Mobius综合征中文文献计量学分析

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Abstract Objective To investigate the clinical characteristics and research progress of Mobius syndrome in newborns.Methods China Journal Full-text Database (CNKI), Wanfang Database (WAN-FANGDATA), VIP Database (CQVIP) and Pubmed Database were searched for related literature that was published between the inception of these databases and March 2018 with such key words as facial paralysis, abducens nerve paralysis and congenital abnormalities. Literature in Chinese that met the inclusion criteria was also collected, and data on cranial nerve damage, abnormalities in other parts of the body, chromosome examination, head MRI examination, abnormalities during pregnancy, delivery and family history was classified and analyzed. Clinical characteristics as well as treatment and prognosis of Mobius syndrome reported in China were analyzed.Results Thirty-three Chinese reports on this syndrome were retrieved and 43 patients(24 males and 19 females) were considered valid. All the patients had symptoms of congenital facial paralysis and abducens nerve paralysis at the same time. What was affected included cranial pairs Ⅵ and Ⅶ, hypoglossal nerve (Ⅻ) pairs (13 cases, 30.23%), oculomotor nerves (Ⅲ), auditory nerves (Ⅷ) , glossopharyngeal nerves (Ⅸ) and vagus or pneumogastric nerves(Ⅹ) (6 cases, 13.95%), trigeminal nerves(Ⅴ) (2 cases, 4.65%). There was no evidence that the olfactory (Ⅰ)and optic nerves (Ⅱ) had been affected by any anomaly in the report we gathered. Magnetic resonance imaging was performed in 18 cases, and 3 cases had positive findings.The result of chromosome analysis in 17 cases was normal. Eight mothers had threatened abortion, abortion failure, colds, medication or X-ray exposure history in early pregnancy. Two cases had family history records. There were few studies with long-term follow-up in China.Conclusions Mobius syndrome is characterized by congenital facial paralysis and strabismus and limitations of eye movement,usually accompanied by other anomalies. There is no effective medical treatment or cure for Mobius Syndrome. Treatment should aim at reduction of symptoms and remain multidisciplinary, with the participation of different health professionals. Identification and intervention should be carried out earlier so as to ensure good outcomes.

Key words： facial paralysis abducens nerve paralysis congenital abnormalities literature analysis

Received: 20 August 2018

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