Non-VACTERL-type anomalies are frequent in patients with esophageal atresia/tracheo-esophageal fistula and full or partial VACTERL association
✍ Scribed by Elisabeth M. de Jong; Janine F. Felix; Jacqueline A. Deurloo; Marieke F. van Dooren; Daniël C. Aronson; Claudine P. Torfs; Hugo A. Heij; Dick Tibboel
- Publisher
- John Wiley and Sons
- Year
- 2008
- Tongue
- English
- Weight
- 86 KB
- Volume
- 82
- Category
- Article
- ISSN
- 1542-0752
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✦ Synopsis
Abstract
BACKGROUND:
The VACTERL association is the nonrandom co‐occurrence of __V__ertebral anomalies, __A__nal atresia, __C__ardiovascular malformations, __T__racheo‐esophageal fistula (TEF) and/or __E__sophageal atresia (EA), __R__enal anomalies, and/or __L__imb‐anomalies. The full phenotype of patients with EA/TEF and other anomalies of the VACTERL spectrum of defects association is not well described in the literature.
METHODS:
Data on patients with EA/TEF seen in two pediatric surgical centers in the Netherlands between January 1988 and August 2006 were evaluated for defects of the VACTERL spectrum as well as non‐VACTERL‐type defects. The presence of two or more defects of the VACTERL spectrum in addition to EA/TEF was the criterion for inclusion in this study. A detailed description was made of all defects.
RESULTS:
Of 463 patients with EA and/or TEF, 107 (23.1%) fulfilled the inclusion criterion, of which seventeen cases had a recognized etiology and were excluded, leaving 90 cases (19.4%) for analysis. Other than the esophagus and the trachea, the vertebrae/ribs and the cardiovascular system were most commonly affected (68.9 and 65.6%, respectively). Interestingly, 70% of cases had additional non‐VACTERL‐type defects, with high occurrences for single umbilical artery (20%), genital defects (23.3%), and respiratory tract anomalies (13.3%).
CONCLUSIONS:
Many patients with EA/TEF and at least two other defects of the VACTERL spectrum also display non‐VACTERL‐type congenital anomalies. Birth Defects Research (Part A), 2008. © 2008 Wiley‐Liss, Inc.