Trigeminal Trophic Syndrome: Diagnosis and Management Difficulties

Abstract

T rigeminal trophic syndrome is a rare complication after peripheral or central damage to the trigeminal nerve, characterized by sensorial impairment in the trigeminal nerve territory and self-induced nasal ulceration. Conditions that can affect the trigeminal nerve include brainstem cerebrovascular disease, diabetes, tabes, syringomyelia, and postencephalopathic parkinsonism; it can also occur following the surgical management of trigeminal neuralgia. Trigeminal trophic syndrome may develop months to years after trigeminal nerve insult. Its most common presentation is a crescent-shaped ulceration within the trigeminal sensory territory. The ala nasi is the most frequently affected site.Trigeminal trophic syndrome is notoriously difficult to diagnose and manage. A clear history is of paramount importance, with exclusion of malignant, fungal, granulomatous, vasculitic, or infective causes. We present a case of ulceration of the left ala nasi after brainstem cerebrovascular accident. CASE REPORTA 52-year-old man was referred to our department for assessment of ulceration of the left ala nasi. He had suffered a cerebrovascular accident in 1999 secondary to left vertebral artery dissection resulting in brainstem infarction. Initial treatment was with warfarin, which was subsequently replaced with aspirin. Identified risk factors included hypertension and hypercholesterolemia.Residual symptoms included left arm weakness, facial numbness, and poor memory. Perception of light touch and pain over the left side of the face was significantly decreased, with an absent corneal reflex. Over the following month, itchiness of the left nostril developed, which provoked intense scratching of the ala. Over the course of 1 year, this resulted in a nonhealing ulcer affecting the left ala of his nose (Fig. 1).A dermatologist diagnosed the condition on the basis of the patient's history before referral. At this stage, the ulcerated area was treated with occlusive dressings and the patient was advised to minimize trauma to the nose. In addition, carbamazepine was commenced to reduce paresthesia. The case was referred to our department in May of 2001 for further management.Initially, the ulcer was debrided and the tissue sent for histologic analysis, which revealed hyperplastic epithelium without evidence of malignancy, infection, or vasculitis. His condition was complicated further by bleeding from the nasal vestibule over the following month. This was attributed to subconscious continuous trauma.A two-stage reconstruction of the ala was planned. In the first stage, the alar defect was corrected with a chondrocutaneous composite graft taken from the right ear, covered externally with a pedicled forehead flap based on the contralateral supratrochlear vessels and nerve. Three weeks later, the second stage was performed comprising division of the pedicle with minor readjustments of the flap (Fig. 2).The patient has been followed for over 2 years after surgery. He remains satisfied with the result and has not developed any further problems...

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.…”

“…6,7 The management and treatment strategies used in TTS differ from those employed to address these more commonly encountered conditions. 4,6 Prompt recognition of TTS is important to prevent delays in diagnosis and initiation of targeted 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.…”

Síndrome trófica trigeminal – Relato de caso e revisão de literatura

“…Cirurgia plástica reconstrutora é utilizada para correção de defeitos, em especial da asa do nariz. 20,21 A STT é uma doença pouco conhecida na literatura neurocirúrgica (de todos os trabalhos utilizados para consulta neste relato, apenas um provém de publicação neurocirúrgica), seu tratamento é difícil e em geral envolve abordagem multidisciplinar. Como a principal etiologia da síndrome é resultado de intervenção direta do neurocirurgião, entendemos que seu reconhecimento deve fazer parte da gama de diagnósticos neurocirúrgicos.…”

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

“…[10][11][12] It is important to clarify that the term "trophic" can be misleading since the ulcerations appear to be self-inflicted by the patient as an attempt to relieve itching and pain; such behavior has also been reported during the sleep. 13…”