Man presents with ophthalmoplegia, proptosis and decreased vision
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A 59-year-old man was transferred from an outside hospital to the University of New Mexico Hospital for acute left eye vision loss and proptosis.
He was admitted 5 days prior and noted gradual vision changes and a “sensation of congestion” around the same time. The patient denied any recent trauma, eye pain, headache, fevers, chills, nausea or emesis.
Source: Yi Ling Dai, MD, and Amar Joshi, MD
His medical history was significant for diabetes, alcoholic liver cirrhosis, pancreatitis, hypertension and chronic left tibial osteomyelitis/hardware infection. He was allergic to penicillin. Medications included insulin and melatonin. His surgical history was significant for a right tibial fixation in April 2022. Family history was noncontributory. He admitted to alcohol use disorder but denied any tobacco or other drug use.
Examination
Upon examination, visual acuity was hand motion without correction in the right eye at near and no light perception in the left eye at near. Pupils were nonreactive with no apparent afferent pupillary defect in either eye. IOPs were normal at 16 mm Hg and 17 mm Hg in the right eye and left eye, respectively. The left eye was noted to have mild proptosis with complete ptosis on external examination. Additionally, sensation within the V1 and V2 distributions was significantly diminished on the left side. Extraocular movements were greatly limited in the left eye with essentially no movement in all directions of gaze (Figure 1) but full in the right eye without pain. The patient was unable to participate in visual fields on confrontation or color vision testing.
On anterior segment exam, the conjunctiva was white and quiet without significant injection. Corneas were clear, and the anterior chambers were deep and quiet without obvious cells. There was a mild nuclear sclerotic cataract in both eyes. The optic nerve appeared healthy without edema in the right eye, and there were some optic disc hemorrhages at the inner rim 360° in the left eye. The rest of the fundus appeared within normal limits.
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Acute vision loss
The differential diagnosis in a patient experiencing vision loss, ophthalmoplegia and proptosis includes vascular, neoplastic, infectious and inflammatory etiologies.
At the top of the differential is intracranial pathologies such as a tumor/mass, given the bilateral vision loss. A cavernous sinus thrombosis may also present with asymmetric ophthalmoplegia but rarely results in such significant vision loss. Other vascular etiologies such as microvascular cranial nerve palsy may result in limited extraocular motility but rarely vision loss. A fulminant case of giant cell arteritis may cause bilateral loss of vision, but the patient’s examination did not show any pallid edema of the optic nerve characteristic of the disease. Inflammatory etiologies such as idiopathic orbital inflammation or thyroid eye disease can cause significant ophthalmoplegia and subsequent compressive optic neuropathy, consistent with the patient’s presentation. However, idiopathic orbital inflammation often presents with significant pain and inflammatory signs, which was not reported by our patient; our patient also did not have the characteristic findings of lid retraction and conjunctival injection often seen in patients with thyroid eye disease. Lastly, infectious etiologies such as sinusitis, bacterial or fungal, can present with ophthalmoplegia and vision loss and must be ruled out in a patient with known risk factors.
Workup and management
The patient was recently admitted and treated for diabetic ketoacidosis at the outside hospital when he noted the vision loss. The patient’s history and clinical findings were highly concerning for acute invasive fungal rhinosinusitis (AIFRS). Diagnostic imaging studies including CT and MRI were obtained. There was absent enhancement of the left middle and superior turbinates, inferior orbital fissure, orbital apex, and along the left optic nerve canal with abnormal signal involving the left optic nerve (Figure 2). There was evidence of intracranial involvement involving the left anterior temporal lobe but no evidence of cavernous or ophthalmic vein thrombosis.
The patient’s HbA1c was 10.5 at time of admission. He was started on IV amphotericin B before transfer. An emergent bedside nasal endoscopic biopsy was done by ENT, which revealed insensate pale left superior, middle and inferior turbinates as well as black necrotic tissue. The patient was subsequently taken to the operating room for surgical debridement. Intraoperatively, the left middle turbinate appeared grossly necrotic, and the left inferior turbinate appeared pale. There was skull base bone necrosis and necrotic tissue around the left orbital apex and posterolateral sphenoid sinus wall. Pathology later confirmed the diagnosis of invasive fungal sinusitis with angioinvasion.
