Académique Documents
Professionnel Documents
Culture Documents
Case report
A R T I C L E I N F O A B S T R A C T
Article history: Tolosa-Hunt syndrome is an idiopathic chronic granulomatous inammatory process commonly
Received 13 February 2013 involving the cavernous sinus and the orbit [1]. Symptoms include unilateral eye pain, ophthalmoplegia,
Received in revised form 5 April 2013 headache, and facial pain in the distribution of the upper divisions of the trigeminal nerve and are highly
Accepted 12 April 2013
responsive to steroid therapy. Gradenigo syndrome describes extension of a middle ear infection to the
Available online 14 May 2013
petrous apex, with trigeminal pain and ophthalmoplegia, typically responsive to antibiotics and often
surgical drainage. We report a case of a 17 year-old girl with apparent Gradenigo syndrome, presenting
Keywords:
with unilateral eye pain, abducens palsy, headache, hearing loss and serous otitis media, who was
Tolosa-Hunt
Gradenigo
ultimately diagnosed with Tolosa-Hunt syndrome.
Otitis media 2013 Elsevier Ireland Ltd. All rights reserved.
Ophthalmoplegia
0165-5876/$ see front matter 2013 Elsevier Ireland Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijporl.2013.04.023
1220 E. Slattery et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 12191221
Fig. 1. Contrast-enhanced T1-weighted images (A, B, C) and FLAIR images (D) obtained at time of initial presentation. (A) Enhancing soft tissue inltrating the right cavernous
sinus. (B) Enhancing soft tissue extending into Meckels cave on the right. (C) Largest deposit of enhancing soft tissue (white open arrow) centered in the right carotid space,
causing severe narrowing of the right internal carotid artery (black arrow) and internal jugular vein. (D) Right mastoid effusion secondary to obstruction of the right
eustachian tube.
signicant improvement in eye pain or diplopia despite aggressive months of immunosuppressive therapy, the patients abducens
treatment for infection, and cultures from the middle ear uid had palsy was completely resolved. The conductive component of her
not grown any pathogenic organisms. After lengthy discussion, we hearing loss had also resolved, although a high frequency
elected to initiate treatment with high-dose prednisone (120 mg/ sensorineural hearing loss persisted in the right ear. Two months
day). Despite the patients negative laboratory tests, her angio- after her initial presentation, the MRI/MRA was repeated and
graphic ndings raised the remote possibility that her symptoms showed complete resolution of both the critical stenosis of the
resulted from an atypical vasculitis. She was maintained on high- internal carotid artery and the inammatory mass that had
dose prednisone for eight weeks after her follow up visit. Within previously been identied in the orbital apex and cavernous sinus
one week, the patients eye pain and headache improved, and her (Fig. 3B). She presented again 9 months after discontinuation of all
diplopia slowly resolved. After recognizing the patients condition immunosuppresion with recurrent symptoms, and repeat scan
to be steroid-responsive, re-examination of the MRI images led us (Fig. 3C) demonstrates recurrence of the soft tissue mass in the
to entertain the diagnosis of Tolosa-Hunt syndrome. Within two right carotid space with recurrent narrowing of the carotid artery.
Fig. 2. Cerebral angiogram performed at time of initial presentation. Frontal (A) and lateral (B) views of the right internal carotid artery (ICA). Black open arrow shows high-
grade smooth stenosis of the petrous segment of the right ICA. White open arrows show smooth narrowing of the distal cervical segment of the right ICA.
E. Slattery et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 12191221 1221
Fig. 3. Contrast-enhanced T1-weighted images demonstrating temporal changes in the largest focus of enhancing soft tissue involving the right carotid space just lateral to
the right longus coli muscle. (A) Initial presentation. Large enhancing soft tissue lesion centered in right carotid space causing narrowing of the distal cervical portion of the
internal carotid artery and obliteration of the internal jugular vein. (B) 2 months later. Near complete resolution of the enhancing mass with mild residual enhancement and
narrowing of the right cervical internal carotid artery. (C) 11 months after initial presentation (89 months after discontinuation of prednisone). Recurrent soft tissue mass
centered in the right carotid space with recurrent narrowing of the cervical internal carotid artery. (D) Nearly two years after initial presentation. Complete interval resolution
of the soft tissue mass in the right carotid space.
