AK Firat

E Ferrante

G Zada

Gilberto Ka Kit Leung

J Alvarez-Linera

J Platz

Jenny Ho

Jenny K. S. Pu

JR Couch

R Forghani

S Albayram

WI Schievink

English

PubMed

Crossref

Acta Neurochirurgica

Pituitary enlargement in spontaneous intracranial hypotension—a diagnostic pitfall

Springer - Publisher Connector

LETTER TO THE EDITOR
Pituitary enlargement in spontaneous
intracranial hypotension—a diagnostic pitfall
Gilberto Ka Kit Leung & Jenny Ho & Jenny K. S. Pu
Received: 11 July 2011 /Accepted: 19 July 2011 /Published online: 29 July 2011
# The Author(s) 2011. This article is published with open access at Springerlink.com
Dear Editor
Spontaneous intracranial hypotension (SIH) is an uncom-
mon condition characterized by the depletion of cerebro-
spinal fluid (CSF) volume [3]. Enlargement of the pituitary
gland may occur in SIH due to compensatory engorgement
of the epidural venous plexus, which is a potential
diagnostic pitfall [2]. We report a 46-year-old woman who
presented with a 2-week history of headache and a partial
right abducens nerve palsy. Her pituitary hormonal profiles
were normal except for raised serum alpha-subunit, follicle
stimulating hormone and luteinizing hormone levels,
compatible with her previous oophorectomy.
Magnetic resonance (MR) studies showed an enlarged
pituitary gland, which was then diagnosed as a non-
functioning pituitary macroadenoma. Less emphasis was
placed on other MR findings including bilateral subdural
effusion, pachymeningeal enhancement, and sagging of the
midbrain, which were suggestive of SIH (Fig. 1). Endo-
scopic transsphenoidal surgery was performed, which
revealed a diffusely enlarged pituitary gland. Biopsy
showed no inflammatory or neoplastic changes. Her
headache and diplopia resolved 2 weeks later. A follow-up
MR study showed complete resolution of the pachymenin-
geal enhancement, bilateral subdural effusion, and pituitary
enlargement. The overall picture was that of SIH that had
resolved spontaneously.
SIH is most commonly due to spontaneous CSF
leakage at the lower cervical and upper thoracic spine.
Mechanical stress, meningeal diverticula, and connective
tissue disorders have been identified as potential risk
factors. The estimated incidence is around 5 per
100,000 per year, with a female predominance and a
peak incidence around the 4th decade [8]. Patients may
present with orthostatic headache, nausea, vomiting, neck
rigidity, and cranial nerve palsies [4]. The characteristic
MR findings include pachymeningeal thickening and
enhancement, tonsillar descent into the foramen magnum,
and subdural effusion [8]. As the craniospinal axis
attempts to maintain volume homeostasis in the face of
continuous CSF loss, dural hyperemia and associated
enlargement of the pituitary gland may occur in up to
two-thirds of the cases [6].
The initial MR findings should have raised the
suspicion of SIH in our patient. On retrospective
enquiry, her headache was indeed postural in nature.
We performed surgery with a view to improve her
abducens nerve palsy. The latter was a recognized
feature of SIH, accounting for up to 80% of reported
ocular manifestations of this syndrome [10]. The optic,
oculomotor, trochlear, and trigeminal nerves may also be
affected [1]. Other unusual pituitary manifestations of SIH
include hemorrhage [9] and partial insufficiency [7].
Interestingly, an unusual case of SIH associated with a
pituitary macroadenoma and subdural hemorrhage has
also been described [5].
The majority of patients with SIH improve on conservative
management, and almost all cases with pituitary enlargement
have normalization of gland size on follow-up MR [2]. For
G. K. K. Leung (*) : J. K. S. Pu
Division of Neurosurgery, Department of Surgery, Li Ka Shing
Faculty of Medicine, The University of Hong Kong,
Queen Mary Hospital,
102 Pokfulam Road,
Hong Kong, China
e-mail: gilberto@hkucc.hku.hk