Discussion
AIFRS is a rare but life-threatening infectious disease with a high mortality rate, ranging from 50% to 80%. Cases with orbital involvement in the United States are around 1.7 cases per 1 million. It is an opportunistic disease most commonly seen in immunocompromised patients; risk factors include diabetes, metabolic acidosis, hematological malignancies, transplant status, chronic immunosuppressive use, HIV/AIDS and malnutrition.
It can be caused by different types of fungi, but most cases of AIFRS involve Aspergillus and/or Mucorales species, including both Rhizopus and Mucor. Clinical presentation may vary depending on the species of fungal infection. AIFRS secondary to Aspergillus species, which is commonly found in soil, presents with a more chronic and indolent picture, with the sphenoid sinus being the most commonly affected site. On the other hand, Mucorales species often presents with an aggressive and fulminant course. Common signs and symptoms include facial swelling (64%), fever (63%), nasal congestion (52%), ophthalmoplegia (50%), facial pain (47%) and headache (46%). The tell-tale sign is black eschar of the nasal turbinates, which was noted on bedside endoscopy in our patient.
Diagnosis is made clinically with histologic confirmation. Aspergillus species is best seen on GMS and/or Schiff stains and branches at acute angles whereas Mucorales species branches at 90°. Diagnostic imaging studies can also aid in the diagnosis. MRI is more sensitive than CT (85% compared with 57%), and a “black turbinate” sign and perisinus invasion are characteristic diagnostic findings.
If diagnosed, AIFRS is a medical emergency due to its high mortality rate. Patients are placed on IV liposomal amphotericin B with or without voriconazole for Aspergillus coverage. Early biopsy with surgical debridement of necrotic tissue and correction of underlying immunocompromising disorder such as blood sugar control should be done urgently. When there is orbital involvement, exenteration has historically been the recommended treatment to remove infected tissue and slow further spread of the infection. However, this has become controversial as exenteration has not been shown to improve survival based on several studies, and new treatment options have become available.
Recently, Ashraf and colleagues introduced outcomes of transcutaneous retrobulbar amphotericin B (TRAMB) injections in cases of AIFRS and advocated for a modified treatment ladder algorithm. First introduced in 2015, TRAMB injection has been shown to have a significantly lower rate of exenteration compared with control, but there was no difference in mortality rate. There was a 4.3% rate of complications including orbital inflammation and/or hemorrhage. Based on these findings, the authors advocated for TRAMB injections in cases of mild to moderate orbital involvement based on clinical examination and orbital imaging.
Clinical course continued
An oculoplastic surgeon was present during the patient’s initial debridement with ENT. Intraoperatively, the infection already involved the skull base with intracranial extension, and the extent of orbital involvement was limited to the apex without involvement of the globe. Therefore, an exenteration was deferred as it would not remove infected tissue or prevent spread of the disease intracranially. The patient was continued on IV amphotericin B and monitored closely. Five days after initial ophthalmic examination, there was progression of vision loss from hand motion to no light perception in the right eye. Dilated fundus examination also showed diffuse retinal ischemia consistent with central retinal artery occlusion of the right eye. Repeat MRI showed interval progression intracranially to the left occipital lobe. The patient was evaluated by neurosurgery and was deemed to be a poor surgical candidate. Eighteen days after his initial presentation, he developed acute right-sided facial droop and altered mental status and was found to have left frontal and anterior temporal infarcts on CT. The patient subsequently died the next day.
- References:
- Ashraf DC, et al. Am J Ophthalmol. 2022;doi:10.1016/j.ajo.2021.05.025.
- Huang YF, et al. J Ophthalmol. 2021;doi:10.1155/2021/9987871.
- Mekonnen ZK, et al. Ophthalmic Plast Reconstr Surg. 2021;doi:10.1097/IOP.0000000000001889.
- Trief D, et al. Br J Ophthalmol. 2016;doi:10.1136/bjophthalmol-2015-306945.
- For more information:
- Edited by Yi Ling Dai, MD, and Teresa P. Horan, MD, of New England Eye Center, Tufts University School of Medicine. They can be reached at ydai@tuftsmedicalcenter.org and thoran@tuftsmedicalcenter.org.