Orbital compartment syndrome following retrobulbar injection of amphotericin B for invasive fungal disease.

PURPOSE: To describe a complication of retrobulbar amphotericin B injections in the treatment of invasive rhino-orbital asperigillosis. OBSERVATIONS: 27 year-old renal transplant recipient presented with a two-week history of headache, binocular diplopia and proptosis of the left eye. Endonasal biopsy on hospital day 3 confirmed the diagnosis of rhino-orbital invasive Aspergillus fumigatus involving the left orbital apex.In addition to systemic antifungal treatment and cessation of immunosuppression, retrobulbar amphotericin B injections (3.5 mg/1 ml) combined with endoscopic local debridement were initiated when the patient developed progressive visual loss. Retrobulbar injections were administered on hospital days 8, 10, 14, 17, and 20. Endoscopic debridement occurred on hospital days 10 and 16.After the fifth retrobulbar amphotericin B injection, the patient developed acute orbital compartment syndrome with intraocular pressures ranging from 47 to 86 mmHg and vision declined to 20/200, requiring emergent lateral canthotomy and superior and inferior cantholysis. Close observation without further intervention resulted in return of vision to 20/20 and normalization of intraocular pressure. CONCLUSION AND IMPORTANCE: Retrobulbar amphotericin B in combination with local debridement may be considered an alternative to exenteration for invasive aspergillosis secondary to reversible immunosuppression. To the authors' knowledge, orbital compartment syndrome secondary to retrobulbar amphotericin B administration has not previously been reported. Patients should be counseled on the risk of severe local inflammation due to amphotericin B. More research is needed to establish the most appropriate dosing, frequency, and duration of retrobulbar amphotericin B injections in the treatment of life-threatening Aspergillus infections.

Temporal Artery Calciphylaxis Presenting as Temporal Arteritis in a Case of Rhinoorbitocerebral Mucormycosis.

Mucormycosis is a rare often fatal opportunistic fungal infection. It is typically described in patients with diabetes in ketoacidotic status and is rare in renal transplant recipients. Calciphylaxis is a rare and highly morbid disease of vascular calcification affecting patients with end-stage renal disease (ESRD). The first case of a renal transplant recipient who was inflicted with both rhinoorbitocerebral mucormycosis and calciphylaxis is reported. A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness. He had undergone renal transplant for ESRD 7 months earlier with resultant immunosuppressive therapy. MRI and nasal biopsy confirmed rhinoorbitocerebral mucormycosis. Immunosuppressive therapy was stopped and antifungal therapy begun. He had orbital exenteration for progressive rhinoorbitocerebral mucormycosis. Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis. Clinical presentation, treatment course, and follow up are discussed.

Orbital schwannomatosis in the absence of neurofibromatosis.

PURPOSE: The aim of this study was to describe 3 cases of primary orbital schwannomatosis without associated systemic neurofibromatosis. METHODS: This is a retrospective interventional study of 3 patients who presented with multiple, distinct masses in the orbit (n = 3) as well as in the hemiface (n = 1). The clinical presentation, imaging features, surgical procedures, and outcomes were defined. RESULTS: Two women and a man presented with of exophthalmos and diplopia. Pain was the most prominent complaint in 2 patients. None of the patients had associated systemic neurofibromatosis by history or examination. Radiologic evaluation with computed tomography or magnetic resonance imaging of orbit revealed multiple well-demarcated intraconal and extraconal masses. Masses were excised, and histopathology confirmed all masses to be schwannomas. Postoperative follow-up was uneventful with alleviation of primary complaints in all patients. CONCLUSIONS: Multiple orbital schwannomas (primary orbital schwannomatosis) may be observed in patients without systemic association of neurofibromatosis. Management includes surgical excision of the tumors to achieve relief from their mass effects.

Masson tumor in the eyelid.

Intravascular papillary endothelial hyperplasia (Masson tumor) is an uncommon vascular lesion that rarely affects the periorbital region. Differential diagnosis from some malignant periorbital tumors is challenging and depends mostly on histopathologic confirmation. We present a case with elevated, multilobular lower eyelid lesion that turned out to be intravascular papillary endothelial hyperplasia.

Iatrogenic cerebrospinal fluid oculorrhea.

Cerebrospinal fluid leakage into the orbit (CSF orbitorrhea) or through the orbit to the exterior (CSF oculorrhea) occurs when there is a communication between orbit and subarachnoid space. It has rarely been described. We report a case of CSF oculorrhea following surgery for recurrent sphenoid wing meningioma. A 67-year-old patient who underwent craniotomy for a recurrent sphenoid wing meningioma complained of tearing from the ipsilateral eye. Fluid collection was observed in the right periorbital area contiguous with craniotomy wound. With wide retraction of eyelids, a 1-cm longitudinal full-thickness laceration through the conjunctiva toward the lateral orbital rim was visualized in the area of the lateral canthal tendon. Steady leakage of pink-tinged serous fluid through the forniceal laceration was observed. With a presumed diagnosis of iatrogenic CSF oculorrhea, the patient was treated conservatively with resolution by postoperative day 6.