Malrotation, Emergency Medicine

Basics

Description

Incomplete rotation and fixation of intestine during embryogenesis during transition from extracolonic position during week 10 of gestation
Risk factor:
- Heterotaxia syndromes
Associated conditions:
- Gastrointestinal anomalies:
  - Duodenal stenosis, atresia, web
  - Meckel diverticulum
  - Intussusception
  - Gastroesophageal reflux
  - Omphalocele or gastroschisis
  - Congenital diaphragmatic hernia
  - Abdominal wall defect
  - Hirschsprung disease
- Metabolic acidosis
- Congenital cardiac anomalies; present in 27% of patients with malrotation; increases morbidity to 61%

Etiology

Duodenojejunal junction remains right of midline
Cecum remains in the upper left abdomen with abnormal mesenteric attachments
Volvulus is complication of malrotation when small bowel rotates around superior mesenteric artery and vein resulting in vascular compromise to midgut
Abnormal anatomy predisposes to obstruction and other conditions
Usually found in combination with other congenital anomalies (70%): Cardiac, esophageal, urinary, anal
Epidemiology:
- 1 in 500 live births
- High mortality in infants: Up to 24%
- Necrotic bowel at surgery increases mortality by 25 .
- Incidence:
  - In neonates, male-to-female ratio 2:1
  - 75% diagnosed newborn period
  - 90% diagnosed by age 1 yr of life
  - Can present during adulthood

Diagnosis

Signs and Symptoms

Neonates:
- Bilious emesis
- Abdominal distention
- Bloody stools
- Constipation/obstipation
- Difficulty feeding
- Poor weight gain
>1 yr: 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
Signs of small bowel obstruction in early infancy
Bilious vomiting associated with abdominal pain
In older children and adults, the most common symptom is abdominal pain
Other pertinent history " acute or chronic abdominal pain, poor feeding, lethargy, malabsorption, chronic diarrhea

Physical Exam

Abdominal exam may show distension from obstruction
Blood in the stool indicates bowel ischemia
Evaluate for congenital anomalies

Essential Workup

Diagnosis is suggested by history and physical exam findings and is delineated by contrast radiography.

Diagnosis 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 distension of stomach and duodenum
Upper GI contrast studies:
- 95% sensitive and 86% accurate
- 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 be very sensitive in experienced hands
- US shows 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
- Overwhelming sepsis
Older children and adults with vague abdominal pain:
- Irritable bowel syndrome
- Peptic ulcer disease
- Biliary and pancreatic disease
- Psychiatric disorders

Treatment

Midgut volvulus may result in need for rapid volume and electrolyte replacement/resuscitation to correct severe hypovolemia and metabolic acidosis.

Pre-Hospital

Rapid transport to ED

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 CV catheter
Initiate broad-spectrum antibiotics for signs of sepsis or peritonitis

Ed Treatment/Procedures

Emergent surgical correction
May require transfer to facility with pediatric surgical expertise when associated with midgut volvulus for:
- Detorsion of volvulus
- Restoration of intestinal perfusion
- Resection of obviously necrotic areas
- Replacement of long segments with questionable vascular integrity back into abdominal cavity for return evaluation and possible celiotomy in 36 hr
Diet:
- NPO

Medication

Broad-spectrum antibiotics prior to surgery
Correct fluid and electrolyte abnormalities
Vasopressors

Follow-Up

Disposition

Admission Criteria

Acute abdomen
Surgical intervention
Significant dehydration
Acidosis
Sepsis
Shock

Discharge Criteria
Stable, asymptomatic, incidental finding without associated condition, although patients are usually admitted

Pediatric surgical evaluation prior to discharge

Issues for Referral
Diagnostic evaluation often requires tertiary care pediatric hospital with pediatric surgical and pediatric radiologic expertise.

Followup Recommendations

As dictated by pediatric surgical service

Pearls and Pitfalls

Early recognition of child with acute abdomen
Prompt treatment of acidosis and shock
Prompt referral to appropriate facility

Additional Reading

Applegate KE. Evidence-based diagnosis of malrotation and volvulus. Pediatr Radiol. 2009;39:S161 " S163.
Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2010.
Lampl B, Levin TL, Berdon WE, et al. Malrotation and midgut volvulus: A historical review and current controversies in diagnosis and management. Pediatr Radiol. 2009;39:359 " 366.
Nehra D, Goldstein AM. Intestinal malrotation: Varied clinical presentation from infancy through adulthood. Surgery. 2011;149:386 " 393.
Shew SB. Surgical concerns in malrotation and midgut volvulus. Pediatr Radiol. 2009;39:S167 " S171.

Codes

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)

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