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Case Reports in Otolaryngology

Volume 2020, Article ID 9439184, 5 pages
https://doi.org/10.1155/2020/9439184

Case Report
Gradenigo’s Syndrome with Carotid Septic Stenosis

Ana Sousa Menezes ,1 Daniela Ribeiro,1 Filipa Balona,2 Ricardo Maré,3 Cátia Azevedo,1
Jaime Rocha,4 and Luı́s Dias1
1
Department of Otorhinolaryngology-Head and Neck Surgery, Hospital de Braga, Braga, Portugal
2
Department of Pediatrics, Hospital de Braga, Braga, Portugal
3
Department of Neurology, Hospital de Braga, Braga, Portugal
4
Department of Neuroradiology, Hospital de Braga, Braga, Portugal

Correspondence should be addressed to Ana Sousa Menezes; ana4644@gmail.com

Received 13 November 2019; Accepted 4 February 2020; Published 20 February 2020

Academic Editor: Abrão Rapoport

Copyright © 2020 Ana Sousa Menezes et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is
properly cited.

Gradenigo’s syndrome was firstly described in 1907 by Giusseppe Gradenigo and is defined as the clinical triad of suppurative
otitis media, ipsilateral abducens nerve palsy, and pain in the distribution of the first and the second branches of the trigeminal
nerve. Since the advent of antibiotics, the incidence of this potentially life-threatening complication has diminished, but oc-
casional cases still occur. We herein report a pediatric case of otitis media associated with Gradenigo’s syndrome complicated by
ipsilateral septic cavernous sinus thrombosis and infectious arteritis of the internal carotid artery.

1. Introduction 2. Case Presentation

Gradenigo’s syndrome (GS) has always been considered a An 8-year-old girl was admitted to the emergency depart-
classical condition, well known by many but currently seen ment with a 10-day history of left-sided otalgia, otorrhea,
by very few. In fact, GS has become a rare diagnosis in the and ipsilateral hearing loss and new onset strabismus and
modern antibiotic era [1–3]. binocular diplopia since the day of admission. She was under
Although being a rare and relatively forgotten process of paracetamol and ibuprofen every 4 hours since the begin-
years past, GS can be potentially life-threatening with sig- ning of the disease, with fever noticed only in day 4 and had
nificant mortality. Cerebral vascular thrombosis is a serious taken 1 dose of cotrimoxazole without medical prescription.
neurological complication of otitis media and occurs sec- She had been vomiting since the previous night, with no
ondary to spread of the infection to the underlying bone. headache or ocular pain. Her medical history was unre-
To the best of our knowledge, very few case reports have markable. Physical examination revealed suppurative left-
been published describing venous sinus thrombosis in GS, and sided acute otitis media, bilateral conductive hearing loss on
only two reported the commitment of the carotid artery [4–10]. acumetry with tuning forks, ipsilateral abducens nerve palsy
We believe it is of utmost importance to raise awareness (Figure 1), and a fever of 38.0°C. Sensation to light touch and
of these vascular complications of GS, which may allow for pinprick in the distribution of the fifth (trigeminal) cranial
early detection and treatment of one of the most serious nerve was preserved. No other focal neurological deficit was
complications of otitis media. Additionally, this case report present. Meningeal signs were negative.
emphasizes the importance of appropriate radiological in- The patient underwent contrast-enhanced computed
vestigation and timely treatment, which in our case included tomography (CT) which revealed left mastoiditis and a left
surgery and medical therapy. petrous apicitis (apical petrositis) with ipsilateral septic
2 Case Reports in Otolaryngology

Figure 1: Left lateral rectus palsy as demonstrated by the patient’s

inability to abduct her left eye.

