Trigeminal trophic syndrome: Report of a case and review of the published work

Abstract

Trigeminal trophic syndrome is a rare complication of trigeminal nerve injury that causes facial ulceration, anesthesia and paresthesia in the same trigeminal dermatomes. We present a case of a 65-year-old woman with a history of meningioma resection 18 years prior who presented 16 years later with an intractable ulceration around her left nasolabial sulcus. Pain and light-touch sensations around the ulcer were decreased. She admitted to frequent manipulation due to a crawling sensation. A skin biopsy showed acanthotic changes and a decreased number of peripheral nerve fibers. Trigeminal trophic syndrome was diagnosed. Carbamazepine was not effective, and the ulcer persisted at 7 months after the initial presentation. We reviewed 36 English-language publications from 2003 to 2012, and analyzed 61 cases of trigeminal trophic syndrome, including this patient. The mean age was 53.3 ± 19.7 years (range, 6-91). The right side of the face was more commonly affected (57%) than the left side. The ala nasi were involved in 48 cases (79%), followed by the cheek in 17 cases (28%). A corneal lesion was observed in 11 cases (18%), suggesting the importance of ophthalmologic consultations. The two major etiologies were trigeminal nerve ablation (18 cases; 30%) and cerebrovascular accidents (18 cases; 30%). The latent period ranged from days to 30 years. Gabapentin and carbamazepine were frequently administrated with variable efficacy. Application of thermoplastic dressings or negative pressure wound therapy demonstrated favorable outcomes. Surgery was an option with a high recurrence rate. Trigeminal trophic syndrome remains a clinical challenge.

Trigeminal trophic syndrome: Analysis of the number of peripheral nerve fibres and blood vessels in the lesional skin

“…3 In addition, it has been reported that semaphorin 3A is strongly expressed in the lesion, suggesting that peripheral nerve fibres are negatively regulated by semaphorin 3A. 4 However, in our patient the numbers of neurofilament + or PGP9.5 + (Thermo Fisher, Rockford, IL, USA) peripheral nerve fibres in the periphery of the ulcer were significantly increased compared with those in normal individuals (neurofilament: Fig. 1c-e, PGP9.5: Fig.…”

“…The physical examination requires the patient undergo a general skin examination 4 by a non-skin specialist. No currently available laboratory tests adequately detect leprosy.…”

Trigeminal trophic syndrome: an updated review

“…Decreased sensation often accompanies these paresthesias, which leads to severe disfigurement with repetitive manipulation of the area; however, one case series reported 50% of their patients had significant associated pain . The ulcers may be single or multiple, focal or extensive, and are reported more often on the right side of the face . The syndrome most commonly affects the second (maxillary) division of the trigeminal nerve and most commonly arises at the junction of the first and second divisions of the trigeminal nerve at the ala nasi .…”

“…Soft tissue scarring can draw the upper lip into a characteristic sneer . Eye lesions are more common than expected; TTS can often result in eyelid involvement, typically manifesting as ulceration with corneal exposure that responds poorly to surgical intervention given poor tissue healing …”

“…Later, it was noticed that some of these patients developed ulcers on or to the side of nostrils after the procedures . There have been approximately 184 cases described between 1933 and 2012 …”