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VACTERL association: a case report highlighting multisystem anomalies and the critical need for multidisciplinary
Mo'ath Iyad Milhem1, Saja Zaki Al-Junaidi1, Abedallah Emad Jabareen1
1Faculty of Medicine, Hebron University, Hebron, Palestine.
Introduction:
VACTERL association is a rare congenital disorder characterized by multisystem anomalies. Neonates are particularly vulnerable, and early recognition with coordinated multidisciplinary care is crucial, especially in resource-limited settings.
Case Presentation:
A Palestinian female neonate was born with respiratory distress, greenish aspiration, and failure to pass meconium. Clinical and imaging findings revealed type 3 tracheoesophageal fistula (TEF), polycystic kidney disease, cloacal anomaly, and persistent pulmonary hypertension (PPHN). Following stabilization in the NICU, the patient developed abdominal distension and oliguria. Ultrasound confirmed bilateral enlarged, cystic kidneys, and obstruction. Urgent surgical exploration revealed a 4 cm anterior gastric wall perforation, which was repaired, and a double-barrel colostomy was performed. TEF ligation and chest tube placement were also completed. Despite intensive care and surgical intervention, the neonate succumbed at 52 hours of life due to severe cardiovascular instability.
Clinical Discussion:
This case illustrates the complexity and variability of VACTERL presentation. The presence of severe anomalies like polycystic kidneys and PPHN contributed to the poor prognosis. Early antenatal diagnosis, coordinated surgical care, and NICU support are essential for survival in such cases.
Conclusion:
This case underscores the life-threatening nature of VACTERL association, highlights the importance of early diagnosis, prenatal screening, multidisciplinary care, and parental counseling, and emphasizes the challenges faced in resource-limited settings.
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