Willis et al.: Treatment Options in Trigeminal Trophic Syndrome. A Multi-Institutional Case Series. Case Report

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1 The Laryngoscope VC 2011 The American Laryngological, Rhinological and Otological Society, Inc. Case Report Treatment Options in Trigeminal Trophic Syndrome: A Multi-Institutional Case Series Mark Willis, MD; William W. Shockley, MD; Steven R. Mobley, MD Trigeminal trophic syndrome (TTS) is an uncommon medical condition that may be encountered by otolaryngologists and facial plastic surgeons. TTS begins with damage to the trigeminal nerve or its central sensory connections, causing anesthesia in a dermatomal distribution. With repeated scratching and manipulation, an ulceration occurs, often in the alar region. In this multi-institutional report, we summarize a small series of patients with TTS. Treatment options are discussed along with a review of the relevant literature. Although rare, it is important that practicing otolaryngologists be familiar with the classic signs and symptoms of this condition in order to avoid delays in diagnosis and treatment. Key Words: Trigeminal Trophic Syndrome, nasal reconstruction, nasal ulceration, facial plastic surgery. Level of Evidence: 4. Laryngoscope, 121: , 2011 INTRODUCTION Trigeminal trophic syndrome (TTS) is a relatively rare condition. The disease process involves self-manipulation/destruction of the nasal region following stroke, denervation of the trigeminal nerve, or treatment of trigeminal neuralgia. The clinical features of the disease typically involve one or more painless, crescent-shaped ulcers in the alar region (Fig. 1). More severe cases can involve tissue loss and may spread to the cheek and upper lip. Typical treatments include neurologic evaluation, behavioral modification, medical management, and surgical repair. Despite these multiple modalities, the ulcers frequently recur and pose a dilemma for clinicians. The following cases underscore the importance of the clinician s familiarity with the disease to ensure prompt diagnosis and treatment. CASE 1 A 49-year-old Caucasian woman presented to the University of Utah Facial Plastic Surgery Clinic with right alar retraction and partial destruction of the alar From the Division of Otolaryngology Head and Neck Surgery, Department of Surgery (M.W., S.R.M.), University of Utah School of Medicine, Salt Lake City, Utah, and the Department of Otolaryngology Head and Neck Surgery (W.M.S.), University of North Carolina School of Medicine, Chapel Hill, North Carolina, U.S.A. Editor s Note: This Manuscript was accepted for publication June 22, The authors have no funding, financial relationships, or conflicts of interest to disclose. Send correspondence to Dr. Steven R. Mobley, 50 North Medical Drive 3C120, Salt Lake City, UT steven.mobley@hsc.utah.edu DOI: /lary rim (Fig. 1A). She first noted the retraction seven years prior, following a vertebral artery dissection that left her with decreased sensation in the right side of her face. She proceeded to have recurrent episodes of bleeding and ulceration of the right ala. This expanded to include the medial right cheek and subsequently led to destruction of the right alar rim. Before proceeding with reconstruction, the cause of the alar destruction was investigated. The first suspicion was that a neoplastic process had eroded the nostril and was possibly inspiriting deeper into the mid-facial tissues. Biopsies and magnetic resonance imaging (MRI) of the head and neck were obtained but were not consistent with a neoplastic process. Immunohistochemical staining was also negative for malignancy or an autoimmune process. Laboratory tests, including CBC, CMP, c-anca, p-anca, and RF, were all normal or negative. The case was presented at a multidisciplinary dermatologic board, and the diagnosis of TTS was reached. A reconstructive plan was designed using a paramedian forehead flap (PMFF). Because the defect was through-and-through, the technique of a three-stage PMFF as described by Menick and Burget 1 was chosen. The first stage reconstituted the inner lining with a full-thickness skin graft and the external lining with a PMFF. In the second stage, the flap was debulked and further shaped, and support was added from a large auricular cartilage graft (Fig. 2). The flap was divided and inset three weeks later. In the three months following the final stage, the affected vestibular opening began to slowly narrow. The nostril stenosis was addressed using a technique that

