肢骨紋狀肥大（melorheostosis）－病例報告與文獻回顧

MELORHEOSTOSIS-CASE REPORT & REVIEW OF LITERATURE

黃巖華(Yan-Hwa Hwang) ； 周宜宏(Yi-Hong Chou) ； 刁翠美(Chui-Mei Tiu) ； 張遵(Tsuen Chang)

《中華放射線醫學雜誌》 7卷4期 (1982/12) Pp. 323-331

摘要

肢骨紋狀肥大是以滴蠟狀流動性骨質肥厚爲本病之特徵，呈線狀沿著長骨之長軸分佈，在放射線學上有典型之特徵可作確定之診斷，本病頗爲罕見，第一次正式之文獻報告爲1922年Ler及Joanny所提出，其後一直到1975年正式文獻報告之病例不超過二百個肢骨紋狀肥大之病因不明，無家族性傾向，男女兩性發生率相同。四肢長骨最常被侵犯，肋骨、脊椎及頭骨較少被侵犯。被侵犯關節之運動不能及痛爲最常見之症狀。此病之症狀及放射線學上的特徵在小孩及成人不太一樣，小孩關節痛不像成人那樣常見和厲害，在成人之骨肥厚發生在骨膜下(Sub- periosteal Pattern )或骨皮質外(extra cortical Pattern)，而在小孩骨肥厚發生在骨內膜(endosteal Pattern)。骨核子掃描在此病可以見到攝取值之增加，而在斑駁狀骨脆病(Osteopoikilosis)及紋狀骨病(Osteopathia striata)則不見增加，是以核子醫學在必要時可作進一步之鑑別診斷。

並列摘要

Melorheostosis presents as sclerotic bones along part or all of a tubular bone simulating the flow of wax down a burning candle. Roentgen study always confirms the diagnosis. It is a very rare disease. Leri and Joanny presented the first case in 1922. There were altogether less than 200 cases in the literaturell 1975. The etiology is unknown. There is no familial tnedency and no sexual predominance. Usually melorheostosis is limited to the long bones of extremities with less tendency for the ribs, vertebrae and the skull. Limited mobility for the involved joints and pain over the involved areas are the usual symptoms. However the clinical and radiological presentation differs in children and adults. Pain is not so severe and frequent in children as that in the adults. In adults the sclerotic bone change takes place in the subperiosteal and extracortical areas while that in children takes place in the endosteal area. Increase uptake is noted in bone scanning which helps in differentiating melorheostosis from osteopoikilosis and osteopathia striata.