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Patients with myelomeningocele may often suffer from severe spasticity. Surgical treatment of the underlying pathology such as hydromyelia and tethered cord may be successful, but failures are not uncommon. Those cases may offer a surgical challenge since further therapeutic options are limited. We present the case of a 7-year-old boy with myelomeningocele and related conditions suffering from severe spasticity and pain in his lower limbs. Surgical efforts with untethering and posterior fossa decompression failed to improve the symptoms. A test with 25 microg intrathecally delivered baclofen showed a total relief of spasticity and pain so that a pump for continuous baclofen delivery was implanted. During 32 months of follow-up, his spasticity has been under excellent control on 55-157 microg baclofen per day. Continuous delivery of intrathecal baclofen may be a surgical option to consider in patients with myelomeningocele and severe spasticity., (Copyright 2003 S. Karger AG, Basel)

Purpose: We investigate the long-term outcome using external urethral sphincter dilation for high risk myelomeningocele., Materials and Methods: Since 1984 external urethral sphincter dilation was performed in 25 patients with myelomeningocele who demonstrated passive leak point pressure greater than 40 cm. H2O and/or poor bladder compliance. Mean followup from the first dilation was 8.4 years. Overall 2.4 dilations were performed per patient (range 1 to 8). Cystometrography, imaging study and continence status were evaluated retrospectively., Results: Overall external urethral sphincter dilation produced durable improvements in mean leak point pressure (60.9 versus 34.4 cm. H2O), capacity (119.8 versus 233.3 ml.), initial compliance (11.5 versus 28.4 ml./cm. H2O) and terminal compliance (1.1 versus 7.7 ml./cm. H2O). Categorical analysis revealed 3 groups in terms of outcome. Group 1 consisted of 11 patients (44%) who demonstrated durable improvements in urodynamic parameters as well as preservation of the upper tracts. These patients demonstrated a 2-step compliance pattern on pre-dilation cystometrography, in which elevated leak point pressure was associated with excellent initial compliance. Group 2 consisted of 5 patients (20%) who failed to maintain safe leak point pressure and whose upper tracts deteriorated, including 4 who eventually underwent augmentation cystoplasty. This group demonstrated a 1-step hypertonicity in which elevated leak point pressure was associated with a steep pressure increase during early filling. Group 3 consisted of 9 patients (36%) who responded minimally in terms of leak point pressure reduction but whose upper tracts remained well preserved. They demonstrated a high pressure instability pattern associated with excellent baseline compliance., Conclusions: External urethral sphincter dilation provides an effective long-term solution for select high risk myelomeningocele cases. Those who demonstrate elevated leak point pressure and poor bladder compliance at the time of external urethral sphincter dilation are less likely to respond, suggesting that the bladder may have already undergone irreversible changes due to high outlet resistance. Patients who demonstrate instability patterns are less likely to respond to external urethral sphincter dilation in terms of leak point pressure reduction but the upper tracts appear to be well preserved.

Infants with myelomeningocele continue to be a management dilemma for urologists. This article discusses many of the current questions that surround the newborn with myelomeningocele. What radiologic studies should be performed and when? How does the clinician determine if bladder drainage is adequate or requires altering? If a problem is identified what are the surgical options? When should urodynamics be performed? Ultimately how one manages, follows and initiates treatments in the newborn will have a significant effect on the long-term morbidity seen in this population of children.

In order to describe the attitudes towards treatment of severely diseased newborns a questionnaire presenting four fictive situations concerning infants with myelomeningocele, trisomy 13, Down's syndrome and epidermolysis bullosa lethalis was mailed to all physicians employed at obstetric or paediatric departments in Denmark. The questionnaire was designed in two versions, differing as regards the parents' situation and attitude towards treatment in two of the situations. Each version was sent to half of the sample. Of 954 questionnaires 664 (69.6%) were completed and returned. The parents' situation and attitude towards treatment played a role in forming the treatment choice to a significant proportion of the respondents, although the majority seemed to form their decisions independently of this factor. In the situations presenting the infants with myelomeningocele and Down's syndrome there was controversy concerning the level of active treatment, while only a minority would provide active treatment in the two other situations. In the case of epidermolysis bullosa lethalis most respondents would provide morphine in doses that could unintentionally hasten death, while few were in favour of legalizing active euthanasia.

