Surgical Resection of a Cerebral
Arteriovenous Malformation for
Treatment of Superficial Siderosis:
Case Report
Raymond I. Haroun, M.D., Khan W. Li, A.B., and Daniele Rigamonti, M.D.
Department of Neurological Surgery, Johns Hopkins Hospital, Baltimore, Maryland

Haroun RI, Li KW, Rigamonti D. Surgical resection of a cerebral
arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurol 2000;53:554 – 8.
BACKGROUND

To our knowledge, there are only two reported cases of
cerebral arteriovenous malformation associated with superficial siderosis. In both cases, the patients were
asymptomatic and were discovered on retrospective review of magnetic resonance imaging.
CASE DESCRIPTION

We describe a case of superficial siderosis in a 47-year-old
male with a remote history of closed head injury that presented with progressive hearing loss, cerebellar ataxia, and
urinary incontinence. Lumbar puncture was indicative of
active subarachnoid bleeding. Cerebral angiography revealed a small vascular malformation that was resected.
RESULTS

Pathological examination confirmed the diagnosis of the
arteriovenous malformation. Six months after surgery the
patient is neurologically stable with no further progression of clinical signs or symptoms.
CONCLUSIONS

We report the first case of surgical resection of an intracranial arteriovenous malformation for the treatment of
superficial siderosis. We emphasize the necessity of a
detailed evaluation when superficial siderosis is suspected to localize and resect potential bleeding sources,
because the disease is progressive and often irreversible.
© 2000 by Elsevier Science Inc.
KEY WORDS

Arteriovenous malformation, subarachnoid hemorrhage, superficial siderosis.

uperficial siderosis is a rare clinical syndrome
that develops from recurrent subarachnoid
hemorrhage. In cases where a source of bleeding
has been identified, dural abnormalities (47%) and
tumors (35%) are the most commonly associated

S

Address reprint requests to: Dr. Rigamonti, Department of Neurological
Surgery, Meyer 5-181, 600 N. Wolfe St., Baltimore, MD 21287-8811.
Received November 24, 1999; accepted March 6, 2000.
0090-3019/00/$–see front matter
PII S0090-3019(00)00217-2

pathology [4]. Less often, vascular malformations
(18%) have been found to be the source of hemorrhage [4 – 8,10,14,15]. One report in the literature
briefly mentions two cases of cerebral arteriovenous malformations (AVM) associated with superficial siderosis [12]. In both cases, the patients
had no symptoms or signs of the clinical syndrome
and were diagnosed by a retrospective review of
magnetic resonance imaging (MRI) studies.
We report a case of superficial siderosis in a
middle-aged man who presented with progressive
hearing loss, cerebellar ataxia, pyramidal signs, and
urinary incontinence. Characteristic hemosiderin
deposition on magnetic resonance imaging confirmed the diagnosis. Cerebral angiography identified a small right frontal vascular malformation,
which was surgically resected. To our knowledge,
this is the first report of surgical resection of a
cerebral vascular malformation for treatment of superficial siderosis.

Case Report
A 47-year-old man presented to our institution with
an 8-year history of progressive hearing loss and a
5-year history of progressive gait difficulty and urinary incontinence. Past medical history was significant for severe closed head injury from a motor
vehicle accident at 18 years of age that kept him
hospitalized for several months. At that time, the
patient had loss of consciousness and complex orthopedic trauma complicated by a left ulnar neuropathy. No intracranial or spinal procedures were
performed. Initial evaluation of the patient’s hearing loss at a tertiary care center did not reveal the
etiology of the patient’s symptoms. Further diagnostic studies between 1994 and 1996 included MRI
of the brain and spinal cord that showed an incidental small cervical syrinx at C6-7, enlargement of
© 2000 by Elsevier Science Inc.
655 Avenue of the Americas, New York, NY 10010

Siderosis and Vascular Malformations

1

Surg Neurol 555
2000;53:554 –8

Axial T2-weighted MR images at the level of the lateral ventricles (A) and midbrain (B) showing extensive
hypointensity around the brainstem, cerebellar folia, and sylvian fissure, which represents superficial siderosis.

