Medicina Repostabout 7 years ago

Trigeminal trophic syndrome

Trigeminal trophic syndrome is a rare disease resulting from injury to the trigeminal nerve. It is characterised by par- and anaesthesia leading to self-manipulation and ulcerative lesions in the trigeminal area. Differential diagnoses are malignancies as well as infectious and autoimmune diseases. Our case presents a severe form of trigeminal trophic syndrome as a late complication of sub-occipital vestibular schwannoma extirpation. An 84-year old female patient presented to our clinic complaining of progressive loss of vision on the right eye. Over 20 years ago, she had had a right-sided vestibular schwannoma extirpated via a sub-occipital approach with simultaneous ventriculoperitoneal shunt placement. Several years later, the patient started to complain of par- and hypaesthesia resulting in a habit of repetitive self-manipulation within the trigeminal area. The self-manipulating behaviour had been aggravated over the past years due to progress of a dementia syndrome. On physical examination, an extensive right-sided facial defect could be seen affecting the upper lip, cheek, ala nasi, the eyeball as well as the right forehead. Furthermore, after ophthalmologic consultation, right-sided amaurosis was diagnosed. After a gerontopsychiatric evaluation, the patient was initialised on medical therapy with sertraline and carbamazepine. Simultaneously, local wound cleaning and an intravenous antibiotic therapy were performed for infection prophylaxis. Possibly aggravated by the dementia syndrome, self-manipulation continued despite conservative measures, leading to progress of the facial defect with complete destruction of the eye ball. For that reason and due to the high risk of infection with potential intracranial spread, it was decided that a surgical wound debridement with exenteratio orbitae be performed. Closure of the large surgical defect required a large tissue bulk. Therefore, a free vascular flap transfer was performed in collaboration with the department for plastic and reconstructive surgery using an anterolateral thigh ALT flap with vascular anastomosis to the ipsilateral facial artery.