Surgical problems in patients with VATER-associated anomalies

Area of Science:

• Pediatric Surgery
• Neonatology
• Clinical Genetics

Background:

• Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a severe congenital anomaly.
• VATER association (Vertebral, Anal, Tracheo-Esophageal, Renal, and Limb anomalies) frequently co-occurs with EA/TEF.
• This study examines outcomes in patients with EA/TEF and multiple VATER anomalies.

Purpose of the Study:

• To review the clinical course and outcomes of patients with EA/TEF and multiple VATER anomalies.
• To evaluate the effectiveness of different surgical approaches.
• To identify factors influencing survival and long-term morbidity.

Main Methods:

• Retrospective review of 14 patients diagnosed with EA/TEF and at least two other VATER anomalies between 1960 and 1980.
• Analysis of patient demographics, associated anomalies, interventions, and outcomes.
• Surgical interventions included preliminary gastrostomy, primary esophageal repair, delayed esophageal repair, and cervical esophagostomy.

Main Results:

• 70% of patients were preterm or low birth weight.
• Common associated anomalies included vertebral (7), anorectal (11), limb (9), and renal (9).
• Eight patients died, with 6 deaths occurring between 1960-1970. Four of 9 primary repairs survived, while 2 of 3 delayed repairs survived.
• Three survivors experienced growth retardation below the 5th percentile.

Conclusions:

• EA/TEF with VATER association presents significant challenges, often compounded by prematurity and multiple anomalies.
• While surgical outcomes have improved over time, aggressive nutritional support and management of chronic conditions are crucial for survivors.
• Delayed esophageal repair may offer better survival rates in select cases.