Gorham-Stout Disease and Cerebrospinal Fluid Otorrhea

Abstract

Objective and Importance: Gorham-Stout disease is a rare entity characterized by vascular proliferation causing local destruction of bone tissue. Owing to its low incidence and variable clinical presentation, the diagnosis requires a high degree of awareness by the clinician. Clinical Presentation: We present the case of a 2-year-old boy diagnosed of Gorham-Stout syndrome with involvement of the temporal bone and secondary cerebrospinal fluid (CSF) leakage. Intervention: Because of the CSF leakage, the patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased. Conclusion: Gorham-Stout disease is a rare condition that can affect the skull base and even present with CSF leakage.

Intracranial hypotension and hypertension: reversible Chiari malformation due to dynamic cerebrospinal fluid abnormalities in Gorham-Stout disease. Case report

“…Our literature search found only 13 patients with GSD who presented with abnormalities in CSF and intracranial pressure ( Table 1). [1][2][3]5,[7][8][9]11,12,[14][15][16]18 In most patients, a CSF leak was identified from the osteolytic skull base bone. There has been no case report of a patient with GSD complicated by a CSF leak from the thigh.…”

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.…”

Complex single step skull reconstruction in Gorham’s disease - a technical report and review of the literature

“…symptoms Total of 4000cGy 24 months, arrest of disease process with new bone formation Mawk et al [ 18 ] 7 Male Right skull base and cervical spine Neck pain, lymphatic fluid within middle ear spaces and paranasal sinuses. Surgery, 4140 cGy 3 months, no clinical or radiological progression Plontke et al [ 34 ] 54 Female Skull Base Right hearing loss Cranio-cervical stabilisation, radiation total 30,6 Gy 8 months, no clinical or radiological progression Girn et al [ 15 ] 2 Female Skull base Clinical signs mimicking raised intracranial pres- sure and deafness Halo application disease process did not respond to palmidronate and radiotherapy ( Five courses of radiotherapy with a dose of 35Gys in 20 fractions) Continuous disease process, death Schiel et al [ 35 ] 14 Female Posterior wall of the maxillary sinus, the orbit and base of the skull as fas as the apwx of the os petrosus Right maxillary pain Removal of right palatal mucoperiosteum and 40 Gy total 77 months, No evidence of further bone lysis Hernández-Marqués et al [ 36 ] 2 Male Temporal bone Secondary cerebrospinal fluid (CSF) leakage Patient required two surgical interventions. The second intervention included mastectomy and placement of a patch and a lumbar drainage device during 50 days, after which the leakage ceased - Mowry et al [ 37 ] 29 Female Left temporal bone Intermittent aural fullness, egophony, tinnitus bilaterally - - Tsutsumi et al [ 38 ] 82 Female Bilateraly parietal regions Painless scalp depressions Open biopsy for histological verification - …”