Secondary tethered cord syndrome in patients with myelomeningocele

Myelomeningocele is associated with other neurological abnormalities, including hydrocephalus, Chiari II malformations, syringomyelia, and secondary tethered cord syndrome. Tethered cord syndrome occurs because of abnormal attachment of the spinal cord to the caudal dural sac, causing cord ischemia. Occasionally, symptoms of progressive neurological deterioration may occur and can significantly affect the long-term outcome of these patients. Proper management of patients with myelomeningocele requires long-term follow-up and evaluation of signs of neurological deterioration that suggest secondary tethered cord syndrome. Treatment of these patients may target symptoms, such as urological intervention for bladder dysfunction, or it may target the tethered cord itself. Recently, many studies have shown that tethered cord release can significantly improve symptoms in these patients.     

Peter: an infant with a myelomeningocele.

Caring for a child with a myelomeningocele is not a task that one person can accomplish on his own. An interdisciplinary team approach, early parental involvement in the baby's care, a thorough knowledge of community resources, and parental understanding of the long range implications will greatly influence and affect Peter's early years. With support from both professionals and relatives and friends, it is hoped that they will develop a positive attitude to this challenge and continue to demonstrate their love and concern for Peter.     

Spinal sonography and magnetic resonance imaging in patients with repaired myelomeningocele: comparison of modalities.

The goals of this study were to evaluate the feasibility of using ultrasonography of the spine in the follow-up evaluation of patients with repaired myelomeningocele at birth and to compare sonography with the accepted modality of magnetic resonance imaging. Over a period of 4 years we performed 165 sonographic studies in 101 patients; 107 sonographic studies had MR imaging results for comparison. We collected our data prospectively. The quality of the sonograms was good in 110 of 129 studies, acceptable in 17 of 129, and poor in two of 129. The sonographic examinations failed in 33 of 165 studies (20%). Concordant information was obtained between ultrasonography and magnetic resonance imaging in the following percentage of studies: level of the distal end of the cord in 82%, position of the cord in the canal in 59%, presence of hydromyelia in 63%, cord duplication in 96%, adhesions in 16%, intradural mass in 37%, cord measurements in 85%, and dural sac measurements in 83%. At the lumbosacral level, we saw no cord pulsation in 57% of the studies in patients with cord adhesions and in 20% of those without adhesions. At the lower thoracic level, we saw no pulsation in 35% of the studies in patients with cord adhesions and in 7% of those without adhesions. Postoperative studies of cord release surgery in eight patients showed varied findings. We conclude that in those patients who have a spinal defect or interlaminar space allowing proper visualization of the lumbosacral spinal canal, ultrasound can provide fairly similar information to that obtained with magnetic resonance imaging of that area with no need for sedation and at a reduced cost. Ultrasonography seems more sensitive than magnetic resonance imaging in the detection of cord adhesions, which is particularly relevant in the diagnosis of tethering.     

Stress and coping in families of children with myelomeningocele.

This pilot study examined differences in and relationships between parent health-related stressors (child care needs and parental concerns), daily hassles, and coping strategies of 17 mothers and 17 fathers of preschool and school-age children with myelomeningocele (MMC). Help related to play was the most frequent need among mothers; fathers needed the most help with school activities. Among the greatest concerns of both parents were their child's future and sufficient income. Too many things to do was a frequently mentioned hassle by both parents. Having faith in God was the coping strategy mentioned most often by both parents.     

Infection stones in patients with myelomeningocele and ileal conduit urinary tract diversion

