An 80-year-old male with diabetes mellitus, mild diabetic retinopathy, and mild dementia underwent reported uncomplicated, bilateral FESS for chronic sinusitis with polyposis at an outside hospital. He was provided an outpatient prescription for amoxicillin-clavulanic acid upon outpatient surgical discharge. Initial pathology specimen within the immediate postoperative period from the outside hospital was reported as non-invasive Aspergillus. He was seen by his original surgeon at the outside facility on postoperative day 3 without visual complaints or unexpected facial pain. On postoperative day 4, the patient reported sudden right-sided vision loss and presented to the walk-in ophthalmology clinic at the primary author’s tertiary care facility. An ophthalmology exam found no perception of light on the right and an abducens nerve palsy. Pupils were equal and reactive to light bilaterally with no pallor of the optic disc and normal ocular pressures. He was sent immediately to the emergency department for imaging, whereupon the otolaryngology team evaluated the patient. Intranasal exam by otolaryngology was only notable for moderate edema of the nasal mucosa without necrosis or pallor. There was no decreased sensation of the palate or midface. Computed tomography was performed and notable for violation of the lamina papyracea (Figure ). Preoperative imaging from the outside facility was not available for comparison. Magnetic resonance imaging suggested acute ischemic findings of the right optic nerve with diffusion restriction at the proximal optic nerve without evidence of thrombosis or hemorrhage. Subsequent imaging after admission noted an abnormal T2 signal without canalicular segment enhancement. There was, however, some adjacent enhancement extending to involve the optic and nerve sheaths in the orbital apex and superior orbital fissure. The enhancement of the right-sided nerve sheaths was thought to be contributing both to the vision loss and the abducens nerve palsy that was defining the OAS. In addition to those mentioned, the neurosurgery, neurology, and internal medicine teams evaluated the patient. It should be noted that repeated images were obtained at several time points due to poor images from motion artifact that did delay care. The clinical and radiologic findings were determined by the treating teams to be most suggestive of the surgical trauma from the FESS at the outside facility and not of an infectious origin. A non-surgical approach was initially pursued. The patient was provided broad-spectrum antibiotics and high-dose steroids to decrease inflammation with close neurologic monitoring. Re-evaluation by otolaryngology on flexible nasal endoscopy did not show any change in findings of the nasal mucosa.\nOn postoperative day 9 from the original FESS, the patient developed sudden vision loss of the left eye. An ophthalmology exam noted no light perception bilaterally, worsening ophthalmoplegia on the right with decreased supraduction, adduction, and infraduction in addition to the complete abducens palsy. These findings suggested now the involvement of the oculomotor and trochlear nerves. The posterior ocular exam now showed mild optic nerve pallor on the right with good color on the left and no evidence of edema. Repeat computed tomography and magnetic resonance imaging noted no new bony destruction; however, there was persistent inflammation of the right optic canal. An emergent multidisciplinary team meeting was held between all consulting specialties and infectious disease was also consulted as the initial diagnosis of traumatic neuropathy was questioned. The patient then underwent emergent FESS with otolaryngology and neurosurgery with decompression of both optic nerves, sphenoidotomy, septectomy, and mucosal stripping (Figure ). The lamina papyracea and bone adjacent to the root of the right orbital apex were dehiscent as had been suggested on imaging (Figure ). Additionally, there was a large amount of absorbable packing in the posterior ethmoid cavity on the right.\nA final culture from the initial FESS at the outside hospital was re-evaluated by the infectious disease team, which revised the diagnosis of Aspergillus to that of S. apiospermum. This pathologic diagnosis was made on postoperative day 10 (postoperative day 1 from emergent FESS). The patient was started on high-dose voriconazole and amphotericin B. The patient continued on maximum medical therapy and continued to worsen clinically and radiologically regarding the infection. Unfortunately, after prolonged hospitalization, he suffered a myocardial infarction and subsequent severe gastrointestinal bleeding. He passed away on postoperative day 47 from the initial FESS after being transferred to inpatient hospice services.
