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Gradenigo Syndrome: Case Report and Review of Literature

Article  in  Neurobiologia · March 2009

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________________________________________________________________________________Neto,
Case Report A.R.S.; et AL.

Gradenigo Syndrome: Case Report and Review of

Literature
Ângelo r. Silva Neto1; Mércia J. Bezerra1; Antônio Rafael Galvão2; Thiago A. F. Rocha3; Marcelo Glauber F.
Pereira3; Victor V. Almeida Ferreira3; Maria Cláudia S. Farias3.

ABSTRACT

Gradenigo´s syndrome is a rare complication of otitis media. Its recognition is important to prevent
intracranial complications. It must be take into account in cases of abducens and facial palsy associated with
otologic infection. We report a case of a 8 year-old girl whose clinical history and radiological findings were
compatible with apical petrositis and Gradenigo´s Syndrome. A review of this challenger and serious
condition is timely to neurological community.
Key words: Gradenigo Syndrome; petrous apicitis; Otitis media.

RESUMO

A Síndrome de Gradenigo é uma rara complicação de otite média. Seu reconhecimento é importante
para prevenção de complicações intracranianas. Deve ser lembrada em casos de paralisia dos nervos facial
e abducente associadas com infecção otológica. Relatamos um caso de uma criança de oito anos cuja
história clínica e achados radiológicos eram compatíveis com petrosite apical e síndrome de Gradenigo.
Palavras chave: otite média, osso petroso, síndrome de Gradenigo.

1
Pediatric Neurosurgery – Hospital Infantil Varela Santiago – Natal/RN – Brazil
2
Otorrinolaringologist - Hospital Infantil Varela Santiago – Natal/RN – Brazil
3
Medical Students – Universidade Federal do Rio Grande do Norte

INTRODUCTION CASE REPORT

G radenigo´s syndrome nowadays is a

rare complication of media otitis in
children. There are few cases of this pathology
reported in papers of neurosurgery. We are
reporting a typical case below with conservative
management and a review of literature.
A 8-year-old child was admitted to our
hospital with a complaint of left fronto-temporal
headache and strabismus of twenty days. The
patient had a history of recurring otitis for the
previous two years. The headache was pulsing and
fronto-temporal associated to auricular pain. With

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the emergence of left eye medial deviation and
on examination the patient showed good general
state, a temperature of 37.8 ° C with a significant
left facial palsy, associated with left eye medial
deviation, suggesting the involvement of the cranial
nerve VI (fig.1). Her pupils were equals and reacted
to light. A left otoscopy revealed disruption of the
tympanic membrane with a purulent discharge and
blood lines. The rest of the neurological examination
was normal.
Laboratory tests showed: Hemoglobin:
11.2g/dl; Haematocrit: 33%; Leukocytes:
8.500/mm3 with 51 segmented, 46 lynphocytes, 2
monocytes and 1 Eosinophile; platelet count was
287,000/mm3. Cultures of left ear were normal.
diplopia, the patient sought medical assistance.
The diagnosis of complicated acute otitis media with
petrositis was considered.
A myringotomy with insertion of a
tympanostomy tube was performed on the 3rd day of
hospitalization and an antibiotic treatment initiated
with amoxicillin/clavulanate for 21 days as well as
physiotherapy .There was a gradual symptom
improvement and reversal of deficits in less than 30
days(fig.4).

Fig.2 – Ct scan showing enhancement of left petrous

apex

Fig.1-left ocular palsy

A computed tomographic (CT) scan of the

brain obtained with the administration of contrast
showed inflammatory changes in the left petrous
apex, with dense enhancement on the contrast, and
no signs of intradural dissemination(fig.2). Magnetic
resonance imaging (MRI) of the brain revealed a
low signal in T1-weighted, and a expansile
enhancing , apparently contained by the periosteum,
involving the left petrous apex(fig.3). Slight dural tail
was present. Other cranial structures were normal.
Fig.3 – MRI on T1-Weighted.

