Fetal Surgery for Spina Bifida

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Developed by The Center for Fetal Diagnosis and Treatment at The Children's Hospital of Philadelphia, this guide provides an overview of fetal fetal surgery for myelomeningocele (MMC), the most common and severe form of spina bifida, including confirmation of the diagnosis, patient criteria for fetal surgery, the surgical procedure, delivery and follow-up care.

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Fetal Surgery for Spina Bifida

  1. 1. Fetal Surgery for Myelomeningocele 1-800-IN UTERO (468-8376) • fetalsurgery.chop.edu
  2. 2. A Guide to Specialized Fetal MMC Surgical Repair Since December 2010, fetal surgery for myelomeningocele (MMC), the most common and severe form of spina bifida, has been offered as a standard of care at The Children’s Hospital of Philadelphia. This is one of the most exciting developments in the history of treatment for birth defects. An extremely complex procedure available only to qualified candidates, fetal surgery for MMC requires significant commitment on the part of mothers who choose to go forward with it and extensive surgical experience to perform successfully. Mothers who choose fetal surgery require comprehensive counseling on the condition and the risks involved in the procedure. This guide provides an overview of fetal MMC repair, including confirmation of the diagnosis, patient criteria for fetal surgery, the surgical procedure, delivery and follow-up care. We welcome the opportunity to discuss individual candidates for referral.
  3. 3. Types of Spina Bifida 2• MMC (Myelomeningocele) s• Myeloschisis 1• Lipomeningocele• MyelocystoceleOpen neural tube defectssuch as myelomeningoceleand myeloschisis aretreatable by fetal repair. Fetus with myelomeningoceleClosed neural tube defects 1. Part of the spinal cord and spinal nerves, usually encased in a sac, protrudesuch as lipomeningocele and through an opening in the back and are exposed to the amniotic fluid.myelocystocele are not 2. Arnold-Chiari II Malformation • The brain stem (hindbrain) descends, or herniates, into the spinal canal in the neck and blocks the circulation oftreatable by fetal repair. cerebrospinal fluid. This can cause a damaging buildup of fluid in the brain called hydrocephalus.
  4. 4. Facts About Myelomeningocele (MMC)• Most common and serious form of spina bifida Long-term• Primary failure of neural tube formation (closure) Consequences• Genetic and micronutrient causes • Hydrocephalus• 1 per 2,000 live births, or 1,500 babies in U.S. each year • Hindbrain herniation complications• 14% die before age 5; 2/3 due to hindbrain • Ventriculoperitoneal herniation complications shunting• 85% require shunts; 45% of shunts develop • Paralysis and cognitive complications within 1 year impairments • Orthopaedic malformations• Up to 4% recurrence risk in subsequent pregnancies (such as club foot) • Bladder and bowel incontinence • Sexual dysfunction • Social and emotional challenges • Lifelong quality-of-life issues
  5. 5. Motor Impairment Related to Level of Defect Cervical 1 2 3MMC can occur Areas below the level of 4 5at any level in the defect will be affected. 6 7the developing 8 This illustration shows a Thoracic 1spine, but occurs defect at the L 4-5 level. 2 3most often in 4 5the lumbosacral 6 7region. 8 9 10The higher the 11defect on the 12 Skinspine, the more Lumbar 1 Muscle Areas Affected Sac 2severe the Hip 3complications. 4 Knee Exposed nerves Ankle (dorsiflexors) 5 Great Toe Sac Sacral 1 Ankle (plantarflexors) 2 3 Bowel & Bladder 4 5
  6. 6. Steps in Making the Diagnosis 1. Maternal serum alpha-fetoprotein (MSAFP) test. Abnormally high AFP levels can suggest neural tube defect. 2. Level II ultrasound to confirm spinal defect, determine level of lesion, confirm features in the brain that indicate spina bifida and assess deformities such as talipes (club foot). 3. Amniocentesis to confirm presence of elevated amniotic fluid alpha-fetoprotein (AFAFP) levels and acetylcholinesterase (AChE), which indicates open (vs. closed) neural tube defect. Amniocentesis is required to be considered for fetal surgery.
