Signs and Symptoms
- Asymptomatic until volvulus occurs
- Neonates:
- Bilious emesis
- Abdominal distention
- Bloody stools
- Constipation/obstipation
- Difficulty feeding
- Poor weight gain
- Infants: Abdominal pain followed by bilious emesis
- Older children and adolescents:
- Chronic vomiting
- Intermittent colicky abdominal pain
- Diarrhea
- Hematemesis
- Constipation
- May not exhibit abnormal physical findings at time of presentation (50-75%)
- Adults: Symptoms vague and nonspecific
- General
- Dehydration
- Acidosis
- Peritonitis
- Ischemic bowel
- Sepsis, shock
History
- Vomiting in infant is the most common sign, but may or may not be bilious
- Bilious vomiting associated with abdominal pain
- Other pertinent history - acute or chronic abdominal pain, poor feeding, lethargy, malabsorption, chronic diarrhea
- In early infancy: Signs of small bowel obstruction
- In older children and adults: The most common symptom is abdominal pain
Physical Exam
- Abdominal exam may show distention from obstruction, tenderness, peritonitis
- Blood in the stool indicates bowel ischemia
- Evaluate for associated congenital anomalies
Essential Workup
Diagnosis is suggested by history and physical exam findings and is delineated by contrast radiography
Diagnostic Tests & Interpretation
Lab
- CBC
- Venous blood gas
- Electrolytes, BUN, creatinine, glucose
- Urinalysis/urine culture
- Type and screen
- Prothrombin time, partial thromboplastin time, international normalized ratio
- Lactate
Imaging
- Plain abdominal radiographs:
- Diagnostic in <30%
- Volvulus likely if accompanied by:
- Duodenal obstruction
- Gastric distention with paucity of intraluminal gas distal to volvulus in complete volvulus
- Generalized distention of small-bowel loops
- “Double-bubble sign” can be seen on upright film from partial duodenal obstruction causing distention of stomach and duodenum
- Upper GI contrast studies:
- Gold stand ard for diagnosis: 93-100% sensitive for diagnosis of malrotation and 56-79% sensitive for diagnosis of volvulus
- Findings:
- Absence of ligament of Treitz or on the right side of the abdomen with misplaced duodenum
- Dilation of proximal duodenum with termination in conical or beak shape
- Spiral or corkscrew appearance of duodenum with volvulus
- Proximal jejunum on right side of abdomen (although readily displaced in neonates)
- Thickening of small-bowel folds
- Contrast enema:
- Can be useful to determine position of cecum in equivocal cases
- Evaluates position of cecum in midline of upper abdomen or to left of midline
- >20% false-negative results
- Ultrasound:
- US can show abnormal relationship between superior mesenteric artery and vein in malrotation
- “Whirlpool” sign on Doppler US of superior mesenteric artery and vein twisting around the base of mesenteric pedicle seen in volvulus
- Normal ultrasound does not exclude malrotation
- CT:
- Little benefit in infants and children
- More likely to be used for diagnosis in adults
Differential Diagnosis
- Early life:
- Hirschsprung disease
- Necrotizing enterocolitis
- Intussusception
- Children with acute abdominal pain and peritoneal signs:
- Appendicitis
- Intussusception
- Sepsis
- Older children and adults with vague abdominal pain:
- Irritable bowel syndrome
- Peptic ulcer disease
- Biliary and pancreatic disease
- Psychiatric disorders
Volvulus may result in need for rapid volume and electrolyte replacement/resuscitation to correct severe hypovolemia and metabolic acidosis
Prehospital
Rapid transport to ED with fluid resuscitation as appropriate
Initial Stabilization/Therapy
- ABCs
- NS (0.9%) IV fluid bolus (20 mL/kg) for shock, sepsis, or dehydration
- Consider nasogastric tube
- 2 IVs and /or central venous catheter
- Initiate broad-spectrum antibiotics for signs of sepsis or peritonitis
ED Treatment/Procedures
- Emergent surgical consultation/correction
- May require transfer to facility with pediatric surgical expertise when malrotation is associated with volvulus for:
- Detorsion of volvulus
- Restoration of intestinal perfusion
- Resection of obviously necrotic areas
- Diet:
Medication
- Broad-spectrum antibiotics prior to surgery
- Correct fluid and electrolyte abnormalities
- Vasopressors
Disposition
Admission Criteria
- Acute abdomen
- Ill-defined diagnosis
- Surgical intervention
- Significant dehydration
- Sepsis
Discharge Criteria
Usually admitted. Stable, asymptomatic, incidental finding without associated condition, signs or symptoms.
- Pediatric surgical evaluation prior to discharge
Issues for Referral
Diagnostic evaluation and surgical intervention often requires tertiary care pediatric hospital with pediatric surgical and pediatric radiologic expertise
Follow-up Recommendations
As dictated by pediatric surgical service
- CarrollAG, KavanaghRG, Ni LeidhinC, et al. Comparative effectiveness of imaging modalities for the diagnosis of intestinal obstruction in neonates and infants: A critically appraised topic . Acad Radiol. 2016;23(5):559-568.
- LamplB, LevinTL, BerdonWE, et al. Malrotation and midgut volvulus: An historical review and current controversies in diagnosis and management . Pediatri Radiol. 2009;39(4):359-366.
- NehraD, GoldsteinAM. Intestinal malrotation: Varied clinical presentation from infancy through adulthood . Surgery. 2011;149(3):386-393.
- StanescuAL, LiszewskiMC, LeeEY, et al. Neonatal gastrointestinal emergencies: Step-by-Step approach . Radiol Clin North Am. 2017;55(4):717-739.
See Also (Topic, Algorithm, Electronic Media Element)
ICD9
751.4 Anomalies of intestinal fixation
ICD10
Q43.3 Congenital malformations of intestinal fixation
SNOMED
29980002 Congenital malrotation of intestine (disorder)
25617003 Congenital duodenal obstruction due to malrotation of intestine (disorder)
253786009 Congenital volvulus (disorder)
37528004 Malrotation of cecum (disorder)