the fourth ventricle, and hypoplastic cerebellar tonsils. Moreover, the T2-weighted MRI of the brain in
1994 revealed superficial hypointensity along the
brainstem, cerebellum, and Sylvian fissures, which
was consistent with superficial siderosis (Figure 1A,
B). The superficial siderosis was thought to be a
result of the patient’s previous trauma and no further diagnostic evaluation or intervention was pursued. In 1998, the patient was referred to our institution for evaluation of his myelopathy.
EXAMINATION
Neurological examination revealed bilateral ptosis,
miosis on the left, and bilateral hearing loss, left
worse than right. In addition, there was loss of
sensation in the lower cervical and upper thoracic
dermatomes, prominent gait ataxia, and hyperreflexia of the lower extremities. A repeat MRI of the
brain and spinal cord revealed extensive hemosiderin deposition in the sylvian fissures and along the
pial surface of the brainstem, cerebellum, and spinal cord. The previously noted cervical syrinx at
C6-7 was unchanged (Figure 2). A diagnosis of superficial siderosis was entertained and further diagnostic evaluation included cerebral angiography,
which revealed a small vascular malformation in

the posterior aspect of the right frontal lobe (Figure
3). A CT myelogram and a detailed spinal angiogram
excluded other potential sources of bleeding (spinal AVM, nerve root avulsion). Lumbar puncture
revealed xanthochromic CSF with 2000 erythrocytes/mm3 and an elevated ferritin level. On the
basis of these studies, the patient was diagnosed
with superficial siderosis, secondary to recurrent
bleeding of a cerebral vascular malformation.
OPERATION
Preoperatively, stereotactic cerebral angiography
was used to localize the lesion. A right frontal craniotomy was performed over the lesion and revealed thickened dura, which was reflected. A xanthochromatic arachnoid membrane lay over the
lesion. Two arterial feeders entered the lesion on its
lateral aspect and converged into one large distal
trunk. On its medial aspect a single draining vein
ran superiorly into the sagittal sinus. After careful
dissection, the feeding and draining vessels were
ligated and the lesion was resected in a core of
tissue measuring 2 ⫻ 1.5 ⫻ 1.5 cm. Intraoperative
angiography confirmed the complete obliteration of
the lesion. Surgical pathology confirmed the diagnosis of arteriovenous malformation.

556 Surg Neurol
2000;53:554 –8

Sagittal T2-weighted MR image of the cervical spinal
cord showing a hypointense rim of hemosiderin
around the spinal cord. Note the atrophy of the cerebellar
folia and the hyperintense syrinx at C6-7 (arrow).

2

POSTOPERATIVE COURSE
The patient had no complications postoperatively.
At 6 months after surgery, the patient has had no
further progression of clinical signs or symptoms.
Subjectively, the patient feels that his symptoms
are improving.

Discussion
Superficial siderosis is caused by recurrent bleeding
into the subarachnoid space that results in the deposition of hemosiderin in the leptomeninges and subpial layers of the brain and spinal cord. A source of
bleeding is found in more than half of all cases [4].
The syndrome is characterized by the clinical triad of
sensorineural deafness (95%), cerebellar ataxia (88%),
and pyramidal signs (76%) [4,13]. Less common findings include dementia, bladder dysfunction, anosmia,
and sensory deficits [4]. Although our patient’s history of hearing loss, ataxia, and urinary incontinence
is classic for the syndrome, the long interval between
initial onset of symptoms in 1990 and the establishment of a diagnosis in 1998 emphasizes the slow progression of the disease and its insidious nature. More-

