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The natural history of cord tethering in transitional LMMC remains unclear. Not all children suffer deterioration,and, in a significant proportion, function is not normal at birth. Surgery, as it is currently practiced, is generally safe but does not confer long-term immunity from deterioration. The risk of deterioration, its pattern, and its timing are related in part to the morphology of the malformation. Patients with asymmetrical malformations may exhibit unilateral functional neurological or orthopedic abnormalities, which conspire with normal neurodevelopment to render these abnormalities apparent at an early age. Symmetrical malformations present later in childhood in association with bilateral and/or urinary dysfunction. The rate of functional deterioration in patients following surgery appears to be equal to or is slower than the rate of deterioration in patients who do not undergo surgery. Many patients will require more than one untethering procedure to address evolving functional impairment. Structural abnormalities require end organ-specific orthopedic or urological interventions. A formal structured multidisciplinary monitoring team is required to provide clinical and functional surveillance. Such monitoring is required following operative untethering and debulking for the life of the patient. The author reviewed current literature to define the timing and pattern of deterioration prior to and following initial cord untethering in patients with transitional LMMC, as well as the operative burden that these children bear in exchange for optimized function.

Introduction: The development of techniques to close open neural tube malformations prior to birth has generated great interest and hope for fetal interventions and their outcomes. To plan a randomized trial, as is being discussed at three centres in the United States, the determination of what constitutes a clinically significant improvement in outcome is critical. To date, preliminary observations from two centres suggest that improvements may occur, not in spinal cord function as originally postulated, but in the extent of the hindbrain hernia and the frequency that shunting is required to control hydrocephalus., Purpose: The determination of what outcome would constitute an important and clinically significant difference in outcome to be achieved by fetal intervention for myelomeningocele., Method: Parents of patients and patients treated in our myelomeningocele clinic were surveyed using a structured and validated tool. From the perspective of a recommendation to a close friend or family member, the interviewees were asked to quantify on a scale from 0 to 100 the chance of specific outcomes (need for a shunt, need for a wheelchair, change of urinary incontinence) that a fetal operation would need to predictably achieve., Results: Responses were obtained from 77 patients/families. The fifty percentile response in each study dimension was as follows: the chance of needing a shunt was 12 % (range 0 - 50 %), the chance of needing a wheelchair was 8 % and the chance of being incontinent was 5 % (range 0 - 25 %)., Conclusions: Fetal interventions will have to achieve significant improvements in the control of hydrocephalus, mobilization and continence over postnatal treatment to be justified.

Introduction: Tethering is assumed to be the primary cause of deterioration seen in children with transitional lipomyelomeningocele as they age. The inevitability of deterioration has led to recommendations for prophylactic interventions to stabilize or prevent further clinical deterioration., Objective: Determine the frequency and patterns of functional deterioration observed after successful untethering in patients with transitional lipomyelomeningocele and compare functional outcomes with what is known regarding untreated patients., Methods: Fifty patients having transitional LMMC, treated at a single institution and followed in a multidisciplinary clinic were retrospectively reviewed to determine their clinical status prior to untethering, and the time to development of new symptoms or signs following untethering., Results: 82% of patients were diagnosed and 78% underwent untethering prior to one year of age. All patients had a cutaneous lumbosacral lipoma, 22 patients were considered normal at presentation and 28 showed abnormalities on clinical examination. Forty-nine patients were untethered successfully and all were available for follow-up ranging from 2 to 138 months (mean 39 months). Acute morbidity was limited to transient neurogenic bladder dysfunction and minor wound complications. Late clinical deterioration occurred in the majority of patients. Orthopedic and neurological deterioration occurred over the first 60 months following untethering and urological deterioration occurred thereafter., Conclusions: Functional loss after untethering is common and the pattern of loss is likely a reflection of the ability to detect abnormalities in this infant population. Untethering does not usually result in permanent acute morbidity, and does not prevent longer-term functional deterioration. The ratio of asymptomatic to symptomatic patients at follow-up in this operative series is similar to age-matched historical series of untreated patients.

Little is known about the long-term prognosis of patients with cervical myelocystoceles and meningoceles. In this study, we report the long-term follow-up (average 10 years and 8 months) of 8 patients with cervical myelocystoceles and meningoceles. Neurologic, orthopedic, urologic and psychosocial status was assessed on long-term follow-up. The results indicate that in patients with cervical myelocystoceles, neurologic deficits become noticeable in the first year of life as the infant matures. Motor deficit was common in these patients, whereas sensory and urologic deficits did not occur. We believe that this is related to underlying myelodysplasia. In addition, patients with cervical myelocystoceles tend to have significant orthopedic problems. In contrast, patients with cervical meningoceles do not have neurologic deficits, but do develop mild orthopedic problems., (Copyright 2000 S. Karger AG, Basel)

Placode untethering in myelomeningocele patients can result in improvement and/or stabilization of neurological function, spinal curvature and pain. This paper reviews the outcome of untethering procedures in 24 patients to determine the frequency of subsequent functional deterioration. Decreased range of movement, joint stiffness and changes in muscle tone were the commonest indications for surgical intervention, occurring in 15 patients. Untethering resulted in improvement in 8 patients, stabilization of progression in 6 and continued deterioration in 1 patient. Two patients previously untroubled with spasticity became symptomatic within 3 months of the procedure. Changes in ambulation were present preoperatively in 9 patients. Stabilization was observed in 4 and improvement in 5. One of the patients, who had improved, deteriorated during the 1st year of follow-up. Alterations in bladder capacity and continence occurred in 7 patients. Improvement was seen in 2 patients, deterioration in 2 and no change in 3. Of the patients who improved, 1 subsequently deteriorated again within the 2nd postoperative year. Of those patients who had stable bladder function preoperatively, 6 subsequently deteriorated despite untethering. Pain was a less frequent symptom, occurring in 6 patients. Six patients became pain-free within 3 months of untethering. Two patients who did not have pain preoperatively had pain at the operative site persisting for up to 3 months postoperatively. Of the 20 patients having a single untethering procedure to date, 11 have further symptoms that can be attributed to retethering.