cavernous sinus thrombosis and infectious arteritis and

stenosis of the internal carotid artery (Figure 2).
Ophthalmological assessment confirmed left lateral
rectus paralysis (Figure 1), with normal biomicroscopy,
fundoscopy, and normal visual acuity.
Blood tests revealed elevated inflammatory parameters
Figure 2: Axial temporal bone computed tomography (CT) scan
with a white blood cell (WBC) count of 26200/μL with 24500 showing opacification of the left middle ear and mastoid and
neutrophils/μL (93.4%) and C-reactive protein of 88.8 mg/L. expanded fluid-filled petrous apex.
Renal function and clotting were unremarkable.
Magnetic resonance imaging (MRI) showed a diffuse
inflammatory enhancement in the left mastoid air cells and the
petrous apex with extraosseous extension of the inflammatory
process to the petroclival suture and dura adjacent to the clivus
and petrous apex on the same side (Figures 3 and 4). Also,
inflammatory intense enhancement in the left carotid canal
with stenosis of about 50% of the petrous, lacerum, and
cavernous segments, and intense enhancement and swelling of
the cavernous sinus was seen (Figures 3–8).
The patient was diagnosed with GS based on her clinical
presentation, which included acute suppurative otitis media
with involvement of the sixth cranial nerve, and based on the
evidence of petrous apicitis (apical petrositis) on the CT. The
imaging studies also revealed the presence of cavernous
sinus thrombosis and carotid septic stenosis. Other differ-
ential diagnosis of petrous apicitis including congenital
cholesteatoma, intracranial abscess, lateral sinus thrombosis,
cholesterol granuloma, temporal bone osteomyelitis, and
neoplastic or granulomatous disease were excluded Figure 3: Axial STIR sequence magnetic resonance imaging (MRI)
according to the clinical and radiological presentation shows a diffuse bilateral inflammatory involvement of middle ear-
[10, 11]. mastoid with extension of the inflammatory process to petrous
Empirical intravenous antibiotic therapy with ceftriax- apex on the left side.
one (100 mg/kg/day), vancomycin (60 mg/kg/day, with a
need to increase the dose to 90 mg/kg/day to reach thera-
peutic levels), and metronidazole (40 mg/kg/day) was star- She completed 13 days of ceftriaxone (suspended due to
ted. Ofloxacin 0.3% ear drops were also initiated. rash) and 21 days of vancomycin and metronidazol with
Considering the internal carotid arteritis with significant complete resolution of clinical findings. Tapering of meth-
stenosis, corticoid therapy with methylprednisolone (2 mg/ ylprednisolone was uneventful. Antiplatelet therapy with
kg/day) and antiplatelet therapy with aspirin (4 mg/kg/day) aspirin was continued for 3 months.
were decided. The abducens nerve palsy totally disappeared 3 days after
She was submitted to left canal-wall-up mastoidectomy admission.
and myringotomy with tube placement with intraoperative The WBC count and C-reactive protein decreased and
findings of abundant purulent middle-ear effusion and the microbial culture from the middle-ear fluid isolated
granulation tissue in the mastoid cavity and middle ear. Streptococcus pyogenes susceptible to penicillin and eryth-
Postoperative management included intermediate care unit romycin. The blood culture was negative.
monitoring for the first week, followed by transfer to the A contrast-enhanced cranial MRI 10 days after admis-
pediatrics ward. sion revealed partial regression of inflammatory signs in the
Case Reports in Otolaryngology 3

Figure 4: Coronal T2-weighted image shows inflammatory in-

volvement of the left petrous apex and Meckel cavity with petrous Figure 6: Coronal contrast-enhanced T1-weighted image shows a
carotid stenosis. heterogeneous abnormal enhancement of left cavernous sinus.

Figure 7: TOF 3D MRI angiography at admission, showing

Figure 5: Coronal T2-weighted image reveals an expanded and narrowing of the petrous, cavernous, and terminal segments of the
heterogeneous cavernous sinus. left internal carotid artery corresponding to left internal carotid
arteritis.
mastoid cells and left petrous apex with total repermeabi-
lization of the carotid artery (Figure 9) always seen, and symptoms should be taken in the context of
On outpatient follow-up, at 3 months following hospital all the patients’ presenting symptoms, signs, and investi-
discharge, she presented complete clinical and radiological gations [12].
remission, without long-term sequelae. Our patient did not present facial sensation impairment.
The grommet tube was still in place at consultation, with Likewise, in Gradenigo’s original case series of 57 patients,
otherwise normal tympanic membrane. Pure tone audi- more than half of the cases did not follow the classical triad [1].
ometry was also normal at 4 months. Here, we highlight the importance of recognizing the
classical triad and not forgetting GS association with the
3. Discussion septic vascular commitment of the cavernous sinus and
carotid artery.
We report an atypical and potentially life-threatening case of Advances in imaging have assisted diagnosis and
GS. As previously reported, the typical triad of GS is not monitoring of GS [13]. In fact, any child presenting with
4 Case Reports in Otolaryngology

