Reversal of Visual Loss From Skull Base Osteomyelitis in a Pediatric Patient

A previously healthy 9-year-old girl with sickle cell trait presented with one day of vision loss in the left eye preceded by one week of frontal headaches and pain on eye movement. On initial examination, best-corrected visual acuities were 20/30 in the right eye and 20/50 in the left eye.

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Reversal of Visual Loss From Skull Base Osteomyelitis in a Pediatric Patient Kevin K. Ma, MD, Caroline D. Robson, MD, Eric D. Gaier, MD, PhD, Ryan Gise, MD A previously healthy 9-year-old girl with sickle cell trait presented with one day of vision loss in the left eye preceded by one week of frontal headaches and pain on eye movement. On initial examination, bestcorrected visual acuities were 20/30 in the right eye and 20/50 in the left eye. Ishihara color plates were full on the right and diminished to the test plate only on the left. A relative afferent pupillary defect was present on the left without anisocoria. Confrontational visual field testing revealed a dense inferior defect in the left eye. There was 2-mm ptosis and mild edema of the left upper eyelid. Extraocular motility, exophthalmometry, cranial nerve examination, intraocular pressure, and anterior segment examinations were normal. Dilated funduscopy revealed subtle hyperemia and fullness of the left optic disc. FIG. 1. Radiographic findings of skull base osteomyelitis on presentation. A–C. Contrast T1 coronal enhanced, fat-suppressed MR images demonstrate abnormal enhancement of the sphenoid bone (asterisks). A. Shows cavernous sinus thrombosis (short arrow) and an asymmetrically small signal void within the left internal carotid artery (arrowhead). B. Shows the peripherally enhancing epidural empyema (long arrow). C. Reveals the empyema and enhancement extending into the left optic canal (long arrow). D. CT sagittal reformat shows cortical destruction of the sphenoid bone (arrowheads). E. 3D time-offlight MRA MIP image shows severe vasospasm of the left (arrow) greater than right internal carotid arteries (short arrows).CT, computed tomography; MIP, maximum intensity projection. Department of Ophthalmology (KKM, EDG, RG), Massachusetts Eye and Ear, Boston, Massachusetts; Harvard Medical School (KKM, CDR, EDG, RG), Boston, Massachusetts; Division of Neuroradiology (CDR), Department of Radiology, Boston Children’s Hospital, Boston, Massachusetts; Department of Ophthalmology (EDG, RG), Boston Children’s Hospital, Boston, Massachusetts; and Department of Brain and Cognitive Sciences (EDG), Picower Institute for Learning and Memory, Massachusetts Institute of Technology, Cambridge, Massachusetts The authors report no conflicts of interest. Address correspondence to Ryan Gise, MD, Department of Ophthalmology, Boston Children’s Hospital, 300 Longwood Avenue, Boston, MA 02115; E-mail: [e-mail] e728 A computed tomography (CT) examination from the referring hospital revealed opacification of the left ethmoid and bilateral sphenoid sinuses. Subsequent more detailed head CT angiography, sinus CT, and brain and orbital MRI without and with gadolinium revealed diffuse erosion, signal abnormality, and enhancement of the sphenoid body, planum sphenoidale, and basisphenoid (Fig. 1A–D). A 5-mm epidural empyema extended across the floor of anterior cranial fossa (Fig. 1B). Purulent material extended into the left optic canal (Fig. 1C). Ma et al: J Neuro-Ophthalmol 2021; 41: e728-e730 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence FIG. 2. Postoperative progression of optic atrophy. A–C. retinal nerve fiber layer (RNFL; left) and ganglion cell layer (GCL; right) analyses 11 days (A), 19 days (B), and 57 days (C) after surgery. Average GCL volumes were 1.03 mm3 in the right eye and 0.96 mm3 in the left eye (A); 1.07 mm3 in the right eye and 0.83 mm3 in the left eye (B); and 1.07 mm3 in the right eye and 0.74 mm3 in the left eye (C). In addition, bilateral cavernous sinus thrombosis (Fig. 1A) and severe vasospasm of the left greater than right internal carotid arteries were noted (Fig. 1E). Intravenous ceftriaxone, vancomycin, and metronidazole were started, and