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Table 1 Review of reported cases of cranial involvement in Gorham’s syndrome

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

Reference Age of onset Gender Location Symptoms Treatment F/U outcome
Chiang et al. [19] 46 Male Occipital bone No neurol. Symptoms. - -
Wildförster et al. [21] - female Squamofrontal No neurol. symptoms   -
Zhang et al. [20] 40 Male Parietooccipital region No neurol. symptoms   Stable
Kawasaki et al. [22] 29 Female Left temporal bone, facial mandibular and vertebral bones Pain, hoarsenes, swallowing disturbace, postural instability of the neck and associated dyspnea and dysphagia, deafness and dumbness, left facial palsy, loss of vision At age 34 years radiation therapy (total, 31 Gy) was performed. At age 35 years, further irradiation of the skull base (total, 28 Gy) was tried, but the osteolytic lesion expanded further. At the age of 36 years posterior cranio-vertebral fixation, tracheotomy and gastrostomy were performed because of postural instability of the neck and Death
Chai et al. [23] 38 Male Fronto-parietal - Cranioplasty  
Lo et al. [24] 23 Male Left parietal region No neurol. symptoms Left parietal craniectomy was performed, Reconstructive surgery with artificial bone graft will be scheduled in the next hospital course, 3 months. Stable
Papeix et al. [25] 40 Male Parietal pone No neurol. symptoms Radiotherapy and surgery -
Rao et al. [26] 20 Female Left parietal bone No neurol. symptoms - -
Frankel et al. [27] 14 Female Calvarium Rhinorrhoe, sensorineural hearing loss, immobile left palate, atrophy of the left side of the tongue with fasciculations 2340 cGy in 13 fractions 12 months, stabilisation and sclerosis
Hasegawa et al. [28] 49 Male Left parietal bone Headache Surgery -
Parihar et al. [29] 35 Female Left parietal bone No neurol. Symtoms Left parietal craniotomy, cranioplasty -
Iyer et al. [30] 58 Female Frontal bone Headache, vomiting, delirium, rhinorrhoea, meningitis Pt. refused surgery -
Girisha et al. [31] 16 months Female Calvaria Developmental delay, failure to thrive - -
Kurczynski et al. [32] 14 Female Left orbit, zygoma, mandible, sphenoid, and occiput Left enophthalmia Radiotherapy with 2000 rad to the entire skull, mandible, and upper cervical vertebrae 24 months, no further progression, slight remineralization
Khorsovi et al. [33] 62 Male Occipital bone No neurol. 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 -