Abstract
Open spina bifida aperta (myelomeningocele [MMC]) is the most frequent nonlethal congenital birth defect of the central nervous system, seen in 3 to 4 per 10,000 live births in the United States every year. It is a midline vertebral defect that exposes the contents of the spinal column to the outside environment. The cause of spina bifida is not fully clear and may involve genetic, metabolic, and environmental influences. Many infants with MMC also have the Chiari II malformation due to ongoing loss of cerebral spinal fluid from the lesion and herniation of the brainstem through the foramen magnum, resulting in obstructive hydrocephalus. The widespread availability of prenatal sonographic diagnosis has facilitated the diagnosis of systemic genetic abnormalities, and in some patients, timely fetal surgery may reduce the need for postnatal ventriculoperitoneal shunt placement. These patients need multidisciplinary management to reduce the morbidity and optimize outcomes.
| Original language | English (US) |
|---|---|
| Title of host publication | Principles of Neonatology |
| Publisher | Elsevier |
| Pages | 462-469 |
| Number of pages | 8 |
| ISBN (Electronic) | 9780323694155 |
| DOIs | |
| State | Published - Jan 1 2023 |
Keywords
- acetylcholinesterase
- alpha fetoprotein
- ambulation
- amniotic fluid
- ankle dorsiflexion
- anticholinergics
- banana sign
- birth defect
- bladder augmentation
- bladder outlet obstruction
- brainstem herniation
- Chiari II
- chromosomal anomaly
- cognitive loss
- corpus callosum
- cortical dysplasia
- CSF
- cutaneous ectodermal structures
- decreased bladder capacity
- decreased compliance
- detrusor overactivity
- detrusor-sphincter dyssynergia
- dorsal dysraphism
- echocardiogram
- ectoderm
- embryogenesis
- endoderm
- endoscopic third ventriculostomy
- enemas
- Escherichia coli
- fetal surgery
- fixed hip flexion
- folate metabolism
- foramen magnum
- gastrulation
- genetic abnormalities
- global hypotonia
- glucose metabolism
- glucose transport
- group B Streptococcus
- horizontal closure
- inclusion dermoids
- inspiratory stridor
- intradetrusor botulinum toxin A
- knee extension
- lemon sign
- lower extremity contractures
- lumbodorsal fascia
- Management of Myelomeningocele Study
- mesenchymal structures
- mesoderm
- methylation cycle
- midbrain distortion
- midline dorsal neural structures
- midline vertebral defect
- myelocystoceles
- neural placode
- neural tube defects
- neurocognitive difficulties
- neurogenic bladder
- neurogenic bowel
- neuronal closure
- noncommunicating hydrocephalus
- notochord
- obstructive hydrocephalus
- orthopedic abnormalities
- oxidative stress
- pes cavus
- pouched fecal diversion
- primary neurulation
- retinoid metabolism
- scoliosis
- secondary neurulation
- sensorimotor function
- spina bifida aperta
- spinal cord tethering
- spinal fluid
- spino-medullary junction
- Staphylococcus
- tectal beaking
- tethering
- transulfuration
- ventricular shunt
- ventriculitis
- ventriculoperitoneal shunt
- vertical closure
- vitamin B
- vocal cord palsies
- vocal cord paralysis
- Z-plasty closure
ASJC Scopus subject areas
- General Medicine