Surgical management of myelocystocele: a single-center experience with long-term functional outcomes.

Objective: Myelocystocele (MCC) is an uncommon form of skin-covered spinal dysraphism. The authors aimed to present long-term functional outcomes of patients treated for MCC with and without associated abnormalities of cloacal development (ACD).

Methods: All patients with MCC and at least one tethered cord release (TCR) treated at a single institution between 1982 and 2019 were retrospectively reviewed. Demographic, operative, and functional outcome data were analyzed.

Results: Of 51 children with MCC, 30 (58.8%) had MCC only and 21 (41.2%) had associated ACD (MCC/ACD). Thirty-two patients (62.7%) had undergone one TCR, while 19 patients (37.3%) had multiple TCRs. Urinary continence assessment was possible in 41 patients (80.4%), and bowel continence assessment was possible in 43 patients (84.3%) who were either older than 6 years or toilet trained. Although patients with MCC only were more likely to void volitionally (p = 0.0001), there was no difference in overall bladder continence based on the presence of ACD (p = 0.15) or the need for additional untethering procedures (p = 0.15). Those with MCC only were more likely to have overall bowel continence (p = 0.0001) and not require any management (p = 0.002), while those with MCC/ACD were more likely to have an ileostomy (p = 0.01). Of the 30 patients with MCC only, 29 (96.7%) were able to ambulate in the community. Of 21 patients with MCC/ACD, 14 (66.7%) were able to ambulate in the community, 5 (23.8%) were not ambulating, and 2 (9.5%) were therapeutic ambulators. A greater proportion of children in the MCC cohort were ambulating in the community (p = 0.01). There was no difference in community ambulation based on the number of TCRs (p > 0.99), but those with multiple TCRs were more likely to use braces (p = 0.01) and require lower-extremity orthopedic surgery (p = 0.01).

Conclusions: Patients born with an MCC, with or without an associated ACD, attained long-term favorable outcomes in bladder and bowel continence and ambulation.