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Spina bifida is a congenital malformation of the spinal cord that can take various forms. On this page you will find information on the most common forms - lipomyelomeningocele, myelomeningocele and filum terminale lipoma (FTL) - as well as on modern diagnostic and therapeutic options. You will also find an overview of prognoses and interdisciplinary treatment approaches.
Lipomyelomeningocele is a form of closed malformation of the spinal canal (so-called occult spina bifida or closed dysraphia). In comparison to closed dysraphia, in which the skin over the malformation is closed, open dysraphia (also known as spina bifida aperta or myelomeningocele) is when the skin on the back is open and the spinal cord and nerves are exposed.
In the case of a lipomyelomeningocele, the vertebral bone and the meninges do not close during embryonic development (during pregnancy) and a fatty growth (so-called lipoma) of the subcutis leads to adhesions and adhesions of the spinal cord to the nerves and the surrounding area. This leads to traction on the spinal cord (so-called tethered cord), which can lead to neurological deficits.
Lipomyelomeningocele is the most common form of occult dysraphism. Lipomas in the spinal cord, such as lipomyelomeningocele, occur in approximately 1 in 4000 live births, with lipomyelomeningocele accounting for approximately 80 % of cases.
The cause of lipomyelomeningocele is unknown. Risk factors associated with lipomyelomeningocele include poorly controlled gestational diabetes, maternal obesity and certain medications (especially anti-epileptic drugs).
Typical signs of lipomyelomeningocele are a soft, painless bump on the back. Other changes to the skin that can occur in connection with a lipomyelomeningocele include an asymmetry of the buttocks (so-called «gluteal fold deviation»), a reddish discolouration of the skin (so-called «haemangioma»), increased hair growth in the area of the bump or an indentation in the area of the coccyx (so-called «non-simple dimple»).
As a rule, babies do not yet show any neurological symptoms. However, neurological symptoms can occur with age, including loss of bladder and bowel function (incontinence and/or constipation), loss of strength and sensation, loss or increase in reflexes and problems with the legs (open skin, club feet, hip dislocations, etc.).
Older children may also experience pain more frequently. This is limited to the lower back and increases with all movements that stretch the back (and therefore the spinal cord).
A lipomyelomeningocele is usually recognised at birth by the skin signs described above. The suspicion is often confirmed by ultrasound. A magnetic resonance imaging (MRI) scan of the back should then be carried out in order to determine the exact extent of the lipomyelomeningocele and plan the treatment.
Surgical removal of the lipoma and decompression of the nerves and spinal cord is the treatment of choice to prevent or limit neurological deficits. This operation is performed prophylactically, ideally at the age of six to nine months, i.e. before it is known whether neurological symptoms are present.
The aim of the operation is to relieve the spinal cord as early as possible so that no neurological symptoms develop or, if symptoms are already present, so that they do not progress. The child's back is opened and the lipoma in the spinal cord is carefully removed. To do this, one or two additional vertebral bodies are usually opened in addition to those already open due to the disease. The spinal cord is relieved (so-called «untethering») so that there is no longer any tension on the spinal cord. The spinal cord is then closed again and the overlying meninges, muscles and skin are sutured shut.
The operation is performed with so-called neuromonitoring, which means that the activity of the nerves is recorded so that the surgeon knows where he is and which structures need to be protected.
Complications rarely occur during these operations. These can include a hole in the meninges through which cerebrospinal fluid leaks, wound infections and, in very rare cases, new neurological deficits (1%). In rare cases, so-called «re-tethering» can occur after the operation, i.e. renewed adhesion of the spinal cord with a recurrence (relapse or renewed growth) of the lipoma or with the scar tissue. This in turn can be associated with neurological deficits such as loss of bladder function, malpositioning of the back or extremities and pain.
A further operation (re-untethering) is only indicated if a neurological deterioration can be objectified.
During the entire hospital stay, your child will be looked after by our paediatric neurosurgery team at UKBB. Your child will arrive one day before the operation, will be examined again by our team and the anaesthetists and can then sleep at home if you wish. The next day, your child will be operated on and then transferred to the intensive care unit for monitoring.
On the day of the operation, one of our parent carers from the parent support service (BELOP) at UKBB will be at your disposal. They will accompany you until your child has fallen asleep and show you where your child will wake up. In the meantime, they will help you to find your way around the hospital. As soon as your child no longer needs close monitoring (usually the following day), he or she will be transferred to the paediatric surgery ward.
In order to minimise the risk of cerebrospinal fluid leaks, your child will lie as flat as possible for the first 48 hours after the operation, after which they can be fully mobilised again immediately. As a rule, your child will recover from the operation after five to seven days and will also be allowed to go home during this period. You may be with your child during the entire time and contact the paediatric neurosurgery team with any questions, who will visit you and your child every day.
