Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings

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  • The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia; and The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
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Object

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.

Abbreviations used in this paper:

DC = dermoid cyst; fMMC = fetal myelomeningocele; IC = inclusion cyst; LE = lower extremity; MOMS = Management of Myelomeningocele Study; NIH = National Institutes of Health; TCS = tethered cord syndrome.

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Contributor Notes

Address correspondence to: Mark P. Johnson, M.D., The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia 5th Wood Center, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. email: johnsonma@email.chop.edu.
  • 1

    Adzick NS, & Harrison MR: Fetal surgical therapy. Lancet 343:897902, 1994

  • 2

    Argenta LGS, , Marks M, & Proffer P: P52: Intraspinal epidermal cyst following intrauterine surgery as cause of difficult to heal myelomeningocele repair. Plastic Surgery 2003, The Premier Educational Experience San Diego, Abstract number 3628,

    • Search Google Scholar
    • Export Citation
  • 3

    Bannister CM: The case for and against intrauterine surgery for myelomeningoceles. Eur J Obstet Gynecol Reprod Biol 92:109113, 2000

  • 4

    Bowman RM, , McLone DG, , Grant JA, , Tomita T, & Ito JA: Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34:114120, 2001

  • 5

    Brezner A, & Kay B: Spinal cord ultrasonography in children with myelomeningocele. Dev Med Child Neurol 41:450 455, 1999

  • 6

    Bruner JP, , Tulipan N, , Paschall RL, , Boehm FH, , Walsh WF, & Silva SR, et al. : Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:18191825, 1999

    • Search Google Scholar
    • Export Citation
  • 7

    Chadduck WM, & Roloson GJ: Dermoid in the filum terminale of a newborn with myelomeningocele. Pediatr Neurosurg 19:8183, 1993

  • 8

    Chakrabortty S, , Oi S, , Yoshida Y, , Yamada H, , Yamaguchi M, & Tamaki N, et al. : Myelomeningocele and thick filum terminale with tethered cord appearing as a human tail. Case report. J Neurosurg 78:966969, 1993

    • Search Google Scholar
    • Export Citation
  • 9

    Danzer E, , Gerdes M, & Bebbington MW: Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther [in press] 2008

    • Search Google Scholar
    • Export Citation
  • 10

    Danzer E, , Johnson MP, , Bebbington M, , Simon EM, , Wilson Rd, & Bilaniuk LT, et al. : Fetal head biometry assessed by fetal magnetic resonance imaging following in utero myelomeningocele repair. Fetal Diagn Ther 22:16, 2007

    • Search Google Scholar
    • Export Citation
  • 11

    Danzer E, , Johnson MP, , Wilson RD, , Flake AW, , Hedrick HL, & Sutton LN, et al. : Fetal head biometry following in-utero repair of myelomeningocele. Ultrasound Obstet Gynecol 24:606611, 2004

    • Search Google Scholar
    • Export Citation
  • 12

    Danzer E, , Sydorak RM, , Harrison MR, & Albanese CT: Minimal access fetal surgery. Eur J Obstet Gynecol Reprod Biol 108:313, 2003

  • 13

    Guidetti B, & Gagliardi FM: Epidermoid and dermoid cysts. Clinical evaluation and late surgical results. J Neurosurg 47:1218, 1977

  • 14

    Haberl H, , Tallen G, , Michael T, , Hoffmann KT, , Benndorf G, & Brock M: Surgical aspects and outcome of delayed tethered cord release. Zentralbl Neurochir 65:161167, 2004

    • Search Google Scholar
    • Export Citation
  • 15

    Herman JM, , McLone DG, , Storrs BB, & Dauser RC: Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome. Pediatr Neurosurg 19:243249, 1993

    • Search Google Scholar
    • Export Citation
  • 16

    Hudgins RJ, & Gilreath CL: Tethered spinal cord following repair of myelomeningocele. Neurosurg Focus 16:2 E7, 2004

  • 17

    Iwasaki M, , Yoshida Y, , Shirane R, & Yoshimito T: [Spinal dermoid cyst secondary to myelomeningocele repair: a case report.]. No Shinkei Geka 28:155160, 2000. (Jpn)

    • Search Google Scholar
    • Export Citation
  • 18

    Johnson MP, , Sutton LN, , Rintoul N, , Crombleholme TM, , Flake TM, & Howell LJ, et al. : Fetal myelomeningocele repair: shortterm clinical outcomes. Am J Obstet Gynecol 189:482487, 2003