2 Fig. 1. Photographs from the initial clinic visits. Three-quarter views are shown for all three patients: (A) case 1; (B) case 2; (C) case 3. has been previously described. 2 Briefly, this technique involves excision of the obstructive scar tissue and stenting of the internal nostril with a thermoplastic splint that is held in place with a through-and-through bolster technique for approximately two weeks (Fig. 3). The long-term results have been good. The patient was most recently seen in the office 20 months from her original presentation and six months from her nostril stenosis repair. She is maintaining good aesthetic form, contour, and functional nasal airflow through the affected side. The ulceration has not recurred (Fig. 4). CASE 2 A 29-year-old male presented to the University of North Carolina (UNC) Facial Plastic Surgery Clinic with an erythematous, ulcerative lesion along the right nasal ala. Eighteen months prior, he was involved in a rollover motor vehicle accident and subsequently had hypesthesia over the right side of his face. He had lost his left eye from a prior injury. Ten months after the trauma, an erythematous lesion occurred along the right nasal ala. The lesion began to crust and slowly progressed to an erosive lesion with significant tissue loss (Fig. 1B). Initially, there was suspicion of malignancy, and a biopsy was performed, which showed chronic inflammation. At this point the patient was referred to UNC. Given the patient s history, the appearance of the lesion, and the negative biopsy, a diagnosis of TTS was made. The patient was counseled to keep the area moist with topical petrolatum ointment. Once complete epithelialization occurred and the area remained free of crusting or ulceration for six months, reconstruction was considered (Fig. 5). The patient was taken to the operating room for a staged nasal reconstruction using a right PMFF, conchal cartilage graft, and septal mucosal flap (Fig. 6). His postoperative course was complicated by distal necrosis of his septal flap and partial loss of the cartilage graft, likely related to his smoking a habit not revealed until after surgery. He developed a right nasal stenosis and two months later underwent debulking of the flap, a composite graft, and repair of the stenosis. This was followed by division and inset of the flap (Fig. 7). His final procedure consisted of revision of his right alar deformity, thinning of the supra-alar soft tissues, and additional cartilage grafting to the right nasal alar margin (Fig. 8). He was lost to follow-up shortly after the last procedure. Fig. 2. Introperative photographs illustrating the second stage of the reconstruction. Preoperative designs from the lateral (A) and base (B) views are shown as well as the postoperative base view (C). 713

3 Fig. 3. Clinic photographs, two weeks after the final state of reconstruction. The base view (A), three-quarter view (B), and base three-quarter view (C) are shown. Fig. 4. Photographs from the final clinic visit. Three-quarter (A), standard frontal (B), and base view (C) images are shown. CASE 3 A 52-year-old female was diagnosed with trigeminal neuralgia 10 years prior to presentation to the UNC Facial Plastics Clinic. Seven years prior to presentation, she underwent a trigeminal ablation procedure with resultant right hemi-facial anesthesia. Approximately one year prior to presentation, a raw area that would intermittently bleed and crust appeared at the base of her right nasal ala. She was referred to UNC after extensive workup at an outside facility failed to determine the cause of the lesion. When seen in clinic, she described numbness of the right face and admitted to unconscious manipulation of the lesion. Physical exam demonstrated absence of her right nasal ala and columellar shift to the right (Fig. 1C). Her cheek exhibited erythema and crusting. Based on these findings and her history of trigeminal nerve ablation, the diagnosis of TTS was made. Initial treatment included cleaning with halfstrength hydrogen peroxide followed by application of petrolatum ointment. A psychiatric referral was also made for behavioral modification, and extensive counseling was undertaken regarding the need for the patient to abstain from manipulating the area for a minimum of six months, with a goal of complete epithelialization with the absence of any inflammatory signs for that six-month period. The area of concern was monitored clinically until it had been quiescent for 10 months. At that time, reconstructive options were presented to the patient. 714 Fig. 5. Right lateral photograph taken after healing of the ulcer.

4 Fig. 6. Intraoperative photographs from the first stage of the paramedian forehead flap reconstruction: (A) nasal alar defect; (B) design and incision of forehead flap; (C) flap sutured into position. Fig. 8. Intraoperative photographs of the last revision procedure. (A) Sculpting of supra-alar soft tissues with insertion of additional cartilage graft at alar margin; (B) bolster in place; (C) base view. Secondary to the patient s employment status and other social issues, she opted to delay reconstruction. She has done well keeping the area of original ulceration quiescent by the regular use of good hygiene and petrolatum ointment as needed. TABLE I. Causes of Trigeminal Nerve Damage Implicated in Cases of Trigeminal Trophic Syndrome. Iatrongenic Organic Infectious/Other Fig. 7. Photographs at follow-up visit after division and inset of flap. Right lateral (A), standard frontal (B), and base views (C). Trigeminal ablation Cerebral infarction Mycobacterium leprae neuritis Gasserian ganglion injection Acoustic neuroma Herpes zoster ophthalmicus Craniotomy Astrocytoma Trauma Thermo- or Meningioma Idiopathic electrocoagulation Spinal cord degeneration Syringobulbia Postencephalic parkinsonism 715

