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Trigeminal Trophic Syndrome

  • Charles H. Dicken
    Correspondence
    Address reprint requests to Dr. C. H. Dicken, Department of Dermatology, Mayo Clinic Rochester, 200 First Street SW, Rochester, MN 55905
    Affiliations
    Department of Dermatology, Mayo Clinic Rochester, Minnesota
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      Seven cases of trigeminal trophic syndrome are reported. In this rare condition, neurotrophic ulcers occur on the face, especially in the ala nasi area in a dermatome of the trigeminal nerve that has been rendered anesthetic, usually as a complication of trigeminal ablation by surgical means or injection for treatment of trigeminal neuralgia. The period from time of trigeminal nerve injury to onset of the ulcer varies from weeks to several years, with a mean of 1 to 2 years. Self-induced trauma is believed to produce the tissue destruction. Once the ulcers appear, they are extremely persistent.
      TTS (trigeminal trophic syndrome)
      Trigeminal trophic syndrome (TTS)
      • Weintraub E
      • Soltani K
      • Hekmatpanah J
      • Lorincz AL
      Trigeminal trophic syndrome: a case and review.
      has been previously reported as “trigeminal neurotrophic ulceration,” “trigeminal neuropathy with nasal ulceration,” “trophic ulceration of the ala nasi,” and “ulceration en arc.” In this syndrome, the most characteristic ulcer is nasal. The defect is typically crescentic, with loss of the ala nasi corresponding to the area of the nose lacking cartilage (Fig. 1). This loss may lead to necrosis of the cartilage framework. If the ulceration extends onto the cheek and upper lip, the scarring and tissue loss can draw the upper lip into a characteristic sneer (Fig. 2). Trophic ulcers can also develop in areas such as the forehead, scalp, and cheek (Fig. 3).
      Figure thumbnail gr1
      Fig. 1 (case 1)Crescentic or y-shaped ala nasi tissue loss and some loss of upper lip tissue.
      Figure thumbnail gr2
      Fig. 2 (case 6)Extensive loss of ala nasi, with typical crescentic defect. Extension of tissue loss onto upper lip draws lip into characteristic sneer.
      Figure thumbnail gr3
      Fig. 3 (case 3)Neurotrophic ulcers on forehead, cheek, and upper lip, with sparing of ala nasi area.
      The ulcers develop in a dermatome of the trigeminal nerve rendered anesthetic by a surgical or other process involving the trigeminal nerve or its central sensory connections. Paresthesias and self-induced trauma to the area further support the diagnosis of TTS.
      Treatment has not been successful. Possible insight into the self-induced nature of the damage can be helpful. A protective dressing such as a hydrocolloid dressing (Duoderm) or a protective device can be effective, but usually only for a brief period.

      CASE MATERIAL

      The data from seven patients with disorders typical of TTS who were examined at the Mayo Clinic are reported in Table 1. Of the seven patients, five had TTS as a complication of surgical ablation by rhizotomy or alcohol injection into the gasserian ganglion for treatment of trigeminal neuralgia. An astrocytoma and a car accident caused the trigeminal nerve changes in one patient each. Another patient was also in a car accident, but the ulcers developed after injection of glycerol.
      Table 1Data From Seven Patients With Trigeminal Trophic Syndrome
      CaseAge (yr)
      Age at onset of ulcer.
      and sex
      Side of faceCauseLatent period (mo)Area of ulcerBiopsies (no.)Surgical repairDuration of ulcer (yr)Treatment
      125 FLAstrocytoma7Nose2Nasal reconstruction and flap4Hydrocolloid dressing, protective device
      231 FRFractures due to car accident3Nose, forehead, scalp4Nasal reconstruction7Hydrocolloid dressing, antibiotics
      337 FLFractures due to car accident, glycerol injections2Nose, chin, cheek, forehead1None4Vitamins, vegetable shortening, antibiotics, protective device
      456 FRAlcohol injections12Nose2None28X-ray, nose shield, amitriptyline hydro-chloride, fluphenazine
      566 MRAlcohol injection, retrogasserian neurectomy60Bridge of nose1Excision1Excision
      672 FLAlcohol injection, radio frequency therapy24Nose2Nasal reconstruction and flap, revision of flap, cartilage graft20Antibiotics, hydrocolloid dressing, haloperidol
      777 FLTrigeminal rhizotomy10Eyelid, forehead, scalp0None74Hydrocolloid dressing
      * Age at onset of ulcer.
      Six of the seven patients were women. The mean age at the onset of ulcers was 52 years, and the range was 25 to 77 years. Four lesions were on the left and three were on the right side of the face. Three patients had only ala nasi ulcers. Two had ala nasi ulcers and involvement of other areas of that side of the face. In one patient, only the bridge of the nose was affected, and another had no nasal lesions but had ulcers on the eyelid, forehead, and scalp.
      A biopsy was done in all but one of the patients, and four underwent multiple biopsies. All specimens showed chronic inflammation and no cancer. In three patients, nasal reconstruction was unsuccessful.
      The mean latent period-the time for the ulcer to occur after trigeminal nerve damage-was 17 months, and the range was 2 to 60 months. The mean duration of the ulcer was 64 months, with a range of 3 to 336 months.

