AIM The morbidity associated with osteoporosis and fractures in children and adolescents with spina bifida highlights the importance of osteoporosis prevention and treatment in these patients. The aim of this study was to examine the occurrence and pattern of bone fractures in paediatric patients with spina bifida.METHOD We reviewed the data of all paediatric patients with spina bifida who were treated in our centre between 1999 and 2008.RESULTS One hundred and thirteen patients were included in the study (63 females, 50 males; mean age 10y 8mo, SD 4y 10mo, range 6mo-18y). The motor levels were thoracic in six, upper lumbar in 22, lower lumbar in 42, and sacral in 43 patients. Of the 113 patients, 58 (51.3%) had shunted hydrocephalus. Thirty-six (31.8%) were non-ambulatory (wheelchair-dependent [unable to self-propel wheelchair] n=3, wheelchair-independent [able to self-propel wheelchair] n=33), 13 were partial ambulators, 61 were full ambulators, and three were below the age of walking. Fortyfive fractures were reported in 25 patients. The distal femur was the most common fracture site. Statistical analyses showed that patients with higher levels of involvement and in wheelchairs had a significantly increased risk of having a second fracture (p<0.001). Spontaneous fractures were the principal mechanism of injury, and an association was identified between fracture mechanism, type of ambulation, and lesion level: the fractures of patients with higher levels of motor functioning and those in wheelchairs were mainly pathological (p=0.01). We identified an association between risk of a second fracture, higher motor level lesion, and non-ambulation. There was an increased risk of having a second fracture after a previous spontaneous fracture (p=0.004).INTERPRETATION Data in this study indicate a high prevalence of fractures in patients with spina bifida.In congenital paraplegia caused by myelomeningocele, the body lacks the usual axial burden on the legs, muscular activity, and sensation. 1 There is a direct influence of working muscles on the remodelling process of the growing tibia. Postmortem studies of patients with spina bifida reveal a thin and atrophic cortex of the tibia with a diminished number of Haversian systems and large remodelling cavities. 2 In fact, patients with spina bifida have lower bone mineral density and are at risk of pathological bone fractures. 3 The clinical status of children with spina bifida can be significantly worsened by the effects of secondary osteoporosis, including fractures and subsequent further immobilization, and the leg bones of patients with spina bifida have an additional risk of fractures after inactivity (e.g. postoperatively). 1,[4][5][6] Our primary aim in this study was to examine the frequency and characteristics of fractures in paediatric patients with spina bifida. A secondary aim was to identify other variables that may contribute to the presence of fractures, such as neurological level, shunted hydrocephalus, syringomyelia, type of ambulation, mechanism of injury...
The prevalence and morbidity associated with osteoporosis and fractures in patients with spina bifida (SB) highlight the importance of osteoporosis prevention and treatment in early childhood; however, the issue has received little attention. The method for the selection of appropriate patients for drug treatment has not been clarified. Objective: To review the literature concerning fracture risks and low bone density in paediatric patients with SB. We looked for studies describing state-of-the-art treatments and for prevention of secondary osteoporosis. Methods: Articles were identified through a search in the electronic database (PUBMED) supplemented with reviews of the reference lists of selected papers. The main outcome measures were incidence of fractures and risk factors for fracture, an association between bone mineral density (BMD) and occurrence of fracture, risk factors of low BMD, and effects of pharmacological and non-pharmacological treatments on BMD and on the incidence of fractures. We considered as a secondary outcome the occurrence of fractures in relation to the mechanism of injury. Results: Results indicated that patients with SB are at increased risk for fractures and low BMD. Risk factors that may predispose patients to fractures include higher levels of neurological involvement, non-ambulatory status, physical inactivity, hypercalciuria, higher body fat levels, contractures, and a previous spontaneous fracture. Limitations were observed in the number and quality of studies concerning osteoporosis prevention and treatment in paediatric patients with SB. The safety and efficiency of drugs to treat osteoporosis in adults have not been evaluated satisfactorily in children with SB.
Introduction: Children with myelomeningocele (MMC) are usually subjected to multiple surgeries. However, the number and type of surgeries are not the same in every patient with MMC over time. This report summarizes the surgical interventions in a cohort of several ages. Materials and methods: Data on all of the patients with MMC, aged from 1 year and 10 months to 21 years and 11 months, were retrospectively reviewed at the Dona Estefânia Hospital in Lisbon, Portugal. Data were collected by chart review and individual interviews. The factors analyzed were demographics, ambulatory status, neurological level of involvement, shunt status, Arnold-Chiari malformation type II, surgical history, and occurrence of fracture. The surgical interventions were categorized as neurosurgical, orthopedic, urinary, ulcer repair and others. Results: A total of 84 alive were eligible and enrolled. The average age was 14 years and six months. A total of 59 patients received shunts (all but one ventriculoperitoneal). In the study group, the 84 patients required 663 surgeries. Neurosurgical interventions were the most frequent surgical procedure and predominated during the first 2 years of life. Surgical interventions related to shunts were the most common neurosurgical interventions. Orthopedic surgeries were more frequent in the age group 6-12 years. Urological surgeries were done mainly after 6 years of age. Surgical repair of pressure ulcers was more common after 12 years of age. Conclusions: Our study brings to light the complexity of this condition, with multiple surgeries among patients with MMC.
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