Gorham‐Stout disease of the temporal bone

Abstract

Gorham-Stout (GS) disease is a rare disease of the bone and is also known as massive osteolysis. Less than 200 cases have been reported in the world literature. A 29-year-old female with a diagnosis of GS disease was identified. She complained of aural fullness and tinnitus bilaterally. Demineralization and moth-eaten changes of the osseous structures of the skull base and posterior fossa were prominent. The left mastoid air cells were opacified and erosion extended to the left jugular foramen, left hypoglossal canal, left stylomastoid process, and left eustachian tube. The radiographic findings and brief literature review are presented.

Gorham–Stout disease of the temporal bone involving the temporomandibular joint

“…There have been a few case reports in the world literature that describe this rare entity involving the temporal bone 3 5 – 7 However, it is notable that only one such case has been associated with sensorineural hearing loss, similar to this report 6 . There are no cases reported on Gorham–Stout disease isolated to the TMJ, although the mandible cortex and zygoma-maxilla complex are known to be involved 8 .…”

“…There is a spectrum of presentations because of the varied osseous deformities associated with Gorham–Stout disease, but the disease is mostly expected to be self-limiting over time. Two reports have also mentioned spontaneous regression of the disease, but this is uncommon 7 9

Gorham–Stout disease can affect any head and neck bony structures Symptoms vary depending on the bone affected, and include hearing loss and temporomandibular joint dislocation This disease should be considered when imaging shows osteolytic involvement Treatment includes conservative, medical, radiotherapy or surgical approaches, depending on disease progression severity

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Gorham-Stout Disease and Cerebrospinal Fluid Otorrhea

“…The affected bones are located mainly in the head and neck and in most of the reported cases the mandible and cervical spine are involved [13]. Only few cases have described involvement of the temporal bone [14]. To our knowledge, no other cases have been reported to present with CSF leakage.…”

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

“… 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 - …”

“…Skull involvement may lead to progressive headache, migraines [ 5 ], nausea, vomiting, otitis media or recurrent episodes of meningitis secondary to chronic cerebrospinal fluid leakage [ 5 , 49 ]. Furthermore, some patients with temporal bone involvement may have auricular fullness, tinnitus, hearing loss or deafness [ 15 , 34 , 37 ]. Vertebral column involvement leading to pathological fractures, spine deformities and/or paraplegia has also been reported [ 7 , 24 , 50 ].…”