J. Ho
Department of Radiology, Queen Mary Hospital,
Hong Kong, China
Acta Neurochir (2011) 153:2445–2446
DOI 10.1007/s00701-011-1099-x
refractory cases, myelography is the study of choice to
identify the site of spinal CSF leakage, followed by definitive
treatments such as epidural blood patching, percutaneous
placement of fibrin sealant, and open surgical repair.
In summary, SIH should be considered in patients who
present with postural headache and the characteristic MR
findings. Initial conservative management should be adopted,
which is effective in the majority of cases. The present case
illustrates the importance of careful history taking and clinical
examination as well as an awareness of this diagnostic pitfall.
Conflicts of interest None
Open Access This article is distributed under the terms of the
Creative Commons Attribution Noncommercial License which per-
mits any noncommercial use, distribution, and reproduction in any
medium, provided the original author(s) and source are credited.
References
1. Albayram S, Asik M, Hasiloglu ZI, Dikici AS, Erdemli HE,
Altintas A (2011) Pathological contrast enhancement of the
oculomotor and trigeminal nerves caused by intracranial hypoten-
sion syndrome. Headache 51:804–808
2. Alvarez-Linera J, Escribano J, Benito-Leon J, Porta-Etessam J,
Rovira A (2000) Pituitary enlargement in patients with intracranial
hypotension syndrome. Neurology 55:1895–1897
3. Couch JR (2008) Spontaneous intracranial hypotension: the syn-
drome and its complications. Curr Treat Options Neurol 10:3–11
4. Ferrante E, Savino A, Sances G, Nappi G (2004) Spontaneous
intracranial hypotension syndrome: report of twelve cases.
Headache 44:615–622
5. Firat AK, Karakas HM, Firat Y, Kahraman B, Erdem G (2006)
Spontaneous intracranial hypotension with pituitary adenoma. J
Headache Pain 7:47–50
6. Forghani R, Farb RI (2008) Diagnosis and temporal evolution of
signs of intracranial hypotension on MRI of the brain. Neurora-
diology 50:1025–1034
7. Platz J, Glucker T, Gratzl O, Woydt M (2007) Spontaneous
intracranial hypotension: case report with subdural hematomas,
steroid dependency and clinical improvement after myelography.
Zentralbl Neurochir 68:87–90
8. Schievink WI (2006) Spontaneous spinal cerebrospinal fluid leaks
and intracranial hypotension. JAMA 295:2286–2296
9. Spero M, Lazibat I, Stojic M, Vavro H (2011) Normal pressure
form of the spontaneous intracranial hypotension: a case report
with pituitary enlargement and asymptomatic pituitary haemor-
rhage. Neurol Sci PMID 21484357
10. Zada G, Solomon TC, Giannotta SL (2007) A review of ocular
manifestations in intracranial hypotension. Neurosurg Focus 23:E8
Fig. 1 a, b Post-contrast T1-weighted coronal images showing an
enlarged and homogeneously enhancing pituitary gland, and diffuse
pachymeningeal enhancement (arrows); c coronal FLAIR image
showing the bilateral subdural effusion (arrows)
2446 Acta Neurochir (2011) 153:2445–2446


A review of ocular manifestations in intracranial hypotension. Neurosurg Focus

Altintas A

Diagnosis and temporal evolution of signs of intracranial hypotension on MRI of the brain.

Normal pressure form of the spontaneous intracranial hypotension: a case report with pituitary enlargement and asymptomatic pituitary haemorrhage.

Rovira A

Spontaneous intracranial hypotension syndrome: report of twelve cases.

Spontaneous intracranial hypotension with pituitary adenoma.

Spontaneous intracranial hypotension: case report with subdural hematomas, steroid dependency and clinical improvement after myelography.

Spontaneous intracranial hypotension: the syndrome and its complications.

Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.

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Pituitary enlargement in spontaneous intracranial hypotension—a diagnostic pitfall

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