Objective: The objective of this study was to know the incidence, mode of presentation, clinical and microbiological aspects, as well as the management of ventriculo-peritoneal shunt malfunction., Patients and Methods: A retrospective study was carried out where thirty-seven children with malfunctioning shunts were studied. The mechanical causes of shunt malfunction included disconnection, fracture and obstruction, while infection was considered as either a clinical or analytical change (blood and/or CSF)., Results: The mean age of the patients was 5.5 years. The most common indication for the insertion of the ventriculoperitoneal shunt was congenital hydrocephalus. The average stay in the ICU was 8.2 days. Mechanical dysfunction was detected in 25 patients (68%) and infectious etiology in 12. The most common clinical manifestations were vomiting, fever and headache. CSF biochemical alteration was found in 8 of the infected group. Staphylococcus was isolated in six of the 8 cases. The ventriculoperitoneal shunt was changed during the first 24 hours in 17 of 25 patients (68%) with mechanical malfunction, while 11 cases with an infective cause (92%) were managed with an external shunt. The interval between the insertion of the ventriculoperitoneal shunt and the malfunction was over 6 months in 20 cases (54%)., Conclusions: Ventriculoperitoneal shunts are the main method for treating hydrocephaly, although they are not without complications which may require surgical procedures. Appropriate surgical technique, asepsis, as well as prophylactic antibiotics are essential to decrease the incidence of complications.

Myelomeningocele is the most common congenital malformation of the nervous system and despite its complexity and involvement of multiple organs is compatible with long survival. The peculiar characteristics of this malformation expose myelomeningocele patients to acute and chronic care problems with effects in quality of survival. In order to evaluate the quality of the follow-up of spina bifida patients in a pediatric hospital, the authors examined 54 patients attending the neurosurgical outpatient unity of a pediatric hospital. The lack of a multidisciplinary spina bifida clinic in Rio de Janeiro forced the patients to pursuit for complimentary medical and paramedical care outside the hospital with significant effects in the quality of survival. In consequence, only 25% of the patients were able to walk and community ambulation was nearly absent. Only 66.6% had a regular rehabilitation program and nearly 50% had routine orthopedics consultations. Almost half of the patients had no urological referral at all and 75% were incontinent, with recurrent urinary infections ranging 72.2%. The rates of neurosurgical complications were similar to those observed in the literature. We concluded that the quality of survival of patients with neural tube defects is strongly influenced by the adverse socio-economical conditions and the lack of a spina bifida multidisciplinary clinic.

In 106 patients with myelomeningocele, which were fitted with orthotic devices, we analyzed the influence of level of neurologic deficit, scoliosis and contractures of the lower extremities on different walking parameters, e.g. orthotic wearing time, duration of walking and velocity, step length and mobility status. The most important factor is the level of neurologic deficit. From a functional point of view a bilateral hip dislocation can be neglected. Whereas impairment of walking ability is strongly correlated with scoliosis of more than 50 degrees and unilateral hip dislocation; therefore both conditions should be treated operatively.

The advances in surgical technique for closure of myelomeningocele and its complications, mainly neurogenic bladder, have resulted not only in a increased lifespan and a better quality of life for these patients, but also a progressively higher number of women are reaching childbearing age and becoming pregnant. The reports of pregnancy in these women are still sporadic. In spite of that, obstetricians must be aware of this situation in order to manage it accordingly. In the course of these pregnancies major complications seldom occur. However there are problems (high-risk of neural tube defects in the offsprings, urinary tract complications, muscular-skeletal disorders and psychosociological problems) that should not be ignored and that may influence the prognosis of these pregnancies. The authors present three case reports with different outcomes.

The aim of this study was to recognize the possible psychological advantages when children with a severe CNS disorder like myelomeningocele (MMC) are given very early rehabilitation treatment. One hundred and seven newborns with MMC seen between 1971-1992 were prospectively analysed with respect to two different therapeutic approaches. The children born during the period 1971-1980 did not receive very early therapeutic rehabilitation treatment, whereas those born during the period 1981-1992, received this treatment. In the latter group, special attention was paid to support an improvement in the difficult relationship between the parents and the child with MMC as well as between parents and caregivers. The following statistically significant differences between the two treatment programmes were found: (1) all children achieved independent locomotion at 5 years, in the very early intervention group, compared to only 35% (P < 0.001) in the group without this programme. Orthopaedic operations in the first-mentioned group were markedly reduced; (2) urological surgery decreased drastically in the group with very early urodynamic rehabilitation. Thus, there were 0.6 operations per patient in the older group, but only 0.06 operations per patient in the younger one (P < 0.001); (3) normal schooling was reached by 76% (22/29) and social continence by 80% (23/29) of the children with very early interventional therapy. In the older group only 54% reached normal schooling (P < 0.05) and 29% social continence (P < 0.001). The very early co-ordinated medical and physiotherapeutic rehabilitation treatment of children with MMC usually reduces the psychosocial stress and improves the quality of life of these children and of their families.

