with barium enema “bird beak” appearance at site of rotation “coffee bean” appearance of bowel failure of duodenum to cross midline = malrotation Differential Diagnosis Intussusception Intestinal atresia Treatment Malrotation without volvulus elective Ladd procedure Malrotation with volvulus (symptomatic or acute abdomen) emergent endoscopic decompression emergent surgical decompression PREVENTION Prevention if patient has heterotaxy, screen for malrotation elective Ladd procedure if malrotation without volvulus Complications Bowel necrosis and perforation Sepsis Prognosis Most with resolution of symptoms after surgery Good prognosis mortality rate 3-9%, worse with premature neonates bowel necrosis