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Detailed information of MALROTATION
MALROTATION
DEFINITION:
A congenital disorder of the intestine characterized by
incomplete rotation of the intestines during fetal development which
may result in acute or chronic, partial or complete bowel
obstruction.
EPIDEMIOLOGY:
- incidence: ?
- age of onset:
- usually in the first year of life with peak before 2 months
of age
- risk factors:
- malrotation is associated with:
- abdominal wall defects (diaphragmatic hernia,
gastroschisis, omphalocele)
- abdominal heterotaxia
- asplenic-polyspenia congenital heart malformation syndrome
anamalad
- duodenal stenosis or atresia
- small intestinal atresia
PATHOGENESIS:
CLINICAL FEATURES:
1. Gastrointestinal Manifestations
1. Bowel Obstruction
- acute or chronic, partial or complete
- bilious emesis
- cyclic or recurrent vomiting and/or abdominal pain
- 25-50% of adolescents are asymptomatic
2. Complications
- malnutrition
- fat malabsorption
- protein-loosing enteropathy
- midgut volvulus with ischemia, necrosis of the small
bowel, and shock
INVESTIGATIONS:
1. Imaging Studies
1. Small Bowel Follow-Thru
- initial procedure performed in a nonobstructed patient
- small bowel on the right side of the abdomen
- colon on the left side of the abdomen
- duodenojejunal junction (ligament of Treitz) lies to the
right of the vertebral column
- cecum is not in the right lower quadrant
- a sharp cutoff with a curved narrowing in the distal
duodenum may indicate an obstruction secondary to a volvulus
2. Barium Enema
- malposition of the cecum - midline or left of midline in
the upper abdomen (position of cecum is normal in 10% of
patients)
3. Abdominal X-Ray
- may easily miss malrotation or volvulus
- double bubble sign with obstruction
MANAGEMENT:
1. Surgery
- emergency reduction of volvulus if present
- surgical correction of the malrotation
ADDITIONAL REFERENCES:
1. Rudolph's Pediatrics (19th Edition); p. 1032-1033 (1991).
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Pediatric Database - MALROTATION
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