[14]. Recently, some authors have advocated for nonsurgical

intervention with intravenous antibiotic therapy [15]. In our
case, surgical treatment was decided, combined with anti-
biotic therapy due to the severity of clinical presentation
with vascular commitment. Indeed, surgical debridement is
necessary in very severe cases or cases resistant to medical
treatment alone [15]. Another case report by Janjua et al. of a
patient with GS with epidural abscess and internal carotid
arteritis has described a good response to myringotomy and
grommet insertion combined with antibiotic and antiplatelet
therapy [10]. However, the authors considered mastoidec-
tomy unnecessary given the partially opacified mastoid air
cells in the CT, which was not our case.
Regarding the choice of antibiotics for treatment of GS,
most authors advocate for the use of a cephalosporin an-
tibiotic along with metronidazole with or without the ad-
dition of vancomycin [10, 11]. Empirical intravenous
antibiotics should cover common agents involved in bac-
Figure 8: Axial diffusion-weighted imaging revealing the absence terial mastoiditis (Staphylococcus aureus, Streptococcus
of restricted diffusion of water within the cavernous sinus and the pneumoniae, Streptococcus pyogenes, and Pseudomonas
temporal bone, excluding the presence of abscess and aeruginosa) and anaerobic organisms can also be considered
cholesteatoma. [16, 17]. In our case, ceftriaxone, metronidazol, and van-
comycin were chosen preoperatively for empiric broad-
spectrum coverage for the most commonly seen organisms.
Our case report stands out for its relatively short intravenous
antibiotic therapy duration. Previous reports have described
variable intravenous antibiotic therapy between 10 and 64
days [12, 15]. Undoubtedly, petrositis caused by infection is
equivalent to osteomyelitis, which needs intensive and
prolonged antibiotic treatment to avoid relapse [15]. In our
case, we considered the 21 days of antibiotic therapy suf-
ficient given the complete resolution of symptoms in the
days after surgery.
Considering the MRI finding of internal carotid arteritis,
with significant carotid narrowing within the petrous bone,
antiplatelet therapy with low-dose aspirin and corticoid
therapy with methylprednisolone was decided. However, the
decision of using antiplatelet therapy and not using anti-
coagulant therapy despite the associated finding of intra-
cranial venous thrombosis was not taken lightly.
Anticoagulant therapy use is controversial in septic cerebral
venous sinus thrombosis in children [18]. A Cochrane re-
view of anticoagulation for cerebral venous sinus thrombosis
Figure 9: TOF 3D MRI angiography 10 days after treatment reveals
total recalibration of the internal carotid artery.
including two small randomized-controlled trials (79 pa-
tients) found a nonsignificant trend towards reduced death
and disability in the anticoagulated group [19]. Although
there are no pediatric randomized-controlled trials, anti-
acute otitis media with suspected intracranial complication coagulants are often used and seem to be safe and beneficial
should undergo imaging testing. CT scan is the first choice of in cases of cerebral venous sinus thromboses [18–20]. In our
imaging, since it is widely available and detects abnormal- patient, given the presence of carotid arteritis, the increased
ities in bone structures, including destruction of trabecular risk of carotid artery rupture and septic embolism with
bone and erosion of the petrous apex. MRI is more sensitive anticoagulation was a concern, having been decided not to
in detecting dural thickening and enhancement as well as initiate hypocoagulation and the use of antiplatelet therapy
intracranial complications and has the advantage of avoiding instead.
unnecessary radiation to the patient. A MRI angiography
may be performed to rule out signs of sinus thrombosis 4. Conclusion
[4, 13].
The ideal treatment for petrous apicitis is controversial GS is seldom seen in modern medicine, thanks to wide-
and typically depends on the severity of clinical presentation spread antibiotic use for the treatment of acute otitis media.
Case Reports in Otolaryngology 5

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