Hussain Mohamed Musheer, Arif K Salauddin
Department of Orthopaedics, Yenepoya Medical College, Mangalore, Karnataka, India -575018.
J Orthop Case Rep. 2015 Jan-Mar;5(1):45-7. doi: 10.13107/jocr.2250-0685.253.
There have been fewer than 150 cases of Bizarre Parosteal Osteochondromatous proliferation (Nora Lesions) reported in the literature to date and no significant reports on angular deformities caused by this lesion. Nora's lesion can easily be misdiagnosed as osteochondroma or chondrosarcoma and therefore inappropriately managed. Although this condition classically appears in the second or third decade, we present to you a three year old boy who presented with multiple bony swellings over the hand and feet that caused an angular deformity of the involved digits. In the case report we have detailed the angular deformities and its treatment outcome.
A three year old boy presented with valgus deformity of middle finger of right upper limb with an associated ulnar bony swelling at the level of middle phalanx which was noticed two years before and was progressing rapidly since last three months. On physical examination the swellings were found to be bony hard, midly tender and found to have been causing an angulare deformity of the digits. The initial suspicion was of osteochondromatous lesion however the excision biopsy showed the lesion to be of the rare entity of bizarre parosteal osteochondromatous proliferation. 1 year follow up showed no progression of angular deformity of the operated digits.
Bizarre osteochondromatous proliferation is an entity that can rarely present in children which needs to be identified and tackled early to prevent the onset of deformities. Almost all cases warrant surgical intervention and the type of excision varies with the type of lesion.
迄今为止，文献报道的奇异型骨旁骨软骨瘤样增生（诺拉病变）病例不足150例，且尚无关于该病变所致角状畸形的重要报道。诺拉病变很容易被误诊为骨软骨瘤或软骨肉瘤，从而导致治疗不当。尽管这种疾病典型地出现在第二或第三个十年，但我们在此向您介绍一名三岁男孩，他的手和脚上出现了多个骨肿胀，导致受累手指出现角状畸形。在病例报告中，我们详细描述了角状畸形及其治疗结果。
一名三岁男孩，右上肢中指出现外翻畸形，在中指指骨水平伴有尺侧骨肿胀，该肿胀于两年前被发现，自过去三个月以来迅速进展。体格检查发现肿胀质地坚硬如骨，有轻度压痛，且已导致手指角状畸形。最初怀疑是骨软骨瘤病变，但切除活检显示该病变是罕见的奇异型骨旁骨软骨瘤样增生。1年随访显示，手术手指的角状畸形没有进展。
奇异型骨软骨瘤样增生是一种在儿童中很少出现的疾病，需要尽早识别和处理，以防止畸形的发生。几乎所有病例都需要手术干预，切除类型因病变类型而异。
