title: Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings.
abstract: OBJECTIVE
  The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure.
  
  
  METHODS
  Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up.
  
  
  RESULTS
  Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4-93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12-63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal.
  
  
  CONCLUSIONS
  Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.
authors.affiliation: The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia; and The University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
authors.forename: Enrico
authors.initials: E
authors.lastname: Danzer
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
authors.forename: Natalie E
authors.initials: NE
authors.lastname: Rintoul
authors.forename: Deborah M
authors.initials: DM
authors.lastname: Zarnow
authors.forename: Erin S
authors.initials: ES
authors.lastname: Schwartz
authors.forename: Jeanne
authors.initials: J
authors.lastname: Melchionni
authors.forename: Linda M
authors.initials: LM
authors.lastname: Ernst
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
authors.forename: Leslie N
authors.initials: LN
authors.lastname: Sutton
authors.forename: Mark P
authors.initials: MP
authors.lastname: Johnson
mesh_terms: D002648:Child; D002675:Child, Preschool; D003884:Dermoid Cyst / Q000453:epidemiology* / Q000473:pathology; D005260:Female; D005315:Fetal Diseases / Q000601:surgery*; D005333:Fetus / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008159:Lumbar Vertebrae; D008297:Male; D008591:Meningomyelocele / Q000601:surgery*; D011183:Postoperative Complications; D012189:Retrospective Studies; D013120:Spinal Cord Neoplasms / Q000453:epidemiology* / Q000473:pathology; D013904:Thoracic Vertebrae; D016896:Treatment Outcome