The 2 major urological objectives in treating the child with myelomeningocele are to preserve renal function and achieve continence. We report our success in managing these cases with nonsurgical therapy. From 1981 to 1991, 45 patients with myelomeningocele 1 to 15 years old were evaluated urodynamically before and after initiating nonsurgical treatment. Pretreatment urodynamics identified 2 groups. Group 1 consisted of 31 patients with leak point pressure of 40 cm. water or more of whom 10 had grades II to V/V vesicoureteral reflux and 4 had moderate or severe hydronephrosis. Group 2 consisted of 14 incontinent patients with leak point pressure of less than 40 cm. water and no reflux or hydronephrosis. Both groups were treated with intermittent catheterization, anticholinergic medications and fluid restriction. Within a mean followup of 5.5 years nonsurgical intervention resulted in lowering maximum detrusor pressure at maximum cystometric capacity to less than 40 cm. water in 22 of 31 group 1 patients (71%), and in resolving vesicoureteral reflux in 7 of 10 (70%) and hydronephrosis in 3 of 4 (75%) group 1 patients. Of 4 patients in group 1 with persistent high grade vesicoureteral reflux or severe hydronephrosis 3 (10%) required augmentation cystoplasty. With nonsurgical management 18 patients (40%) were completely continent, 18 (40%) required 2 or less pads daily and 9 (20%) required more than 2 pads daily. Nonsurgical management alone was effective in preserving the upper urinary tract in 90% of patients and it provided satisfactory continence in 80%. Surgical management should be reserved for the minority of patients whose upper tract changes do not resolve and for those whose degree of continence is not satisfactory with nonsurgical management.

The purpose of this paper is to describe the medical attention that is given to patients affected by myelomeningocele in Spain. We have used information extracted from the 'Multicentre study of patients suffering from spina bifida in Spain'. This material was collected between 1986 and 1988 from experiences with 1500 patients all over our country. 52.9% of the patients were male, and 47% female, and of the total number, 72.2% were under 15 years of age. Most of these patients attended public hospitals (78.5%) and on average each one had been admitted about seven times. The cost of hospitalisation of the sample has been assessed. Urology, orthopaedic surgery and rehabilitation are the specialties most involved. The most frequent complications were those related to the urinary system (66.1%).

We report a four year follow-up of 39 of 47 infants born after pre-labour Caesarean section and 68 of 79 born vaginally. Loss of motor function due to late complications was more frequent in the Caesarean section group (Fisher's Exact; P = 0.004). However, the means of the differences between the X-ray levels (measured as the last intact vertebral arch seen on standard anteroposterior roentgenograms of the spinal column) subtracted from the motor levels still favour Caesarean section (mean = 3.24; SD = 2.7) over vaginal delivery (mean = 1.2; SD = 2.7) (Student's t-test; P = 0.0003). The frequencies of other complications, death and neonatal meningitis, were not significantly different. Another 38 infants born by Caesarean section after labour were more paralysed (mean of X-ray and motor difference = 1.8, SD = 2.2) following rupture of amniotic membranes than those with intact amniotic membranes with or without labour (mean = 3.4; SD = 2.2) (Student's t-test; P = 0.0067). The differences between X-ray and motor levels for patients born by Caesarean section with intact amniotic membranes and without labour (mean = 3.6; SD = 2.4) were not significantly different from those with labour and intact amniotic membranes (mean = 2.89; SD = 1.5). The number of new cases of meningomyelocele presenting to our clinic has decreased from an average of 30 per year between 1970 and 1987 to 14 between 1988 and 1992.

Data from 1500 patients affected by spina bifida have been collected in a multicentre study in Spain from 1986 to 1988. This paper is concerned with a part of the data, specifically regarding the walking of patients and certain factors that influence the prognosis for ambulation. The neurological level of lesion most commonly present was lumbosacral (65.6%). Hydrocephalus was frequent (62% of the sample), and has significant implications regarding prognosis for walking. Hip dislocation (32%) and scoliosis (22.9%) were orthopaedic problems noted in our sample. Pressure sores appeared in 31.7% of the patients. The onset of walking ability has been studied; 15% of the sample started walking at an age older than 5 years. Finally, different modalities of ambulation are described according to the use of ancillary devices, and in independence in activities of daily living.