A 59-year-old man with a 1-month history of headache was examined in an otolaryngological clinic of a general hospital. The patient’s anterior nasal septum was swollen, and he was diagnosed with nasal septal abscess and admitted to the hospital the same day. Enhanced computed tomography (CT) revealed a swollen, ring-enhanced lesion in the anterior nasal septum (Fig. ). He was treated with incisional drainage of the abscess and antibiotic drip for 1 month (fosfomycin, ampicillin/sulbactam, ceftriaxone, and meropenem). In addition, insulin-based therapy was started because the patient had uncontrolled diabetes and hemoglobin A1c of 13.3%. His symptoms persisted despite 1 month of antibiotic drip. Pus from the nasal septum was cultured three times. Culture tests detected low concentrations of Capnocytophaga spp. and Serratia marcescens, which were sensitive to the antibiotic drip. No fungi were detected. The septal lesion was biopsied twice and showed granulation-like tissue with infiltrated inflammatory cells (nothing indicating malignancy or fungus). Despite the antibiotic drip for >1 month, diplopia developed due to bilateral abducens nerve palsy, with subsequent impairment of right eye movement and eventual visual loss. The patient was then referred to our department.\nThe patient’s initial visit revealed a saddle nose. Nasal fiberscope revealed bulging of the nasal septum and granulation-like tissue on the right side of the nasal septum. The patient’s left eye fixed at the central position and the supraduction and adduction of the right eye were limited. His visual acuities were light perception in the right eye and “count fingers” in the left eye. The functions of the 5th, 7th, and 8–12th cranial nerves were intact.\nThe patient’s hemoglobin A1c was 9.2%. Plain CT showed a poorly marginated lesion with soft tissue density at the superior nasal septum, which was invading the anterior cranial fossa. Another mass with soft tissue density appeared in the left maxillary sinus (Fig. ). A spinal tap and cerebrospinal fluid culture were immediately performed, and the results revealed elevated glucose levels (130 mg/dL) and cell counts (mononucleosis 19/m3, polynucleosis 1/m3). No pathogen was detected by microscopic examination or culture methods. Owing to the possibility of a fungal and/or bacterial infection, we prescribed an antibiotic (ampicillin/sulbactam) and antifungal (amphotericin B) drip. Subsequently, we performed diagnostic endoscopic sinus surgery under general anesthesia. Histopathological examination revealed that the ball-like lesion in the left maxillary sinus harbored fungi, with filaments slightly thinner than those of Aspergillus spp. (Fig. ). A commercially available fungal DNA detection kit (Robust HotStart ReadyMix PCR Kit; KAPA biosystems, Inc., U.S.A.) was used to detect pathogens in tissues from the nasal septal lesion and ball-like fungal lesion in the left maxillary sinus. A broad-range polymerase chain reaction (PCR) was performed targeting the highly conserved sequence of the ribosomal RNA genes in fungal DNA shared by most fungi. Internal transcribed spacer (ITS) regions in ribosomal DNA were then amplified by PCR using primers ITS1F (5′-TCCGTAGGTGAACCTGCGG-3′) and ITS4R (5′-TCCTCCGCTTATTGATATGC-3′). PCR products of 150–500 bp were obtained and sequenced to match a known DNA sequence from a reference database (BLAST: Basic Local Alignment Search Tool). The organism detected by PCR and tissue cultures was S. apiospermum. Subsequent enhanced magnetic resonance imaging (MRI) revealed strongly enhanced lesions of the nasal septum, ethmoid sinuses, bilateral orbital apexes, and cavernous sinus. The dura along the anterior and bilateral middle cranial fossa was thickened and well-enhanced. (Fig. ). The diagnosis was orbital apex syndrome and hypertrophic pachymeningitis secondary to fungal nasal septal abscess caused by S. apiospermum.\nDrug susceptibility testing (Table ) led us to change the antifungal drip to voriconazole (500 mg/day). Since voriconazole had no apparent effect on the patient’s symptoms after four days, we surgically resected the lesion under general anesthesia.\nUsing endoscopy-assisted open skull base surgery, we removed the fungal ball-like lesion and the mucosa of the left maxillary sinus. We then incised the anterior border of the nasal septum and removed the mucosa of the bilateral ethmoid and sphenoid sinuses. After anterior craniotomy, we resected the anterior skull base between both sides of the ethmoid tegmen, from the frontal table anteriorly to the optic chiasm posteriorly. We avoided the extirpation of the eye ball or removal of the orbital content, because the medial bony wall of the orbit was intact.\nThe resected nasal septum was thickened and necrotic, strongly suggesting infectious inflammation and necrosis of the tissue. Mild mucosal edema was observed in the bilateral ethmoid and sphenoid sinuses. No fungal invasion or pus was observed. The dura was thinned just above the affected part of the cranial fossa, suggesting that the infection and inflammation had spread to the dura; however, there was no apparent dural deficit, and the dura was preserved. Following resection, we reconstructed the affected anterior cranial fossa with pericranium.\nThe patient’s headache decreased and almost disappeared 3 weeks postoperatively. His preoperative mild fever and inflammation normalized, and he was discharged on postoperative day 29. The patient continued oral voriconazole (400 mg/day) until 12 months post-surgery, when bilateral ocular movements completely improved; however, loss of visual acuity persisted. At 22 months post-surgery, a follow-up MRI demonstrated that the enhanced lesion in the orbital apex diminished. Thirty months has passed without any evidence of recurrence until now. The timeline of the patient’s illness is illustrated in Fig. .