Garvan John D, Trantalis John N
Orthopaedic Department, The Sutherland Hospital, Caringbah, New South Wales, Australia.
ANZ J Surg. 2003 Dec;73(12):1065-7. doi: 10.1046/j.1445-2197.2003.t01-4-.x.
McCune-Albright Syndrome is a rare condition characterized by endocrine abnormalities, precocious puberty, pigmented skin lesions and polyostotic fibrous dysplasia with consequent fractures and limb deformity. Patients with this syndrome might have had multiple operations on a limb and might also have extensive internal fixation in-situ. We review the case of a 41-year-old woman with McCune-Albright syndrome, who presented with a pathological fracture of her left femur below a long plate and screws. Our management of this challenging problem included the use of a retrograde femoral nail, which, because of the need to retain pre-existing internal fixation, had to be locked proximally through a hole in a femoral plate. This technique, combined with reaming, and thus bone grafting of the fracture, and also perioperative infusions of pamidronate, allowed an early recovery and return to premorbid function for the patient. In the present study we detail our technique and discuss its advantages over other possible methods of treatment.
McCune-Albright综合征是一种罕见疾病,其特征为内分泌异常、性早熟、皮肤色素沉着病变以及多骨型纤维性发育不良,进而导致骨折和肢体畸形。患有该综合征的患者可能已对肢体进行过多次手术,且可能还存在广泛的原位内固定。我们回顾了一例41岁患有McCune-Albright综合征的女性病例,她出现了长钢板和螺钉下方左股骨的病理性骨折。我们对这个具有挑战性问题的处理包括使用逆行股骨钉,由于需要保留原有的内固定,该逆行股骨钉必须通过股骨钢板上的一个孔在近端锁定。这项技术,结合扩髓以及由此进行的骨折部位植骨,还有围手术期输注帕米膦酸盐,使患者得以早期康复并恢复到病前功能。在本研究中,我们详细介绍了我们的技术,并讨论了其相对于其他可能治疗方法的优势。