Long-term outcome of terminal myelocystocele patients

Introduction: A terminal myelocystocele, a closed form of a neural tube def ect (NTD), can present as a large, fully epithelialized, cystic lumbosacral mass containing fat, cerebrospinal fluid (CSF) and neural tissue. The spin al cord terminates at a neural placode wherein the central canal opens into a CSF-filled cavity that is distinct from fluid in the subarachnoid space surrounding the spinal cord. This form of NTD, in our experience, was only associated with major caudal cell mass abnormalities, as these patients oft en have maldevelopment of the lower spine, pelvis, genitalia, bowel, bladde r, kidney and the abdominal wall. This study will describe the clinical man ifestations, surgical management and long-term outcome of our terminal myel ocystocele patients. Methods: To characterize this rare entity, a 13-year r etrospective review was undertaken at our institution. Results: Nine patien ts with terminal myelocystoceles were identified. In all cases, there were multiple congenital defects including cloacal exstrophy, imperforate anus, omphalocele, pelvic deformity, equinovarus or renal abnormality. Only 1 out of 9 patients has required a shunt for hydrocephalus. The main goal of neu rosurgical intervention was to reduce the size of the mass, which can slowl y enlarge over time. The spinal cord was also untethered, although these pa tients have no chance of bowel or bladder control. With a mean follow-up of 63 months, all patients remained neurologically stable. Impairment of lowe r extremity function is usually severe. However, some patients were ambulat ory with the aid of a walker or orthotic device. All patients required a pr olonged hospital stay as well as multiple operations prior to initial disch arge. Conclusion: Experienced, multispecialty care is needed to optimize th e long-term outcome of these complex patients. Copyright (C) 2000 S. Karger AG, Basel.