The prognosis for children with a lipomyelomeningocele is generally very good, but is related to the symptoms and limitations that occur (spinal deformities, bladder dysfunction). The children have normal intelligence and often a very good quality of life. Despite treatment, some children show bladder and/or rectal dysfunction, back pain, scoliosis and other neurological deficits.
The lipoma is a low-grade lesion and is not life-threatening. Children who have undergone surgery are cared for and treated by several specialists in paediatric neurosurgery, paediatric neurology, paediatric urology and paediatric neuro-orthopaedics in our interdisciplinary consultation at the UKBB every year for years.
Stricker S, Eberhardt N, Licci M, Greuter L, Raphael G, Soleman J. Wound closure with a mesh and liquid tissue adhesive (Dermabond Prineo) system in paediatric spine surgery: a prospective single-centre cohort study incorporating parent-reported outcome measures. J Neurosurg Pediatr. Sept 2022. doi.org/10.3171/2022.8.PEDS22270
Neural tube defects that lead to a so-called open spine («spina bifida») are among the most common congenital diseases. Myelomeningocele is the most common form of open spine and describes an open cleft in the spinal column with an accompanying skin defect above it, through which the meninges and spinal cord can be seen or come out.
This is due to a lack of/restricted closure of the nerve plate during neurulation, the formation of our nervous system including the brain and spinal cord, during early development. This in turn leads to the defect in the spinal column and spinal cord, which is then visible as an open back.
Myelomeningocele occurs between 1-7 per 1000 births and can occur due to a variety of factors (individually or in combination). One of the best known risk factors for an open spine is a folic acid deficiency; genetic factors (whereby a familial clustering is recognisable, approx. 2% recurrence rate), female gender, poorly controlled gestational diabetes, maternal obesity, certain medications (especially antiepileptic drugs), exposure to pesticides or various syndromes (trisomy 13 & 18, Meckel-Gruber syndrome, Roberts syndrome, Jarcho-Levin syndrome, HARD syndrome) can also lead to myelomeningocele.
Nowadays, an open back is usually diagnosed during pregnancy as part of a check-up. This is detected as part of the standard screening examination using ultrasound. In addition, the folic acid level is measured and, if there is an increased suspicion, the alpha-feto protein (AFP) in the mother's blood is analysed. This combination enables a relatively reliable detection of a myelomeningocele. It is rarely discovered at birth.
Children with a myelomeningocele show an open back at birth, in which the two nerve plates and often a sac, which emerges from the back and contains the meninges and cerebrospinal fluid, can be seen. This defect occurs mainly in the lower parts of the spinal column, but can theoretically occur at any level of the spinal column.
Exposure of the spinal cord can lead to neurological deficits in the musculature and sensation as well as autonomic functions such as loss of bladder and rectal control. Finally, in around 50-60% of cases, myelomeningocele can lead to a Hydrocephalus (hydrocephalus) and the associated problems. In addition, practically all children with a myelomeningocele show a so-called Chiari II malformation, a low position of the cerebellar tonsils (so-called tonsils), which, however, rarely requires treatment.
An open back is usually detected during pregnancy check-ups. This is detected as part of the standard screening examination using ultrasound. In addition, the folic acid level is measured and, if there is an increased suspicion, the alpha-feto protein (AFP) in the mother's blood is analysed. This combination enables a relatively reliable detection of a suspected myelomeningocele.
In the case of an abnormal pregnancy screening, further genetic examinations (e.g. the search for other anomalies etc.) and an MRI are often carried out.
In the rare case that an open back is not discovered during the prenatal check-up, the characteristic signs described above will appear at birth, enabling the diagnosis to be made.
All children born with an open spine must be operated on within 24-48 hours. A meningomyelocele is usually diagnosed during pregnancy and a team of consultants from various specialist disciplines can discuss the options with you in detail.
In principle, there is also the option of performing an operation in the womb (so-called foetal surgery). This is an operation in which the uterus is opened and then operated on in the womb itself. This results in a slight improvement in development and a lower rate of hydrocephalus, but only shows a slight improvement in quality of life. In addition, there are the maternal risks of the operation, which include premature birth, placental abruption and inflammation of the egg membrane (chorioamnionitis).
At the UKBB, we use the operation on live-born children. A neurological examination is carried out immediately after birth to determine the extent of the child's neurological limitations. The open back is then surgically closed within the first 24-48 hours of life. In addition, the children receive antibiotic therapy to prevent an infection in the central nervous system.
The child is placed on its stomach under anaesthetic and the back is carefully opened. The respective layers of the musculature, the spinal cord, the dura (meninges) and the skin of the back are reconstructed and joined together. The back is then closed again. If hydrocephalus is present, this will also be treated; further information on this can be found at on our website under «Hydrocephalus».
