Trigeminal trophic syndrome: A systematic review.

OBJECTIVES: To systematically report and document Trigeminal Trophic Syndrome (TTS), characterize its clinical presentation, diagnostic tests performed, outline management strategies, outcomes; and highlight the role of otolaryngologists in the tissue diagnosis of this rare syndrome. DATA SOURCES: PubMed/Medline, Scopus, and Cochrane databases. REVIEW METHODS: PubMed/Medline, Scopus, and Cochrane databases were systematically reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to identify all cases of TTS published with an English translation from inception to December 2020. RESULTS: A total of 142 articles describing 214 patients with TTS were included in the analysis. There was a female predominance (62.9 %) and a median age of 57 (range 1-93) years at presentation. A trigeminal neurological insult was identified in 200 (93.5 %) cases. The most common triggers for TTS were treatment for trigeminal neuralgia (35.7 %) and cerebrovascular accident (21.6 %). Self-inflicted trauma occurred in 137 (64 %) patients. Biopsy was done in 123 (57.5 %) patients. Patient education, barrier devices, and medications to address parasthesias were the most common treatment strategies. The majority of patients (72.5 %) received multimodal therapy. Surgery was performed in 35 (22.7 %) patients. Treatment outcomes were discussed in 120 (56.1 %) patients. CONCLUSIONS: TTS is a rare condition with poorly understood pathophysiology. It should be suspected in a patient with non-healing facial ulceration and altered sensation within the trigeminal nerve distribution. Biopsy of the ulcer is important to confirm the diagnosis and exclude malignancy. Treatment options include conservative and pharmacologic measures, and less frequently surgery.