Malrotation
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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%.
- Gastrointestinal anomalies:
Etiology
- Duodenojejunal junction remains right of midline
- Cecum remains in the upper left abdomen with abnormal mesenteric attachments
- Volvulus is a 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
- Congenital cardiac anomalies are present in 27% of patients with malrotation
- 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
- 10% may present after age 1 and can present during adulthood
-- To view the remaining sections of this topic, please log in or purchase a subscription --
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%.
- Gastrointestinal anomalies:
Etiology
- Duodenojejunal junction remains right of midline
- Cecum remains in the upper left abdomen with abnormal mesenteric attachments
- Volvulus is a 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
- Congenital cardiac anomalies are present in 27% of patients with malrotation
- 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
- 10% may present after age 1 and can present during adulthood
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