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

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

-