Gorham-Stout病合并脊柱畸形:1例报告和综述（开放获取）

Gorham-Stout disease with spinal deformity: a case report and literature review (Open Access)

摘要:

Gorham-Stout 病 （Gorham-Stout disease, GSD） 是特发性骨溶解症的一种，罕发脊柱受累。GSD 的发病机制尚不明确，病程多变，诊断和治疗极具挑战性。当骨溶解引发病理性骨折、畸形时，及时进行手术干预可促进新骨形成，控制骨溶解进展。在本报道中，患者以多个腰椎骨溶解合并畸形为特征，接受了药物治疗以及 3 次手术干预。在末次手术时，病椎邻近软组织发生了浸润性破坏，严重的出血迫使笔者简化了手术方案。经过 26 个月的随访，患者的骨溶解得到了控制，脊柱畸形未再进展。多椎骨受累的 GSD 病具有很高的进展潜能，即便畸形较轻，一期长节段固定融合仍是较为稳妥的手术方式。此外， 本文对相关文献进行综述。

Abstract:

Gorham-Stout disease (GSD) is a sort of idiopathic osteolysis disease, in which with involvement of spine is rare. The diagnose and treatment of GSD are particular challenging as the pathological mechanism is currently unclear and the course of disease is variable. When the osteolysis has resulted in pathological fracture or deformity, early surgical intervention is able to facilitate new bone formation and arrest the progression of osteolysis. In this paper, a patient who was characterized by multiple osteolysis of lumbar spine with deformity was treated by medication and three-time surgical interventions. During the last surgery, the soft tissues adjacent to the damaged vertebrae were diffusively destructed. The massive hemorrhage had forced us to simplify the surgical procedure. With a 26-month follow-up, we observed that the osteolysis and the deformity were halted. The progression potential is high in the GSD with multiple vertebrae involved. Even the deformity is mild, one-stage long-segmental fixation and fusion remains a reliable choice.