Infection renal calculi are serious complications of urinary tract diversion in patients with myelomeningocele. During a 12-month period, 10 renal units containing calculi were managed surgically. The kidneys were in patients with multiple previous operations and medical problems. Nine of the ten stones had a staghorn configuration, filling the renal pelvis with calyceal extensions. Nephrectomy was necessary for a nonfunctional kidney and a conventional nephrolithotomy was done in one case. Endourologic techniques were used for treatment of eight renal units. Endourologic methods depended on placement of a percutaneous nephrostomy tube. The nephrostomy tract was dilated, then renal calculi were disintegrated under direct vision using ultrasound, and the stone fragments were removed. Six of eight kidneys (75%) were rendered stone-free using these methods. The other two kidneys had residual stone fragments located in isolated calyces with little potential for obstruction. Skin breakdown occurred in two patients, and two patients required transfusions. These results compared favorably with the results of conventional open surgery. Major advantages of percutaneous nephrolithotomy were evident during the postoperative period. Since there was minimal incision discomfort, patients were easily mobilized and there were no pulmonary problems. Patients were able to resume oral intake on the day of surgery and usually resumed full activities within one week of discharge. Percutaneous methods are a valuable aspect of a total therapeutic program for infection renal calculi in patients with myelomeningocele.     

Abdominal problems in patients with spinal cord lesions.

The physiatrist faces two major difficulties when dealing with abdominal problems in spinal cord-injured patients: (1) realizing when there is a serious problem; and (2) determining the etiology of the problem. Patients are presented which demonstrate these difficulties. One patient with a ruptured appendix and the periappendicial abscess had only mild symptoms whereas another patient with severe abdominal pain, rigidity and rebound tenderness had a viral enteritis. The neurologic innervations of the abdomen and the various signs and symptoms appearing in cord-injured patients with abdominal problems are described. A methodical evaluation procedure for acute problems in paraplegic patients is presented.     

Ambulation in the adolescent with myelomeningocele. I: Early childhood predictors

Birth records identified 192 children with myelomeningocele born in Minnesota in the years 1966-1970. In 1981 current ambulation, neurologic level, and early motor achievement were determined in 77 of the surviving 80 children by chart review and questionnaire. Of these 77, 20 were not walking at all, one was walking only in therapy, and the remaining 56 were community ambulators (16 of these occasionally used wheelchairs). The actual distance walked daily varied from 100 to 5,000 meters in this group, with 19 of these community ambulators never walking as far as around a block. Five children never walked, 16 had stopped walking by 1981, and another six, although still walking, were doing less than they had previously. In 20 of the 22 who showed a decrease in total walking between the ages of ten and 15, the beginning of the decline was associated with a period of immobilization, demonstrating the potentially adverse effects of immobilization in these children. The ability to walk outdoors independently and to use a wheelchair by age seven predicted ambulation as an adolescent correctly for 87% of the children. In contrast, neurologic level predicted adolescent mobility correctly for only 71%.     

Managing dysphagia. Special problems in patients with neurologic disease

Swallowing is a brief but intricate process. When this process is interrupted, as in patients with neurologic disorders, problems such as aspiration and risk of malnutrition can occur. The authors of this article discuss an individualized approach to evaluation and management of neurogenic oropharyngeal dysphagia. Three cases illustrate the diversity of causes, signs and symptoms, and clinical course.     

Physical activity capacity in children with myelomeningocele

Thirty-three children (10 to 15 years of age) with myelomeningocele were studied to determine isometric muscle strength of hip and knee extension, range of hip and knee extension, usual and maximal ambulatory velocities, maximal aerobic capacity, and the energy cost (oxygen uptake) of ambulation versus wheelchair usage in subjects who both walked and wheeled. Subjects were placed into one of four groups depending on their level of motor function (those with motor levels at L2 and above, L3-4, L5 to sacral, and without motor deficit). Maximal ambulatory velocity correlated with strength of the hip extensor (r = 0.85) and knee extensor (r = 0.81) muscles. All variables were found highly dependent upon the level of motor function. Subjects with the greatest deficits had the most significant impairments and vice versa. It was found, however, that subjects without motor deficit had impairments in the usual speed of ambulation (9%), maximal running velocity (20%), and VO2 max (13%). These deficits were due to the measured strength deficits in these subjects as compared to normal values. This finding underscores the importance of quantitative assessment of muscle strength as significant deficits in strength can be missed with manual muscle testing. In those subjects who walked and wheeled, wheeling was found much more energy efficient. Wheeling at 4.8 km/hr was 11% more efficient than walking at 33% that velocity.