Zhang Hanwen, Han Chaofan, Pang Daming, Yong Hai, Yang Jincai, Yin Peng, Zhou Lijin
Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.
Front Surg. 2022 Aug 8;9:981025. doi: 10.3389/fsurg.2022.981025. eCollection 2022.
Gorham-Stout syndrome is an uncommon condition with a varied clinical presentation and unclear cause that is characterised by a proliferation of lymphatic capillaries and severe regional osteolysis. Spinal and visceral involvement increases the syndrome's morbidity and mortality rates. Here, we report about a male patient with Gorham's disease who developed local kyphosis and neurological disorders due to massive osteolysis.
A 13-year-old male patient presented with progressive kyphosis and massive osteolysis of the thoracic vertebrae. Halo-pelvic traction and vertebral column resection osteotomy were performed to reconstruct the spine and prevent disease progression. The entire lesion was resected, and an artificial vertebra filled with allograft bone was used to achieve temporary stability. Although the patient presented with chylothorax following surgery, which required thoracic drainage, the patient did achieve a satisfying outcome.
Limited by the number of GSS cases with spinal involvement and chylothorax manifestations, halo-pelvic distraction as a preoperative preparation and vertebral column resection osteotomy provide a novel avenue for managing this disease.
戈勒姆-斯托特综合征是一种罕见疾病，临床表现多样，病因不明，其特征为淋巴管增生和严重的局部骨质溶解。脊柱和内脏受累会增加该综合征的发病率和死亡率。在此，我们报告一名患有戈勒姆病的男性患者，因大量骨质溶解而出现局部脊柱后凸和神经功能障碍。
一名13岁男性患者出现进行性脊柱后凸和胸椎大量骨质溶解。进行了头盆牵引和脊柱切除截骨术以重建脊柱并防止疾病进展。切除了整个病变组织，并使用填充同种异体骨的人工椎体实现临时稳定。尽管患者术后出现乳糜胸，需要进行胸腔引流，但患者最终取得了满意的结果。
受脊柱受累和乳糜胸表现的戈勒姆-斯托特综合征病例数量限制，头盆牵引作为术前准备和脊柱切除截骨术为治疗该疾病提供了一种新途径。
