Multidisciplinary Spina Bifida Clinic Research Articles

Does Surgical Release of Secondary Spinal Cord Tethering Improve the Prognosis of Neurogenic Bladder in Children With Myelomeningocele?

The diagnostic criteria for secondary tethering of the spinal cord in children with prior closure of an open spinal defect are not well defined. Urological, neurological or orthopedic deterioration during followup remains the only strong indicator for the diagnosis of this condition. However, the outcome of untethering surgery cannot always be predicted. The aim of this study was to determine neurourological outcome after secondary untethering surgery in children with myelomeningocele. Of 401 children with myelomeningocele followed at our multidisciplinary spina bifida clinic between 1996 and 2005, 56 (14%) were diagnosed with secondary tethering of the spinal cord (median age at diagnosis 4.1 years). The diagnosis was based on urological and neuro-orthopedic deterioration in 58% and 42% of children, respectively. Preoperative urological findings were compared with 6-month postoperative findings. Febrile urinary tract infection, upper tract dilatation and vesicoureteral reflux were preoperatively documented in 48.2%, 19% and 30% of the 56 children, respectively. At 6 months postoperatively urine cultures were sterile in 34% of patients and upper tract dilatation completely resolved in 45%. For all grades of vesicoureteral reflux complete resolution occurred in 47% of patients. Urodynamic parameters in terms of cystometric bladder capacity and detrusor leak point pressure substantially improved 6 months after untethering surgery (125 vs 170 ml and 69.1 vs 47.5 cm H2O, respectively, p < 0.05). Assessment of urodynamic findings in 19 children at 1 year failed to demonstrate a significant change in these parameters. A subgroup analysis according to patient age at untethering surgery revealed urodynamic improvement was more significant in children who were diagnosed and treated before age 7 years. Secondary tethering of the spinal cord is a major risk factor for urological and neurological deterioration in children with myelomeningocele. Close urological surveillance remains the most valuable tool in the early diagnosis. Our study shows that secondary untethering surgery may significantly improve urological outcome.

Are myelodysplastic children receiving sufficient health care in Turkey? An analysis of the problems in primary management and their impact on neuro-urological outcome

Objective To investigate the problems in the primary care of children with spina bifida and to analyze their impact on neuro-urological outcome in Turkey. Information from mothers about the disease, difficulties in getting medical services, availability and efficacy of prenatal ultrasound, and folic acid intake was also assessed. Patients A retrospective review of 476 children registered in the pediatric urology section of our institutional multidisciplinary spina bifida clinic between 1996 and 2005 was made. All children were assessed for the time of primary repair, time to first neuro-urological visit, and compliance to follow up. A phone interview was made with 166 mothers to obtain data regarding their educational status, supplementary folic acid intake before/during gestation, compliance to obstetric follow up, prenatal diagnosis and counseling, information about the importance of neuro-urological surveillance, and difficulties in neuro-urological follow up. Results Two-thirds of the mothers had an educational status of elementary school or lower. Phone interviews revealed inadequate obstetric follow up in 42% and a low prenatal diagnosis rate (49%) in those under regular follow up. Chart review revealed a significant delay in timing of primary surgical closure (mean 3 months and 2.9 years for open and closed lesions, respectively) and first neuro-urological follow-up visit (mean 1.8 years and 9.7 months after primary repair for open and occult lesions, respectively). Reasons for delayed closure were misguided advice of the nurse/midwife involved in delivery and inability to obtain tertiary health care. Socioeconomic inadequacy of the families and inefficacy of the health insurance system were the most important factors impairing follow up. Conclusions Serious problems exist in the prevention, prenatal diagnosis and primary management of children with myelodysplasia in Turkey. As a consequence, neuro-urological follow up starts relatively late, which adversely affects the urological prognosis.

