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

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  • 1 Division of Pediatric Neurosurgery, Ann & Robert H. Lurie Children’s Hospital of Chicago;
  • | 2 Department of Neurological Surgery, Northwestern University Feinberg School of Medicine;
  • | 3 Division of Pediatric Urology, Ann & Robert H. Lurie Children’s Hospital of Chicago;
  • | 4 Department of Urology, Northwestern University Feinberg School of Medicine;
  • | 5 Division of Pediatric Orthopedics, Ann & Robert H. Lurie Children’s Hospital of Chicago; and
  • | 6 Department of Orthopedics, Northwestern University Feinberg School of Medicine, Chicago, Illinois
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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.

ABBREVIATIONS

ACD = abnormalities of cloacal development; CIC = clean intermittent catheterization; CMG = cystometrogram; MCC = myelocystocele; MMT = manual muscle testing; RBUS = renal/bladder ultrasound; TCR = tethered cord release; VATER = vertebral anomalies, anal atresia, tracheal-esophageal atresia, renal/radial anomalies.

Diagram from Behbahani et al. (pp 488–496).

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