Spina bifida – colloquially called “open back” – (thesaurus synonyms: Congenital cranial deformity with hydrocephalus and with spina bifida; Anomaly of the aquaeductus cerebri with spina bifida; Anomaly of the Sylvian aqueduct with spina bifida; Atresia of the apertura lateralis ventriculi quarti with spina bifida; Atresia of the apertura mediana ventriculi quarti with spina bifida; Atresia of the aquaeductus cerebri with spina bifida; Atresia of the foramen luschkae with spina bifida; Atresia of the foramen magendii with spina bifida; Atresia of the aqueduct of Sylvius with spina bifida; Dorsal spina bifida; Dorsal spina bifida with hydrocephalus; Dysrhaphy syndrome; Dysrhaphy disorder; Absence of cranial bone with hydrocephalus and with spina bifida; Split spine; Hydranencephaly with spina bifida; Hydrancephaly with spina bifida; Hydrocele spinalis; Hydrocele spinalis with hydrocephalus; Hydrocephalus congenitus with dorsal spina bifida; Hydrocephalus congenitus with lumbar spina bifida; Hydrocephalus congenitus with lumbosacral spina bifida; Hydrocephalus congenitus with sacral spina bifida; Hydrocephalus congenitus with spina bifida; Hydrocephalus congenitus with thoracic spina bifida; Hydrocephalus congenitus with thoracolumbar spina bifida; Hydrocephalus congenitus with cervical spina bifida; Hydromeningocele; Hydromeningocele with hydrocephalus; Hydromyelocele; Hydromyelocele with hydrocephalus; Hydrocephalus with spina bifida in the newborn; Congenital obstruction of the foramen gastendii with spina bifida; Congenital obstruction of aquaeductus cerebri with spina bifida; Congenital deficient spinal closure with hydrocephalus; Congenital obstruction of aquaeductus cerebri with spina bifida; Congenital spinal fissure; Lumbar spina bifida with hydrocephalus; Lumbar spina bifida without hydrocephalus; Lumbosacral spina bifida; Lumbosacral spina bifida with hydrocephalus; Deficient spinal closure; Meningomyelocele; Meningomyelocele with hydrocephalus; Meningocele; Meningocele with hydrocephalus; Meningomyelocele; Myelomeningocele; Myelomeningocele of the spinal cord; Myelomeningocele with hydrocephalus; Myelocele; Myelocele with hydrocephalus; Myelocystocele; Myelocystocele with hydrocephalus; Neural tube defect; Obstruction of foramen interventriculare Monroi with spina bifida; Open spine; Rachischisis; Rachischisis with hydrocephalus; Rhachischisis; Spinal cord hernia; Spinal cord hernia with hydrocephalus; Sacral spina bifida with hydrocephalus; Sacral spina bifida without hydrocephalus; Cranial anomaly with hydrocephalus and with spina bifida; Cranial bone agenesis with hydrocephalus and with spina bifida; Cranial bone anomaly with hydrocephalus and with spina bifida; Septum of aqueductus cerebri with spina bifida; septum of Sylvian aqueduct with spina bifida; spina bifida; spina bifida aperta; spina bifida cystica; spina bifida with hydrocephalus a. n.k. ; Spinal hydromeningocele; Spinal meningocele; Stenosis of aqueductus cerebri with spina bifida; Syringomyelocele; Syringomyelocele with hydrocephalus; Thoracic spina bifida with hydrocephalus; Thoracic spina bifida without hydrocephalus; Thoracolumbar spina bifida; Thoracolumbar spina bifida with hydrocephalus; Spinal fissure with hydrocephalus; Vertebral fissure; Cervical spina bifida with hydrocephalus; Cervical spina bifida without hydrocephalus; ICD-10 Q05. -: Congenital malformations of the nervous system) refers to an embryonic closure disorder in the area of the spinal column (malformation of the neural tube; neural tube defect) toward the cranial (“upward” or “toward the head“) or caudal (“downward”) of varying severity. “Spina” translates to “spine” or “thorn” and refers to the spinous process of the vertebral body. “Bifidus” means “split in two” or, in reference to the clinical picture, “vertebral fissure.” Spina bifida develops between the 19th and 28th day of embryonic development. During this period, called “primary neurulation”, the neural tube is formed from the neural plate and closed. The following manifestations of spina bifida (SB) are distinguished:
- Spina bifida totalis (rachischisis) – very rare form; most severe degree of cleft formation; the neural tissue lies bare, a skin covering is missing
- Spina bifida partialis
- Spina bifida occulta (SBO; “occulta” = hidden, not visible).
- Bipartite vertebral arch, in which the spinal cord with its spinal meninges is not involved (without an opening)
- This form is usually diagnosed only by chance during X-rays or an examination of the back – it is not visible from the outside
- in bedwetting children this form of the disease is often found
- Spina bifida aperta (SBA; “aperta” = open, visible).
- In addition to incomplete closure of the posterior vertebral arches, the spinal meninges and/or spinal cord are involved in the cleft formation:
- Myelocele – exposed spinal cord (rare).
- Spina bifida cystica
- Meningocele – spinal cord membranes bulge out of the spinal canal through the vertebral arch space under the skin; spinal cord and spinal nerves are in place; intact outer skin; no neurologic deficits
- Myelomeningocele (MMC) – spinal cord membranes and spinal cord are located outside the vertebral arch and are visible as a protrusion under the skin (Zele); paraplegia of varying severity, neurological malformations; hydrocephalus (hydrocephalus; pathological enlargement of the fluid-filled fluid spaces (cerebral ventricles) of the brain) is found in an average of 72% of patients depending on the lesion level (MMC affects approximately 10% of all spina bifida cases)
- In addition to incomplete closure of the posterior vertebral arches, the spinal meninges and/or spinal cord are involved in the cleft formation:
- Spina bifida occulta (SBO; “occulta” = hidden, not visible).
After heart defects, neural tube defects are among the second most common congenital (congenital) malformations. Localization: most often spina bifida occurs in the lumbar region (lumbar region) and in the region of the os sacrum (sacrum) (in about 80% of cases). Sex ratio: girls are affected slightly more often than boys. The prevalence of clinically manifest spina bifida aperta is 0.1% (Germany; Ireland: 0.7%) and up to 15% for spina bifida occulta. The incidence (frequency of new cases) in Central Europe is between 1-3 per 1,000 births, but increases significantly if there is a child with spina bifida in the family. Course and prognosis: The effects of spina bifida depend on the severity of the malformation. People suffering from spina bifida are either hardly or very severely physically impaired. The localization of the malformation (at what level of the spinal column?) and whether parts of the spinal cord and nerves have protruded through the vertebral fissure in the form of a sac are also decisive for the extent. Children suffering from spina bifida aperta usually require lifelong medical care. Nevertheless, life expectancy can be high. The intelligence of those affected is usually not impaired (if, in addition, there are no brain malformations). Spina bifida occulta has no particular medical significance, so therapy is not required (except for neurologic deficits).