Squamous cell carcinoma in neglected lumbar myelomeningocele successfully managed by multidisciplinary surgical reconstruction

Abstract

Squamous cell carcinoma (SCC) arising within an untreated myelomeningocele (MMC) represents an exceptionally rare but devastating complication of this common neural tube defect, with only 15 cases documented in English literature prior to this report. We present the 16th documented case of SCC in a neglected lumbar MMC and provide a comprehensive critical appraisal of all 15 previously published cases to elucidate epidemiological patterns, treatment heterogeneity, and critical gaps in standardized management protocols. The current case involves a 19-year-old male with a neglected lumbosacral MMC and hydrocephalus who had received only ventriculoperitoneal shunt placement, without surgical closure of the underlying myelomeningocele defect. Over time, chronic ulcerations and repeated infections lead to malignant transformation. Magnetic resonance imaging revealed an Arnold–Chiari II malformation and an extensive mass infiltrating the spinal cord from T11 to S1. En bloc resection with durotomies and reconstruction using bilateral V-Y advancement flaps with latissimus dorsi muscle advancement achieved a complete oncological excision. Adjuvant locoregional radiotherapy was administered for high-risk patients. At two-year follow-up, the patient remained disease free. This case underscores the importance of early MMC closure and vigilant long-term surveillance in resource-limited settings to prevent Marjolin-type degeneration.