the patient underwent emergent endoscopic left ethmoidectomy and sphenoidotomy. Intraoperative samples grew Streptococcus intermedius. Bedside examination on postoperative day 1 revealed a complete afferent pupillary defect and the absence of light perception (NLP) in the left eye. Her examination remained unchanged for 10 daily examinations. On the 11th day, vision returned at the count-fingers level and improved to 20/20 in the left eye with full Ishihara color plates over the subsequent 4 days. A relative afferent pupillary defect remained in the left eye. Optical coherence tomography (OCT) 19 days postoperatively revealed relative thinning of ganglion cell layer (GCL) compared with the unaffected eye with preserved peripapillary retinal nerve fiber layer (RNFL) thickness (Fig. 2A). At 57 days, diffuse RNFL thinning in the left eye was evident (Fig. 2B). Kinetic perimetry was normal in both eyes 27 days postoperatively. The patient completed a 6-week course of intravenous ceftriaxone and metronidazole without complication and with near complete resolution radiographically. DISCUSSION To the best of our knowledge, this is the first reported case of a unilateral optic neuropathy secondary to skull base osteomyelitis (SBO) in an otherwise healthy pediatric patient. SBO is a rare but potentially Ma et al: J Neuro-Ophthalmol 2021; 41: e728-e730 life-threatening complication of uncontrolled chronic otitis externa and, less commonly, sinusitis (1,2). SBO very rarely affects children, and most severe cases involve elderly patients with diabetes, immunosuppression, or other comorbidities (1,3). Winter et al (4) described 2 adult cases of unilateral optic neuropathy from Pseudomonas-associated SBO leading to NLP vision without recovery. By contrast, the patient presented in this article recovered visual acuity from NLP postoperatively to 20/20 despite infection-related and/or surgery-related optic nerve injury evident on OCT. Consistent with previous observations in optic neuropathy secondary to severe pediatric infections (5), our patient recovered relatively rapidly compared with adults with SBO (3). However, complete recovery of central visual acuity from NLP is remarkable and likely reflects the lack of systemic comorbidities, surgical accessibility of the infectious source, and timely intervention. Given the recovery of vision, we postulate that the mechanism of visual loss was either related to compression from the empyema surrounding the optic nerve, infectious perineuritis, or a combination of these. This case underscores the utility of a timely multidisciplinary approach with prompt surgical intervention and aggressive antimicrobial therapy to preserve visual function in osteomyelitis of the skull base. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: K. Ma, E. Gaier, and R. Gise; b. Acquisition of data: K. Ma, E. Gaier, and Ryan Gise; c. Analysis and interpretation of data: K. Ma, E. Gaier, and R. Gise. e729 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence Category 2: a. Drafting the manuscript: K. Ma, E. Gaier, and R. Gise; b. Revising it for intellectual content: K. Ma, E. Gaier, and R. Gise. Category 3: a. Final approval of the completed manuscript: K. Ma, E. Gaier, and R. Gise. REFERENCES 1. Blyth CC, Gomes L, Sorrell TC, da Cruz M, Sud A, Chen SCA. Skull-base osteomyelitis: fungal vs. bacterial infection. Clin Microbiol Infect. 2011;17:306–311. e730 2. Chandler JR, Grobman L, Quencer R, Serafini A. Osteomyelitis of the base of the skull. Laryngoscope. 1986;96:245–251. 3. Rothholtz VS, Lee AD, Shamloo B, Bazargan M, Pan D, Djalilian HR. Skull base osteomyelitis: the effect of comorbid disease on hospitalization. Laryngoscope. 2008;118:1917–1924. 4. Winter TW, Anwar Z, Lam BL, Schatz NJ, Sternau LL, Guy JR. Optic nerve involvement from Pseudomonas aeruginosa– associated skull base osteomyelitis. JAMA Ophthalmol. 2013;131:253–255. 5. Bajor A, Lang CP, Bültmann E, Framme C, Hufendiek K. Orbital complication of acute sinusitis : orbital cellulitis in a 10-year-old child [in German]. Der Ophthalmologe. 2017;114:365–369. Ma et al: J Neuro-Ophthalmol 2021; 41: e728-e730 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.