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- Title
Heterotaxy and intestinal rotation anomalies: 20 years experience at a UK regional paediatric surgery centre.
- Authors
Cullis, Paul; Siminas, Sotirios; Salim, Adeline; Johnson, Robert; Losty, Paul; Cullis, Paul S; Losty, Paul D
- Abstract
<bold>Background: </bold>The association of heterotaxy with intestinal rotation anomalies is well described. However debate exists with regard optimal management notably should 'asymptomatic' bowel rotation anomalies undergo operation? The present study therefore sought to determine: (1) the risk(s) of volvulus in patients diagnosed with heterotaxy and (2) define morbidity associated with operation for 'asymptomatic' anomalies in a fragile patient cohort with co-existent congenital heart disease.<bold>Methods: </bold>Medical case record reviews of ALL heterotaxy patients born during January 1993-December 2013 and attending a UK paediatric centre were analyzed.<bold>Results: </bold>Of a total of 92 patients, 16 (17.4%) cases underwent foregut imaging studies. Three examinations were performed in 'symptomatic' patients. Twelve studies reported 'abnormal anatomy' with only five patients undergoing surgical correction. No complication(s) were recorded after Ladd's operation to correct defects. A single fatality occurred within 30 days postoperatively from cardiac failure. In 87 patients in whom Ladd's operation was not undertaken, no single patient developed intestinal volvulus (median length of follow-up 27.2 months, total 446.1 person years).<bold>Conclusion: </bold>This study strongly supports a 'watchful waiting' policy for heterotaxy patients. Many children who ultimately die from heart disease may avoid unnecessary abdominal surgery.
- Subjects
UNITED Kingdom; HETEROTAXY syndromes; PEDIATRIC surgery; INTESTINAL physiology; MEDICAL records; HEALTH outcome assessment; SURVIVAL analysis (Biometry); TREATMENT effectiveness; VOLVULUS; DISEASE complications; DIAGNOSIS
- Publication
Pediatric Surgery International, 2015, Vol 31, Issue 12, p1127
- ISSN
0179-0358
- Publication type
journal article
- DOI
10.1007/s00383-015-3755-3