Intestinal malrotation, midgut volvulus, and gastric volvulus

Intestinal malrotation is a congenital abnormality characterized by abnormal or incomplete coiling and/or fixation of the midgut during embryologic development. The most common types of malrotation are nonrotation and incomplete rotation. Malrotation can cause midgut volvulus, which is a life-threatening condition in which the small intestine twists around the superior mesenteric artery and is a common cause of mechanical intestinal obstruction in neonates and infants. Malrotation and midgut volvulus most commonly manifest in newborns but can occur at any age. Bilious vomiting is a hallmark symptom of midgut volvulus, often accompanied by other signs of bowel obstruction (e.g., abdominal pain and distention). If left untreated, midgut volvulus can lead to bowel ischemia; features include tachycardia, hypotension, hematochezia, and peritonitis. Urgent surgical consultation is required for patients with suspected midgut volvulus. An upper GI series is the gold standard for diagnosing midgut volvulus and may show the corkscrew sign in the duodenum. Definitive treatment is surgical detorsion using the Ladd procedure.

Gastric volvulus is a distinct condition characterized by more than 180° rotation of the stomach along one of its axes. Manifestations of acute gastric volvulus include severe epigastric pain and distention, retching without vomiting, and an inability to pass a nasogastric tube (i.e., Borchardt triad). Abdominal x-rays and contrast studies can support the diagnosis. Definitive treatment involves urgent surgical detorsion with gastropexy to prevent recurrence.

See “Sigmoid volvulus and cecal volvulus” for more details on volvulus in adults.

For a normal intestinal rotation sequence, see “Rotation of the midgut.”

Epidemiology ^[10]

• Estimated to occur in ∼ 1 in 500 live births
• True incidence likely higher, as it may be asymptomatic or manifest with nonspecific symptoms in older children

Pathophysiology ^[11][12][13]

Intestinal malrotation is a congenital anomaly caused by incomplete or abnormal rotation of the midgut and/or fixation during embryogenesis.

• Nonrotation
  • The entire colon is left-sided; the entire small bowel is right-sided.
  • The mesenteric attachment has a wider base.
• Incomplete rotation
  • The cecum remains fixed in the RUQ by peritoneal bands (Ladd bands).
  • The mesenteric base is narrow.

Clinical features ^[3][10][14]

• Neonates and infants: typically present with acute manifestations
  • Clinical features of midgut volvulus
  • Features of duodenal obstruction (bilious vomiting without abdominal distention)
• Older children: : ranges from asymptomatic to chronic intermittent abdominal pain or early satiety and weight loss ^[14]

Consider intestinal malrotation in older children with unexplained nonspecific GI symptoms. ^[15]

Diagnosis ^[1][10][16]

• Upper GI series (gold standard): abnormal duodenojejunal junction (DJJ) position
  • Anteroposterior view: DJJ should lie to the left of the spine at the level of the duodenal bulb.
  • Lateral view: DJJ should course posteriorly in the retroperitoneum.
• Ultrasound: superior mesenteric vessel displacement ^[6][16]

A normal ultrasound does not exclude malrotation.

Treatment ^[10][14][17]

Symptomatic patients

• Initial management
  • Suspected midgut volvulus: Begin initial management of midgut volvulus.
  • Suspect bowel obstruction: Begin initial management of bowel obstruction.
  • Other stable symptomatic patients: Provide supportive treatment as needed.
• Definitive treatment: Ladd procedure
  • Perform urgently for midgut volvulus or bowel obstruction.
  • For other patients, consider timing on a case-by-case basis in consultation with a specialist.

Do not delay treating patients presenting with suspected midgut volvulus as it is a time-dependent surgical emergency.

Asymptomatic (incidental finding) ^[10][17]

• Children: Prophylactic Ladd procedure is often recommended due to the risk of volvulus.
• Adults: surgery or observation depending on age, comorbidities, and surgical risk

Complications ^[10]

• Midgut volvulus
• Internal hernia

Midgut volvulus most commonly occurs as a complication of intestinal malrotation in neonates and infants. Rarely, it can occur in adults as a complication of surgery (e.g., bariatric surgery).

Epidemiology

• Incidence: 1:6000 live births in the US
• Age: : most commonly occurs in neonates and infants ^{[2][18][19][20]}

Midgut volvulus and Malrotation are more common in Minors, while SigmOid volvulus is more common in Older individuals.

Pathophysiology ^[21]

Involves twisting of the bowel around its mesentery which leads to:

• Closed-loop mechanical bowel obstruction → accumulation of gas and feces within the loop → increased intraluminal pressure → impaired capillary perfusion of the bowel → bowel strangulation, ischemia, and gangrene
• Torsion of the mesenteric vascular pedicle → occlusion/thrombosis of mesenteric vessels → bowel strangulation, ischemia, and gangrene

Etiology

The following applies to children; see “Special patient groups” for etiology in adults.

