Aangeboren afwijkingen van de wervelkolom en het ruggemerg (dysraphisme)

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Spina bifida

Spina bifida is een ontwikkelingsstoornis van de wervelkolom en het ruggenmerg. Er zijn verschillende vormen die met verschillende neurologische stoornissen gepaard gaan.

Spina bifida occulta
De processus spinosus van een of meerdere wervels ontbreken. De bekleding van de huid is intact, maar een verkleuring van de huid, lokale haargroei, huidaanhangsels, vetkussentjes of een terugtrekking van de huid wijzen op de verborgen spinale misvormingen. Ook een lipoom (adipeuze verbinding tussen het ruggenmerg en het subcutane vet) of diastematomyelia (gespleten ruggenmerg) kunnen onderliggend aanwezig zijn. In sommige gevallen kan slechts een verdikt uiteinde van het koord van het ruggenmerg (filum terminale) de oorzaak zijn van tractie op het ruggenmerg.

Deze afwijkingen hinderen de verdere evolutie van het ruggenmerg, waardoor neurologische problemen ontstaan in de loop van de ontwikkeling van het kind.

Spina bifida aperta
De zogenoemde open rug is de meest voorkomende vorm van defect van de neurale buis en ontwikkelt zich tussen de 21e en de 28e dag van zwangerschap. Het wordt waarschijnlijk veroorzaakt door verschillende factoren, maar dankzij de toediening van foliumzuur vóór de conceptie en door een verbeterde prenatale diagnose, daalt het aantal nieuw gediagnosticeerde gevallen op het tijdstip van de geboorte.

De onvolledig gesloten neurale buis presenteert zich uiteindelijk hetzij bedekt met de huid (meningocele) of meestal als een massa met cerebrospinale vloeistof en de onontwikkelde neurale plaat aan de oppervlakte (myelomeningocele).

De neurologische afwijkingen zijn afhankelijk van de plaats en de uitbreiding van het defect en zijn onomkeerbaar. In de meeste gevallen gaat een spina bifida aperta gepaard met andere afwijkingen zoals een waterhoofd (hydrocefalie), Chiari misvorming, syringomyelie en tethered cord syndroom. Naast deze neurologische afwijkingen kunnen verdere defecten op orthopedische gebied voorkomen, zoals holvoeten, heup luxaties, scoliose en kyfose. Ook verstoorde blaasfunctie kan zich voordoen.
Voor deze kinderen is niet alleen chirurgie voor het defect (bij voorkeur binnen de eerste 48 uur na de geboorte) noodzakelijk maar zijn ook andere operaties nodig. Een nauwe samenwerking binnen een gespecialiseerd centrum is aan te raden om de best mogelijke ontwikkeling van het kind te garanderen.

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Spina bifida

Spina bifida means a developmental disorder of the spine and the spinal cord. There are different forms with accordingly various neurological deficits.

Spina bifida occulta

The processus spinosus of one or several vertebrae are missing. The covering skin is intact.

But discoloration of the skin, abnormal hair growth, skin appendices, fat pads, or a retraction of the skin draw attention on the hidden spinal deformities, which can be a bening tumour such as an epidermoid or a dermoid. Also lipoma (fatty connection between the spinal cord and the subcutaneous fat) or diastematomyelia (split spinal cord) may be the underlying pathology. In some cases only a fatty thickened end of the string of the spinal cord (filum terminale) is the cause of traction on the spinal cord.

Those abnormalities prevent the normal ascension of the cord, which will cause neurological problems in the course of the child’s development. An early presentation of the patient will help to

Spina bifida aperta

The so-called open spine/back, is the most common form of defects of the neural tube and develops between the 21st and 28th day of pregnancy. It is most probably caused by different factors. With the administration of folic acid prior to conception and through improved prenatal diagnosis, the number of newly diagnosed children at the time of birth is falling.

The incomplete closing of the neural tube presents eventually covered with skin (meningocele) or mostly appears like a mass containing cerebrospinal fluid and the undeveloped neural plate (myelomeningocele).

The neurological deficits are dependent on the location and extension of the defect and are irreversible. In most cases, spina bifida aperta occurs along with other defects such as hydrocephalus, Chiari malformation, syringomyelia, and tethered cord syndrome. Besides these neurological deformities, further defects in the orthopaedic field may occur, such as high arches, hip luxations, scoliosis and kyphosis. Furthermore, in the field of general surgery with inguinal hernia and in the urological sector with urinary bladder disorders.

