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Malrotation, Emergency Medicine

Basics

Description

- 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

- >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%)

- 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

- 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

- 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

  • Incomplete rotation and fixation of intestine during embryogenesis during transition from extracolonic position during week 10 of gestation
  • Risk factor:
  • Associated conditions:Gastrointestinal anomalies:Duodenal stenosis, atresia, webMeckel diverticulumIntussusceptionGastroesophageal refluxOmphalocele or gastroschisisCongenital diaphragmatic herniaAbdominal wall defectHirschsprung diseaseMetabolic acidosisCongenital 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 birthsHigh mortality in infants: Up to 24%Necrotic bowel at surgery increases mortality by 25 .Incidence:In neonates, male-to-female ratio 2:175% diagnosed newborn period90% diagnosed by age 1 yr of lifeCan present during adulthood

Diagnosis

Signs and Symptoms

  • Neonates:Bilious emesisAbdominal distentionBloody stoolsConstipation/obstipationDifficulty feedingPoor weight gain
  • >1 yr: Abdominal pain followed by bilious emesis
  • Older children and adolescents:Chronic vomitingIntermittent colicky abdominal painDiarrheaHematemesisConstipationMay not exhibit abnormal physical findings at time of presentation (50 " 75%)
  • Adults: Symptoms vague and nonspecific
  • General:Dehydration, acidosisPeritonitisIschemic bowelSepsis, 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 obstructionGastric distention with paucity of intraluminal gas distal to volvulus in complete volvulusGeneralized 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% accurateFindings:Absence of ligament of Treitz or on the right side of the abdomen with misplaced duodenumDilation of proximal duodenum with termination in conical or beak shapeSpiral or corkscrew appearance of duodenum with volvulusProximal 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 casesEvaluates 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 handsUS 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 volvulusNormal ultrasound does not exclude malrotation
  • CT:Little benefit in infants and childrenMore likely to be used for diagnosis in adults

Differential Diagnosis

  • Early life:Hirschsprung diseaseNecrotizing enterocolitisIntussusception
  • Children with acute abdominal pain and peritoneal signs:AppendicitisIntussusceptionOverwhelming sepsis
  • Older children and adults with vague abdominal pain:Irritable bowel syndromePeptic ulcer diseaseBiliary and pancreatic diseasePsychiatric 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 volvulusRestoration of intestinal perfusionResection of obviously necrotic areasReplacement of long segments with questionable vascular integrity back into abdominal cavity for return evaluation and possible celiotomy in 36 hr
  • Diet:

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)