95 Case Report: A Rare Case of Juvenile Hyaline Fibromatosis Presenting with Bilateral Acro-Osteolysis
نویسندگان
چکیده
Abstract Aim We report a rare case of juvenile hyaline fibromatosis (JHF) leading to acro-osteolysis bilateral hands in 54-year-old male patient. Method JHF is an autosomal recessive condition resulting from mutation the ANTXR2/ CMG2 gene. It results accumulation amorphous deposits skin and other organs. Other cardinal manifestations include gingival hyperplasia, flexion contracture large joints lytic lesions long bones. This right-handed window cleaner was referred specialist hand unit due debilitating nodular on his hands. X-ray demonstrated apparent osteolysis multiple distal phalanges The patient experiencing difficulties with gripping, barriers completing activities daily living performing competently within occupation. Results Currently, there no cure for this condition. Our underwent total nine operations over 10 years. Key indications surgery included ulcerations or optimising functional aesthetic outcomes. Radiotherapy chemotherapy were also attempted limited beneficial results. Conclusions has highlighted that important differential be considered by surgeons when presented hand. chronic disease requires multidisciplinary team approach addressing both social physical disease. likelihood high. Therefore, careful stratification surgical intervention coupled shared decision-making throughout process fundamental.
منابع مشابه
Idiopathic multicentric osteolysis with acro-osteolysis. A case report.
We report a case of multicentric massive osteolysis. A 52-year-old woman presented with a three-year history of progressive deformities of the hands. She had osteolytic lesions of the metacarpals and metatarsals, and resorption of the terminal phalanges. During follow-up over four years osteolysis spread to affect the ribs, clavicles, mandible, and long bones. There was no family history of any...
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ژورنال
عنوان ژورنال: British Journal of Surgery
سال: 2023
ISSN: ['1365-2168', '0007-1323']
DOI: https://doi.org/10.1093/bjs/znad258.238