congenital pseudarthrosis of the clavicle

  • 文章类型: Case Reports
    先天性锁骨假关节(CPC)是一种病因不明的罕见疾病,这是由锁骨骨化核联合过程的失败引起的。这是沙特阿拉伯记录的第一个CPC双胞胎实例,第四,总体。在第33周,一对双胞胎过早出生时患有呼吸窘迫综合征。他们都被送进了新生儿重症监护室。拍了胸部的X光片,锁骨畸形被发现了.因为这对双胞胎是通过剖腹产出生的,排除了锁骨外伤性骨折.对家庭成员进行了评估,在父亲身上也发现了同样的缺陷,突出遗传倾向。
    Congenital pseudarthrosis of the clavicle (CPC) is a rare disorder with an unknown etiology, which is caused by a failure of the clavicle ossification nuclei union process. This is the first CPC twin instance documented in Saudi Arabia, and the fourth overall. In the 33rd week, a set of twins was born prematurely with respiratory distress syndrome. They were both admitted to the neonatal intensive care unit. X-rays of the chest were taken, and the clavicular deformity was discovered. Because the twins were born via a cesarean section, a traumatic clavicular fracture was ruled out. The family members were evaluated, and the same defect was discovered in the father as well, highlighting a genetic predisposition.
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  • 文章类型: Case Reports
    先天性锁骨假关节(CPC)很少见。它主要影响右侧,原因不明。大多数报告的病例是在新生儿期以外诊断的。沙特阿拉伯仅报告了两例CPC病例,两者都是在童年时期被诊断出来的。这里,我们介绍了一个沙特男性新生儿的案例,右侧的CPC。由于顺利的剖宫产和无痛性锁骨肿块,因此在出生后不久就做出了诊断。胎儿更喜欢将头部保持在右侧位置,这可能是对CPC中右侧优势的合理解释。
    Congenital pseudarthrosis of the clavicle (CPC) is rare. It predominantly affects the right side for an unknown reason. Most of the reported cases are diagnosed outside the neonatal period. Only two CPC cases have been reported in Saudi Arabia, where both were diagnosed during childhood. Here, we present the case of a Saudi male newborn with right-sided CPC. The diagnosis was made shortly after birth because of the uneventful cesarean delivery and painless clavicular lump. Fetuses prefer keeping their head in a right lateral position which may be a plausible explanation for the right-side predominancy in the CPC.
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  • 文章类型: Journal Article
    背景:先天性锁骨假关节(CPC)是一种罕见的先天性实体,病因和发病机制尚未解决。迄今为止,已报告了近250例病例。CPC的特征是出生时锁骨中部有明确的缺陷,通常在儿童后期或青春期畸形明显时被诊断出来。手术管理是有争议的,尤其是无症状儿童,与文献中报道的各种技术。
    方法:我们报告了一例6岁男孩在小学入学体检时被诊断为CPC的病例。手术治疗包括假关节病清创术,第三管板内固定,和桶形单皮质髂骨自体移植。
    结果:术后9个月获得完整的骨愈合,术后4年和3个月观察到令人满意的功能和外观。
    结论:我们认为,采用桶形单皮质髂棘自体移植重建是治疗CPC的一种有效且可重复的手术技术。
    BACKGROUND: Congenital pseudarthrosis of the clavicle (CPC) is a rare congenital entity with unresolved aetiology and pathogenesis. Nearly 250 cases have been reported to date. CPC is characterized by a definite defect in the mid-clavicle at birth and is usually diagnosed when the deformity becomes evident in late childhood or adolescence. Surgical management is controversial, especially in asymptomatic children, with various techniques reported in the literature.
    METHODS: We report a case of a 6-year-old boy who was diagnosed with CPC during a medical examination for primary school enrollment. Operative treatment included debridement of pseudoarthrosis, internal fixation with third tube plate, and barrel-shaped mono-cortical iliac crest autograft.
    RESULTS: A complete bone union was obtained 9 months after the operation, and satisfactory function and cosmetic appearance were observed 4 years and 3 months postoperatively.
    CONCLUSIONS: In our opinion, reconstruction with barrel-shaped mono-cortical iliac crest autograft was an effective and reproducible surgical technique to treat CPC.
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  • 文章类型: Journal Article
    BACKGROUND: Congenital pseudarthrosis of the clavicle is rare and popular surgical options include excision of the non-union, iliac crest bone grafting and stabilisation with either a fully threaded pin or stabilisation with a reconstruction plate.
    METHODS: Between 1995 and 2009, ten patients with congenital pseudarthrosis of the clavicle were retrospectively reviewed for outcome of two different forms of surgical management. Five patients had excision of the non-union, iliac crest bone grafting and stabilisation with a fully threaded pin (group A) and the other five patients had excision of the non-union, iliac crest bone grafting and stabilisation with a reconstruction plate (group B). One patient in the second group had bilateral pseudarthrosis.
    RESULTS: In group A, three patients achieved radiological union at a median of 6 months. Two patients failed to unite at the pseudarthrosis and one of them had further surgery with a reconstruction plate in order to achieve union. In group B, all five patients (six clavicles) achieved radiological union at a median of 3 months. All patients had painless full range of shoulder movement and were engaging in unrestricted activities.
    CONCLUSIONS: Excision of the pseudarthrosis, autogenous iliac bone grafting and stabilisation with plate achieves union quicker and with lower incidence of complications compared to excision of the pseudarthrosis, autogenous iliac bone grafting and stabilisation with fully threaded pins.
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