• Intestinal malrotation
• Intestinal bands and/or adhesions (e.g., Ladd bands)
• Megacolon (Hirschsprung disease, Chagas disease)

Clinical features ^[3][14]

• Bilious vomiting with abdominal distention in neonates and infants
• Features of duodenal obstruction: bilious vomiting without abdominal distention
• Signs of bowel ischemia: hematochezia, hematemesis, hypotension, and tachycardia

Bilious vomiting in a lethargic newborn suggests bowel ischemia. ^[14]

Abdominal examination may have limited utility, as it is difficult to assess for tenderness and rebound tenderness in neonates and infants.

Diagnosis ^{[1][2][3][10]}

Approach

• Ill-appearing or unstable (e.g., signs of shock, signs of bowel ischemia)
  • Begin initial management of midgut volvulus.
  • Defer imaging and expedite surgery.
• Stable
  • Consider abdominal ultrasound with Doppler as an initial test for newborns with bilious vomiting. ^[22][23]
  • Obtain upper GI series (gold standard)
  • Consider small bowel follow-through as an adjunct if upper GI series is equivocal.
  • Obtain laboratory studies as adjuncts.

Do not delay surgery for imaging in ill-appearing or unstable children with suspected midgut volvulus. ^[14]

Laboratory studies ^[6]

• CBC with differential
• Blood glucose
• Electrolytes
• Renal and liver function tests
• Capillary blood gas
• Lactate
• Blood gas

Imaging findings

• Upper GI series
  • Incomplete duodenal obstruction ^[14]
  • Corkscrew duodenum ^[6]
  • Bird beak sign ^[2]
• Abdominal x-ray: nonspecific findings; may show radiological signs of bowel obstruction and a gasless abdomen ^[3]
• Abdominal ultrasound with Doppler: whirlpool sign ^[6]
• Small bowel follow-through: may show abnormal position of the cecum ^[5][7]

Differential diagnosis

• Neonates and infants with recurrent vomiting: duodenal atresia and stenosis; , hypertrophic pyloric stenosis
• Neonates with features of bowel ischemia and/or gangrene: necrotizing enterocolitis
• Older children with abdominal pain and vomiting: intussusception
• Older children and adults with nonspecific symptoms: GERD, chronic mesenteric ischemia, food allergy
• See “Differential diagnosis of lower gastrointestinal bleeding in children.”

Management ^[10]

Initial management of midgut volvulus

• ABCDE approach
• Urgent surgery consult for immediate surgery
• NPO status
• Bowel decompression (e.g., nasogastric tube on low to intermittent suction)
• IV fluid resuscitation and electrolyte repletion as needed
• Broad-spectrum empiric antibiotic therapy

Continue stabilization during transport to the operating room to maximize intestinal salvage. ^[14]

Definitive treatment ^[2][4][6]

• Expedite emergency surgery.
• Ladd procedure: a procedure involving bowel detorsion, Ladd band division, mesenteric widening, cecum and colon repositioning, and appendectomy.

Special patient groups

Children ≥ 1 year old ^[10]

• Clinical features
  • Insidious presentation over days to months
  • Intermittent vomiting and/or abdominal pain
  • Solid food intolerance
  • Chronic diarrhea
  • Signs of biliary obstruction or pancreatitis
• Treatment: Ladd procedure ^[20]

Adults ^[24]

• Epidemiology: rare in adults
• Etiology: may result from undiagnosed congenital malrotation, prior surgery, or adhesions. ^[25][26][27]
• Clinical features: can be acute (signs of bowel ischemia) or intermittent (e.g., postprandial pain, nausea)
• Diagnosis
  • Imaging: CT abdomen with contrast ^[24]
  • Findings: "Whirl sign" or abnormal positioning of the duodenojejunal junction. ^[28]
• Treatment
  • Ladd procedure with resection of necrotic bowel
  • Prior bariatric surgery requires a different surgical approach (e.g., additional closure of mesenteric defects).

Internal hernias or midgut volvulus may present similarly in patients with prior bariatric surgery. ^[29][30]

Background ^[14][31]

• Rare condition in which the stomach rotates > 180° along the organoaxial or mesenteroaxial axis.
• This leads to gastric outlet obstruction and potential ischemia, necrosis, or perforation.

Etiology ^[31]

• Lax gastric ligaments (e.g., gastrocolic, gastrohepatic)
• Anatomical disorder (e.g., paraesophageal hernia; , Bochdalek hernia in children)
• Gastric defects (e.g., gastric ulcer, gastric carcinoma) ^[6]

Clinical features ^[14][32]

• Acute: Borchardt triad ^[14]
  • Inability to pass a nasogastric tube
  • Retching without vomiting
  • Severe epigastric pain and distention
• Chronic: intermittent symptoms of gastric outlet obstruction

Diagnosis ^[32]

• Abdominal x-ray
  • Large round gas bubble in the upper abdomen or chest with an air-fluid level
  • Gasless distal bowel
• CT chest and abdomen
  • Dilated stomach; may be positioned in the thoracic cavity
  • Swirl sign (esophagus and stomach rotated around each other on transverse plane)

Treatment ^[32]

• Initial management
  • Similar to the initial management of midgut volvulus
  • Includes gastric decompression (see “Gastric outlet obstruction.”)
• Surgery: detorsion and gastropexy

Complications ^[14]

• Strangulation; of the stomach, leading to ischemia and perforation
• Sepsis