By Dr.Ravindra Patil
Myelomeningocele is one of the forms of spina bifida and described as a sac containing liquid and nerve tissue protruding from the back. It occurs when there is an incomplete closure of the spinal cord during the first month of pregnancy.
Myelomeningocele generally occurs during embryonic development due to incomplete closure of the spinal neural tube during the first month of pregnancy. It ultimately leads to an exposed neural tissue or meninges with a fluid-filled sac that protrudes at the affected vertebral level.
Myelomeningocele can cause several neurological deficits and complications which are dependent on the involved vertebral site. It can lead to devastating morbidity and multiple disabilities; hence, the prognosis is often worse if diagnosed late or left untreated.
Causes of myelomeningocele are many and include environmental, maternal, and genetic factors.
It is the branch of medicine which deals with the incidence, distribution, and possible control of diseases and other factors relating to health. The prevalence of myelomeningocele is approximately 0.8 to 1 per 1000 live births worldwide.
In the new-born found to be suffering from myelomeningocele, there is a sac extending from the vertebral column, covered with meninges, filled with cerebrospinal fluid and neural tissue. The clinical symptoms are dependent on the site of the spinal lesion at the vertebral column. The higher the level affected, the more deficits occur, and the worse the future of the patient.
Myelomeningocele is one of the most prevalent causes of neurogenic bladder in children.
Patients can have a life-long functional disabilities like paraplegia, sphincter dysfunction, motor and sensory impairments. Some might experience abnormal sexual development in adulthood.
Renal, respiratory, and cardiac complications are common causes of death in patients with spina bifida and Myelomeningocele.
A cerebellar tonsillar herniation [where a part of the small brain protrudes through foramen magnum] may occur in myelomeningocele. Other complications are hydrocephalus and syringomyelia. There are many more complications.
The degree of neurological impairment predominantly depends on the affected spinal cord level.
The diagnosis of myelomeningocele is possible in the first trimester of pregnancy when the foetus in inside the mother’s womb by ultrasonography. It is non-invasive, safe and effective. A careful examination of the foetal spine myelomeningocele if present. Test of the amniotic fluid may show higher levels of alpha-fetoprotein.
Other features of spina bifida that are visible through ultrasound include hydrocephalus, microcephaly, small-shaped cerebellum, and abnormal cranial bones.
Fast MRI gives a better visualization of the spine and neural elements as well as other organ systems associated with myelomeningocele and spina bifida.
Once the diagnosis has made, early surgical repair of the spinal lesion is essential in preventing further deficits and neurological damage. Surgery before the birth of the baby has been proven to be more effective than surgery on the new-born to reduce the occurrence of complications in life of the children.
The myelomeningocele sac is everted and placed within the dural canal. The closure of the fascia then achieves the repair. Small-sized lesions are coverable by primary repair. However, the closure of large-sized myelomeningoceles could require either a flap or graft repair. Radiological imaging is done to exclude other associated anomalies.
Myelomeningocele although easily diagnosed because of the sac protruding from the back, can be confused with many other birth defects like these:
The above are rare and complicated disorders best handled by specialists.
Delayed diagnosis of myelomeningocele is associated with poor future of the child and lower rates of survival. Moreover, delayed and neglected management has links to various complications such as lifetime dependence, immobility, functional disability, muscle weakness, bladder, and bowel dysfunction etc. The presence of other disorders, such as hydrocephalus, increases the mortality rate in patients with myelomeningocele.
Complications of myelomeningocele are either surgical or non-surgical.
Surgical complications include infections at the site of the lesion, bleeding, delayed wound healing, re-tethering of the spinal cord, and cerebrospinal fluid leakage.
Non-surgical complications are:
Postoperative care primarily depends on the type of surgical procedure. If surgery is done before birth and foetal delivery is preterm, the baby should stay at a specialized neonatal intensive care unit in a latex-free environment. Subsequently, coordinated medical care should be followed to prevent other complications related to prematurity or foetal surgery.
Although foetal surgery gives better results than postnatal surgery, patients and mothers are at a higher risk of complications during and after the surgery.
To preserve the functional neurological level and improve the quality of life, long-term rehabilitation care is required.
Early surgical intervention before or within 24 to 48 hours after birth is essential to prevent more damage. It can improve the quality of living if followed by integrated medical care.
Nevertheless, children might require frequent medical evaluation and hospital visits to address their needs and complaints. Early medical management for children complaining of learning and cognitive disabilities in schools can help them prepare for their future education. Parents should be aware that the child might show a particular area of disability.
Having a child with spina bifida would increase the risk of having another affected child in subsequent pregnancies. To avoid the risk of another affected pregnancy, genetic counselling should be required before conception.
Premarital and antenatal counselling should be a consideration for high-risk individuals. Regular antenatal examination and visits are essential to follow foetal development and address possible foetal and maternal complications.
Increasing awareness about spina bifida is significant to improve the outcomes of patients in their society. The good news is: 94% of spina bifida adults have finished a high school degree, and about 62% have completed a college degree in the USA.
Hence, there are no boundaries for patients to learn or be part of their community.
However, ignorance about the disease and its possible complications in addition to delaying therapeutic interventions and not providing adequate medical services would lead to more difficulties in the future and impact the quality of life.
Samarth Neuro and Superspeciality Hospital has 100+ beds & specializes in emergency surgery for neurological issues/disorders & diagnostics.
Samarth Neuro and Superspeciality Hospital
