neurosurgical considerations in spina bifida debbie k. song, m.d. gillette children’s specialty...
TRANSCRIPT
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Neurosurgical Considerations in Spina Bifida
Debbie K. Song, M.D.
Gillette Children’s Specialty Healthcare
St. Paul, MN
Spina Bifida Association of Iowa
Education Day 2015
March 28, 2015
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Neurosurgical Issues in Spina Bifida
Spina bifida / myelomeningoceleClosure of myelomeningoceleHydrocephalusChiari II MalformationTethered Spinal CordSyringomyelia
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Spina Bifida
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Myelomeningocele Incidence: ~1-2/1000 live births Genetic risk
2-3% risk of having child with myelomenigocele after having a prior pregnancy affected by myelomeningocele
6-8% if 2 affected siblings Increased risk when close relatives have pregnancies affected by
myelomeningocele Risk factors
Prior pregnancy affected by myelomeningocele Maternal folic acid deficiency
0.4 mg/day if no history of neural tube defect 4 mg/day if prior pregnancy with neural tube defect
Pregestational maternal diabetes mellitus Certain antiseizure medications: valproic acid, carbemazepime
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Myelomeningocele closure
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Adzick NS et al, N Engl J Med. 2011 March 17; 364 (11):993-1004
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Hydrocephalus
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Hydrocephalus Affects 85% of people with myelomeningocele5-10% of babies with MMC have overt
hydrocephalus at birth If it occurs, hydrocephalus usually does so by 6
months of ageMay become clinically evident after closure of
back at birthShunt to treat hydrocephalus in myelomeningocele
Some work looking at endoscopic third ventriculostomy (ETV) as an alternative
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Catheter in ventricle through burr hole in skull Reservoir/valve (separate or in combination) Distal catheter draining into:
Peritoneal cavity Pleural cavity Right atrium Gallbladder, sagittal sinus, fallopian tube…
Antibiotic impregnated catheter Anti-siphon devices Programmable valves
Shunts – basic configuration
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Ventriculoperitoneal shunt for hydrocephalus
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Neurosurgery follow-up important Why was the shunt placed? What kind of valve (programmable? Setting?) History of shunt surgeries – malfunctions, infections? What does child look like during a shunt malfunction? Copies of scans on file for local ER/future comparison Do ventricles get larger when shunt malfunctions?
Managing a child with hydrocephalus
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Chiari II malformation
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Chiari II malformation Present in 75-90% of patients with
myelomeningocele Constellation of anomalies:
Hindbrain herniation (downward displacement of parts of the medulla, cerebellum, 4th ventricle into spinal canal)
Brainstem abnormalities Low-lying venous sinuses Small posterior fossa
Chiari II related to hydrocephalus development Intrauterine repair of myelomeningocele
reduces incidence of Chiari II (MOMS trial)
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Chiari II malformation Leading cause of mortality in infants with myelomeningocele 15-30% of patients symptomatic, requiring surgery Presentation in infants:
Apnea, swallowing problems, stridor, lower cranial neuropathies High-pitched, hoarse, weak cry Vocal cord palsy, hypotonia Recurrent aspiration pneumonia, nasal regurgitation
Presentation in older patients: Headache, neck pain Balance/coordination problems Increased upper extremity spasticity , weakness
Make sure shunt is working!
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Tethered Spinal Cord
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Tethered Spinal Cord
Everyone with repaired myelomeningocele will have radiographic tethered cord
~1/3 of patients with spina bifida will require tethered cord release surgery
Assess for symptoms: neurologic, orthopedic, urologic
Clinical symptoms often driven by growth spurt
Make sure shunt is working!
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Syringomyelia
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Syringomyelia CSF within the spinal cord Present in 40-80% of patients with myelomeningocele Symptomatic in 2-5% of patients; assess for clinical change! Presentation overlaps with tethered cord and Chiari II
symptoms: Upper extremity weakness, loss of function Hand deformity neck or back pain Worsening scoliosis Spasticity Ascending motor loss in the legs
Treatment options: syrinx shunt, Chiari decompression, tethered cord release
Make sure shunt is working!
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Final Thoughts
Shunt care is important Clinical deterioration warrants investigation…starting
with shunt function Overall clinical picture is more important than imaging Be proactive and accountable for you/your child’s
healthcare