COMPLICATIONS ASSOCIATED WITH CLEAN INTERMITTENT CATHETERIZATION IN CHILDREN WITH SPINA BIFIDA

Clean intermittent catheterization (CIC) is commonly used in the management of voiding dysfunction associated with neurospinal dysraphism. We determined the incidence of genitourinary complications associated with this technique in a population of children with spina bifida. The charts of all children younger than 13 years attending a multi-disciplinary spina bifida clinic between 1987 and 2002 were reviewed. Children in whom CIC had been performed for a minimum of 5 years were identified. Catheterization was performed with a polyvinyl chloride catheter and a water-based lubricant. All genitourinary complications that had occurred in this group were recorded. The caregiver of each patient was then contacted by telephone to confirm the accuracy of our data. There were 32 females and 27 males identified in whom CIC had been performed for a minimum of 5 years. Mean duration of CIC was 10.5 years (range 5 to 15). Of the patients 45 (76%) learned to perform CIC independently at a mean age of 8 years (range 4.7 to 15.3). Two complications were gross hematuria and a false passage in the bulbar urethra. Both complications occurred in males early in the course of CIC, and while being performed by a caregiver. Neither complication was associated with long-term sequelae. The incidence of genitourinary complications associated with CIC in children with spina bifida is low. We identified 2 complications during a period of 570 patient-years for an incidence of 3.5 complications/1000 patient-years (95% confidence interval -1.3, 8.3) of observation.

Myelomeningocele and Tethered Cord: Caring for an Aging Population

Neurosurgeons outside pediatric centers are sometimes called upon to treat patients with spinal dysraphism. Children with spinal dysraphism are living longer. Upon entering adulthood they often become ineligible for care in pediatric facilities having a robust resource base focused on management of this complex multiorgan-system disease. Additionally, a percentage of patients will present in adulthood with spinal cord tethering of various etiologies. There is very limited data to recommend that adults should be followed in a multidisciplinary spina bifida clinic. There is even less data to suggest whether this care should be provided in the adult or the pediatric setting. We will discuss the pediatric model and its implications relevant to adults with spinal dysraphism. We feel that adults with complex spinal dysraphism should be followed in a multidisciplinary spina bifida clinic.

Beyond multidisciplinary care: a new conceptual model for spina bifida services.

Medical care for people who are born with spina bifida (myelodysplasia) is complex and challenging, but the goal of such care is to optimize health and function for people with this condition. Despite years of expert agreement that multidisciplinary care is the medical standard for individuals with spina bifida, this model does not seem to go far enough towards accomplishing the goal of improved function as well as health for all people with spina bifida. Advances in the medical and surgical management of people with spina bifida and society's increased desire to include those with disabilities in family and community life has heightened our expectations for functional/participatory improvement for people with this condition. Some large spina bifida programs have added a separate rehabilitation component to the comprehensive package of services offered to people with spina bifida. The problem with adding further services to the traditional multidisciplinary spina bifida program is the increased complexity and potential for fragmentation that it presents, particularly for the patient and family. We performed a MEDLINE literature search to find information on integrating these services. Although several articles address the importance of either the multidisciplinary spina bifida clinic or a rehabilitation approach, there is little information about integration of medical and rehabilitation services. We propose a new model for spina bifida services throughout the lifespan that integrates the medical and functional/rehabilitative perspectives. Our model for conceptualizing spina bifida services builds on the WHO framework for coding disability (ICIDH-2). This framework includes not only health and functional perspectives, but a participation dimension as well. Furthermore, our new model emphasizes the importance of anticipatory guidance and transitional planning as an integrating dimension applicable across the lifespan. The model is based on three main conceptual dimensions--comprehensiveness, coordination and longitudinality. We are using the model to create standards of care, integrate services and improve their efficiency, and to develop policies regarding spina bifida services. Through this effort we hope to provide better medical and rehabilitation services to those with spina bifida and meet the complex challenge the condition presents throughout the lifespan.

Acompanhamento ambulatorial de pacientes com mielomeningocele em um hospital pediátrico

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.

Investigative Techniques and Renal Parenchymal Damage in Children with Spina Bifida

The case records of 72 patients attending a multidisciplinary spina bifida clinic were reviewed to discover the prevalence of renal parenchymal damage. 19.4% of the total population had such damage. More detailed analysis of the group according to age showed that the prevalence of parenchymal damage in the over ten year olds (27.3%), was twice that of the under five year olds (13.3%) but the same as the prevalence of "at risk" patients (26.6%) in this group. On the basis that most renal parenchymal damage occurs early we conclude that the current approach to investigation and treatment is reducing the incidence of renal parenchymal damage in this population. Further reduction would require the early identification of the high risk bladder before the onset of hydronephrosis.