Pathological long‐bone fractures in residents with cerebral palsy in a long‐term care facility in South Africa

Abstract

A high incidence of long‐bone fractures has been observed in children and young adults with quadriplegic cerebral palsy in residential care. This study aimed to determine factors that contribute to these fractures and to institute preventive treatment. Twenty individuals (12 males, eight females) of a cohort of 88 residents with spastic quadriplegia in residential care in Gauteng, South Africa who had sustained fractures were compared with a random sample of age‐matched control participants (10 males, 10 females) from the same facility. Participants ranged in age from 6 to 29 years (median 17.5 years). The majority of fractures were in the upper extremities. There was radiological and biochemical evidence of rickets and osteomalacia in both groups. However, the severity of the disease was more pronounced in the group with fractures. There was a significant relation (p=0.002) between the number of fractures and the use of anticonvulsant therapy (ACT). Three months of vitamin D administration (calciferol 5000iu/day) resulted in a marked clinical improvement. There were no fractures during this period in either group. In addition, the mean serum calcium (Ca) and phosphate (Pi)levels increased (Ca from 2.17 to 2.35 mmol/L and Pi from 1.13 to 1.66 mmol/L) and mean total alkaline phosphatase level decreased (from 1123 to 423U/L). We concluded that vitamin D deficiency was the major factor contributing to the occurrence of fractures in this population. Unless sunlight exposure can be guaranteed, vitamin D supplementation should be considered for children and adults in residential care, especially if they are on ACT, even in areas with year‐round sunshine.

Review of Fractures and Low Bone Mass in Children with Cerebral Palsy

“…A study showed that all 20 institutionalized children and young adults with quadriplegic CP and a history of long-bone fractures had radiological and biochemical evidence of rickets or osteomalacia, and vitamin D treatment resulted in marked clinical improvement with no recurrence of fracture during the treatment period. 5 Growth hormone may also be deficient in children with CP. A recent large-scale study of 46 children with CP aged between 3 and 11 years showed that 70% of these children lacked normal growth hormone secretion.…”

“…17 Furthermore, a significant relationship between the number of pathological long bone fractures and the use of AEDs was demonstrated in institutionalized residents with CP. 5 In summary, severe neurological impairment (non-ambulatory status, GMFCS level V), severe joint contracture, a history of fracture, prolonged immobilization (particularly the use of a hip spica), malnutrition (Ryles tube feeding or gastrostomy, low body weight z-score) and use of AEDs are known to be associated with an increased fracture risk in children with CP.…”

“…Multidisciplinary feeding team approach 43 Early use of Ryles tube, surgical correction of gastro-esophageal reflux or gastrostomy Abnormal vitamin D metabolism: Low vitamin D status 5 Use of AEDs 5,9,17,21 Ensure adequate vitamin D intake Vitamin D treatment for rickets or osteomalacia 5 Use of AEDs with the lowest impact on bone metabolism 44 Vitamin D and calcium supplementation in children receiving AEDs 45…”

“…In one study, all 20 children and young adults with CP and pathological long bone fractures showed radiological and biochemical evidence of rickets or osteomalacia. 5 Besides feeding difficulties and inadequate exposure to sunlight, the use of antiepileptic drugs (AED) also contributes to low vitamin D status. Local studies in children with CP showed that 30% of children living in a community had epilepsy 16 and 63% of children living in institutions were taking AEDs.…”

“…4 A higher prevalence rate of 23% was reported in 88 children with quadriplegic CP. 5 The fracture incidence was estimated to be 4.8 per 100 personeyears among one study of 261 children with moderate-to-severe CP. 6 Thus, the fracture incidence in children with CP is much higher than that in the general paediatric population.…”