Statistics from Altmetric.com
Aspergillus infections of the paranasal sinuses are classified as invasive or non-invasive.1 The vast majority are non-invasive with a good prognosis; however, the invasive type behaves as a malignant neoplasm with bone destruction, orbital and intracranial extensions, and a high mortality rate.2–4
We present one patient with invasive paranasal aspergillosis that extended into the orbit and cranial cavities, and who has survived for 9 years and 1 month.
Along with headaches and periorbital pain beginning in July 1993, a 64 year old woman noticed a decrease vision in her right eye and visited us on 7 September 1993. She had poorly controlled diabetes mellitus. Her corrected visual acuity was 20/30 right eye and 20/20 left eye, and her critical flicker fusion frequency was 25 Hz right eye and 32 Hz left eye. A central scotoma with a relative afferent pupillary defect was present in the right eye. The right optic disc was slightly swollen.
A computed tomographic scan disclosed a mass in the right posterior ethmoid and sphenoid sinuses with destruction of the lateral wall of the sphenoidal sinus and the enlargement of the superior orbital fissure. The mass extended to the right orbital apex and the right cavernous sinus. Magnetic resonance imaging showed an enhanced mass, isointense on T1 weighted and isointense and partially hypointense on T2 weighted images (Fig 1).
Endoscopic right sphenoidectomy was performed on 18 September, and Aspergillus was histopathologically identified in a small mass isolated from the sphenoid sinus mucosa (Fig 2). Intravenous fluconazole (400–800 mg/day), oral flucitocine (8000 mg/day), and daily transnasal sinus irrigation with amphotericin B were started on 22 September. However, she lost all vision in her right eye on 4 October.
She was transferred to the department of neurological surgery on 13 October. Antifungal therapy was continued with the addition of oral itraconazole (200 mg/day). Carotid angiography demonstrated that stenosis of the right ophthalmic artery. A frontal craniotomy was performed on 1 November, and a fibrous granulomatous lesion was found in the right posterior ethmoid sinus, sphenoid sinus, cavernous sinus, and orbit that extended to the right optic nerve. The orbit was necrotic and the sphenoid sinus was severely eroded. Cultures and histopathological examinations were negative for Aspergillus.
The patient underwent debridement and removal of the frontal bone on 24 December because of the development of an epidural abscess. In February 1994, the abscess had extended from the right cavernous sinus to the temporal lobe and bacterial cultures were positive for Pseudomonas aeruginosa. The abscess subsided but still persisted following antibiotics and antifungal therapy.
On 13 September 1994, orbital exenteration, intracranial exenteration, and skin plasty were performed. Her diabetes mellitus is fairly well controlled by insulin injection and she has been free of aspergillosis for 9 years and 1 month after the initial presentation.
To the best of our knowledge, survival for more than 9 years of a patient with invasive paranasal aspergillosis has not been reported. The longest previous case of invasive paranasal aspergillosis is for 8 years and 2 months.1
The prognosis of this disease depends on the location and the duration of the infection, and patient’s immunological status.3,4 Sphenoidal aspergillosis is aggressive nature because of the close relation to the skull base.3 Her poorly controlled diabetes mellitus could be a risk factor for the onset of Aspergillus infection; however, at present it is fairly controlled, presumably contributing to this long survival.
The sphenoid sinus mucosa was positive for Aspergillus but it was not found at the time of the intracranial surgery probably because of the extensive antifungal therapy before surgery and/or the low viability of the mycelium in a fungus ball.5
The long term survival of this patient with orbital-paranasal aspergillosis despite intracranial extension is attributed to early diagnosis, optimal antifungal therapy, complete surgical debridement, and the improvement in the patient’s systemic condition.