Rhinocerebral zygomycosis in Pakistan: clinical spectrum, management, and outcome.
OBJECTIVE
To study the disease spectrum and salient management features of 36 patients with histopathologically-confirmed rhinocerebral zygomycosis seen at our academic center over a 16-year period.


METHODS
Retrospective review of patients admitted to the Aga Khan University Hospital in Karachi, Pakistan from January 1991 to December 2006 with histopathologically-confirmed zygomycosis of the head and neck.


RESULTS
Mean patient age was 40 +/- 5.0 years (range, 34-63 years), and 23 (64%) patients were male. Thirty-two (89%) patients were referred from clinical services other than otolaryngology. Underlying predisposing conditions included diabetes mellitus (21 patients), haematologic diseases (9), and renal failure (6). Twenty (55%) patients had limited sinonasal disease, ten (28%) had orbital involvement, and six (17%) had intracranial extension. All patients underwent rigid nasal endoscopy and biopsy, and black necrotic tissue was seen in 22 (61%) instances warranting endoscopic or open surgical debridement. Four of 6 patients undergoing open surgery required orbital exenteration. Overall patient survival was 56% (20/36 patients). Diabetic patients had improved survival (17/21, or 81%) compared to patients with haematologic disorders (3/9, or 33%) (p = 0.001). All six patients with intracerebral disease died. Eighteen of the 22 (82%) patients treated with surgery plus amphotericin B survived vs. two of 14 (14%) receiving amphotericin B alone (p < 0.001).


CONCLUSIONS
In rhinocerebral zygomycosis, an aggressive, multidisciplinary, diagnostic and therapeutic approach that utilizes CT or MRI staging, and combines endoscopic or open surgical debridement with amphotericin B-based antifungal therapy offers the best chance of recovery.
Departments of Otolaryngology, The Aga Khan University, Karachi, Pakistan.
Mubasher
M
Ikram
Moghira
M
Iqbal
Muhammad Aslam
MA
Khan
Erum
E
Khan
Mahnaz
M
Shah
Raymond A
RA
Smego
D000328:Adult; D000666:Amphotericin B / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D001706:Biopsy; D001927:Brain Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000382:microbiology / Q000628:therapy; D003646:Debridement; D004724:Endoscopy; D005260:Female; D006784:Hospitals, Teaching; D006801:Humans; D008297:Male; D008875:Middle Aged; D009089:Mucor / Q000302:isolation & purification; D009668:Nose Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000382:microbiology / Q000628:therapy; D010154:Pakistan / Q000453:epidemiology; D012189:Retrospective Studies; D012233:Rhizopus / Q000302:isolation & purification; D016019:Survival Analysis; D016896:Treatment Outcome; D020096:Zygomycosis / Q000175:diagnosis / Q000453:epidemiology / Q000382:microbiology / Q000628:therapy
D000935:Antifungal Agents; D000666:Amphotericin B