AB0775 MANY RARE AND SERIOUS LOCALIZATIONS IN ONE CASE WITH HEREDITARY EXOSTOSES
نویسندگان
چکیده
Background: Multiple hereditary exostoses (MHE) is an inherited disorder of bone growth. People who have MHE grow or boney bumps on their bones that vary in size, location and number. Some localizations are rare but potentially serious such as ribs, spine pelvis due to proximity important structures. Objectives: The aim this case report was illustrate the clinical radiographic features some MHE. Methods: We reporting a thirty one-year-old male with severe Results: A affected by multiple exostoses., not followed up, came our consultation complaining about chest pain. On examination, he walks limp because unequalness his limbs.The osteoarticular examination revealed numerous palpable osseous masses at distal proximal end 2 arms, forearms, thighs legs right iliac wing. Neurological vivid deep tendon reflexes, bilateral positive Hoffmann’sign Babinski reflex associated hemiparesis. pulmonary exam normal. Medullary MRI showed medullary compression spinal cord atrophy sign myelopathy c1 c2 segment. ct –scan exostosis, two largest lesions were located second rib measuring 45mm * 35mm axially 52mm height compromising blood vessels, retroperitoneal growing mass 126 94 mm 100 leading entrapment axial skeleton abdominal organs. It also confirmed thickening cartilage cap suggestive malignant chondrosarcomatous transformation. Conclusion: Exostoses most common benign tumors. Its complications can cause functional disability even vital risk. References: [1]Eke GK, Omunakwe HE, Echem RC. Hereditary 15-year-old boy: review literature. Niger J Paediatr. 2016;43(4):295. Disclosure Interests: None declared
منابع مشابه
Acetabular dysplasia associated with hereditary multiple exostoses. A case report.
Hereditary multiple exostoses is an autosomal dominant disorder characterised by multiple osteochondromata, most commonly affecting the forearm, knee and ankle. Osteochondromata of the proximal femur have been reported to occur in 30% to 90% of affected patients with coxa valga in 25%. Acetabular dysplasia is rare but has been described. This is the first report of a patient requiring surgical ...
متن کاملHereditary multiple exostoses.
Hereditary multiple exostoses (HME) is a skeletal disorder which primarily affects enchondral bone during growth. It is characterised by multiple exostoses, usually arising in the juxtaepiphyseal region of the long bones. Exostoses that affect "almost every bone in the body" were first mentioned by John Hunter in his Lectures on the principles of surgery in 1786.1 The first family affected by H...
متن کاملManifestations of hereditary multiple exostoses.
The solitary osteochondroma, a common pediatric bone tumor, is a cartilage-capped exostosis. Hereditary multiple exostosis is an autosomal dominant disorder manifested by the presence of multiple osteochondromas. Linkage analysis has implicated mutations in the EXT gene family, resulting in an error in the regulation of normal chondrocyte proliferation and maturation that leads to abnormal bone...
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ژورنال
عنوان ژورنال: Annals of the Rheumatic Diseases
سال: 2021
ISSN: ['1468-2060', '0003-4967']
DOI: https://doi.org/10.1136/annrheumdis-2021-eular.3296