Haroun et al

over, some of the patient’s clinical findings were
attributed to a remote history of trauma from a motor
vehicle accident, such as the cervical syrinx that may
have caused the suspended sensory levels at C7-T4.
The diagnosis of superficial siderosis is most easily confirmed by MRI. In our patient, a T2-weighted
image revealed the presence of a hypointense rim
along the pial surface of the brainstem, cerebellum,
and spinal cord, that was consistent with the deposition of hemosiderin. This led to cerebral angiography and the localization of the source of bleeding,
a right frontal vascular malformation. Because of
the patient’s history of trauma and the rarity of
cerebral vascular malformations associated with
superficial siderosis, the patient underwent computed tomographic (CT) myelography and complete spinal angiography to exclude other sources
of bleeding. There have been several reports in the
literature of superficial siderosis associated with
spinal AVMs [5,14,15] and traumatic dural injury
from nerve root avulsions [2,17].
An aggressive approach to the localization of potential sources of bleeding is warranted for several
reasons. First, the neurological deficits of superficial siderosis are progressive and often irreversible
[4]. Second, there is no effective medical therapy
currently available for the treatment of superficial
siderosis [1,8,13,16]. Desferroxiamine, an iron chelator, and trientine, a copper chelator, have been
used in the treatment of superficial siderosis. Unfortunately, their administration over varying time
periods did little to improve symptoms [4,8,13,16].
Third, surgical ablation of a potential bleeding
source may arrest intermittent subarachnoid hemorrhage [17] and normalize the CSF profile [9]. Although our patient has a remote history of trauma,
the results of the lumbar puncture and evidence of
recurrent bleeding from the cerebral vascular malformation as seen at surgery confirm that this AVM
was the reason for the patient’s superficial siderosis. We recommended surgery as the most likely
therapeutic intervention capable of preventing further decline in the patient’s neurological status.
To our knowledge, there only are 11 reported
cases of aneurysm or vascular malformation causing superficial siderosis in the world literature (Table 1). In three of these cases, the patients had no
clinical symptoms [7,11] and in a fourth case insufficient data were presented to confirm the diagnosis
[3]. The remaining seven cases of symptomatic superficial siderosis are comprised of three patients
with spinal AVMs (two cervical, one thoracic), three
cases of periventricular venous malformations (two
third ventricle, one fourth ventricle), and one case
with an anterior communicating artery aneurysm.

Siderosis and Vascular Malformations

Surg Neurol 557
2000;53:554 –8

A lateral angiogram after
injection of the right internal carotid artery revealing a
posterior frontal vascular malformation (arrow) with a draining vein to the sagittal sinus
(arrowhead).

3

Given the propensity of many CNS lesions to
bleed, it is surprising that symptomatic superficial
siderosis is not more common. The rarity of symptomatic superficial siderosis is emphasized by Offenbacher’s radiographic review of 8843 consecutive head MRIs covering a seven-year time period. In
that study, only 13 patients (0.15%) had MRI evidence of superficial siderosis, and of those patients
only two (0.023%) were symptomatic [12]. The rarity of superficial siderosis is explained by its
etiology— chronic low-grade intermittent bleeding

1

over many years leading to the deposition of hemosiderin in the subpial layers of the CNS.
Unfortunately, it is impossible to determine
which patients with CNS lesions are likely to develop superficial siderosis. Given the rarity of the
disease, our case illustrates that an aggressive approach to the localization and resection of potential
bleeding sources is warranted in the treatment of
superficial siderosis. This case also is unique in that
a vascular malformation of the cerebral convexity is
the etiology of the superficial siderosis. Moreover,

Vascular Malformations and Aneurysms Associated with Superficial Siderosis

YEAR

AUTHOR

TYPE

LOCATION

NOTES

1. 1947
2. 1962
3. 1967
4. 1969
5. 1969
6. 1983
7. 1988
7. 1991
8. 1996
9. 1996
10. 1996
11. 1998

Cammermeyer
McGee et al.
Foncin et al.
Hughes, Oppenheimer
Hughes, Oppenheimer
Pinkston et al.
Koeppen, Dentinger
Parnes, Weaver
Offenbacher et al.
Offenbacher et al.
Hashimoto, Hoyt
Schievink et al.

Aneurysm
Venous malformation
Spinal AVM
Aneurysm
Aneurysm
Spinal AVM
Posterior fossa AVM
Posterior fossa AVM
Cerebral AVM
Cerebral AVM
Cavernous angioma
Spinal AVM

Vertebral
Thalamus-periventricular
Thoracic
Anterior comm. artery
Middle cerebral artery
Cervical
Inferior medullary velum
Inferior medullary velum
Lateral ventricle
Not specified
Lateral ventricle
Cervical

Insufficient clinical data

Asymptomatic
Same case reported
in two articles.
Asymptomatic
Asymptomatic

558 Surg Neurol
2000;53:554 –8

Haroun et al

it is the first report of resection of a cerebral arteriovenous malformation for the treatment of superficial siderosis.
The authors would like to thank Richard Clatterbuck, M.D.,
Ph.D. and Pamela Talalay, Ph.D. for thoughtful review of the
manuscript.