  7. 7. Rationale for Fetal MMC Surgery Results of Management of Myelomeningocele Study (MOMS)*Spinal cord damage showing outcomes after prenatal repair vs. postnatal repair:is progressive duringgestation. Fetal MMC • Reduced need for ventricular shunting at 12 months of age (40% of prenatal surgery group, compared to 83% ofsurgery may prevent postnatal surgery group)further damage and • Reduced incidence or severity of neurologic effects, e.g.,reverse the hindbrain impaired motor and sensory function of legsherniation feature • Improved ambulation at 30 months of ageof the Arnold-Chiari II • Reversal of hindbrain herniation component of Arnold-Chiari IImalformation. malformation Before surgery After surgery*N. Scott Adzick, M.D. et al.,“A Randomized Trial ofPrenatal versus Postnatal Repair of Myelomeningocele,”N Engl J Med. 2011 Mar 17;364(11):993-1004.Epub 2011 Feb 9.
  8. 8. When is Fetal Surgery for MMC an Option?Partial list of inclusion criteria:• Singleton pregnancy• Maternal BMI < 35• Confirmed presence of elevated AFAFP and AChE• Confirmed normal genetic testing• Absence of associated fetal anomalies• Myelomeningocele at level T1 through S1• Arnold-Chiari II malformation There is an extensive list of (hindbrain herniation) inclusion and exclusion criteria• Gestational age at time of fetal surgery for fetal MMC repair at CHOP. ≤ 25 weeks, 6 days For a complete listing, visit• Maternal age ≥ 18 years fetalsurgery.chop.edu/spinabifida or call 1-800-IN UTERO (468-8376).
  9. 9. Evaluation at the Center for Fetal Diagnosis and Treatment Diagnostic testing:A comprehensivemultidisciplinary • High-resolution level II ultrasound to confirm location of MMCevaluation. and assess for other birth defects • Ultrafast fetal MRI to confirm presence of Arnold-Chiari II malformation and screen for evidence of other brain or spinal abnormalities and any other structural defects not related to MMC • Fetal echocardiogram to evaluate heart structure and function continued s Ultrasound Fetal MRINear Right:Ultrasound image showingmyelomeningocele sac containingneural elementsFar Right:Fetal MRI showing hindbrainherniation, myelomeningocele saccontaining neural elements and noextra-axial cerebrospinal fluid
  10. 10. Evaluation at the Center for Fetal Diagnosis and Treatment continued Patient counseling and education: • Review of spina bifida and associated medical problems, and prenatal and postnatal options with obstetrician and neurosurgeon • Physical exam of mother, review of medical history and clearance for surgery by anesthesiologist and obstetrician • Review of pre- and postnatal care and monitoring • Neonatology counseling about possible preterm birth • Review of medications necessary before, during and after prenatal surgery • Social work psychosocial evaluation to assess readiness for surgery, coping mechanisms and family support • Review of surgical procedure (if opting for fetal surgery) and its risks with fetal surgeon. Risks include uterine scarring, membrane separation, infection, bleeding, prematurity and fetal demise.
  11. 11. Fetal MMC Surgery • Mother and fetus receive general anesthesia • Fetal surgeon performs laparotomy and uterus is opened with uterine stapling device that pinches off all blood vessels and keeps membranes tacked up to muscle layer of uterus • Sterile intraoperative ultrasound is performed by maternal-fetal medicine specialist • Fetus’ back is rotated into view • Fetal cardiologist performs continuous fetal heart monitoring by echocardiography • Pediatric neurosurgeon resects MMC sac from exposed spinal cord (neural placode) and skin edges, returns cord to spinal canal, and closes surrounding tissue and skin over defect • Uterine and abdominal incisions are closedN. Scott Adzick, M.D. et al.,“A Randomized Trial of Prenatalversus Postnatal Repair ofMyelomeningocele,” N Engl J Med.2011 Mar 17;364(11):993-1004.Epub 2011 Feb 9.
