Unidad Médica de Alta Especialidad, Servicio de Ortopedia Pediátrica, Hospital de Pediatría "Dr. Silvestre Frenk Freund", Centro Médico Nacional "Siglo XXI", Instituto Mexicano del Seguro Social, Mexico City, Mexico.
Unidad de Investigación en Epidemiología Clínica, Hospital de Pediatría "Dr. Silvestre Frenk Freund", Centro Médico Nacional "Siglo XXI", Instituto Mexicano del Seguro Social, Avenida Cuauhtémoc 330. Colonia Doctores Delegación Cuauhtémoc C.P, 06720, Mexico City, Mexico.
BMC Pediatr. 2019 Jun 24;19(1):206. doi: 10.1186/s12887-019-1561-0.
Gorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis (variant of idiopathic nonhereditary osteolytic disease) with a slow progression, which is self-limiting for some years. It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix. The aim of this study is to present the clinical features of this disease, as well as the importance of prompt diagnosis and treatment, with a review of the reported cases.
We describe two cases of Gorham-Stout disease between 2013 and 2017 with surgical interventions, follow-up and results. Case one involves an 11-year-old male with involvement of the left iliac bone, with adequate evolution after a surgical procedure with a lyophilized cadaveric tricortical bone allograft. Case two involves a 6-year-old male with cervical spine C1-C3 repercussion; in the protocol for surgical treatment, he presented with signs of spinal cord compression and died.
Diagnosis of Gorham-Stout disease is made by exclusion, and its clinical presentation varies widely, from spontaneous remission to a fatal outcome.
Gorham-Stout 病是一种罕见的疾病，其特征为单发且广泛的 IV 型溶骨性病变（特发性非遗传性溶骨性疾病的一种变异），进展缓慢，在数年内具有自限性。它的特征为复发性血管肿瘤，破坏解剖结构，血管通道在骨内增殖，导致骨基质的破坏和吸收。本研究的目的是介绍这种疾病的临床特征，以及及时诊断和治疗的重要性，并对报道的病例进行回顾。
我们描述了 2013 年至 2017 年间的两例 Gorham-Stout 病病例，均接受了手术干预、随访和结果评估。病例一为 11 岁男性，左侧髂骨受累，在接受冻干同种异体三皮质骨移植的手术治疗后，病情得到了充分的缓解。病例二为 6 岁男性，颈椎 C1-C3 受累；在手术治疗方案中，他出现了脊髓压迫的迹象，并死亡。
Gorham-Stout 病的诊断是通过排除法确定的，其临床表现差异很大，从自发性缓解到致命结局都有可能。