Epiphysiolysis from a series of 253 patients under regular control in the Spina Bifida Unit at our hospital has been analysed from 1967 to the present time. From that total there have been 18 physeal fractures in 9 of the myelomeningocele patients. The clinical characteristics, diagnostic difficulties and therapeutic criteria for this type of lesion are considered, and we suggest the need to use suitable orthoses whenever the patient is able to walk, and to temporarily increase the level of orthotisation in patients if the physis of the knee is affected.

Decisions about the role of cesarean section, forceps, and vacuum delivery in obstetrics continue to be a challenging part of daily obstetric practice. Five interesting papers on this topic are reviewed, including a comparison of international rates of cesarean sections and vaginal births after cesarean section, the method of choice for the delivery of the low-birth-weight fetus presenting as vertex, the advantages of a prelabor cesarean section for a fetus diagnosed with meningomyelocele, and the appropriate role for the midforceps delivery in the 1990s.

It has frequently been noted that high-level paralysis precludes community ambulation for children with myelomeningocele. Of the 87 children in our population with high-level paralysis, however, 45 (52%) did achieve community ambulation by 5 years of age, while 42 (48%) did not. There was no significant difference between these two groups by sex, shunt status, scoliosis, or hip surgery prior to 5 years of age. Among the community ambulating group, there were significantly (p less than 0.001) more children who were not retarded, had received physical therapy for walking, and had compliant parents. We concluded, therefore, that community ambulation is a realistic goal for many children with myelomeningocele and high-level paralysis.

Of the 102 consecutive children with myelomeningocele who were treated by intermittent catheterisation between 1977 and 1985, 89 were investigated with regard to the development of the upper urinary tract. The median follow-up period was 6.7 years. Dilatation and vesicoureteric reflux were assessed by means of repeated urography and voiding cystourethrography. Of the 49 children with normal radiology at the start of catheterisation, all but 2 remained normal. In 27 with isolated dilatation of the upper urinary tract or reflux at the start, 17 subsequently became normal. Dilatation together with reflux was seen in 13 patients at the start; 3 became normal, 4 still had reflux and in 6 patients both dilatation and reflux persisted. Intermittent catheterisation thus had a positive effect on the upper urinary tract in the vast majority of cases. In children with both dilatation and reflux, however, there is a risk of deterioration and they should be followed up closely.

Whether to treat or not to treat a high neural tube lesion presents a significant ethical dilemma. In the past two decades, the medical practice regarding the approach to a neonate with a neural tube defect has gone through a series of philosophical and therapeutic changes. Medical, ethical, religious, and legal systems have grappled inconclusively with the various aspects of these issues in recent years. In this article, the pros and cons of relevant medical and ethical considerations are analyzed, and a decision-making process is outlined. It is suggested that in order to enhance the ability of the responsible care provider and the parents to reach reasonable and morally defensible decisions, a properly organized decision-making process ought to be adhered to. Each individual patient should be analyzed according to the following major categories: medical data, basic ethical and religious principles, legal and/or institutional regulations, and physician-parents relationship. Based on current medical knowledge and on pertinent ethical reasoning, it is argued and recommended that almost always, if possible, aggressive management should be favored.

Our experience with 23 cases of fetal myelomeningocele provides preliminary information on the outcome of these infants diagnosed in utero and managed by a multidisciplinary team. The mean age of diagnosis was 23.7 weeks (range, 16 to 34 weeks). Mean gestational age at delivery was 36.9 weeks in the 14 patients who elected to continue their pregnancies. None of the 11 infants with lumbosacral or sacral lesions developed significant ventriculomegaly before term. Of the three patients diagnosed with thoracolumbar lesions, two had progression of ventriculomegaly necessitating early delivery at 32 to 34 weeks of gestation. These preliminary findings suggest that a coordinated prenatal and neonatal approach appears to result in a favorable prognosis for infants with myelomeningocele, but that neonatal complications are common, requiring careful monitoring and aggressive management.

The ambulant long term care for children with meningomyeloceles requires the interdisciplinary cooperation of pediatricians, pediatric neurologists, surgeons, orthopedists, radiologists and psychologists, social workers, physiotherapists and orthopedic mechanics. We present the concept for our out-patient consulting hour for 154 children and adolescents with meningomyeloceles, meningoceles and other forms of paraplegia, and the program of medical examinations for the detection of urologic malformations and the consequences of neurogenic disturbance of bladder function, neurologic complications (seizures, tethered cord etc). and dysfunction of liquor shunts. Additional items are the control of physiotherapy and the regular and adequate prescription and inspection of orthoses. The rate of complications is presented: revision of shunts 60%, seizures 17%, cerebral abnormalities 16%.

