An atypical case of trigeminal trophic syndrome: A legal medicine perspective in medical responsibility

Abstract

Background:Trigeminal trophic syndrome is a rare complication of peripheral or central damage to the trigeminal nerve characterized by anesthesia, paresthesia and a secondary persistent facial ulceration.Methods:We describe the case of a 40-year-old woman with previous history of Le Fort I osteotomy for a class III malocclusion who developed trigeminal trophic syndrome. Atypically, the cutaneous symptoms appeared bilaterally and 8 years after surgery.Results:Differential diagnosis was based on clinical history, tissue biopsy and serologic evaluation. Atypical findings could be linked to the surgical burdens of Le Fort I osteotomy, a procedure characterized by a bilateral incision on the maxillofacial bones with a reasonable probability of causing a bilateral injury of the peripheral branches of the trigeminal nerve.Conclusion:Although the long delay between trigeminal trophic syndrome onset and surgery and the absence of adequate medical evidence cannot confirm a link with previous surgery in this case, the increasing number of maxillofacial surgery cases suggests that this complication may be more frequent in the next decades, and thus, involved specialists should be aware of this condition as a possible complication of maxillofacial surgery procedures.

Trigeminal Trophic Syndrome—A Unique Clinical Presentation of a Rare Condition

“…Trigeminal trophic syndrome frequently presents with the following triad of symptoms: trigeminal anesthesia, facial paresthesias, and crescent-shaped ulceration of the lateral nasal ala. [2][3][4]8 Unilateral facial ulcers in the setting of former trigeminal injury are pathognomonic for TTS; however, the primary causative condition may not be clear and many clinicians do not realize that the etiology of the lesions is a neurologic problem. 5,9 The majority of cases are caused by iatrogenic damage (eg, rhizotomy) to the trigeminal nerve.…”

“…8,12 Although histologic assessment is not specific, it rules out infection (herpes simplex/zoster, leprosy, syphilis, mucormycosis), granulomatosis, vasculitis, lymphomas, and skin cancer. 3 Once TTS is the most likely diagnosis, management should be multidisciplinary including behavioral modification, neurologic evaluation, pain management, medical treatment, surgical repair, and psychological management. 5,6,11,13 Patient insight into the self-induced mechanism of injury is a key component of treatment.…”

“…Trigeminal trophic syndrome results from damage to the central or peripheral components of the trigeminal nerve pathway leading to facial dysesthesia and trigeminal hypoesthesia or anesthesia. [2][3][4] Selfinflicted manipulation and trauma to the dysesthetic skin leads to cutaneous injury and ulcer development. 5 Diagnosis is often delayed because the clinical presentation is similar to more frequently encountered diagnoses in otolaryngology, including neoplasms (basal cell and squamous cell carcinoma), cutaneous vasculitis, infectious processes (herpetic ulcer), granulomatosis with polyangiitis, and pyoderma gangrenosum.…”

Bilateral Trigeminal Trophic Syndrome

“…Stroke is another common cause of TTS, especially in the case of Wallenberg lateral medullary syndrome and posterior cerebral artery stroke. The time period between the injury to the trigeminal pathway and onset of TTS can differ from 2 weeks to 30 years with the median interval of 1 year [3].…”