Myelomeningocele: long-term neurosurgical treatment and follow-up in 202 patients

Object. In this paper the authors focus on the long-term management of myelomeningocele (MMC) and its associated conditions. Methods. During a 25-year period, 220 consecutive patients with MMC underwent surgical repair. There were 203 cases (92%) of enlarged ventricles, but only 171 cases (78%) of hydrocephalus. Seven infants (3%) presented with early brainstem dysfunction; two improved after ventricular drainage, whereas five required craniocervical decompression. Results. Overall, five neonates (2%) died; 215 patients (98%) were eventually discharged from the hospital and of these, 202 (94%) remained in the authors' outpatient program. During the follow-up (range 1-25 years, mean 9.3 years), 96 patients required shunt revision (63% of shunts); 16 patients (8%) experienced late brainstem dysfunction that was treated by shunt placement in eight and by craniocervical decompression in the other eight; 137 patients (68%) harbored hydrosyringomyelia, but only six required surgical treatment; 40 patients (20%) presented symptoms of tethered cord, but surgical detethering was indicated in just 22 patients (11%). There were five deaths (2%), and severe adjunctive neurological morbidity was reported in 18 patients (9%) (owing to various causes). "Social" results have been evaluated by extrapolating 38 patients older than 15 years of age whose initial lesions were below L-2: 37 (97%) of these patients were fully independent, had "social urinary continence," and attended normal schools. Conclusions. Currently, many patients with MMC reach adulthood and social continence; self-care may be expected in a large percentage of cases. Nevertheless, there are many associated neurological conditions that have to be faced, and a coordinated network of care remains necessary throughout the patient's life. Moreover, these patients often present with so many peculiarities that indications for treatment, choice of proper techniques, and results are not always clear and evident.