In utero repair of myelomeningocele: Experimental pathophysiology, initial clinical experience, and outcomes | Congenital Defects | JAMA Surgery | JAMA Network
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1.
Meuli  MMeuli-simmen  CYingling  CD  et al.  Creation of myelomeningocele in utero: a model of functional damage from spinal cord exposure in fetal sheep.  J pediatr surg. 1995;301028- 1033PubMedGoogle ScholarCrossref
2.
Meuli  MMeuli-simmen  CHutchins  GM  et al.  In utero surgery rescues neurological functional at birth in sheep with spina bifida.  Nat med. 1995;1342- 347PubMedGoogle ScholarCrossref
3.
Paek  BFarmer  DWilkinson  C  et al.  Hindbrain herniation develops in surgically created myelomeningocele and is prevented by prenatal repair in fetal lambs.  Am j obstet gynecol. 2000;1831119- 1123PubMedGoogle ScholarCrossref
4.
Nicolaides  KHCampbell  SGabbe  SGGuidetti  R Ultrasound screening for spina bifida: cranial and cerebellar signs.  Lancet. 1986;272- 74PubMedGoogle ScholarCrossref
5.
Cambell  SAllan  LGriffien  D  et al.  The early diagnosis of fetal structural abnormalities. Lerski  RAMorley  P Ultrasound '82 proceedings of the third meeting of the world federation for ultrasound in medicine and biology. Oxford, England Pergamon Press1983;547- 563Google Scholar
6.
Nyberg  DAMack  LA The spine and neural tube defects. in: nyberg da, mahoney bs, pretorius dh, eds.  Diagnostic ultrasound of fetal anomalies. Chicago, Ill Yearbook Medical1990;146- 202Google Scholar
7.
Heffez  DSAryanpur  JHutchins  GMFreeman  JM The paralysis associated with myelomeningocele: clinical and experimental data implicating a preventable spinal cord injury.  Neurosurgery. 1990;26987- 992PubMedGoogle ScholarCrossref
8.
Mclone  DGKnepper  PA The cause of chiari ii malformation: a unified theory.  Pediatr neurosci. 1989;151- 12PubMedGoogle ScholarCrossref
9.
Villablanca  JRHovda  DA Developmental neuroplasticity in a model of cerebral hemispherectomy and stroke.  Neuroscience. 2000;95625- 637PubMedGoogle ScholarCrossref
10.
Adzick  NSSutton  LNCrombleholm  TMFlake  AW Successful fetal surgery for spina bifida [letter].  Lancet. 1998;3521675- 1676PubMedGoogle ScholarCrossref
11.
Tulipan  NBruner  JP Myelomeningocele repair in utero: a report of three cases.  Pediatr neurosurg. 1998;28177- 180PubMedGoogle ScholarCrossref
12.
Bruner  JPWalsh  WFTulipan  N Open fetal repair of myelomeningocele improves neurologic outcome in the neonate [abstract].  Am j obstet gynecol. 1999;1803150Google ScholarCrossref
13.
Sutton  LNAdzick  NSBilaniuk  LT  et al.  Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele.  JAma. 1999;2821826- 1831PubMedGoogle ScholarCrossref
14.
Bruner  JPTulipan  NPaschal  RL  et al.  Fetal surgery for myelomeningocele and the incidence of shunt dependent hydrocephalus.  JAma. 1999;2821819- 1825PubMedGoogle ScholarCrossref
15.
Simpson  JL Fetal surgery for myelomeningocele: promise, progress, and problems.  JAma. 1999;2821873- 1874PubMedGoogle ScholarCrossref
16.
Tulipan  NSutton  LNBrunner  JP  et al.  The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus.  Pediatr neurosurg. 2003;3827- 33PubMedGoogle ScholarCrossref
17.
Vanderwall  KJBruch  SWMeuli  M  et al.  Fetal endoscopic ("fetendo") tracheal clip.  J pediatr surg. 1996;311101- 1104PubMedGoogle ScholarCrossref
18.
Harrison  MRAdzick  NS Fetal surgical techniques.  Semin pediatr surg. 1993;2136- 142PubMedGoogle Scholar
19.
Bond  SJHarrison  MRSlotnick  RNAnderson  JFlake  AWAdzick  NS Cesarean delivery and hysterotomy using an absorbable stapling device.  Obstet gynecol. 1989;7425- 28PubMedGoogle Scholar
20.
Holmes  NMNguyen  HTHarrison  MRFarmer  DLBaskin  LS Fetal intervention for myelomeningocele: effect on postnatal bladder function.  J urol. 2001;1662383- 2386PubMedGoogle ScholarCrossref
Paper
August 2003

In utero repair of myelomeningocele: Experimental pathophysiology, initial clinical experience, and outcomes

Author Affiliations

From the Departments of Surgery (Drs Farmer, Lee, and Harrison) and Neurosurgery (Drs von Koch, Peacock, Danielpour, and Gupta) and the Fetal Treatment Center (Drs Farmer, van Koch, Lee, and Harrison), University of California, San Francisco.

Arch Surg. 2003;138(8):872-878. doi:10.1001/archsurg.138.8.872
Abstract

Hypothesis  Experimental work raises the possibility that in utero repair of myelomeningocele (mmc) may improve lower extremity, bladder, and bowel function, ameliorate the arnold-chiari malformation, and decrease the need for postnatal shunting.

Design  We previously developed fetal lamb models to create and reverse lower extremity damage and the arnold-chiari malformation in utero. we then applied our extensive experience with fetal surgery, including fetal endoscopic (fetoscopic) surgical manipulation, to develop techniques for mmc repair.

Setting  A tertiary referral center.

Patients  All patients treated between 1998 and 2002 for a prenatally diagnosed mmc.

Interventions  Either fetoscopic mmc repair, fetoscopic patch repair, or limited maternal hysterotomy and microsurgical 3-layered fetal mmc repair was performed.

Main outcome measures  Gestational age at delivery, survival, neurologic outcome, and need for ventricular shunting at 1 year.

Results  Complete fetoscopic repair was accomplished in 1 fetus. two other fetuses underwent partial fetoscopic procedures. the remaining 10 patients underwent limited maternal hysterotomy and microsurgical 3-layered fetal mmc repair. four of 13 patients died, and the mean gestational age at delivery of 11 fetuses born alive was 31 weeks. five of 9 required ventricular shunting by age 1 year. in 2 patients, lower extremity function improved by more than 2 vertebral levels compared with prenatal ultrasonography. five of 10 patients who lived longer than 3 weeks required postnatal wound revision within 7 days after birth.

Conclusions  Fetoscopic repair, although feasible, does not yet yield optimal surgical results. open surgical repair before 22 weeks' gestation is physiologically sound and technically feasible. one third of patients appear to be spared the need for a shunt at age 1 year, but improvement in distal neurologic function is less clear. additionally, fetal mortality is associated with this procedure. our results complement the data published by groups at children's hospital of philadelphia, in pennsylvania, and vanderbilt university, nashville, tenn. a national institutes of health–sponsored prospective randomized trial is now underway at these 3 centers to compare fetal repair with postnatal repair.

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