REFERENCES
1. Arnaud A, Hermosilla E, Ferrer X, Devoize JL, Rajabally Y, Lagueny A. Case of superficial hemosiderosis
of the central nervous system treated with trientine.
Rev Neurol 1998 (Fr);154:243–5.
2. Bonito V, Agostinis C, Ferraresi S, Defanti CA. Superficial siderosis of the central nervous system after
brachial plexus injury. J Neurosurg 1994;80:931– 4.
3. Cammermeyer J. Deposition of iron in paraventricular areas of human brain in hemochromatosis. J Neuropathol Exp Neurol 1947;6:111–27.
4. Fearnley JM, Stevens JM, Rudge P. Superficial siderosis of the central nervous system. Brain 1995;118:
1051– 66.
5. Foncin JF, Gaches J, Saimot L, Wolinetz E. A case of
marginal hemosiderosis of the central nervous system secondary to medullary angioma. Rev Nuerol
1967 (Fr);116:685–90.
6. Hashimoto M, Hoyt WF. Superficial siderosis and episodic fourth nerve paresis. Report of a case with
clinical and magnetic resonance imaging findings.
J Neuro-Ophthalmol 1996;16:277– 80.
7. Hughes JT, Oppenheimer DR. Superficial siderosis of
the central nervous system: A report of nine cases
with autopsy. Acta Neuropathol (Berl) 1969;13:56 –74.
8. Koeppen AH, Dentinger MP. Brain hemosiderin and
superficial siderosis of the central nervous system.
J Neuropathol and Exp Neurol 1988;47:249 –70.
9. Matsumoto S, Kang Y, Sato S, Kawakami Y, Oda Y,
Araki M, Kawamura J, Uchida H. Spinal meningeal
melanocytoma presenting with superficial siderosis
of the central nervous system. Case report and review of the literature. J Neurosurg 1998;88:890 – 4.
10. McGee DA, Van Patter HJ, Morotta J, Olszewski J.
Subpial cerebral siderosis. A report of two cases.
Neurology 1962;12:108 –13.
11. Offenbacher H, Fazekas F, Reisecker F, Schmidt R,
Payer F, Lechner H. Superficial siderosis of the spinal
cord: A rare cause of myelopathy diagnosed by MRI.
Neurology 1991;41(1987– 89).
12. Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G. Superficial siderosis of the central nervous
system: MRI findings and clinical significance. Neuroradiology 1996;38:S51– 6.

13. Parnes SM, Weaver SA. Superficial siderosis of the
central nervous system: A neglected cause of sensorineural hearing loss. Otolaryngol Head Neck Surg
1992;107:69 –77.
14. Pinkston JW, Ballinger WE, Lotz PR, Friedman WA.
Superficial siderosis: A cause of leptomeningeal enhancement on computed tomography. J Comput Assist Tomogr 1983;7:1073– 6.
15. Schievink WI, Apostolides PJ, Spetzler RF. Surgical
treatment of superficial siderosis associated with a
spinal arteriovenous malformation. J Neurosurg 1998;
89:1029 –31.
16. Stevens I, Petersen D, Grodd W, Poremba M, Dichgans
J. Superficial siderosis of the central nervous system.
A 37-year follow-up of a case and review of the literature. Eur Arch Psychiatry Clin Neurosci 1991;241:57–
60.
17. Tapscott SJ, Eskridge J, Kliot M. Surgical management
of superficial siderosis following cervical nerve root
avulsion. Ann Neurol 1996;40:936 – 40.

COMMENTARY

This case report by Haroun et al provides a concise
review of superficial siderosis. The authors illustrate the comprehensive radiographic assessment
(MRI studies of the brain and spinal cord, cerebral
and spinal angiography, and CT myelography) that
is necessary for the evaluation of these patients.
However, radiographic testing may be unrevealing
in nearly 50% of patients, and other causes should
be considered [2]. Superficial siderosis may be
asymptomatic. This report serves as a reminder
that superficial CNS siderosis needs to be considered in patients with bilateral sensorineural hearing
loss [1] as well as ataxia and pyramidal signs.
Thomas J. Leipzig, M.D.
Neurosurgeon
Indianapolis, Indiana
REFERENCES
1. Hsu WC, Loevner LA, Forman MS, Thaler ER. Superficial siderosis of the CNS associated with multiple cavernous malformations. AJNR 1999;20:1245– 8.
2. Mascalchi M, Salvi F, Pirini MG, D’Errico A, Ferlini A,
Lolli F, Plasmati R, Tessa C, Villari N, Tassinari CA.
Transthyretin amyloidosis and superficial siderosis of
the CNS. Neurology 1999;53:1498 –1503.

ou are a professional; you are a physician. Act, dress, and
think like one.

Y