Background: VACTERL association is a rare, non-random cluster of at least three congenital anomalies involving Vertebral, Anorectal, Cardiac, Tracheoesophageal, Renal, and Limb development. It affects approximately 1 in 10,000–40,000 live births, with an unclear aetiology—though disrupted Sonic Hedgehog (Shh) signalling has been implicated.We present a case of postnatally diagnosed VACTERL in a preterm neonate, comprising bilateral superior vena cavae (SVCs), imperforate anus, tracheoesophageal fistula (TOF), oesophageal atresia (OA), and pre-axial polydactyly. The striking presence of bilateral SVCs, rarely associated with VACTERL, introduced early diagnostic uncertainty and procedural difficulty.Case Presentation Summary: A male infant was born at 33 weeks’ gestation via emergency caesarean to healthy, non-consanguineous Nigerian parents, due to intrauterine growth restriction, cerebral redistribution, and abnormal Dopplers. Maternal history included gestational diabetes and pre-eclampsia. He required resuscitation at birth (Apgar scores of 1, 8, 9). Failed nasogastric tube insertion raised immediate concern for OA. Examination revealed unilateral pre-axial polydactyly and imperforate anus.He was transferred at 6 hours of life to a regional surgical centre, where imaging confirmed OA with TOF, abnormal bowel gas, and distal colonic narrowing. On day 0 of life (DOL), he underwent thoracotomy with TOF ligation, gastrostomy for long-gap OA, and sigmoid-descending colostomy. Re-anastomosis and ‘pull-through’ surgery due to long oesophageal gap is planned for 3 months of life.VACTERL association was diagnosed postnatally based on tracheoesophageal, anorectal, limb and later cardiac anomalies. On DOL4, chest X-ray (Figure) following central venous catheter (CVC) insertion unexpectedly revealed a persistent left SVC (PLSVC). Subsequent echocardiography confirmed bilateral SVCs, a vascular anomaly scarcely associated with VACTERL. This finding complicated CVC placement and highlighted the importance of early cardiac imaging in neonates.Cranial and renal ultrasounds, and spinal X-ray were normal. The extra digit autoamputated after bedside clipping (DOL 14). Chromosomal microarray was normal. Spinal ultrasound and micturating cystourethrogram are pending to complete VACTERL screening.Learning Points Discussion: Cardiac anomalies present in 40–80% of VACTERL cases and contribute significantly to associated morbidity and mortality. Our case highlights bilateral SVCs, an exceptional but crucial finding, identified only after repeated concerns about CVC positioning. PLSVC is the most common thoracic venous anomaly, occurring in 0.3% of the general population and in 3-11% of congenital heart disease patients. Its association with VACTERL is uncommonly reported, and true prevalence remains unknown. Thus, suggesting such vascular anomalies may be under-recognised in the VACTERL spectrum.This diagnostic gap has practical implications. In our case, the missed identification of bilateral SVCs led to multiple unnecessary line insertions. Diagnostic challenges are only exacerbated by its typical asymptomatic presentation. Additionally, prenatal VACTERL diagnosis remains challenging and does not consistently improve outcomes. Instead, it often results in earlier delivery (by 2 weeks) and higher neonatal mortality risk.This case illustrates the phenotypic diversity of VACTERL, reinforces the need for comprehensive postnatal anomaly screening, and highlights the value of early cardiac imaging when multiple congenital anomalies are present. A PLSVC should be considered a necessary differential when CVCs appear to track unusually over the left thorax.
