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Meningomyelocele (Spina Bifida)

The terms meningomyelocele and spina bifida are used synonymously. It is an important movement system dysfunction in childhood and ranks second after CP as the cause of disability. It is a complex syndrome affecting the musculoskeletal, reproductive, and urinary excretory systems.

In the first weeks of embryonic life, it emerges as a result of the fact that the spinal cord and back part of the spine do not close. It is usually recognized at birth by the presence of an external sac in the back or waist region. The sac contains the spinal cord and spinal cord sheaths, and nerve roots.

 


The treatment of children with meningomyelocele is usually multidisciplinary. First of all, four main deficits should be emphasized:

1. Hydrocephalus. It occurs in 90% of cases.

2. Paraplegia or paraparesis. There is no or very little movement in the legs. It is accompanied by sensory disturbances.

3. Neurogenic bladder and bowel

4. Spinal deformities and bone anomalies


  Disorders observed in meningomyelocele (spina bifida)


The risk of having a child with meningomyelocele is 1 in a thousand. The risk increases 50 times in recurrent pregnancies of children with spina bifida. Since folic acid reduces the incidence of spina bifida by approximately 70% and reduces its severity if it occurs, it should be taken as medicine or as a cereal product enriched with folate, if available.


The course of the Disease: 

The functional walking status of the patient differs according to the motor level. If the level is in the lower back area, it means that the patient will remain wheelchair dependent. As the motor level goes down, the probability of the child standing and even walking with the devices increases.


TREATMENT

Although the treatment of meningomyelocele (spina bifida) is initially in the form of closure of the sac, rehabilitation is the basis of the treatment. Surgical intervention may be required in advanced ages. Rehabilitation continues before and after surgery.

Rehabilitation


Early period: It begins with the birth of the child. Early rehabilitation includes bladder and bowel care, prevention of contractures, hip dislocations and spinal deformities, normal mobility with orthoses, and wheelchair use.


First of all, the family is told about the positioning, holding, and transfer of the newborn. In addition, joint range of motion (ROM), posture, and trunk strengthening exercises are taught for the extremities involved, especially for children born with hip and knee contractures. At an early age, joint range of motion exercises is performed at every diaper change. Bed position should be given to provide passive stretching against tense hip flexors and spinal restrictions. Babies with high lesion levels may need a sitting and support device suitable for their bodies. These devices protect the surgical area and help with trunk and head control.


Late period: In the long term, treatment is focused on the central nervous system (cerebrospinal cord), bladder, intestines, and musculoskeletal system.


Central nervous system: Most of these children have hydrocephalus and may develop neurological dysfunction above the level of Meningomyelocele. It is essential to start treatment to detect, correct and control these problems as early as possible. One of them is the Arnold Chiari II malformation. It is the downward displacement of both the cerebellum and brain stem into the cervical canal. Very serious symptoms such as wheezing, difficulty in swallowing, aspiration, periodic breathing during sleep, and central apnea develop. In severe cases, surgery is required.


 Arnold Chiari malformation 


Another one is hydromyelia. Hydromyelia or enlargement of the central canal is similar to the enlargement of the ventricles seen in hydrocephalus. The most common symptoms are rapidly progressive scoliosis, upper extremity weakness, spasticity, and motor weakness in the lower extremities. Its treatment is surgery.


Another problem with the spine and spinal cord is tethered cord syndrome. It has been reported in 11-15% of children undergoing meningomyelocele repair. Diagnosis is made with progressive symptoms. The most common ones are spasticity, and strength, and mobility changes. Back pain is also common. Surgical treatment can stop the progression of symptoms. The mean age at diagnosis of the tethered cord is 6 years.


Urinary system: Overflow incontinence may occur in meningomyelocele; The bladder fills and empties, but because there is no innervation to induce contraction, there is a steady, slow overflow of urine when the bladder is overfilled. This can lead to infection and kidney damage. The patient may have to use a pad for continuous urine leakage.


