Surgical management of Gorham‑Stout syndrome involving the cervical spine with bilateral pleural effusion: A case report and literature review

Abstract

Gorham-Stout syndrome (GSS) is a rare disease characterized by spontaneous and progressive osteolysis caused by benign proliferation of lymphatic vessels or capillaries. It most commonly occurs in children or young individuals without any inherited predisposition. GSS most commonly affects the shoulder girdle, pelvis, ribs and skull. Its diagnosis is mainly based on radiological and pathological findings. The present study reports on the case of a 22-year-old male patient diagnosed with GSS involving the C1-T1 vertebrae accompanied by bilateral pleural effusion. Resection of the occipital and cervical vertebral lesions and spinal reconstruction using an internal fixator were successfully performed via the posterior approach. After the surgery, the patient received bisphosphonate treatment and vitamin D supplementation. The pleural effusion gradually decreased. At the 18-month follow-up visit, no evidence of new bone obstruction was present and the patient had no neurological sequelae.

Endoscopic Endonasal Dural Reconstruction for a Cerebrospinal Fluid Leak in the Middle Cranial Fossa of a Patient with Gorham-stout Disease with Skull Base Defect

“…GSD 1 - 11 ) and generalized lymphatic anomaly (GLA) 4 , 8 , 10 ) are rare and intractable diseases of unknown etiology characterized by diffuse lymphoid infiltration of systemic organs and associated with significant morbidity and mortality. GSD, also known as vanishing bone disease , 5 ) is considered related to GLA and may feature similar lesions in internal organs, including osteolytic lesions.…”

“…Gorham-Stout disease (GSD) is a rare disorder characterized by massive osteolysis, usually in a unicentric pattern in isolated bones. 1 - 11 ) It features gradual bone destruction by angiomatosis and overstimulation of osteoclasts and may advance into neighboring soft tissues. The efficacy of surgical treatment for cerebrospinal fluid (CSF) leaks in adults with GSD and skull base defects is uncertain because of the paucity of surgical reports.…”

Utility of the spinal instability neoplastic score to identify patients with Gorham-Stout disease requiring spine surgery

“…[5,15] e progressive nature of the disease necessitates multiple revision surgeries in half of reported cases and complications such as neurologic deficit, chylothorax, and a high rate of mortality are common. [1,4,8,9,15] In patients with spinal lesions, it is critical for the treating physician to identify patients at risk of catastrophic instability. ese patients undoubtedly benefit from the early involvement of a spine surgeon to assess stability and instigate prompt access to surgical care.…”

Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases

“…Spinal lesions can also be managed surgically if the lesion leads to spinal instability, deformity or pain. Posterior spinal stabilization with screws and rods associated with decompression, or anterior stabilization with plate and screws to induce vertebral fusion, can be used in the most severe cases of cervical and thoracic deformities (101,115,117,144,145,146,147,148,149). The use of vertebroplasty has to be considered when the spine is involved, but the osteolytic lesion does not lead to severe deformity or to major instability (98,137).…”

“…Surgery also plays a key role in thoracic complications, such as chylothorax or even minor pleural fluid leakage, which have been treated with pleural drainage (119,149,151,152), pleurectomy, pleurodesis (64,153,154) and/ or thoracic duct ligation or embolization (14,27,65,155). In most of the cases, the best outcome has been obtained by combining these surgical techniques rather than using them in isolation (154,156).…”