García-Guzmán AE, Aparicio-Rodríguez JM

Language: Spanish
References: 10
Page: 96-104
PDF size: 209.09 Kb.

ABSTRACT

Introduction. VACTERL association was described in the literature in 1982. At the beginning only four clinical characteristics were mentioned in comparison with six currently described; (V) Vertebral, (A) anal malformations, (C) cardiac anomalies, (TE) Traqueo-Esophaeal fistula, (R) Renal malformations, (L) limbs anomalies. This congenital association has 0.3 to 2.1 cases per 10,000 newborn. In México, few clinical cases have been reported, the main goal of this study is to analyze the different clinical patterns among 14 patients with this genetic disease in a third level pediatric Hospital in Mexico.
Material and Methods. A Descriptive, retrospective and transversal study was performed among 14 patients diagnosed with VACTERL (with a minimal of three clinical features).
Results and Discussion. Men predominated, with a 3.6:1 ratio, 79% was captured on the first day of life, VACTERL was presented in association with triads and tetrads 35.71 % each, 3 pentads with 21.42% and 7.14% hexads. the features of VACTERL that occurred most frequently were anal malformations in 83% being the most common one with anorectal malformation fistula, with the same percentage cardiac malformations were present, of these, 92% presented more of a congenital heart disease, the most common being Inter Auricular Communication 67%. NO VACTERL abnormalities were present in more than half of all cases (57%) being the most common genital ambiguity report karyotype and annular pancreas (14.28%)It is important to know the characteristics of VACTERL and the different ways that may appear to establish an early diagnosis, since more than half of the features of VACTERL resolved with surgical correction situation may benefit the quality of life of patients. The VACTERL association in this study was observed with several different clinical symptoms: A. Three and four clinical symptoms 35.71% B. Five clinical symptoms 21.42% C. Six clinical symptoms 7.14% It is important to have a earlier diagnosis with a medical and surgical treatment to ensure a better quality of life for all patients with these congenital malformations.

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