Early- and long-term surgical outcomes in 109 children with lipomyelomeningocele

Childs Nerv Syst. 2021 May;37(5):1623-1632. doi: 10.1007/s00381-020-05000-y. Epub 2021 Jan 6.

Abstract

Aim: To determine the functional outcomes and risk factors for outcomes following surgery for lipomyelomeningocele (LMMC).

Methods: Data from 109 children with LMMC who underwent surgery from January 2008 to December 2017 were retrospectively studied to evaluate functional outcomes and possible risk factors for early- (at discharge from hospital or within 1 month of surgery) and long-term outcomes after surgery.

Results: There were 53 boys and 56 girls with median age of 36 months (IQR 12-90 months; range, 4 months to 18 years) at surgery. At presentation, neurological function was normal (asymptomatic group) in 28 (25.7%) children while there was neurological dysfunction (symptomatic group) in 81 (74.3%). Near total or radical excision of lipoma was done in 71 (65.1%) children. Six (5.8%) children had deterioration of neurological function in the post-operative period with recovery of function in three of them. There were no statistically significant risk factors for early outcomes. At a mean follow-up of 62.5 months (IQR 35-82 months; range, 12-146 months), 21 (27.6%) of the 76 symptomatic group children (including 59.3% with incomplete bladder dysfunction) showed improvement, 52 (68.4%) remained the same, while 3 (4%) deteriorated. In 31 children (symptomatic group) who developed symptoms after 1 year of age, early surgery after development of symptoms was associated with better chances of recovery (p = 0.0008). In the asymptomatic group, 23 (88.5%) had normal neurological function at mean follow-up of 57.5 months (IQR 30-77 months; range, 12-141 months) and 3 (11.5%) had neurological deterioration. The mean time to late deterioration (re-tethering) from surgery was 51.3 months (IQR 24-75 months; range, 24-84 months). Presence of syrinx on initial MRI (p = 0.008) and partial resection of the lipoma (p = 0.02) were independent risk factors for delayed deterioration.

Conclusion: Radical resection of LMMC probably helps in preserving neurological function in > 90% of children at long-term follow-up. Fifty-five percent of children > 2 years of age with incomplete bladder dysfunction regained normal function following surgery. In children who develop symptoms after birth, early surgical intervention can reverse the neurological deficits.

Keywords: Lipomyelomeningocele; Spinal lipoma; Surgical outcome; Syrinx.

MeSH terms

  • Child
  • Female
  • Humans
  • Infant
  • Lipoma* / diagnostic imaging
  • Lipoma* / surgery
  • Male
  • Meningomyelocele* / complications
  • Meningomyelocele* / surgery
  • Retrospective Studies
  • Spinal Cord Neoplasms*
  • Treatment Outcome

Supplementary concepts

  • Lipomyelomeningocele