Cord untethering for lipomyelomeningocele: expectation after surgery

Abstract

The natural history of cord tethering in transitional LMMC remains unclear. Not all children suffer deterioration,and, in a significant proportion, function is not normal at birth. Surgery, as it is currently practiced, is generally safe but does not confer long-term immunity from deterioration. The risk of deterioration, its pattern, and its timing are related in part to the morphology of the malformation. Patients with asymmetrical malformations may exhibit unilateral functional neurological or orthopedic abnormalities, which conspire with normal neurodevelopment to render these abnormalities apparent at an early age. Symmetrical malformations present later in childhood in association with bilateral and/or urinary dysfunction. The rate of functional deterioration in patients following surgery appears to be equal to or is slower than the rate of deterioration in patients who do not undergo surgery. Many patients will require more than one untethering procedure to address evolving functional impairment. Structural abnormalities require end organ-specific orthopedic or urological interventions. A formal structured multidisciplinary monitoring team is required to provide clinical and functional surveillance. Such monitoring is required following operative untethering and debulking for the life of the patient. The author reviewed current literature to define the timing and pattern of deterioration prior to and following initial cord untethering in patients with transitional LMMC, as well as the operative burden that these children bear in exchange for optimized function.