During the entire hospital stay, your child will be cared for by our paediatric neurosurgery team at UKBB. After birth, your child will be monitored in the neonatal intensive care unit, treated with antibiotics against possible infections and then usually undergo neurosurgical treatment within a day.
On the day of the operation, one of our parent carers from the Parent Support Service (BELOP) at UKBB will be at your disposal. They accompany the parents until the child has fallen asleep and show the parents where the child will wake up. In between, they will help you to find your way around the hospital.
After the operation, your child will return to the neonatal intensive care unit for monitoring. As soon as your child no longer requires close monitoring, it will be transferred to the mother and child ward. As soon as your child has recovered from the operation, he or she will be allowed to go home with you. You may be with your child during the entire time and contact the paediatric neurosurgery team with any questions.
Children with a myelomeningocele have a good life expectancy, but their mental and physical development is strongly dependent on the level of the myelomeningocele and the accompanying symptoms (e.g. hydrocephalus, spinal deformities, bladder dysfunction).
Most children have normal intelligence, but can show learning disabilities. Some children can walk, others are wheelchair-bound. Bladder and rectal dysfunction is present in up to 60% of affected children, although certain children with myelomeningocele may also show normal function. Approximately 10-25% of children with myelomeningocele develop epileptic seizures over time. This may also occur in association with hydrocephalus associated with myelomeningocele.
Unfortunately, it is very difficult to assess the severity of the neurological and cognitive impairments before birth. Back problems in the sense of scoliosis (crooked back) or tethered cord (adhesion of the spinal cord) can also occur and could lead to further operations in the sense of a scoliosis operation (straightening operation) or an untethering operation (detachment of the spinal cord), after which the symptoms usually improve quickly.
All children with treated myelomeningocele are regularly followed up over the years in our interdisciplinary myelomeningocele consultation at the UKBB. During this consultation, your child will be seen by our paediatric neurosurgery team, paediatric neurology specialists and, if necessary, paediatric urology, paediatric orthopaedics, paediatric nephrology and our social services.
Licci M, Guzman R, Soleman J. Maternal and obstetric complications in foetal surgery for prenatal myelomeningocele repair: a systematic review. Neurosurg Focus. 2019;47(4):E11. doi:10.3171/2019.7.FOCUS19470
Stricker S, Balmer C, Guzman R, Soleman J. Dizygotic opposite-sex twins with surgically repaired concordant myelomeningocele conceived by in vitro fertilisation using intracytoplasmic sperm injection: a case report and review of the literature. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2019;35(4):725-728. doi:10.1007/s00381-018-3990-8
Licci M, Zaed I, Beuriat PA, Szathmari A, Guibaud L, Mottolese C, Di Rocco F. CSF shunting in myelomeningocele-related hydrocephalus and the role of prenatal imaging. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2021;37(11):3417-3428. doi:10.1007/s00381-021-05217-5
Mitra SS, Feroze AH, Gholamin S, Richard C, Esparza R, Zhang M, Azad TD, Alrfaei B, Kahn SA, Hutter G, Guzman R, Creasey GH, Plant GW, Weissman IL, Edwards MS, Cheshier S. Neural Placode Tissue Derived From Myelomeningocele Repair Serves as a Viable Source of Oligodendrocyte Progenitor Cells. Neurosurgery. 2015;77(5):794-802; discussion 802. doi:10.1227/NEU.0000000000000918
Stricker S, Eberhardt N, Licci M, Greuter L, Raphael G, Soleman J. Wound closure with a mesh and liquid tissue adhesive (Dermabond Prineo) system in paediatric spine surgery: a prospective single-centre cohort study incorporating parent-reported outcome measures. J Neurosurg Pediatr. Sept 2022. doi.org/10.3171/2022.8.PEDS22270
The filum terminale is a band of connective tissue that connects the end of the spinal cord (known as the conus) with the meningeal sac containing the spinal cord and the coccyx, thus forming a tension belt for the spinal cord. In an FTL, this ligament becomes «fatty» and its elasticity is restricted, which can lead to the spinal cord being pulled down more strongly (so-called tethered cord syndrome), which in turn can lead to neurological problems. Alongside lipomyelomeningocele, FTL is one of the most common forms of occult spinal dysraphism (a form of open spine). Both forms occur in about 1 in 4000 live births, with lipomyelomeningocele being the most common form of lipoma (about 80 %) and FTL accounting for about 10 % of cases. Girls are more frequently affected than boys. As with the open back, the cause is an error in the development of the nervous system, in which embryonic cells have probably not functioned properly, resulting in a defect in the spinal cord through which fat cells can migrate and spread. However, the exact cause is still unclear. FTL can occur as part of rare syndromes such as VACTERL and Currarino syndrome.