The parents of 50 children with myelomeningocele completed a questionnaire assessing their attitudes toward the medical management of their newborn infants. Of the 47 mothers and 29 fathers interviewed, only 26 were satisfied with the information they had received at the delivery hospital, whereas 61 expressed satisfaction with the information given at the tertiary-care hospital. Satisfaction was significantly more common when parents had been involved in decisions about the management of their newborn infants. The people most commonly cited as being helpful in making decisions were physicians at the tertiary-care hospital, spouses and other family members. The most important factors in reaching a decision were the information received at the tertiary-care hospital and the parents' own personal philosophy and social values. Most parents felt that they should have the final decision about the medical management of their newborn infants.

High myelomeningocele lesions do not preclude an acceptable level of functional walking provided that an integrated programme of surgical treatment and bracing is adopted. Clinical analysis of 100 patients with myelomeningocele shows that the development of the "swivel walker" and "hip guidance orthosis" has been associated with an improved level of function. Over 30 per cent of patients with thoracic lesions and 68 per cent of those with lumbar lesions achieved independent walking. For this reason the criteria used at present by paediatricians to govern the selection of infants for non-active treatment may require reconsideration.

The management of children born with myelomeningocele is now extended over an entire lifespan. Rehabilitation specialists managing these children must understand the initial anatomic dysfunction, its interaction with normal child development, and the effect of normal aging on pediatric-onset disabilities. A model for an interdisciplinary process that keeps the final goals of any child-onset disability in mind is discussed. A review of current medical, surgical, and behavioral interventions for these children is also presented.

The purpose of the study reported here was to determine whether improvement in medical knowledge affects medical students' interviewing skills in each, or any, of the four years of medical school. Nineteen first-year students, 21 second-year students, 18 third-year students, and 20 fourth-year students at the University of Iowa College of Medicine were randomly assigned to an experimental or a control group. The experimental group observed a 45-minute videotape lecture that discussed the practical issues of the diagnosis and management of children with meningomyelocele. All subjects in both groups then completed a multiple-choice examination on meningomyelocele. The interviews were videotaped and then blindly rated. The results of the ratings show that there was no group effect but there was a significant class difference in that the first-year class was less sophisticated than the three other classes. The results suggest that interview skills are not affected by increasing knowledge about medical conditions but do improve with training in interview skills.

A program for early selection and treatment of the infant with myelomeningocele was developed at the University of Oklahoma Health Sciences Center in 1977. Over a 5-year period, 69 babies were evaluated, 36 babies were recommended for early vigorous treatment. Of the 33 babies for whom only supportive care was recommended, five were initially treated at the parents' request, two underwent delayed vigorous treatment, one was subsequently treated by "crisis management," one moved and did not return for follow-up, and 24 received only supportive care. All 24 babies died between 1 to 189 days of age (mean 37 days). The involvement of several physicians and paramedical support personnel is considered essential for this approach of early selection and treatment. Continued support and regular follow-up is necessary for any baby who receives only supportive care. Parents retain legal custody. Although ethical concerns make any approach difficult, the method presented is considered to be the best alternative available at this time.

Sixty-eight patients born with a myelomeningocele were studied in the second and third decades of their lives. All of them had had some orthopaedic surgery, an orthotic program, and other specialized medical and paramedical services. In the group with sacral-level lesions, half were community ambulators; in the group with lower lumbar lesions, a third; in the group with upper lumbar lesions, a tenth; and in the group with thoracic-level lesions, none. The most important factors that determine the ambulatory status of a child with myelomeningocele, besides the level of the lesion, are the motor power within a given neurosegmental level and the extent and degree of orthopaedic deformities.

Cerebrospinal fluid (CSF) eosinophilia was observed in 26 of 404 children after the implantation of shunts for the treatment of internal hydrocephalus. High levels of 65% and 78% were recorded in two cases, which are reported in detail. In the remaining 24 cases, CSF eosinophilia ranging between 1% and 3% was found. None of the cases with CSF eosinophilia had blood eosinophilia. The cases indicate that a reaction to the material used for the shunt should be considered along with possible parasitic infestations in patients with such findings.

Of 114 patients with myelomeningocele 44 (40 per cent) have no significant urologic disability. Intermittent catheterization has been used in 51 children and was helpful in 13 (26 per cent). Cystometrograms and urethral pressure profiles have been useful as predictive parameters. Causes of intermittent catheterization failures are discussed.