Congenital lumbar and umbilical hernias in lumbo-costo-vertebral syndrome with ipsilateral renal agenesis: A rare case report with literature review.

Abstract

<h4>Introduction and importance</h4>Congenital lumbar hernia (CLH) is a rare condition in infancy, often associated with lumbo-costo-vertebral syndrome (LCVS), which combines vertebral, rib, and abdominal wall anomalies. Genitourinary defects are uncommon and rarely documented.<h4>Case presentation</h4>A 1-year-old girl presented with a left-sided CLH, small umbilical hernia, and prominent left anterior ribs protrusion manifesting as a chest wall bulge. Radiologic workup showed T10-T11 hemivertebrae, fused left laminae and ribs (10th-11th), scoliosis convex to the hernia side, and ipsilateral renal agenesis, confirming LCVS with a genitourinary anomaly. Elective open surgical repair included meshplasty of the left lumbar defect and excision of the protruding fused left anterior rib segment through the same incision, with concurrent umbilical hernia repair through a circumferential periumbilical incision. Recovery was uneventful.<h4>Clinical discussion</h4>This case illustrates the syndromic nature of CLH within LCVS and underscores the need to screen for associated anomalies, particularly genitourinary defects such as renal agenesis. Imaging beyond ultrasound is often required to delineate skeletal and myofascial abnormalities. An open approach was favored to allow simultaneous excision of the protruding fused left rib segment and durable mesh reinforcement of a ~5 cm defect; this strategy achieved excellent outcomes. The coexistence of umbilical hernia with LCVS and CLH may be incidental but could broaden the recognized phenotypic spectrum and, to our knowledge, has not been previously reported.<h4>Conclusion</h4>Awareness of LCVS in infants with CLH facilitates evaluation for associated anomalies and guides timely surgical management. Early recognition and repair can yield favorable results.