    • Search Google Scholar
    • Export Citation
  • 19

    Kirsch WM, & Hodges FJ 3rd: An intramedullary epidermal inclusion cyst of the thoracic cord associated with a previously repaired meningocele. Case report. J Neurosurg 24:1018 1020, 1966

    • Search Google Scholar
    • Export Citation
  • 20

    Koen JL, , McLendon RE, & George TM: Intradural spinal teratoma: evidence for a dysembryogenic origin. Report of four cases. J Neurosurg 89:844851, 1998

    • Search Google Scholar
    • Export Citation
  • 21

    Lee GY, , Paradiso G, , Tator CH, , Gentili F, , Massicotte EM, & Fehlings MG: Surgical management of tethered cord syndrome in adults: indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine 4:123131, 2006

    • Search Google Scholar
    • Export Citation
  • 22

    Lu GC, , Steinhauer J, , Ramsey PS, & Faye-Petersen O: Lethal pulmonary hypoplasia after in-utero myelomeningocele repair. Obstet Gynecol 98:698701, 2001

    • Search Google Scholar
    • Export Citation
  • 23

    Lunardi P, , Missori P, , Gagliardi FM, & Fortuna A: Long-term results of the surgical treatment of spinal dermoid and epidermoid tumors. Neurosurgery 25:860864, 1989

    • Search Google Scholar
    • Export Citation
  • 24

    Maher CO, , Goumnerova L, , Madsen JR, , Proctor M, & Scott RM: Outcome following multiple repeated spinal cord untethering operations. J Neurosurg 106:434438, 2007

    • Search Google Scholar
    • Export Citation
  • 25

    Martinez-Lage JF, , Masegosa J, , Sola J, & Poza M: Epidermoid cyst occurring within a lumbosacral myelomeningocele. Case report. J Neurosurg 59:10951097, 1983

    • Search Google Scholar
    • Export Citation
  • 26

    Martinez-Lage JF, , Poza M, & Sola J: Dermoid and epidermoid tumors in myelomeningocele patients. Pediatr Neurosurg 20:274, 1994

  • 27

    Mazzola CA, , Albright AL, , Sutton LN, , Tuite GF, , Hamilton RL, & Pollack IF: Dermoid inclusion cysts and early spinal cord tethering after fetal surgery for myelomeningocele. N Engl J Med 347:256259, 2002

    • Search Google Scholar
    • Export Citation
  • 28

    McLone DG: Continuing concepts in the management of spina bifida. Pediatr Neurosurg 18:254256, 1992

  • 29

    McLone DG: Technique for closure of myelomeningocele. Childs Brain 6:6573, 1980

  • 30

    Nelson MD Jr, , Bracchi M, , Naidich TP, & McLone DG: The natural history of repaired myelomeningocele. Radiographics 8:695706, 1988

  • 31

    Scott RM, , Wolpert SM, , Bartoshesky LE, , Zimbler S, & Klauber GT: Dermoid tumors occurring at the site of previous myelomeningocele repair. J Neurosurg 65:779783, 1986

    • Search Google Scholar
    • Export Citation
  • 32

    Shikata J, , Yamamuro T, , Mikawa Y, & Kotoura Y: Intraspinal epidermoid and dermoid cysts. Surgical results of seven cases. Arch Orthop Trauma Surg 107:105109, 1988

    • Search Google Scholar
    • Export Citation
  • 33

    Storrs BB: Are dermoid and epidermoid tumors preventable complications of myelomeningocele repair?. Pediatr Neurosurg 20:160162, 1994

    • Search Google Scholar
    • Export Citation
  • 34

    Sutton LN, , Adzick NS, , Bilaniuk LT, , Johnson MP, , Crombleholme TM, & Flake AW: Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282:18261831, 1999

    • Search Google Scholar
    • Export Citation
  • 35

    von Koch CS, , Quinones-Hinojosa A, , Gulati M, , Lyon R, , Peacock WJ, & Yingling CD: Clinical outcome in children undergoing tethered cord release utilizing intraoperative neurophysiological monitoring. Pediatr Neurosurg 37:8186, 2002

    • Search Google Scholar
    • Export Citation
  • 36

    Yamada S, , Won DJ, & Yamada SM: Pathophysiology of tethered cord syndrome: correlation with symptomatology. Neurosurg Focus 16:2 E6, 2004

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