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________________________________________________________________________________Neto, A.R.S.; et AL.
Fig.4 – Total recovery of VI nerve palsy.
DISCUSSION
Intracranial complications from adjacent
infections have declined in number during the last
decades (1). Early diagnosis due to more
sophisticated radiological examinations and mainly
due to the appearance and development of potent
antibiotics have produced a considerable reduction
of cases of cerebral empyemas, abscesses, among
other piogenic conditions.
Gradenigo’s syndrome consists of neuro-
logical signals of the involvement of the petrous
apex as a consequence of otitis media. The
incidence rate of acute petrous apicitis has been
reported to occur in two of 100,000 children with
acute otitis media. Giusepe Gradenigo described
the syndrome in 1907 (2), a time when penicillin
had not yet been discovered. The treatment was
always surgical, such as mastoidectomy, external
drainage, myringotomy, and with a high rate of
complications. The clinical signals were
characterized by otitis media, involvement of cranial
nerve VI and the first two divisions of the trigeminal
nerve, leading to paresthesias and retroocular pain,
as well as convergent strabismus with paresis of side
gaze on the affected side. With this classical clinical
form, we can associate other signals, reflecting the
involvement of nerves VIII and VII for example. Even
in Gradenigo’s initial reports, less than a half of the
patients with petrositis presented the classic triad.
The petrous portion the temporal bone has
a triangular shape, with its base facing the mastoid
and the middle-ear, and its apex facing the clivus
and cavernous sinus (3). The mastoid portion has
aerated sections communicating with the middle-ear
(adito-antral) ,and the mastoid (3).The apex is
aerated in 30 to 33% of cases where 60% is filled
with bone marrow. pneumatization is asymmetrical
in 4 to 7% of times. The most apical and medial
portion, however, tends to be more dense. A fold of
medial dura mater of the petrous apex forms
Dorello’s canal, where cranial nerve VI rises toward
the cavernous sinus, related to the petroclinoid
ligament. The ophthalmic and maxillary division of
the trigeminal nerve on the lateral wall of the
cavernous sinus are related closely to the petrous
apex and are commonly affected. The facial nerve in
its mastoid segment can be affected when there is
mastoiditis associated with otitis, reflect a peripheral
facial paresis.
Gradenigo’s syndrome occurs when the
dissemination of an otological infection goes
untreated. In fact it consists of an apical petrositis
which can be acute or chronic. The acute forms
have a more intense pain. The emergence of
the convergent strabismus should quickly lead to
this hypothesis and to the necessity for image
examinations. In chronic forms there is an associa-
tion to prolonged otorrhea, which slightly alleviating
the pain but indicating the perpetuation of the
incorrectly treated infection.
The time between the otitis and onset
of ocular paralysis can vary between 1 week to
3 months, according to the literature. Consequently
many patients may develop the deficit in subtle ways
thus delay the diagnosis. The physical examination
should be directed not only to complaints, but
should also include evidence of cranial nerve
function and in some cases a fundoscopy, taking
into account the possibility of complications that may
develop with intracranial hypertension.
The main isolated agents are Streptococcus
pneumoniae and Pseudomonas aeruginosa. There is
also Proteus mirabilis and Staphylococcus aureus, as
well as mycobacterias. In immunodepressed
patients(7), cases of otitis media can perpetuate and
lead to the development of apical petrositis due to
difficultly treated atypical agents. Even when the
agent is not isolated the use of a broad spectrum of
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antibiotics can treat most of the infections. The
isolation can be difficult, including by culture
methods difficult to cultivate (anaerobic).
A CT scan of the skull is the best
examination, especially with a detailed study of the
temporal bone region. At the start there is
opacification of the aerated cells. In the referred
case, we saw the formation of hypodense collection
with peripheral ring enhancement in the petrous
apex, found from abscessed forms. It also allows
identification of dissemination to the dura mater or
epidural collections when present. In chronic forms
there is erosion of the apical bone, reminiscent of
osteomyelitis.
MRI is complementary to tomography,
particularly useful when there is doubt in diagnosis.
It is characterized by a low signal in T1, with
peripheral enhancement similar to tomography. In
T2 there is a high signal, more intense in chronic
forms, easily recognized when compared to the
usual signal (hyposignal) seen in the mastoid and
petrous apex due to the aerated content. In
situations where the image suggests petrositis or
bone marrow, the fat suppression technique could
be useful (2).
The main differential diagnosis includes
cholesteatoma and mastoiditis. Other diseases
include chondroma, clival chordoma, epidural
abscess, cholesterol cyst and rarely metastases.
When considers of cranial nerve VI palsy, remember
the possibility of a localizing false signal by
intracranial hypertension. This can also happen in
vascular complications such as venous sinus
thrombosis adjacent to the petrous apex. The non-
recognition of Gradenigo’s syndrome can lead to
life threatening intracranial complications such as
epidural empyema of the posterior fossa, cerebral
abscess and cavernous sinus thrombophlebitis.
Currently there is greater tendency for
conservative treatment consisting of hospitalization,
broad-spectrum antibiotics administration and
myringotomy with insertion of pressure equalization
(PE) tubes(10) . The latter allows drainage, with the
help of a tympanostomy, of the collection not only in
the middle-ear and mastoid, but also in the petrous
apex in some cases. Antibiotics should cover both
aerobic and anaerobic bacteria both gram-positive
146
and gram-negative. Whenever possible consider the
cultures, even if the adequate isolation is only
slightly frequent. The improvement of ocular paresis
varies in the literature (4). On average there is a
resolution between the first and second weeks.
Surgical treatment is restricted to refractory
cases, with intense mastoiditis, intracranial
complica-tions and osteomyelitis (2)(10). Patients
that have been inadequately treated with non
culture-directed antibiotics for otitis media can lead
to partially treated infections and to the development
of resistant organisms. Thus they could be consi-
dering to surgery. The approach, if considered,
should remove as much of temporal bone infected
as possible with preservation of hearing and facial
nerve functions. This includes mastoidectomy and
exposition of petrous apex by media fossa.
Circunferential petrosectomy is another option with
low risk of morbity(10).
In view of its rarity as a complication of an
otological disease and its way of presentation
remembers tumoral and surgical lesions, apical
petrositis should be recognized as an entity which
can be treated conservatively thus avoiding costs
and morbidity to a select group of patients. Its
recognition as Gradenigo’s Syndrome is emphasized
here and should be remembered in all cases of
paralysis of cranial nerve VI associated with petrous
lesions.
REFERENCES
1. Sherman SC, Buchanan A. Gradenigo
syndrome: a case report and review of a rare
complication of otitis media. J Emerg Med. 2004
Oct;27(3):253-6.
2. Burston BJ, Pretorius PM, Ramsden JD.
Gradenigo's syndrome: successful conservative
treatment in adult and paediatric patients. J Laryngol
Otol. 2005 Apr;119(4):325-9.
3. Marianowski R, Rocton S, Ait-Amer JL,
Morisseau-Durand MP, Manach Y. Conservative
management of Gradenigo syndrome in a child. Int
J Pediatr Otorhinolaryngol. 2001 Jan;57(1):79-83.
________________________________________________________________________________Neto, A.R.S.; et AL.

4. Villa G, Lattere M, Rossi A, Di Pietro P. Acute positive patient. Arch Intern Med, v.157, n.18, Oct
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Management of Gradenigo Syndrome in a Child. Int
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Streptococcus acidominimus infection in a child
3. 2002.(Lutter, Kerschner et al., 2005)
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