VACTERL association with polycystic kidney disease in a newborn: a case report

Joshuan J Barboza, ,Universidad Señor De Sipán, Escuela De Enfermería Chiclayo, Perú ,Universidad César Vallejo, Facultad De Medicina Trujillo, Perú ,David R Rodríguez Díaz,Norma Del Carmen Gálvez-Díaz,Edwin A Aguilar-Sánchez

doi:10.24265/horizmed.2021.v21n3.11

Abstract

Malformations are important causes of infant mortality, chronic disease and disability in many countries, causing 3.2million disabilities per year. The VATERL association includes vertebral defects, anal atresia, tracheoesophageal fistulaand renal dysplasia, as well as limb abnormalities. This case report aims to provide the general characteristics of theVACTERL association and discuss other cases found in the medical literature. This is the case of a female newborn deliveredat 35 weeks of pregnancy with severe respiratory distress, who was admitted to the neonatal ICU due to hemodynamicdecompensation. She had a history of bilateral polycystic kidney disease found by ultrasound at 25 weeks of pregnancy. Aphysical examination showed vaginal agenesis and imperforate anus. Atelectasis, cardiomegaly and dextroposition of thecardiac silhouette were observed in a radiography. The patient was diagnosed with VACTERL association and imperforateanus. She died 2 days after a respiratory arrest.

Highlights

Malformations are important causes of infant mortality, chronic disease and disability in many countries, causing 3.2 million disabilities per year
Una malformación congénita es la alteración de un tejido causada por una anormalidad genética que afecta directamente a los genes involucrados en su desarrollo [1]
Uno de los aspectos clínicos relevantes en el caso fue la marcada dificultad respiratoria que se presentó desde el nacimiento

Summary

Introduction

Malformations are important causes of infant mortality, chronic disease and disability in many countries, causing 3.2 million disabilities per year. VACTERL (siglas del inglés vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, y limb abnormalities) es una entidad caracterizada por defectos vertebrales, atresia anal, fístula traqueoesofágica y displasia renal, así como anormalidades en las extremidades . El estudio de Piro et al [5] describe la asociación de anomalías genéticas y su correlación con atresia esofágica con o sin fístula gastroesofágica en casos de VACTERL.

Results

Conclusion