      DISCUSSION

      Trigeminal neuralgia is a common disorder in elderly patients, and the pain can make life miserable. Relief may be obtained by cutting the sensory root or by injecting alcohol into the gasserian ganglion. Relief of pain may cause loss of the sensations of pain, temperature, and light touch, with resultant paresthesia in the trigeminal area.
      Most patients with TTS notice a burning, itching, tickling, or crawling sensation, and most admit to picking or rubbing the area. Why only certain patients with facial anesthesia experience paresthesias and why ulcers develop in only a few are unclear issues. Some patients may be prone to habitual picking of the nasal area.
      Once the picking and the ulcer start, the course is usually chronic. In one of the patients, the nasal ulcer had persisted 28 years and was present at the time of death.
      Surgical repair is usually unsuccessful, as in these seven patients. Surgical repair may be worthwhile, however, if tissue with functional innervation and its own blood supply is used.
      • McLean NR
      • Watson AC
      Reconstruction of a defect of the ala nasi following trigeminal anaesthesia with an innervated forehead flap.
      In some patients, insight into the self-induced trauma may be sufficient, but the chronicity of the ulcers suggests that this is not generally true. Use of a device or prosthesis to protect the ulcerated area may be helpful, especially if patients are convinced that the ulcer will not heal if they continue to touch the area.
      Many other treatments have been suggested, including cervical sympathectomy,
      • McKenzie KG
      Observations on results of operative treatment of trigeminal neuralgia.
      ionizing radiation,
      • Loveman AB
      An unusual dermatosis following section of the fifth cranial nerve.
      antibiotics.
      • Howell JB
      Neurotrophic changes in the trigeminal territory: disturbances after operation for trigeminal neuralgia.
      transcutaneous electrical nerve stimulation,
      • Westerhof W
      • Bos JD
      Trigeminal trophic syndrome: a successful treatment with transcutaneous electrical stimulation.
      pimozide,
      • Mayer RD
      • Smith NP
      Improvement of trigeminal neurotrophic ulcération with pimozide in a cognitively impaired elderly woman-a case report.
      diazepam and amitriptyline hydrochloride,
      • Finlay AY
      Trigeminal trophic syndrome.
      and vitamin B supplementation.
      • Philpott OS
      Trophic ulcer complicating operative procedures for the relief of trigeminal neuralgia.
      Seventy-five percent of cases of TTS have been associated with surgical trigeminal ablation by rhizotomy or alcohol injection into the gasserian ganglion.
      • Weintraub E
      • Soltani K
      • Hekmatpanah J
      • Lorincz AL
      Trigeminal trophic syndrome: a case and review.
      Less common causes are vertebrobasilar insufficiency, acoustic neuroma, postencephalitic parkinsonism, syringobulbia, meningioma, astrocytoma, Hansen's disease, and trauma.
      • Weintraub E
      • Soltani K
      • Hekmatpanah J
      • Lorincz AL
      Trigeminal trophic syndrome: a case and review.
      Other Other causes to be considered are herpetic ulceration and other infectious diseases, such as syphilis, yaws, leprous trigeminal neuritis, deep fungi, mycobacteria, rhinoscleroma, and leishmaniasis. Carcinoma is often suspected, and all but one of these seven patients underwent one or more biopsies to rule out this disease. Pyoderma gangrenosum, Wegener's granulomatosis, and midline granuloma are also considerations. Factitial dermatitis should not be the diagnosis if neurologic signs are present.
      TTS is highly recognizable. In a patient with a history of trigeminal nerve damage, the crescentic ulceration of the ala nasi is almost pathognomonic. Of importance, in a small percentage of patients, ulcers may occur in a nonnasal area such as the forehead, scalp, or cheek.
      Patients with TTS may first be examined by primary-care physicians, otolaryngologists, neurologists, neurosurgeons, or dermatologists. If these physicians are aware of TTS, appropriate diagnosis and management will benefit the patient.

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        Trigeminal trophic syndrome: a case and review.
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        Trigeminal trophic syndrome: a successful treatment with transcutaneous electrical stimulation.
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        Improvement of trigeminal neurotrophic ulcération with pimozide in a cognitively impaired elderly woman-a case report.
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        Trigeminal trophic syndrome.
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