Objective: Prenatal ultrasonography can localize the level of the spinal cord malformation, allowing prediction of the potential postnatal neurological deficit and functional prognosis., Methods: This study has two evaluations: (a) a retrospective prenatal review of 26 fetuses with spinal dysraphism (1987-1991), and (b) a follow-up descriptive study of patients (1971-1981) who underwent closure of the spinal lesion and ventricular shunting in the neonatal period., Results: Prenatal ultrasound evaluation enabled the accurate definition of the last intact vertebral level which allows separation of fetuses into three functional groups (last intact level L2, L3-4, L5-sacral). Patterns of ambulation, urinary and bowel continence, and school performance vary according to level of spinal lesion and the neurological deficit. The need for ventricular shunts, the incidence of other spinal malformations and surgical interventions did not vary with the level of the spinal lesion., Conclusions: The functional outcome for patients with myelomeningocele is variable; however, distinct patterns emerge based on the level of spinal dysraphism and the resultant neurological deficit. By relating the level of the fetal spinal lesion to outcome data, more precise functional prognoses can be given to families.

Since 1969, the Meningomyelocele Clinic at British Columbia's Children's Hospital has been serving the needs of children with spina bifida. In 1994, the clinic provided urgent and routine multidisciplinary clinical assessments to 330 patients and families. The purpose of this study was to assess, from the patients' and families' perspective, the appropriateness of the frequency of routine clinic visits and the value of the health services provided. Sixty-five patients/families were interviewed and completed a questionnaire developed by the clinic. Current visit frequency and services provided appear to meet the patient/family needs. The medical and nursing contributions were seen as valuable resources for the patient/families resulting in most patient/families feeling well informed about spina bifida.

The classification and embryogenesis of cystic cervical dysraphic lesions are discussed in the light of the authors' experience and review of the literature. It is felt that these lesions are best described as meningoceles or myelocystoceles, and the use of the term 'myelomeningocele' may be more confusing than clarifying. The authors hypothesize that the cervical meningocele and the myelocystocele are part of a spectrum of the same underlying developmental abnormality, namely limited dorsal myeloschisis, with the eventual abnormality depending on the presence or absence of associated hydromyelia.

The long-term functional outcome of 101 children born with meningomyelocele between 1971 and 1981 was assessed, by a combination of retrospective chart review and follow-up assessments. The children had been managed at birth using a process of nonstandardized selection. Eighty-three of the 101 patients survived after a minimum follow-up of 8.6 years, for a mortality rate of 18%. Forty-four of 83 children (53%) were community ambulators, and this correlated well with the presence of intact quadriceps function. Forty-eight children (58%) attended normal school and were grade-appropriate. Sixty-two of 83 patients (75%) were socially continent of urine, and 71/83 (86%) were socially continent of stool. Hydrocephalus was present in 93 of the 101 children in the study, and 85 children were shunted. Half of the shunted children required a shunt revision in the first year of life, and thereafter the rate of revision decreased, so that after 2 years the risk of revision was approximately 10% per year.

Between 4% and 8% of cases of spina bifida cystica occur in a cervical or cervicothoracic location. Despite a large body of literature concerning spinal dysraphism, there has been little written specifically about patients afflicted with this disorder in a cervical location. Eight children who presented at birth with posterior cervical or cervicothoracic lumps, all of which represented a dysraphic state, are discussed. Two types of abnormalities were noted. Three patients had hydromyelia with an associated myelocystocele herniating posteriorly into a meningocele sac. In these three patients there was an associated Chiari II malformation and hydrocephalus. The other five children had a meningocele in which a band of tissue extended from the posterior aspect of the spinal cord through a defect in the bone and fascia to the posterior part of the meningocele sac itself. No patient had a lesion that could be described as a meningomyelocele. The investigation and surgical management of these conditions are discussed and the need for intradural exploration to untether the spinal cord in the cervical region is stressed.

Apnea is a known complication of the Chiari II malformation presenting in infancy. Obstructive apnea secondary to bilateral abductor palsy or laryngomalacia and centrally mediated expiratory apnea with cyanosis can occur. Observations of 9 patients suggest that these forms of apnea may represent stages in a continuum of brain stem dysfunction due to the combined effects of the hindbrain malformation and its compression, hydrocephalus and progressive arachnoiditis. Obstructive apnea in some patients may be reversed by optimal control of hydrocephalus with or without cervical decompression. These patients may also develop episodes of cyanotic expiratory apnea of central origin (PEAC). This form of apnea does not respond to surgical or medical treatment and may show progressive worsening over time. Five of 6 patients with this form of apneic spell died suddenly, 2 of these died despite full recuscitative efforts. It is recommended that reports of treatment address results for both forms of apnea.