One often becomes aware of an FTL through typical skin signs such as a hairy area on the back (so-called «hypertrichosis»), a reddish discolouration (so-called «haemangioma»), a small indentation in the skin on the back (so-called «non-simple dimple») or a deviation of the fold in the area of the buttocks («gluteal fold deviation»). Rarely are FTLs found incidentally during imaging of the back. Most FTLs detected early (before the age of six months) are asymptomatic, but affected children can develop symptoms with age in approximately 30% of cases, such as loss of bladder function (approximately half of patients are affected) and bowel function (incontinence and/or constipation), loss of strength and sensation (approximately 15%), loss or increase in reflexes and problems with the legs (open skin, club feet, hip dislocations, etc., approximately 30%). Older children may also have more frequent pain, which is limited to the lower back and increases with any movement that stretches the back (and therefore the spinal cord).
FTL is often noticed by chance, among other things by the skin characteristics mentioned above, as it usually causes no symptoms. An ultrasound (only possible in infants) is the primary examination method, but often cannot completely rule out FTL, which is why a magnetic resonance imaging (MRI) scan of the back is performed to confirm the diagnosis. The MRI is also used to determine the indication for surgery, with a so-called low-lying conus (spinal cord end that is too low, below the third vertebral bone) indicating traction on the spinal cord (tethered cord syndrome), which usually leads to the recommendation of surgery.
If FTL has been diagnosed, surgery should be performed to prevent future symptoms. During the operation, the child's back is opened. At UKBB, we opt for a minimally invasive procedure in which the bony vertebral structures are spared as much as possible and practically only muscles and ligaments are opened. The skin incision for this is around 3 cm long. After opening the muscles, bones and ligaments, the meninges are opened and the FTL is accessed. The operation is performed with so-called neuromonitoring, which means that the activity of the nerves is recorded and the surgeon therefore knows where he is and which structures need to be protected. The FTL is stimulated with electricity and as there is no neurological activity in the area of the filum, the measurement should be negative and the filum can be cut. The operation takes about three to four hours. Complications rarely occur during these operations. Among other things, there may be a leak through which cerebrospinal fluid escapes, wound infections and, in very rare cases, new neurological deficits (in 0-1% of cases). In very rare cases, a so-called «re-tethered cord» can occur after the operation, a relapse due to adhesion of the filum to the surgical scar. This leads to renewed stretching of the spinal cord and can be associated with loss of bladder function, misalignment of the back and pain. The re-tethering is detached from the scar by another operation, after which the symptoms usually improve quickly.
Your child will be cared for by our paediatric neurosurgery team at UKBB throughout the entire hospital stay. Your child will arrive one day before the operation, will be examined again by our team and the anaesthetists and can then sleep at home if you wish. The next day, your child will be operated on and then transferred to the intensive care unit for monitoring. On the day of the operation, one of our parent carers from the Parent Support Service (BELOP) at UKBB will be available to you. They accompany the parents until the child has fallen asleep and show the parents where the child will wake up. In between, they will help you to find your way around the hospital.
As soon as your child no longer requires close monitoring (usually the following day), he or she will be admitted to the paediatric surgery ward. In order to minimise the risk of cerebrospinal fluid leaks, your child will lie as flat as possible for the first 48 hours after the operation, after which they can be fully mobilised again immediately. As a rule, your child will recover from the operation after three to five days and will also be allowed to go home during this period.
You can be with your child during the entire time and ask questions to the paediatric neurosurgery team, who will visit you and your child every day.
The prognosis for children with FTL is excellent if the operation is performed early (at the age of six to nine months). Children who have undergone surgery are followed up in our clinic for one to two years, after which the usual paediatric check-ups take place.
Lalgudi Srinivasan H, Valdes-Barrera P, Agur A, Soleman J et al. Filum terminale lipomas-the role of intraoperative neuromonitoring. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2021;37(3):931-939. doi:10.1007/s00381-020-04856-4
Greuter L, Licci M, Terrier A, Guzman R, Soleman J. Minimally invasive interlaminar approach for untethering of fatty filum terminale in paediatric patients - how I do it. Acta Neurochir (Vienna). Published online 2022. doi:10.1007/s00701-022-05204-y
Lalgudi Srinivasan H, Valdes-Barrera P, Agur A, Soleman J, Ekstein M, Korn A, Vendrov I, Roth J, Constantini S. Filum terminale lipomas-the role of intraoperative neuromonitoring. Childs Nerv Syst. 2021 Mar ;37(3):931-939. doi: 10.1007/s00381-020-04856-4.
Stricker S, Eberhardt N, Licci M, Greuter L, Raphael G, Soleman J. Wound closure with a mesh and liquid tissue adhesive (Dermabond Prineo) system in paediatric spine surgery: a prospective single-centre cohort study incorporating parent-reported outcome measures. J Neurosurg Pediatr. Sept 2022. doi.org/10.3171/2022.8.PEDS22270
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