本文選題：先天性脛骨假關(guān)節(jié) + 神經(jīng)纖維瘤病��； 參考：《南華大學(xué)》2016年碩士論文

【摘要】：目的:先天性脛骨假關(guān)節(jié)是一種先天畸形,被列為兒童骨科最大的疑難癥之一。本研究通過資料的整理分析,描述先天性脛骨假關(guān)節(jié)的臨床資料特征。方法:回顧性分析2007年4月至2016年3月來湖南省兒童醫(yī)院治療的先天性脛骨假關(guān)節(jié)患者的臨床資料,包括性別、側(cè)別、假關(guān)節(jié)位置(脛骨上1/3、中1/3、下1/3)、腓骨狀態(tài)(是否有假關(guān)節(jié)、假關(guān)節(jié)位置)、是否合并神經(jīng)纖維瘤病、臨床Crawford分型(I型、II型、III型、IV型)、第一次骨折年齡、分布區(qū)域、是否有神經(jīng)纖維瘤病家族史。結(jié)果:共244例病例、249側(cè)患肢,男170例,女74例。左側(cè)119例,右側(cè)120例,雙側(cè)均發(fā)生者5例。初次就診時平均年齡為42.93±36.019(月)(范圍:16天至17歲),其中初次就診年齡段1歲-2歲,有74例,2歲-3歲年齡段,有44例,6歲-段及3歲-4歲段分別有43例、30例,其它年齡段有53例。假關(guān)節(jié)處第一次骨折的年齡的均值為16.77,標(biāo)準(zhǔn)差為15.867。假關(guān)節(jié)位置在脛腓骨上1/3者4例,在脛腓骨中1/3和脛腓骨下1/3者分別有107例、138例,其中CrawfordⅠ型11例,CrawfordⅡ型22例,CrawfordⅢ型31例,CrawfordⅣ型185例。有腓骨假關(guān)節(jié)者為146例,位置其中以腓骨下1/3最多,有141例,腓骨中1/3及腓骨上1/3分別有3例及2例。來源分布區(qū)域最多的是湖南省的51例,其次為山東省17例。有NF者148例。有NF家族史的病例數(shù)有15例。有、無NF家族史組NF的差異有統(tǒng)計(jì)學(xué)意義(P0.05),男性和女性組NF家族史、側(cè)別、腓骨假關(guān)節(jié)的差異無統(tǒng)計(jì)學(xué)意義(P0.05),男性和女性組的NF差異有統(tǒng)計(jì)學(xué)意義(P0.05)。結(jié)論:湖南省兒童醫(yī)院CPT患者男性多于女性,男女比約2.3:1,2%的病變累及雙側(cè),主要位于脛腓骨中、下1/3,初次就診年齡為42.93±36.019(月),第一次骨折年齡為16.77±15.867(月),60.7%合并NF,6.1%有NF家族史。CPT患者男性與女性的NF的發(fā)生率有差異,有NF家族史與無NF家族史的NF的發(fā)病率存在差異。而男性與女性的側(cè)別構(gòu)成、腓骨假關(guān)節(jié)的分布、NF家族史無差異。
[Abstract]:Objective: congenital pseudarthrosis of tibia is a congenital malformation, which is one of the most difficult diseases in pediatric orthopedics. This study describes the clinical features of congenital pseudarthrosis of tibia. Methods: the clinical data of congenital pseudarthrosis of tibia treated in Hunan Children's Hospital from April 2007 to March 2016 were analyzed retrospectively. Pseudoarticular position (1 / 3 of the upper tibia, 1 / 3 of the middle, 1 / 3 of the lower tibia), fibula status (whether there are pseudoarthrosis, pseudoarthrosis, and neurofibromatosis, clinical Crawford classification, type I, type II, type III, type IV), age of the first fracture, distribution area, Any family history of neurofibromatosis. Results: there were 244 cases (170 males and 74 females) with 249 affected limbs. There were 119 cases on the left side and 120 cases on the right side. The average age of the first visit was 42.93 鹵36.019 (range: 16 days to 17 years old). Among them, the first visit age was 1 year -2 years old, 74 cases were 2 years old 3 years old, 44 cases were 6 years old and 30 cases were 3 years old, and there were 43 cases of 6 years old and 3 years old of 3 years old and 53 cases of other age groups. The mean age of the first fracture was 16.77 and the standard deviation was 15.867. There were 4 cases with 1 / 3 position of pseudarthrosis on tibia and fibula, 107 cases with 1 / 3 of tibia and fibula with 1 / 3 below tibia and fibula, including 11 cases of Crawford type 鈪，

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