Trigeminal Trophic Syndrome: Diagnosis and Management Difficulties

CASE REPORT Trigeminal Trophic Syndrome: Diagnosis and Management Difficulties Ahmad F. Bhatti, F.R.C.S.I., A.F.R.C.S.I. Daniela Soggiu, M.D. Antonio Orlando, F.R.C.S., Dip.E.B.O.P.R.A.S. Bristol, United Kingdom 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 REPORT A 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 From the Department of Plastic and Reconstructive Surgery, Frenchay Hospital. Received for publication July 17, 2005; accepted October 27, 2005. Copyright ©2007 by the American Society of Plastic Surgeons DOI: 10.1097/01.prs.0000293759.03833.88 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 at the site of ulceration. DISCUSSION Trigeminal trophic syndrome, or “ulceration en arc,” is a rare condition and presents as a triad of trigeminal sensory impairment, altered facial sensations, and crescent-shaped ulceration of the ala nasi. McKenzie1 in Canada and Loveman2 in the United States first described the condition in 1933. They recognized that some surgical procedures (principally in the treatment of trigeminal neuralgia) that resulted in trigeminal sensory impairment would develop ulceration of facial skin. When this involved the ala, this could result in full-thickness destruction of the skin and the underlying cartilage. Disclosure: None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this article. www.PRSJournal.com 1e Plastic and Reconstructive Surgery • January 2008 Fig. 1. Preoperative view of the patient at the time of presentation. Fig. 2. Postoperative view. Trigeminal trophic syndrome has been reported in diabetes, tabes, postencephalitic parkinsonism, and vascular disease and after surgery for acoustic neuroma and syringomyelia. Occasionally, the exact cause remains uncertain.3– 6 The patient often develops altered sensations, such as burning or crawling, mostly in the area of 2e sensory overlap between the ophthalmic and maxillary divisions of the trigeminal nerve. These sensations focus the patient’s attention on this area and consciously or subconsciously encourage “picking” to attempt symptomatic relief.7 Clinically, depigmentation, dermatitis, and a dry, atrophic nasal mucosa predispose to epistaxis and crusting. Classically, ulceration of the ala nasi has a “punched-out” appearance of the skin and cartilage and may appear months to years after the insult to the nerve.8 This emphasizes the need for a thorough history, examination, and investigation that includes a tissue biopsy. The diagnosis is mainly one of exclusion. Differential diagnosis includes malignant, infectious, granulomatous, and vascular lesions. The underlying cause is still unknown. It has been hypothesized that autonomic vasomotor dysfunction may play a role in its development. Datta et al.8 have suggested that when the gasserian ganglion is destroyed, sympathetic fibers from the internal carotid artery are somehow disrupted, resulting in a persistent low sympathetic tone. This leads to constant cooling of the skin secondary to vasodilatation and a slower venous return. This in turn results in an unfavorable environment for wound healing. Many treatment options have been proposed. The simplest management is prevention. This can be achieved by advising the patient to restrain from picking the area of irritation and keeping the dry mucosa well lubricated, thereby preventing the sensation of blocked nasal airways. Pharmaceutical treatment regimens involving amitriptyline, diazepam, chlorpromazine, and carbamazepine have been used with limited success.9 –11 They seem to influence both the paresthesia and the behavioral factors involved in this syndrome. Westerhof and Bos successfully treated a trigeminal trophic syndrome patient with transcutaneous electrical nerve stimulation. The patient’s consequent healing was attributed to the enhancement of local blood supply by the transcutaneous electrical nerve stimulation.12 Surgical management has proved difficult in the past. Local flaps using the skin from the anesthetic side tend to shrink and develop recurrent ulceration.13 Anesthesia and concomitant vasomotor dysfunction are prerequisites for these dystrophic lesions. It is suggested that bringing in tissue from the nonanesthetic side with its own blood and nerve supply improves the success of the treatment of trigeminal trophic syndrome.6,7,14 This was the route chosen in this case. Volume 121, Number 1 • Trigeminal Trophic Syndrome CONCLUSIONS It is very difficult to explain the exact underlying mechanism for survival of the flap following pedicle division in the second stage when the flap also becomes anesthetic because of denervation. Recurrence of the ulceration might therefore be anticipated, but this did not occur during follow-up of more than 2 years. We believe that the contralateral skin flap might have assisted healing only during the first stage before division of the pedicle, interrupting the vicious cycle of dysesthesia and self-harm. After division of the pedicle, the flap was rendered anesthetic, thus preventing triggering sensations from stimulating recurrence symptomatology. Ahmad F. Bhatti, F.R.C.S.I., A.F.R.C.S.I. Department of Plastic and Reconstructive Surgery Frenchay Hospital Frenchay Park Road Frenchay, Bristol BS16 1LE, United Kingdom [email protected] REFERENCES 1. McKenzie, K. G. Observations on the operative treatment of the trigeminal neuralgia. Can. Med. Assoc. J. 29: 492, 1933. 2. Loveman, A. B. An unusual dermatosis following section of the fifth cranial nerve. Arch. Dermatol. Syphilis 28: 369, 1933. 3. Karnosh, L. J., and Scherb, R. F. Trophic lesions in the distribution of the trigeminal nerve. J.A.M.A. 115: 2144, 1940. 4. Rosenberg, S. J., and Solovay, J. Trophic ulcer following encephalitis lethargica. Arch. Dermatol. 39: 825, 1939. 5. Freeman, A. G. Neurotrophic ulceration of the face with erosion of the ala nasi in vascular disorders of the brainstem. Br. J. Dermatol. 78: 322, 1966. 6. McLean, L. R., and Watson, A. C. H. Reconstruction of a defect of the ala nasi following trigeminal anaesthesia with an inverted forehead flap. Br. J. Plast. Surg. 35: 201, 1982. 7. 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