Ophthalmic and multidisciplinary management of orbital exenteration for high-grade adenoid cystic carcinoma of the paranasal sinus with skull base extension, in a patient with concurrent metastatic prostate adenocarcinoma

Purpose: To describe the ophthalmic evaluation, oncologic staging, and multidisciplinary surgical management of a patient with high-grade transformed adenoid cystic carcinoma of the left paranasal sinus with orbital, skull base, and cavernous sinus extension, complicated by a separate metastatic prostate primary.

Case Description: A 74-year-old male presented with progressive left periorbital swelling, intermittent tearing, and limitation of ocular motility, ultimately diagnosed with high-grade transformed adenoid cystic carcinoma of the left paranasal sinus (T4a vs. T4b, N2b, M1) with extension into the medial orbit, anterior cranial fossa, cavernous sinus, and pterygoid fossa, with pulmonary metastases. He also carried a diagnosis of metastatic prostate adenocarcinoma to bone. Preoperative imaging (MRI brain/face, PET/CT) demonstrated progressive heterogeneous mass effect along the medial left globe with a necrotic level 1B cervical lymph node concerning for additional nodal metastasis. The preoperative course was complicated by worsening eye pain, prompting bedside lateral canthotomy for concern of orbital compartment syndrome, with symptomatic improvement.

The patient underwent combined ENT/ophthalmology resection: left medial maxillectomy, orbital exenteration, midline septectomy, complete ethmoidectomy and sphenoidotomy, frontal sinusotomy (Draf III), maxillary antrostomy, excision of middle turbinates, and left neck dissection (levels 1A–4), with anterolateral thigh free flap reconstruction of the orbital defect. The postoperative course was notable for new-onset atrial fibrillation with rapid ventricular response (rate-controlled), acute-on-chronic normocytic anemia requiring transfusion, and polymicrobial paranasal sinusitis (Pseudomonas and Klebsiella species) managed with targeted antibiotics. The fellow (right) eye was monitored throughout for a visually non-significant cataract and peripheral retinal pigment epithelial changes, with surgery deferred to protect vision in the remaining eye given the overall prognosis.

Observations: This case highlights the ophthalmologist's dual role in complex oncologic orbital disease — coordinating exenteration for local tumor control while safeguarding visual function in the fellow eye, including acute perioperative recognition and management of orbital compartment syndrome, particularly when a second, unrelated malignancy complicates prognosis and treatment sequencing.

Conclusion: Locally advanced sinonasal malignancy with orbital and skull base invasion requires close ophthalmology–ENT–oncology coordination across the perioperative period, from acute compartment-syndrome recognition through free-flap reconstruction, with a conservative, protective approach to the unaffected eye when systemic prognosis is guarded.

George Nemeth is a medical student at the University of Louisville School of Medicine who has received his Master in Public Health at Baylor University. He has collaborated with physicians at the Tripler Army Medical Center in Honolulu, Hawaii to conduct clinical ophthalmology research and with the University of Louisville to conduct public health research in ophthalmology.