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Malformations and Spina Bifida




Pediatric Neurosurgery is also specialised on the treatment of malformations of brain, spinal cord and their encasement. A large proportion of malformations are disturbances of brain and spinal cord development (so-called Spina Bifida) that require neurosurgical intervention.

Spina bifida is a term for a huge variety of different inborn (congenital)  malformations of the spinal cord and it’s surrounding structures. It is a malformation of the neural tube out of that the spinal cord and surrounding structures evolve. The missing closure of the spine can have a very variable extent. Closure of the neural tube and genesis of the malformation occurs on Day 22-28 of pregnancy, during a process called primary neurolation, when the primitve nervous system is given birth and a tube is formed.

Basically there are two types: Closed and Open Spina Bifida. Whereas the first is a mild variant and treatment is not necessary except for so-called Dermalsinus. the latter presents a open connection of neural structures to the surface. This bears the high risk of direct damage and infection, that can be fatal for the patients.
Depending on the serverity of the malformation and extent of spinal cord affection, children can be affected more or less severe and suffer from neurologic deficits like inability to walk, urinary incontinence, bowel incontinence or spinal deformities.
Associated conditions like brain malformations (Chiari malformation etc.) and especially untreated hydrocephalus can severely impair the prognosis of the intellectual development.
Most severe malformations harbour the risk of neurologic deficits and further deterioration over time due to associated conditions.  Myelomeningoceles and split-cord syndromes result in inborn neurologic impairment and a high risk for hydrocephalus that requires neurosurgical therapy to preserve the children from intellectual disabilities. Understanding of the nature of the malformation as well as it’s anatomy is crucial for successful surgical treatment.

Treatment of malformations like myelomeningoceles or encephaloceles as well as their associated abnormalities (e.g. Chiari malformation of hydrocephalus) are a multidisciplinary task delivered by the Fetal Diagnostic and Therapy Centers. Gynacologists, specialists for prenatal diagnostics, pediatric radiologists, neuropediatricians, pediatric surgeons, neonatologists as well as pediatric neurosurgeons work hand in hand for advice, treatment and long-term assistance to the small patients.

 

Website of Children’s Hospital of Philadelphia, USA

Precise knowledge of anatomy, neural structure and growth is the key for successful treatment of disturbances at these very vulnerable stages of child development.

Due to the cooperation with Pränatalzentrum Leipzig advice on malformations can given by experts on the field.  A cooperation with Children’s Hospital Philadelphia (CHOP, Prof. Flake) is planned to offer detailed information about innovative fetal surgery techniques in the future.

Interdisciplinary cooperation is offered for cases of complex malformations that require pediatric surgical treatment. Continued long-term support and treatment by neuropediatricians and pediatric neurosurgeons are essential for optimal long-term results and to achieve a maximum of quality of life for those children. Pediatric Neurosurgery offers highest competence for secondary surgery after fetal MMC Treatment in case of tethered cord Syndroms. Furthermore, surgical removal of intraspinal lipomas and lipomyelomeningoceles is performed with high safety at high numbers each year offering optimal results and meeting highest medical and scientific standards.

 

Request further information via  Email about Malformations & Spina-Bifida-Clinics in Germany and worldwide:

spinabifida@kinderneurochirurgie-leipzig.de


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