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Table 1 Reported cases of GSD with CSF leakage

From: Gorham-Stout disease affecting the spine with cerebrospinal fluid leakage and Chiari-like tonsillar herniation: a rare case report and review of literature

Authors & Year

Age, Sex

Bone Involvement Causing CSF Leakage

Symptoms

Intracranial pressure

Rhinorrhoea

Chiari-like tonsillar herniation

Treatment of CSF leakage

Follow-up /outcome

Reference

Present case

20, M

Thoracic and lumbar spine

Orthostatic headache

Low–high

None

Yes

EBP

1 year, rebound intracranial hypertension, improved

-

Yoshimoto et al. 2018

19, F

Femur and pelvis

Headache, motor weakness, visual acuity loss, papilledema

Low–high

None

Yes

EBP

1 year, improved

[7]

Morimoto et al. 2013

11, F

Right temporal bone (petrous apex)

Vertigo, headache, and pulsatile tinnitus, nausea, hearing loss

High

None

None

Surgical repair (extradural middle fossa approach with temporal fascia flap)

1 year, improved

[8]

Hosoya et al. 2020

25, M

Temporal bone

Otorrhea, meningitis

-

None

None

Infection control and bed rest

Improved

[12]

Aouad et al. 2022

3, F

Temporal bone

Fever, left otorrhea, swelling of the left neck

Low-rebound intracranial hypertension

None

Yes

Surgical treatment (mastoidectomy, fat graft)

Improved, rebound intracranial hypertension

[9]

Nagashima et al. 2017

25, F

Skull base (Right petrous apex)

Meningitis, headache, hearing loss

Normal

None

Yes

An endoscopic endonasal transsphenoidal approach

Follow-up

[13]

Cushing et al. 2010

12, M

Right petrous apex

Headache, nausea, vomiting, meningitis

Low

None

None

Middle ear and mastoid obliteration

6 months, Improved

[14]

Hernández-Marqués et al. 2011

2, M

Temporal bone

Fever, right otorrhea, vomiting, drowsiness, watery otorrhea, meningitis

-

None

None

Surgical interventions (mastectomy, placement of a patch, a lumbar drainage device)

The leakage ceased

[15]

Maroufi et al. 2022

11, M

Skull base

Neck pain

-

Yes

Cranial settling

Conservative management (bed rest, oral acetazolamide)

-

[16]

Fukayama et al. 2022

14, F

Skull base

Fever, neck pain, headache, vomiting, jaw pain, bacterial meningitis

-

None

None

Sirolimus

6 months, no recurrence of meningitis

[17]

Newland et al. 2008

27, M

Skull base

Chronic intermittent clear nasal discharge, headache

-

Yes

None

A sphenoid obliteration, lateral temporal bone resection, the Eustachian tube was obliterated

-

[18]

Nozawa et al. 2016

6, M

Skull base

CSF leakage, hearing loss, facial palsy

-

Yes

Herniation of cerebellar tissue into the internal auditory canal

Sirolimus

3 years, improved

[19]

Morinaga et al. 2022

33, F

The left temporal and sphenoid bones

Recurrent meningitis

-

None

None

Endoscopic endonasal surgery for dural reconstruction

12 months, improved

[20]

Watanabe et al. 2021

16, M

Temporal bone

Nasal discharge, right ear obstruction, fever, headache

Normal

Yes

None

Fistula closure by transmeatal approach

4 years, stable

[21]

Peragallo et al. 2018

18, F

The right temporal bone

Bacterial meningitis

Low–high

None

Yes

Zoledronic acid

Rebound intracranial hypertension

[22]

Yokoi et al. 2020

14, F

T9-10

Thoracic back pain, headache

Low

None

Yes

Dural repair, a blood patch

6 months, improved

[23]

Suero Molina et al. 2014

30, M

T11 vertebral body

Headache

Low

None

Yes

Neurosurgical management by repair of the dura

12 months, improved

[24]

Adler et al. 2011

7, F

Lumbar spine

Headache

Low

None

Yes

3 transforaminal blood patches

6 months, improved

[25]

Iyer et al. 1979

58, F

Skull (calvarial lesion)

Headache, vomiting, and delirium, meningitis

High

Yes

None

-

-

[26]