Cerebrospinal fluid leakage in Gorham-Stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine

Abstract

<jats:p>Patients with Gorham-Stout disease (GSD), a rare disease of poorly understood etiopathophysiology, suffer from progressive osteolysis. Destruction of bone matrix is caused by lymphatic vessels, which can lead to CSF leakage if parts of bony structures adjacent to CSF spaces are involved. So far, fewer than 200 patients have been reported in the literature; only 4 of these patients presented with CSF leakage. The authors report the case of a 30-year-old man with GSD and CSF leakage due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine. Neurosurgical intervention, including dura repair, was needed. Experimental medical therapy with rapamycin was started, leading to disease control for more than 12 months. Progression of GSD can lead to destruction of the meninges, causing CSF leakage. The authors review 4 other cases reported in the literature and discuss therapeutic options.</jats:p>

A rare case of Gorham-stout syndrome involving the thoracic spine with progressive bilateral chylothorax: a case report

“…Managing chylothorax in GSS includes irradiation therapies, pleurectomy, pleurodesis, thoracic duct ligation and lymphangiomatous tissue excision [2]. Suero et al [18] recommended pleurodesis for intractable pleural effusion. In our case, chest drainage and thoracic duct ligation combined with pleurodesis were performed to relieve the symptoms due to the refractory chylothorax.…”

Vanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit: A case report and review of literature

“… 6 To the best of our knowledge, 13 cases of GD of spine have been reported after 2005. 8 20 21 22 23 24 25 26 27 28 29 30 31 A brief review of these cases with treatment and outcome has been shown in Table 2 . Out of the total 41 reported cases of GD of spine, cervical spine is the most commonly affected with 11 cases involving only cervical spine and additional 6 cases involving cervicothoracic spine.…”

Increased Intracranial Pressure in a Boy with Gorham-Stout Disease

“…Bony lesions proximate to the dura and surrounding CSF spaces can also result in CSF leaks, manifesting as otorrhea and concomitant meningitis [ 11 ]. In addition, there have been several cases in the literature reporting patients with GSD and CSF leaks with subsequent intracranial hypotension requiring surgical repair [ 3 , 4 , 5 , 6 ], with clinical symptoms to include orthostatic headache, nausea, and vertigo. In 1 of these cases, the authors reported a patient with GSD in whom it was felt that intracranial hypertension was the cause of the patient's subsequent CSF leak [ 12 ].…”

“…Gorham-Stout disease (GSD), also known as vanishing bone disease, is a rare disorder, which most commonly presents in children and young adults and is characterized by an excessive proliferation of lymphangiomatous tissue within the bones [ 1 ]. This lymphangiomatous proliferation most frequently affects the cranium and axial skeletal bones [ 2 ] and, due to the proximate location to the dura surrounding cerebrospinal fluid (CSF) spaces, can also result in CSF leaks manifesting as intracranial hypotension with clinical symptoms to include orthostatic headache, nausea, and vertigo [ 3 , 4 , 5 , 6 ]. We are presenting the case of a boy with GSD who presented with headaches due to increased intracranial pressure.…”