Hand function in patients with spina bifida cystica

The Jebsen-Taylor hand function test was performed on 143 patients with myelomeningocele, 8-35 years of age. Compared with normal individuals, matched for sex and age, patients with myelomeningocele demonstrated impaired hand function. Thoracic level and high lumbar level patients exhibited poorer hand function than low lumbar and sacral level patients. Patients who required more than three shunt operations for continued control of hydrocephalus were more likely to have abnormal hand function. Awareness of such deficiencies should enable orthopedic surgeons, therapists, and parents to establish realistic functional expectations and management aims for these children.     

Urological follow-up of adult spina bifida patients

AIMS: The vast majority of the current urological literature understandably has concentrated on the management of children with spina bifida, because in the past the majority did not survive into adulthood. With improvements in the understanding and multidisciplinary care of spina bifida patients it has become a disease of adults. Our aim was to evaluate the current literature to attempt to formulate evidence based guidelines for the management of this difficult group of patients. METHODS: We reviewed the literature on adult urological management of spina bifida, all relevant articles which concentrated on adults and long-term management were studied in full. RESULTS: Renal function may begin/continue to deteriorate into adulthood, becoming the leading cause of adult death. This is thought to occur because of changes in the adult bladder, with increases in storage pressure. Medical and surgical management should aim to preserve renal function as well as the maintenance of continence in the face of the growing and changing urinary tract. Follow-up should be regular and in the context of a specialist multidisciplinary clinic. Despite being unvalidated in the follow-up of adult spina bifida patient's annual serum creatinine, ultrasound and urodynamics are currently the best tools available. CONCLUSIONS: There is no reason why the majority of spina bifida sufferers cannot use their own kidneys for the rest of their lives. This however relies on urological treatment being instigated soon after birth and continuing into adulthood.     

Triple arthrodesis of the foot in spina bifida patients

The feet of 13 spina bifida patients who had undergone triple arthrodesis in adolescence were reviewed at an average of 10 years after operation. Fifteen of 18 feet were considered satisfactory (83%); of the remaining three, two had recurrent planovalgus deformities and one a painful pseudarthrosis. Three feet had required revision of the triple arthrodesis, and there was one postoperative infection. No patient had lost ambulatory status as a result of foot problems and eight of the 10 patients who previously needed calipers were able to discard them or to use lighter ones.     

Coxa vara in spina bifida

In spina bifida the femoral neck can develop either the well-known coxa valga or the hitherto unreported coxa vara. Twenty-three cases of coxa vara in spina bifida are reported. These result from spontaneous separation of the upper femoral epiphysis (10 cases), spontaneous fracture of the femoral neck (three cases) and iatrogenic avascular necrosis of the upper femoral epiphysis (10 cases).     

Charcot arthropathy in spina bifida

This multicenter study was undertaken to identify the prevalence of Charcot arthropathy in the spina bifida population; to evaluate the relationship of neurosegmental level, ambulatory level, and distribution of joint involvement; and to assess treatment results and make treatment recommendations. Sixteen patients were identified with Charcot arthropathy based on clinical and radiographic criteria ranging in age from 9 to 42 years. There were 15 ankles, seven knees, and four hips identified with Charcot arthropathy. Six patients underwent surgery and modification of orthoses, eight had a modification of orthoses only, one had no modification, and one was lost to follow-up. Mean follow-up was 4 years and 9 months (with four good, 17 fair, and five poor results). The best results were seen in 13 compliant patients with a brace modification, whereas poor results were seen in three patients with poor brace compliance. Based on our study, we have noted the prevalence of Charcot arthropathy in spina bifida to be one in 100 cases.     

Epilepsy in patients with spina bifida in the lumbosacral region

This study aimed to assess the relevance of epilepsy and spina bifida in the lumbosacral region. We evaluated 75 patients with spina bifida admitted to the Kyushu University Hospital from 1980 to 2004. Patients were classified as having meningocele (MC, 4 cases), myelomeningocele (MMC, 6), myeloschisis (MS, 45), and lumbosacral lipoma (LL, 20). Nine cases had epileptic disorders, and all showed MS. Meticulous neuroradiological investigations revealed cerebral abnormalities such as polymicrogyria or hypogenesis of the corpus callosum in all epileptic cases. Locations of cerebral abnormalities topographically correlated with areas of interictal EEG abnormalities. Although all epileptic cases had ventriculoperitoneal (VP) shunt for hydrocephalus before the onset of epilepsy, interictal EEG abnormalities could not be explained by location of the VP shunt. In all LL patients, neither history of epilepsy nor cerebral abnormalities were noted on magnetic resonance imaging (MRI). Epileptogenesis in spina bifida patients seemed to correlate with coexisting cerebral abnormalities in MS patients rather than with the VP shunt. However, not all spina bifida patients associated with cerebral abnormalities had epilepsy, and not all cerebral abnormalities were epileptogenic, suggesting that epilepsy in spina bifida patients was multifactorial.     

Femoral neck abnormalities in spina bifida

Femoral neck abnormalities in spina bifida can be of two types: Type A, consisting of widening of the physis and often associated with varus deformity, and Type B, characterized by marked narrowing of the femoral neck, resulting in a typical mushroom appearance. The Type A deformity is usually associated with an abduction contracture of the hip. This physeal lysis seems to be secondary to microtrauma sustained during persistent exercise done by the parents and therapist to overcome the abduction contracture. No treatment is required, even when varus deformity is present. These patients require an orthosis with a pelvic band for ambulation, and their mobility will not be affected by the deformity.