Yadav Garima, Verma Neha, Sachdeva Sarthak, Goyal Arun
Department of Otorhinolaryngology, University College of Medical Sciences and GTB Hospital, Delhi, India.
Department of Otorhinolaryngology, Shaheed Hasan Khan Mewati Government Medical College, Nalhar, Nuh, Haryana India.
Indian J Otolaryngol Head Neck Surg. 2022 Oct;74(Suppl 2):3411-3415. doi: 10.1007/s12070-021-02550-8. Epub 2021 Apr 23.
Garcin's Syndrome is a rare pathology involving multiple cranial nerves in the setting of invasive mucormycosis, usually in immunocompromised patients. Owing to its extremely high mortality rate, clinician should have a high suspicion for diagnosis. This article presents a rare case of Rhino-Orbito-Cerebral Mucormycosis with Garcin's syndrome in a 33-years old male along with a discussion of previously reported cases. The case is discussed in light of scant contemporary literature on the cited subject. A thorough search using the keywords Garcin's Syndrome, Invasive Mucormycosis, Rhino-orbital, Rhino-cerebral mucormycosis, was conducted on Pubmed/MEDLINE, Google scholar, LILACS, medRxiv and Google. A total of six reported cases found in international literature published between 2000 and 2020 were reviewed and analyzed. Garcin's Syndrome is associated with a high mortality rate. In our review, of the total seven patients, only three survived, bringing the collective mortality to 42.85%. Patients had multiple cranial nerve involvement leading to long term sequelae. Our case showed the unique finding of VIII nerve involvement. Early intervention helped in patient survival and better functional outcome. This literature review highlights the distinct clinical nature of the presentation of disease and the importance of prompt diagnosis and early management in the reversal of complications in an otherwise potentially fatal disease.
加欣综合征是一种罕见的病理情况,通常发生在免疫功能低下的患者中,由侵袭性毛霉菌病累及多条颅神经所致。鉴于其极高的死亡率,临床医生应高度怀疑并进行诊断。本文介绍了一例33岁男性患鼻眶脑型毛霉菌病合并加欣综合征的罕见病例,并对既往报道的病例进行了讨论。结合关于该主题的少量当代文献对该病例进行了讨论。使用关键词“加欣综合征”“侵袭性毛霉菌病”“鼻眶型”“鼻脑型毛霉菌病”在PubMed/MEDLINE、谷歌学术、LILACS、medRxiv和谷歌上进行了全面检索。对2000年至2020年间发表的国际文献中报道的总共6例病例进行了回顾和分析。加欣综合征的死亡率很高。在我们的综述中,7例患者中只有3例存活,总体死亡率为42.85%。患者有多条颅神经受累,导致长期后遗症。我们的病例显示了听神经受累这一独特发现。早期干预有助于患者存活并获得更好的功能预后。这篇文献综述强调了该疾病表现的独特临床性质,以及及时诊断和早期管理对于逆转这种潜在致命疾病并发症的重要性。