Trigeminal Trophic Syndrome: A Comprehensive Review of a Surgical Approach

Abstract

: Trigeminal trophic syndrome (TTS) is an exceedingly rare disease that causes facial ulceration, most commonly at the nasal ala. The overall incidence of TTS is not known, with less than 150 cases published in the literature. We searched the United States National Library of Medicine National Institutes of Health (PubMed) using the terms “Trigeminal Trophic Syndrome” and “TTS” as keywords. Publications in all languages were included if an English abstract was available. We reviewed 111 cases of TTS described in 75 publications from 1979 to 2021. Fourteen cases involved surgical reconstruction. Of these, the lesions completely healed in 7 cases (50%), recurred in 5 (36%), and were unspecified in 2 (14%). Reconstruction was completed in a 2 to 3-stage approach in 6 cases; of these, healing without recurrence was observed in 5 cases (83%). When donor tissue from the affected side was used as a basis for reconstruction, healing without recurrence was observed in 2 cases (50%). This is in comparison to the use of contralateral, sensate tissue in which there was healing without recurrence in all 3 cases (100%). The surgical management of TTS remains a topic of controversy. The rates of success remain comparable despite the use of various flap types. However, the use of contralateral, sensate flaps and a staged surgical approach appears to be effective based upon the best available evidence in the literature. Further prospective orretrospective controlled studies are necessary to make more reliable recommendations, though may be challenging given the rarity of TTS.

Trigeminal Trophic Syndrome as a Complication of Herpes Zoster Ophthalmicus

“…There are multiple causes for TTS and the most common are iatrogenic such as therapeutic trigeminal nerve ablation. There are other potential causes including, but not limited to, ischemic medullary or pontine stroke, craniofacial surgery, acoustic neuroma, post-infectious encephalitis, astrocytoma, meningioma, surgical or local trauma, and amyloid deposits in the trigeminal nerve and herpes zoster infection, as is the case for this patient [1][2][3][4][5][6][7]. Most ulcers affect the infraorbital nerve distribution.…”

“…The characteristic clinical triad of TTS is facial skin ulceration, paresthesia, and hypoesthesia or anesthesia [1][2][3][4][5][6][7]. There are multiple causes for TTS and the most common are iatrogenic such as therapeutic trigeminal nerve ablation.…”

“…The period between trigeminal nerve insult and ulceration ranges from weeks to decades [1,4,5]. It's more common in women, and the mean age across different publications tends to fall within the sixth and seventh decades [1,[3][4][5][6]8].…”

“…The precise incidence is not known. It apparently lies somewhere between 150 and 200 cases according to two recent reviews of the syndrome [ 4 , 6 ].…”

“…The surgical management of TTS remains controversial. The use of contralateral, sensate flaps and a staged surgical approach appears to be effective [ 6 ].…”