5 DISCUSSION Trigeminal trophic syndrome was first described by Loveman 3 and McKenzie 4 in The lesions are complications of trigeminal anesthesia that occurs following damage to the sensory root of the trigeminal nerve. Subsequent self-manipulation leads to progressive ulceration of the affected dermatome. The classic clinical presentation of TTS is one or more crescent-shaped ulcers that arise along one side of the nose. Picking of these lesions can lead to tissue loss, which can spread to the cheek and upper lip in extreme cases. Ulcerations can present in other locations in about 13% of cases, including ulcerations of the scalp, forehead, ear, palate, jaw, and cornea. 5 A key feature of TTS is the frequent sparing of the nasal tip, due to its innervation from the ethmoidal branch of the ophthalmic division (V1) of the trigeminal nerve. There are many possible causes of TTS but, most commonly, the condition is seen following ablation treatment for trigeminal neuralgia (Table I). The differential diagnosis of TTS is broad, and reaching a definitive diagnosis largely involves excluding other causes of facial ulceration. These include basal cell carcinoma, squamous cell carcinoma, herpetic ulceration, syphilis, leprosy, Wegener s granulomatosis, and other less common causes. The persistence of these lesions poses a treatment challenge for the clinician. A multidisciplinary approach should be utilized and may include neurologic evaluation, pain management, medical treatment, surgical repair, and psychological management to assist with reduction of self-manipulation. A crucial element of treatment involves the education of the patient as to the underlying cause of the ulcers (digital picking/manipulation) and convincing them that the lesions are selfinduced. Several medications have been reported to show some benefit in the treatment of TTS. Carbamazepine has been reported to reduce the paresthesias associated with the sensorial impairment of the face within 48 hours of initiation of treatment and to promote healing after four weeks of use. 6 Other drugs that have been shown to have limited benefit include vitamin B, 7 diazepam, amitriptyline, 8 chlorpromazine, 9 acyclovir, 10 and injected triamcinolone. 11 Transcutaneous electrical stimulation has also been reported to treat TTS successfully. Westerhof and Bos 12 attributed their findings to an improvement in local blood supply that resulted from the transcutaneous electrical impulse. Surgical treatment has shown mixed results in the past. If local flaps are used from adjacent areas of the face that are anesthetic as well, the repairs tend to shrink and develop recurrent ulceration. 13 However, it has been shown that when regional flaps which have their own blood and nerve supply are used, the longterm results have been good. 14 A successful repair using a PMFF was described by McLean and Watson years ago and was the choice for two of the patients in this series. CONCLUSIONS We present a small series of patients with TTS from two university institutions who underwent treatments ranging from immediate reconstruction, reconstruction following six months of wound care, to observation and wound care without reconstruction. An instructional point from the care of the patients in this report is that, while reaching a diagnosis was initially a dilemma for those not familiar with the disease, all of the patients had presentations that were typical and consistent with other published photos of this disease process. This highlights the importance of the otolaryngologist s awareness of the classic history and visual presentation of TTS, which will help to expedite diagnosis, decrease costly workups, and begin proper medical treatment and reconstructive surgery planning more rapidly. BIBLIOGRAPHY 1. Burget GC, Menick FJ. Aesthetic Reconstruction of the Nose. St. Louis: Mosby; Daines SM, Hamilton GS, Mobley SR. A graded approach to repairing the stenotic nasal vestibule. Arch Facial Plast Surg 2010;12: Loveman A. An unusual dermatosis following section of the fifth cranial nerve. Arch Dermatol Syph 1933;28: McKenzie K. Observations on the results on the operative treatment of trigeminal neuralgia. Can Med Assoc 1933;29: Weintraub E, Soltani K, Hekmatpanah J, et al. Trigeminal trophic syndrome. A case and review. J Am Acad Dermatol 1982;6: Bhushan M, Parry EJ, Telfer NR. Trigeminal trophic syndrome: successful treatment with carbamazepine. 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