Title | Gradenigo Syndrome: Mimicker of Slipped Muscle and Shunt Failure |
Creator | Bonavia L, Jackson J. |
OCR Text | Show Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Gradenigo Syndrome: Mimicker of Slipped Muscle and Shunt Failure Anais Seeds, BS, Sydni L. Coleman, MD, Sasha Strul, MD G radenigo syndrome is classically known as a triad disease consisting of unilateral or bilateral acute otitis media, facial pain, and cranial nerve (CN) 6 palsy (1). This syndrome was first described in 1904 by Guisseppe Gradenigo, who reported symptoms of pain in the trigeminal region, diplopia because of the involvement of the sixth CN, and periostitis (1). It typically arose from untreated progression of otitis media in the postantibiotic era (2). Because of more effective and readily available antibiotics today, Gradenigo syndrome is rarely observed. We present a rare case of bilateral Gradenigo syndrome mimicking slipped muscle poststrabismus repair in a patient with multiple complicating factors to his presentation. Early diagnosis and treatment of this condition prevents further serious complications. A 2-year-old boy with a history of traumatic brain injury and subdural hematomas status postlumboperitoneal (LP) shunt was seen for amblyopia and strabismus treatment. Examination showed amblyopia of the left eye, intermittent exotropia, and a dissociated vertical deviation (DVD). The patient had equal pupils with no apparent afferent pupillary defect, and normal anterior and posterior exams bilaterally. The amblyopia was treated with patching. The patient underwent a bilateral lateral rectus recession (BLR) of 6 mm, left inferior oblique anteriorization, and right inferior oblique recession for a right dissociated horizontal deviation (DHD) of 25 prism diopters (PD), left DVD of 12 PD, and bilateral inferior oblique overaction. On postoperative day 2 (POD#2), sensorimotor examination demonstrated 23 and 22 abduction deficits in the right and left eyes respectively with a right esotropia (ET) of 25 PD. Doll’s head maneuver was done and did not result in any improvement in motility. There were no signs or symptoms of LP shunt malfunction (nausea, vomiting, lethargy, irritability), which the patient had with prior shunt malfunctions. After close monitoring without improvement, exploratory surgery to locate possible bilateral slipped muscle was performed. Forced duction testing intraopera- tively was normal without evidence of restriction. Upon exploration, the lateral recti were found at the expected location (12.5 mm from the limbus both eyes). The patient developed upper respiratory infection (URI) symptoms later on POD#0 (with respect to the second operation). There was no improvement in the abduction deficit in either eye and a 20 PD ET persisted on POD#4. On POD#6 the patient was started on Amoxicillin for a URI. The patient was sent for shunt malfunction workup and his neurosurgeon ordered an MRI, which revealed stable ventricular system findings without evidence of shunt abnormality. The MRI showed incidental bilateral acute otitis media (Fig. 1). By POD#13, after 1 week of Amoxicillin, the abduction deficit in both eyes significantly improved to 21 bilaterally. On POM#1, both the abduction deficit and ET were completely resolved. The patient’s right DHD recurred and required re-recession of the bilateral lateral recti with an uneventful postoperative course. We present a rare case of Gradenigo syndrome. It is clinically known for its classic triad of unilateral or bilateral acute otitis media, facial pain, and CN CN 6 palsy (1). Because of more effective and readily available antibiotics today, Gradenigo syndrome is rarely observed. However, it is critical to make an early diagnosis to properly treat this condition (2). The petrous apex is closely located to both CN 5 and 6 being separated only by thin dura mater. If an ear infection occurs, the area becomes vulnerable and Department of Ophthalmology and Visual Sciences, University of Minnesota, Minneapolis, Minnesota. The authors report no conflicts of interest. Address correspondence to Sydni L. Coleman, MD, Department of Ophthalmology and Visual Sciences, University of Minnesota, 516 Delaware Street SE, Minneapolis, MN 55455; E-mail: Colem799@ umn.edu e410 FIG. 1. MRI of the orbits. A. postcontrast T1 fat-saturated axial image showing normal globes and extraocular muscles without slipped muscle. B. T2 image showing hyperintensity of the middle ear and mastoid cavity, bilaterally (indicated by white arrows). Seeds et al: J Neuro-Ophthalmol 2022; 42: e410-e411 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence inflammation can cause facial pain and diplopia because of CN 5 and 6 involvement, respectively (3). Our patient presented with bilateral CN 6 palsies immediately after BLR surgery. Surgical exploration ruled out a slipped muscle. The presentation was complicated by the presence of a LP shunt and concern for shunt failure. Although the bilateral otitis media would have had to have coincided with his first surgery, he only developed URI symptoms 3 weeks later, and only on subsequent MRI were his bilateral acute otitis media infections discovered. The CN 6 palsies resolved with oral antibiotics. Because of the developmental delay of our patient related to his traumatic brain injury, the presence of facial pain or diplopia was difficult to ascertain. Interestingly, Gradenigo himself reported an original case study of patients with Gradenigo syndrome with less than half of the patients having the full triad of the disease (2). Although Gradenigo syndrome is rare in the postantibiotic era, it deserves consideration especially in cases of CN 6 palsy in patients unable to provide a clear history. Seeds et al: J Neuro-Ophthalmol 2022; 42: e410-e411 STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: A. Seeds, S. Coleman, and Strul; b. Acquisition of data: A. Seeds, S. Coleman, and S. Strul; Analysis and interpretation of data: A. Seeds, S. Coleman, and Strul. Category 2: a. Drafting the manuscript: A. Seeds and Coleman; b. Revising it for intellectual content: S. Coleman and Strul. Category 3: a. Final approval of the completed manuscript: Coleman and S. Strul. S. c. S. S. S. S. REFERENCES 1. Valles J, Fekete M. Gradenigo syndrome. Unusual consequence of otitis media. Case Rep Neurol. 2014;6:197–201. 2. Pedroso JL, Aquino CCH, Abrahão A, de Oliveira RA, Pinto LF, Bezerra MLE, Beuttenmuller Gonçalves Silva A, Bulcão de Macedo FD, Mendes AVdM, Barsottini OGP. Gradenigo’s syndrome: beyond the classical triad of diplopia, facial pain and otorrhea. Case Rep Neurol. 2011;3:45–47. 3. Lutter SA, Kerschner JE, Chusid MJ. Gradenigo syndrome: a rare but serious complication of otitis media. Pediatr Emerg Care. 2005;21:384–386. e411 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
Date | 2022-03 |
Language | eng |
Format | application/pdf |
Type | Text |
Publication Type | Journal Article |
Source | Journal of Neuro-Ophthalmology, March 2022, Volume 42, Issue 1 |
Publisher | Lippincott, Williams & Wilkins |
Holding Institution | Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890 |
Rights Management | © North American Neuro-Ophthalmology Society |
ARK | ark:/87278/s6dfwcbj |
Setname | ehsl_novel_jno |
ID | 2197467 |
Reference URL | https://collections.lib.utah.edu/ark:/87278/s6dfwcbj |