For these children, not only surgery for covering the defect (preferably within the first 48 hours after birth), is necessary but numerous further surgeries, too. A close cooperation with and within a specialised medical center is highly recommended.

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Spina bifida

Spina bifida means a developmental disorder of the spine and the spinal cord. There are different forms with accordingly various neurological deficits.

Spina bifida occulta

The processus spinosus of one or several vertebrae are missing. The covering skin is intact.

But discoloration of the skin, abnormal hair growth, skin appendices, fat pads, or a retraction of the skin draw attention on the hidden spinal deformities, which can be a bening tumour such as an epidermoid or a dermoid. Also lipoma (fatty connection between the spinal cord and the subcutaneous fat) or diastematomyelia (split spinal cord) may be the underlying pathology. In some cases only a fatty thickened end of the string of the spinal cord (filum terminale) is the cause of traction on the spinal cord.

Those abnormalities prevent the normal ascension of the cord, which will cause neurological problems in the course of the child’s development. An early presentation of the patient will help to

Spina bifida aperta

The so-called open spine/back, is the most common form of defects of the neural tube and develops between the 21st and 28th day of pregnancy. It is most probably caused by different factors. With the administration of folic acid prior to conception and through improved prenatal diagnosis, the number of newly diagnosed children at the time of birth is falling.

The incomplete closing of the neural tube presents eventually covered with skin (meningocele) or mostly appears like a mass containing cerebrospinal fluid and the undeveloped neural plate (myelomeningocele).

The neurological deficits are dependent on the location and extension of the defect and are irreversible. In most cases, spina bifida aperta occurs along with other defects such as hydrocephalus, Chiari malformation, syringomyelia, and tethered cord syndrome. Besides these neurological deformities, further defects in the orthopaedic field may occur, such as high arches, hip luxations, scoliosis and kyphosis. Furthermore, in the field of general surgery with inguinal hernia and in the urological sector with urinary bladder disorders.

For these children, not only surgery for covering the defect (preferably within the first 48 hours after birth), is necessary but numerous further surgeries, too. A close cooperation with and within a specialised medical center is highly recommended.

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Spina bifida

Spina bifida means a developmental disorder of the spine and the spinal cord. There are different forms with accordingly various neurological deficits.

Spina bifida occulta

The processus spinosus of one or several vertebrae are missing. The covering skin is intact.

But discoloration of the skin, abnormal hair growth, skin appendices, fat pads, or a retraction of the skin draw attention on the hidden spinal deformities, which can be a bening tumour such as an epidermoid or a dermoid. Also lipoma (fatty connection between the spinal cord and the subcutaneous fat) or diastematomyelia (split spinal cord) may be the underlying pathology. In some cases only a fatty thickened end of the string of the spinal cord (filum terminale) is the cause of traction on the spinal cord.

Those abnormalities prevent the normal ascension of the cord, which will cause neurological problems in the course of the child’s development. An early presentation of the patient will help to

Spina bifida aperta

The so-called open spine/back, is the most common form of defects of the neural tube and develops between the 21st and 28th day of pregnancy. It is most probably caused by different factors. With the administration of folic acid prior to conception and through improved prenatal diagnosis, the number of newly diagnosed children at the time of birth is falling.

The incomplete closing of the neural tube presents eventually covered with skin (meningocele) or mostly appears like a mass containing cerebrospinal fluid and the undeveloped neural plate (myelomeningocele).

The neurological deficits are dependent on the location and extension of the defect and are irreversible. In most cases, spina bifida aperta occurs along with other defects such as hydrocephalus, Chiari malformation, syringomyelia, and tethered cord syndrome. Besides these neurological deformities, further defects in the orthopaedic field may occur, such as high arches, hip luxations, scoliosis and kyphosis. Furthermore, in the field of general surgery with inguinal hernia and in the urological sector with urinary bladder disorders.

For these children, not only surgery for covering the defect (preferably within the first 48 hours after birth), is necessary but numerous further surgeries, too. A close cooperation with and within a specialised medical center is highly recommended.

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