Trigeminal trophic syndrome of all three nerve branches: an underrecognized complication after brain surgery

Abstract

The authors report a case of trigeminal trophic syndrome (TTS) that occurred as a complication of a neurosurgical procedure. Three years after a second surgical treatment for a meningioma of the cerebellopontine angle, this 32-year-old woman developed TTS with progressive skin ulcers on the left ala nasi and the left side of the forehead and chin. Trigeminal trophic syndrome is an extremely rare cause of facial ulceration. It occurs as a consequence of trigeminal nerve damage or impaired central sensory connections. To the authors' knowledge, this is the first report of lesions in the dermatomes of all three branches of the nerve after a neurosurgical procedure. Early recognition of this disorder is important, as treatment is difficult and often unsatisfactory. Many clinicians are not aware of this disease, thus, it may be more common than previously thought. The importance of recognizing and diagnosing TTS, as well as its treatment, are discussed.

Trigeminal trophic syndrome: Report of a case and review of the published work

“…When gabapentin was effective but needed to be replaced due to side effects, tiagabine or oxcarbazepine could be administrated instead . Carbamazepine, which was the second most frequently used medication (seven cases), was quite effective in some patients . Amitriptyline has been used traditionally (four cases) without showing significant efficacy.…”

Trigeminal trophic syndrome: an updated review

“…Ulcer formation is possibly triggered by self‐mutilation in response to a sensation of itching or burning . Other less frequently associated causes include tumors (astrocytoma, meningioma), trauma, craniotomy, infection (herpes, syphilis, leprosy) and syringobulbia . Alcohol injection, surgical ablation, rhizotomy of the sensory component of the trigeminal nerve, coagulation of Gasserian ganglion, vertebrobasilar insufficiency, acoustic neuroma, postencephalitis, and amyloid deposits in the central nervous system and trigeminal nerve have also been reported .…”

Síndrome trófica trigeminal – Relato de caso e revisão de literatura

“…7,8 Raramente, há acometimento das três divisões do NT como em nosso caso. 3,9 O NT pode ser acometido em seu trajeto central ou periférico, mas é o gânglio de Gasser a porção mais comumente envolvida. 2 Há relato de casos idiopáticos, 8 porém a etiologia mais frequentemente relatada são as lesões iatrogênicas para tratamento de neuralgia do trigêmeo (rizotomia por radiofrequência ou alcoolização); outras causas menos comuns incluem: infarto do tronco cerebral, tumores intracranianos (meningiomas, neurinomas do acústico, astrocitomas), herpes zóster, seringobulbia, insuficiência vertebrobasilar, encefalites e traumatismos cranianos.…”

“…Acomete preferencialmente o sexo feminino com incidência de cerca de 2:1 em relação ao sexo masculino, o que corresponde à ocorrência aproximada de neuralgia do trigêmeo. [2][3][4] Há relatos de pacientes com 14 meses de vida, bem como de 93 anos de idade, 11 sendo a sexta década a mais comumente acometida. 4 As lesões são tipicamente úlceras em crescente que se iniciam quase que invariavelmente na asa do nariz, podendo acometer qualquer área dos dermátomos trigeminais.…”

“…Desta forma, estudos sorológicos e análise histopatológica das lesões devem sempre ser incluídas na propedêutica destes pacientes. 2,3,14 Os achados típicos de biópsia não são patognomônicos e incluem hiperplasia epitelial com proliferação vascular e fibrose da derme; atipias celulares ou células gigantes não devem ser encontradas. 4 A STT é um distúrbio de difícil terapêutica.…”