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Anomalies of intestinal rotation in childhood: analysis of 447 cases
F J Rescorla1, F J Shedd, J L Grosfeld
1Department of Surgery, Indiana University School of Medicine, Indianapolis.
Insights
Midgut volvulus, a serious intestinal condition, is common in infants and has a high mortality rate. Early surgical intervention, like the Ladd procedure, is recommended for malrotation to prevent this life-threatening complication.
Area of Science:
- Pediatric Surgery
- Gastrointestinal Surgery
- Congenital Abnormalities
Background:
- Intestinal malrotation is a congenital anomaly affecting infants and children.
- Midgut volvulus, a complication of malrotation, can lead to acute or chronic symptoms and carries a significant mortality risk.
Purpose of the Study:
- To analyze outcomes in infants and children with anomalies of rotation and fixation.
- To evaluate the incidence and management of midgut volvulus in different patient groups.
- To assess the efficacy of the Ladd procedure in preventing midgut volvulus.
Main Methods:
- Retrospective review of 447 infants and children with malrotation.
- Classification into four groups based on clinical presentation: acute midgut volvulus, chronic symptoms, incidental findings, and association with other anomalies.
- Surgical management included Ladd procedure, reduction of volvulus, resection, and appendectomy.
Main Results:
- Acute midgut volvulus (Group A) had a 28% mortality rate.
- Patients with chronic symptoms or incidental malrotation (Groups B and C) underwent Ladd procedure with unrelated deaths.
- Neonates with malrotation associated with diaphragmatic hernia or abdominal wall defects (Group D) had a low incidence of midgut volvulus when not surgically treated for malrotation.
Conclusions:
- Midgut volvulus is a critical condition in infants with high mortality.
- The Ladd procedure is recommended for patients with malrotation presenting with chronic obstructive symptoms or discovered during other surgeries.
- The risk of midgut volvulus appears lower in neonates with abdominal wall defects, possibly due to adhesions from prior operations.
Abstract:
This report concerns 447 infants and children with anomalies of rotation and fixation. Patients were placed in four groups based on initial symptoms. Group A involved 18 patients with acute midgut volvulus. At laparotomy, midgut volvulus was noted and reduction of midgut volvulus and a Ladd procedure were performed in 10 cases and resection was required in 8. There were five deaths (28%). Group B included 54 children with chronic symptoms of intermittent volvulus or duodenal obstruction. Group C involved 44 cases of malrotation observed during exploration for other disorders. Patients in groups B and C underwent a Ladd procedure and appendectomy. There were five unrelated deaths. Group D included 331 neonates with malrotation caused by either diaphragmatic hernia (n = 111) or abdominal wall defects (n = 220). A Ladd procedure was performed on 48 patients with abdominal wall defects and 29 surviving children with diaphragmatic hernia. Only 2 of 172 (1.2%) patients with abdominal wall defects and 1 of 34 (2.9%) patients with diaphragmatic hernia not treated for malrotation had midgut volvulus. Midgut volvulus is more common in infants and is associated with a high mortality rate (28%). Patients with malrotation and chronic obstructive symptoms or those observed during other elective procedures should undergo a Ladd procedure because of the risk of midgut volvulus. The risk of midgut volvulus is low in patients with abdominal wall defects and, probably as a result of adhesions from previous neonatal operations.