Immunosuppression was restarted, and repeat imaging two years This patient demonstrates a case of mistaken identity. We
after presentation shows complete resolution (Fig. 3D). originally diagnosed an infectious complication of chronic otitis
media, Gradenigo syndrome (also referred to as petrous apicitis) and
2. Discussion characterized by middle ear infection extending to the petrous apex,
ear pain, and ophthalmoplegia. When the patients symptoms did
The rst case describing what is now known as Tolosa-Hunt not improve despite aggressive antibiotic therapy, eye pain became
syndrome was published in 1954, where Tolosa described stenosis of the predominant symptom, and severe carotid stenosis was noted
the internal carotid artery as an integral component of the syndrome on MRA, we turned to other possible diagnoses. In retrospect,
[4]. In this original paper, the diagnostic symptoms of severe, perhaps the nal diagnosis of Tolosa-Hunt syndrome could have
continuous retro-orbital pain preceding or coinciding with ipsilateral been made at the outset, although the presence of an opacied
ophthalmoplegia and spontaneous remission with recurrences over middle ear and mastoid ipsilateral to the patients ophthalmoplegia
a period of months to years without evidence of neurological ndings led us to believe that the eye ndings were a result of pathology
outside of the orbit or cavernous sinus were established. Hunt added within the temporal bone. The otolaryngology literature has
the additional criteria of steroid responsiveness in a subsequent contributed little to the understanding of Tolosa-Hunt syndrome,
paper published in 1961. The utility of MRI in identifying Tolosa- and while the temporal bone ndings and hearing loss in this patient
Hunt syndrome has been integrated into the diagnostic criteria over were likely unrelated to her presenting diagnosis, it would be helpful
the last two decades [1,2]. Primary and secondary MRI ndings have to consider the possibility of Tolosa-Hunt syndrome in others who
been described [3]. Primary ndings include the presence of present with new onset ophthalmoplegia, given the high prevalence
homogeneous cavernous sinus enhancement; local increase in of otitis media in children and eagerness to attribute certain cranial
cavernous sinus size; and thickening and bulging of the dural nerve decits to pathology centered in the middle ear. In this patient,
contour on certain MRI sequences (T1 with gadolinium) in the region the source of her cranial nerve decit did not originate in the ear, but
of Meckels cave, the cavernous sinus, and the orbital apex. Secondary at the orbital apex. Fortunately, her outcome was excellent once
ndings include loss of dural contour, narrowing of the cavernous immunosuppressive therapy was initiated.
portion of the internal carotid artery, and extension of soft tissue
enhancement into the superior orbital ssure with involvement of References
the orbital apex. Ophthalmoplegia and carotid stenosis in Tolosa-
Hunt syndrome are believed to be secondary to extrinsic compres- [1] L.B. Kline, W.F. Hoyt, The Tolosa-Hunt syndrome, J. Neurol. Neurosurg. Psychiatry
71 (2001) 577582.
sion of nerves as they course through the cavernous sinus and orbital
[2] The International Classication of Headache Disorders: 2nd edition. Cephalalgia
apex, in part due to the formation of an inammatory pseudotumor 2004;24 Suppl 1:9-160.
in this conned space. Upon review of the MRI studies in the case [3] B. Schuknecht, V. Sturm, T.A. Huisman, et al., Tolosa-Hunt syndrome: MR imaging
presented above, the carotid artery and the structures of the features in 15 patients with 20 episodes of painful ophthalmoplegia, Eur. J. Radiol.
69 (2009) 445453.
cavernous sinus do in fact appear compressed in the region of bright [4] E. Tolosa, Periarteritic lesions of the carotid siphon with the clinical features of a
enhancement, although this nding was not initially appreciated. carotid infraclinoidal aneurysm, J. Neurol. Neurosurg. Psychiatry 17 (1954) 300302.