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1/6. Ossification of the posterior longitudinal ligament in vitamin d-resistant rickets: case report and review of the literature.

    STUDY DESIGN: A case report of cervical myelopathy caused by ossification of the posterior longitudinal ligament in a patient with vitamin d-resistant rickets is presented together with a review of literature. OBJECTIVE: To report the diagnosis of ossification of the posterior longitudinal ligament in a white woman with vitamin d-resistant rickets. SUMMARY OF BACKGROUND DATA: The association between ossification of the posterior longitudinal ligament and untreated vitamin d-resistant rickets has been reported in japan, but infrequently in white populations. In whites, ossification of the posterior longitudinal ligament is closely associated with diffuse idiopathic skeletal hyperostosis. A clear association between ossification of the posterior longitudinal ligament and vitamin d-resistant rickets in white populations has not yet been established. methods: The medical record and imaging studies of a patient treated at the authors' institution for cervical myelopathy caused by ossification of the posterior longitudinal ligament in the setting of treated vitamin d-resistant rickets were reviewed. A medline search of the medical literature between 1966-1999 was performed to identify pertinent studies and similar case reports. RESULTS: The occurrence of spinal stenosis in untreated adults with vitamin d-resistant rickets has been reported in all regions of the spine in Japanese patients. The association between ossification of the posterior longitudinal ligament and untreated vitamin d-resistant rickets was first reported in japan, where ossification of the posterior longitudinal ligament is endemic. This association may be incidental, because reports on ossification of the posterior longitudinal ligament in whites are not as frequent as in Japanese, reflecting the higher prevalence of this condition in japan. CONCLUSION: Ossification of the posterior longitudinal ligament and ossification of the posterior longitudinal ligament associated with deranged calcium or phosphate metabolism may be different pathologic entities sharing a common outcome. Adequate treatment of vitamin d-resistant rickets may not always prevent or reverse ossification of the posterior longitudinal ligament.
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2/6. Cervical spinal cord compression attributable to a calcified intervertebral disc in a patient with X-linked hypophosphatemic rickets: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: X-linked hypophosphatemic rickets is a common inherited phosphate-wasting disorder, but it is a rare cause of spinal cord compression. We present the first reported case of a calcified intervertebral disc causing spinal canal stenosis in X-linked hypophosphatemic rickets. CLINICAL PRESENTATION: A 44-year-old woman presented with paresthesia of her left arm and a loss of grip in both hands. magnetic resonance imaging revealed a calcified intervertebral disc, as well as a posterior osteophytic bar causing marked cervical cord compression at C6/C7. INTERVENTION: An anterior cervical discectomy at C6/C7 and fusion with autologous bone graft were performed. The patient then exhibited significant improvement. CONCLUSION: A review of the 16 published cases demonstrates that thickening of the vertebral laminae, facet joint hypertrophy, and ossification of the intervertebral discs, posterior longitudinal ligament, and/or ligamentum flavum contribute to spinal canal stenosis in X-linked hypophosphatemic rickets. Those changes are caused by the disease itself and are unlikely to be related to long-term vitamin d treatment. Eleven of 16 patients were reported to have experienced favorable outcomes after surgery.
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3/6. Untreated hypophosphatemic vitamin d-resistant rickets with symptomatic ossification of the ligamentum flavum.

    An adult case of untreated hypophosphatemic vitamin d-resistant rickets with symptomatic ossification of the ligamentum flavum in the thoracic region is reported and discussed with regard to the calcium metabolism and the ligamentous ossification. Factors influencing ossification of the ligamentum flavum may be mechanical stress on the spine and an increase in calcium retention.
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4/6. Origin, diagnosis, and treatment of the dental manifestations of vitamin d-resistant rickets: review of the literature and report of case.

    Previous discussions center on early diagnosis, initial treatment, and follow-up therapy for the patient with vitamin d-resistant rickets. Both the medical and dental aspects of treatment for these patients has a long-range effect on the normal developmental patterns. Although treatment is begun at an early age, some rachitic skeletal effects such as minor bowing of the legs and bossing of the skull will invariably be noticed. In patients with controlled rickets the alveolar processes undergo normal development, with apparent normal dental eruption. The poor development and calcification of the alveolus seen in the untreated patient leads to loss of the lamina dura and periodontal ligament of the teeth. patients with resistant rickets possess a functional dentition, although not without inherent defects. Various degrees of fracture and attrition of enamel can be seen, and hypoplasia of dentin is nearly a universal result. Defects extending to the dentinoenamel junction have been shown in repeated cases. Cementum, because of its close relationship with dentin calcification, also appears abnormal. Pulp tissue may undergo abberations of physiology in resistant rickets, although further work in this respect is needed. With respect to the possible dental pathoses seen in this disease, the dental history of the patient with resistant rickets discussed in this report showed that several of the deciduous teeth, possibly the mandibular left second premolar and right first molar, and definitely the maxillary right second premolar and canine and the mandibular left canine had all undergone pulpal degeneration of apparently unknown causation. In the maxillary right second premolar and the mandibular left canine, enamel fractures were clinically and radiographically apparent. However, the maxillary right canine originally had an acute abscess with no defects other than normal, minimal wear facets. No causative factor for its necrosis could be found. Overt enamel fractures in the maxillary right second premolar and the mandibular left canine may have led to microexposures of the pulp with subsequent bacterial pulpal contamination. suppuration present in several of the pulps when first entered during endodontic treatment, as well as chronic fistulas in several areas, support the conclusion that contamination by some means does indeed occur.
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5/6. Neurological involvement in X-linked hypophosphataemic rickets.

    X-linked hypophosphataemic rickets is a familial form of vitamin d resistant rickets in which gross bony and ligamentous changes may occur. Two patients showing severe spinal disease with evidence of spinal cord compression requiring neurosurgical intervention are reported. The management of such lesions may be problematic as cord compression may be found at several levels at presentation, and further difficulties develop after neurosurgical treatment.
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6/6. Thoracic spinal cord compression caused by hypophosphataemic rickets: a case report and review of the world literature.

    vitamin d resistant hypophosphataemic rickets is a rare cause of spinal cord compression. The compression is caused by a combination of thickening of the laminae and calcification of the ligamentum flavum. Modern imaging techniques including CT and MRI provide excellent detail of both the level and degree of compression. MRI is particularly useful for examining the rest of the spinal cord for areas of impending compression and for postoperative follow-up. With careful surgical decompression a full neurological recovery can be achieved.
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