  12. 12. From Fetal Closure to Delivery • Mother remains in hospital 3 –5 days if no complications • Modified bed rest for 3 – 4 weeks; restricted activity until delivery • Weekly ultrasound monitoring • Goal is planned c-section at 37 weeks • Postnatal care in Newborn Intensive Care Unit (NICU)
  13. 13. NICU StayUpon admission, • Head ultrasounds day of life 0 and prior to dischargemultidisciplinary • MRI of brain and spineconsult with: • Daily head circumference• Neurosurgery • Renal and bladder ultrasound day of life 2• Urology • Bladder scans with handheld ultrasound device every 4 hours for first 48 hours to estimate amount of urine in bladder• Orthopaedics and whether baby requires catheter• Physical Therapy • Clean intermittent catheterization if bladder volume is greater• Spina Bifida Clinic than 50 percent of expected volume • Video-urodynamics day of life 2 and then at 2 months of age – special catheter measures pressure when bladder is full; soft catheter in rectum measures abdominal pressure on bladder; uroflow chair measures urine flow rate and time needed to empty bladder • Urinary tract infection prophylaxis • Evaluation for shunt
  14. 14. Follow-upOur Center is committed • Patients receiving follow-up care through CHOP are seento the comprehensive in our Spina Bifida Clinic the nation’s first program to bring a multidisciplinary approach to long-term follow-up.long-term follow-up of The clinic includes experts from pediatrics, nursing,our patients, to provide neurosurgery, orthopaedics, urology, physical therapy,the best care for children social work and genetics.and their families, • Follow-up includes visits every four to six months untiland to continuously gain age 2, then annually, with urodynamic testing and renalnew knowledge so that bladder ultrasounds to ensure kidneys function properlywe can advance care for and bladder function is stable.future generations. • Depending upon location of lesion and outcome after surgery, follow-up may also include: Clean intermittent catheterization; bowel management; lower extremity bracing; physical therapy evaluation and guidance to outside physical therapists and early intervention; pressure sore management; referral to appropriate psychosocial and financial resources; consultation with other subspecialties including ophthalmology, nephrology, nutrition and feeding team, and plastic surgery.
  15. 15. CHOP Experience in Fetal MMC RepairSuspected prenatal diagnosis An unparalleled level ofof myelomeningocele (MMC) continuous experience:since September 1995: • Leaders in development and research of fetal MMC Patient referrals: Over 1,450 repair for over 30 years Patient evaluations: Nearly 700 • First fetal MMC repair performed in 1998 Fetal surgeries: Over 125 • Performed 58 fetal MMC repairs, meeting strictAbout 1 in every 3-4 patients evaluated inclusion criteria, beforeis a candidate for fetal repair. start of MOMS trial • More than 800 fetal surgeries (all diagnoses) for patients from all 50 states and more than 50 countriesCenter for Fetal Diagnosis and Treatment at CHOP, data on file as of 1/31/12
  16. 16. Key Resources The Children’s Hospital of Philadelphia • Center for Fetal Diagnosis and Treatment fetalsurgery.chop.edu/spinabifida 1-800-IN UTERO (468-8376) • Spina Bifida Clinic 215-590-2483 Spina Bifida Association of America www.sbaa.org Centers for Disease Control and Prevention www.cdc.gov/ncbddd/spinabifida Spina Bifida Connection http://spinabifidaconnection.com March of Dimes www.marchofdimes.com
  17. 17. Referrals • Appointments • Information1-800-IN UTERO (468-8376) • fetalsurgery.chop.edu © 2012 The Children’s Hospital of Philadelphia. All Rights Reserved. 5588/1M/02-12

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