Osteomyelitis in an Osteopathia Striata with Cranial Sclerosis Patient
نویسندگان
چکیده
Osteopathia striata with cranial sclerosis (OS-CS) is characterized by linear bone dysplasia at the long bone radiographically and sclerotic change at the cranium. The purpose of this case report is to study the symptoms and treatments of osteomyelitis in a patient with OS-CS. A 41-year-old patient had pus discharge from a fistula at the mental region and increase in radiolucencies with sequestra in panoramic radiograph images. Computed tomography (CT) as well as radiograph images for the whole skeleton were taken. The patient was diagnosed with OS-CS. Sequestrectomy and fistulectomy were performed. The patient recovered and no relapse occurred within six months after surgery. For diagnosis of OS-CS, CT and additional radiograph images for the whole skeleton are required. Because of the increased bone density, this patient is prone to relapse after sequestrectomy. Therefore, the surgeon must minimize trauma with the least incision and exfoliation, and preoperative antibiotics.
منابع مشابه
Osteopathia striata congenita with cranial sclerosis and intellectual disability due to contiguous gene deletions involving the WTX locus.
Osteopathia striata congenita with cranial sclerosis (OSCS) is a skeletal dysplasia caused by germline deletions of or truncating point mutations in the X-linked gene WTX (FAM123B, AMER1). Females present with longitudinal striations of sclerotic bone along the long axis of long bones and cranial sclerosis, with a high prevalence of cleft palate and hearing loss. Intellectual disability or neur...
متن کاملCT of the temporal bone in a patient with osteopathia striata and cranial sclerosis.
The authors present a case of congenital dysplasia affecting the long bones, skull, and other systems in a 7-year-old girl, with special attention to CT of the temporal bone, which showed abnormal ossicle fixation, a narrowed Eustachian canal, thickened sclerotic bone, and a small mastoid antrum and middle ear cavity. CT of the temporal bone can help one distinguish the etiologies of hearing lo...
متن کاملWTX R353X mutation in a family with osteopathia striata and cranial sclerosis (OS-CS): case report and literature review of the disease clinical, genetic and radiological features
Osteopathia striata with cranial sclerosis (OS-CS) or Horan-Beighton syndrome is a rare X-linked dominant inherited bone dysplasia, characterized by longitudinal striations of long bones and cranial sclerosis. Patients can be asymptomatic or present with typical facial dysmorphism, sensory defects, internal organs anomalies, growth and mental retardation, depending on the severity of the diseas...
متن کاملPrenatal diagnosis of osteopathia striata with cranial sclerosis.
Osteopathia striata with cranial sclerosis (OSCS) is a rare X-linked dominant genetic disorder resulting mutation in the WTX gene. Clinically, OSCS presents with linear striations in the metaphyseal region of the long bones and pelvis in combination with sclerosis of the cranium and face. A twenty-seven year old G5T1P3A0L2 woman with a history of periand neonatal infant male deaths was referred...
متن کاملOsteopathia Striata with Concomitant Fibroblastic Osteosarcoma of the Femur. A Potential Malignant Risk?
We report a case of a 33-year old female with an unusual presentation of high-grade osteosarcoma superimposed on osteopathia striata. Few cases of malignancy arising in sclerosing bone dysplasia have been reported in literature. Of the few reported ones, osteosarcoma was associated with melorheostosis in two cases, with osteopoikilosis in the one case, and with combined melorheostosis and osteo...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 36 شماره
صفحات -
تاریخ انتشار 2014