In principle, all newborns are placed on clean intermittent catheterization (CIC), oxybutynin, and antibiotic prophylaxis immediately after closure of the back defect. Immediately after the closure of the back defect, oxybutynin is started with CIC. Most centers discontinue prophylaxis after 1 year of age.


Neurogenic bowel: Intestinal incontinence (fecal incontinence) creates an important limitation in the professional and social life of patients with spina bifida. Both urine and bowel control are necessary to get rid of the constant use of diapers. Intestinal emptying at regular intervals provides significant stool retention.


Musculoskeletal System: Musculoskeletal disorders are common in children with meningomyelocele. Depending on the muscle imbalance, inward and outward rotation of the leg, joint contractures, mild dislocations, scoliosis, and fractures may develop. Surgical interventions for children who cannot stand and walk and children who can walk are different. Less surgery is performed in those who do not have movement in their legs.


Mobility, assisted and sometimes unassisted walking, is achieved by using a wheelchair, or one of the assistive devices that provide support and mobility. Robotic gait training can help walking. Those with motor function loss at L3 and below are successful in community ambulation.


Patient Care and Education


The overall goal of the medical team involved in the treatment of meningomyelocele (Spina Bifida) is to develop and maintain an independent level of function as much as possible.


From birth, the family is informed about care, and exercises for the trunk and affected extremities are taught. Most children develop head control, sitting balance, and some other types of mobility during their first year of life. Particular attention is required to the sitting position. Appropriate chairs are provided to reduce scoliosis.


In the pre-school period, standing and walking are started with braces such as walkers and crutches or an assistive device. Self-care skills such as dressing, and training for transferring to the toilet, tub, car, or chair, and skin examination should be given.


Learning disabilities and other cognitive deficits pose problems during schooling. Precautions may be required for perceptual problems such as poor visual attention, poor tactile perception, and spatial confusion.


Wheelchair sports should be encouraged.


Pressure sores: Pressure sores are seen at a rate of 20-25%, mostly in the skin area where the sensation is lost. The earliest symptom of prolonged pressure is redness. Generally, areas, where body weight is applied with prolonged and unequal pressures, are high-risk areas for pressure ulcers.


Obesity: If the child with spina bifida is obese, they will have more difficulty in losing weight in the following years due to the reduced energy consumption. Guidance and nutrition education associated with diet therapy should begin in infancy.


Psychosocial and social issues: Each individual's capacity to understand and imitate the world is much more complex than the simple scoring system on an intelligence test. But intelligence tests give at least a rough measure. Intelligence test scores are consistent with lesion levels, and those with higher-level lesions have lower scores and those with lower-level lesions have better performance. The family changes irreversibly with the disabled child, and the child is affected by the family's response. The family should be supported in coping with the stresses encountered in the care of the disabled child.


Adult Meningomyelocele

As the survival time of individuals with meningomyelocele increases, it is usual for adult problems to develop. Therefore, neurological changes, renal dysfunction, joint disease, and pressure sores should be checked regularly. Pain is a typical complaint of adults with spina bifida, and back, neck, and leg pain are common.


Osteoporosis (OP) develops secondary to immobilization. There may be extremity fractures due to osteoporosis.


Deformities may progress with advancing age, leading to loss of function and lower extremity deformities.


Adolescents with spina bifida have similar expectations for marriage and fertility as healthy adolescents. Regarding sexual life, it has been reported that women have varying degrees of genital sensation, while most men can erect and ejaculate, none of which is related to the level of motor function. Most adults have satisfactory sexual function. It has been determined that women with meningomyelocele conceive easily, complete the pregnancy without developing any major complications, but experience frequent urinary system infections.


Latex sensitivity develops in individuals with meningomyelocele. If latex allergy is suspected or proven, latex products (catheters, condoms, gloves, etc.) should not be used.


In case of loss of mobility, the environmental communication barriers of the individual must also be resolved.


SELECTED REFERENCES:

- İrdesel J: Nöral tüp defektleri ve rehabilitasyonu (Neural tube defects and rehabilitation). Tıbbi Rehabilitasyon’da (Medical Rehabilitation) (Ed. Oğuz H). Nobel Tıp Kitabevleri, İstanbul, 2015