It is therefore advised that VPS insertion is carried out in a meticulous fashion, with particular attention to preventing infection. Standardised VPS insertion protocols have been shown to decrease the rate of VPS infections, and we recommend that such a protocol be adhered to in any VPS procedure - even more so in this scenario. Once a shunt has been placed, the later development of symptoms or signs of the Chiari malformation or syringomyelia should raise suspicion of shunt failure.
Although the Chiari malformation may appear dramatic on MRI, it rarely becomes symptomatic if the shunt is functioning well. Over the past two decades ETV has gained popularity as a treatment option for non-communicating hydrocephalus. This modality carries a much smaller infection risk and is not dependent on extracranial mechanical drainage; therefore, it is seen by many as a more attractive treatment option. In patients with clear non-communicating hydrocephalus, it is the surgical treatment of choice.
In myelomeningocele, however, there remain some challenges in using this as a first-line option, and it continues to carry a significant failure rate. There are reports in the literature of successful management of hydrocephalus in these patients with ETV combined with choroid plexus cauterisation, particularly in the Third World setting, where reliable access to follow-up for VPS and management of the possible complications may not exist.
The floor of the third ventricle where the endoscopic stoma is made is often abnormal in these patients, there is usually an element of communicating hydrocephalus, and the failure rate of ETV is higher in infants than in older children. Optimisation of hydrocephalus control for the best clinical and cognitive outcomes, especially where there is hindbrain herniation, remains controversial.
At our institution we continue to place a VPS for hydrocephalus in myelomeningocele. For older patients who develop shunt failure, ETV may be an option. Even if VPS or ETV has been successful in treating hydrocephalus in these patients, it is important to maintain close outpatient follow-up, as one should always remain vigilant in case of failure of either procedure. Initially, 3-monthly follow-up is recommended. Once patients are stable, yearly follow-up, preferably in a spinal defects clinic, is ideal.
Pang D. Surgical complications of open spinal dysraphism. Neurosurg Clin N Am ;6 2 Stein SC, Schut L. Hydrocephalus in myelomeningocele. Childs Brain ;5 4 Long-term outcome and complications of children born with meningomyelocele.
Childs Nerv Syst ;8 2 Outcome of patients with meningomyelocele: The Ege University experience. Childs Nerv Syst ;14 3 The cause of Chiari II malformation: A unified theory.
Pediatr Neurosci ;15 1 The Chiari II malformation: Cause and impact. Childs Nerv Syst ;19 Williams HA. Unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida.
Cerebrospinal Fluid Res ; Bruner JP, Tulipan N. Intrauterine repair of spina bifida. Clin Obstet Gynecol ;48 4 Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation.
The higher the defect is located in the spinal column, the greater the number of functions it can potentially affect. Nerve damage can result in varying degrees of paralysis, which can affect mobility, but also bladder and bowel control. It can also cause lack of sensation. With open spina bifida, the lesion is ideally surgically closed within hours after birth by a paediatric neurosurgeon, to prevent infection.
A multidisciplinary approach to care is required, to address any possible additional health issues, such as hydrocephalus and incontinence, but also to ensure the mental health and well-being of those born with spina bifida and hydrocephalus, and their families.
Hydrocephalus is one of the most commonly associated conditions with myelomeningocele, as is a condition called Chiari II malformation , or hindbrain herniation. Hydrocephalus occurs when there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed into the bloodstream. As the CSF builds up, it causes the ventricles to enlarge and the pressure inside the head to increase.
Apart from the above mentioned conditions, other possible issues often associated with spina bifida are:. Due to the reduced mobility, children, adolescents and adults with spina bifida will be at increased risk of obesity.
Combined with the lack of sensation, there is a the risk of skin breakdown and pressure ulcers. A healthy lifestyle and diet are key, as are regular skin checks. Others may need assistance to maximise their learning capacity. The operation for open fetal surgery for MMC repair involves making a small opening in the uterus, then closing the spinal cord opening just like after birth. The womb is repaired and the mother is in the hospital for four to five days.
The surgery is performed between 19 and 26 weeks of the pregnancy. Mothers usually stay locally for about two weeks so that we can monitor the pregnancy. After this, they can return home for delivery. Because of the scar caused by the surgery on the uterus, the baby and all future babies have to be delivered by Cesarean birth. As with any prenatal surgery, there are risks to both the mother and the baby. Our team at the Fetal Care Institute will discuss all of the risks and benefits of the surgery with you and your family, so you can make the best decision for your baby.
The standard care for babies with spina bifida is to repair the defect after birth. The neurosurgeon closes the opening of the spinal cord, and restores the muscle, skin, and tissue to cover it. MMC is a disease affecting many parts of the body. Problems with Hydrocephalus and the Arnold-Chiari malformation need to be followed carefully. Because the spinal cord also affects urine and bowel function, these bodily functions often need to be managed to prevent complications.
Optimally, babies need to be followed in a spina bifida clinic, where a team of specialists work together to help determine the best course of treatment. This is a very specialized clinic in which many doctors of different specialties and nurses are dedicated to the care of these babies. Louis Fetal Care Institute. You may call our team directly at or speak with your doctor about the potential of a referral. At the St. Louis Fetal Care Institute, we understand the fear and confusion that come with the diganosis of a prenatal medical condition.
We share these patient stories to offer comfort, hope, and inspiration from other parents who have been where you are. Know that you are not alone - our team is here to support you every step of the way. SSM Health uses your location to provide you with relevant content, like doctor suggestions and local services.
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