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小儿莫比乌斯综合征的变革性牙科护理:在长期住院期间衔接口腔健康与全身管理

Transformative Dental Care in Pediatric Moebius Syndrome: Bridging Oral Health and Systemic Management During Prolonged Hospitalization.

作者信息

Delgado Renata Zoraida Rizental, Bueno Marcus, Mugayar Leda, Franco Juliana Bertoldi

机构信息

Inclusive Care Clinic, College of Dentistry, University of Illinois, Chicago, Illinois, USA.

Department of Dentistry, Central Institute, Clinical Hospital of Medical School of the University of São Paulo, São Paulo, São Paulo, Brazil.

出版信息

Spec Care Dentist. 2025 May-Jun;45(3):e70031. doi: 10.1111/scd.70031.

Abstract

Moebius syndrome is a rare congenital disorder of unknown etiology. It is characterized by non-progressive paralysis of the facial and abducens cranial nerves due to atrophy of their nuclei, often accompanied by malformations in the upper and lower limbs. This report aims to highlight the critical role of hospital dentistry in managing a 6-year-old patient with Moebius syndrome. The patient was on mechanical ventilation via tracheostomy following a cardiorespiratory arrest at birth and has been hospitalized in a long-term care institution. Feeding and medication were administered via gastrostomy, and she was under a regimen of phenobarbital, phenytoin, and benzodiazepines for epilepsy control. Dental follow-up, initiated at birth, included monthly bed visits and medical team consultations. Key interventions included extraction of deciduous teeth to prevent bronchopulmonary aspiration, management of sialorrhea using scopolamine and botulinum toxin, selective wear of deciduous canines to mitigate self-mutilation, and monitoring drug-induced gingival hyperplasia. This case underscores the essential role of dental care in maintaining oral health, preventing complications such as respiratory infections, and enhancing the quality of life for syndromic patients under prolonged mechanical ventilation.

摘要

梅比厄斯综合征是一种病因不明的罕见先天性疾病。其特征是由于面神经核和展神经核萎缩导致面部和展神经出现非进行性麻痹,常伴有上下肢畸形。本报告旨在强调医院牙科在治疗一名6岁梅比厄斯综合征患者中的关键作用。该患者出生时心肺骤停后通过气管切开进行机械通气,一直在长期护理机构住院。通过胃造口进行喂食和给药,她正在接受苯巴比妥、苯妥英和苯二氮䓬类药物治疗以控制癫痫。从出生开始的牙科随访包括每月的床边检查和医疗团队会诊。关键干预措施包括拔除乳牙以防止支气管肺误吸、使用东莨菪碱和肉毒杆菌毒素治疗流涎、选择性磨除乳牙犬齿以减轻自残行为以及监测药物性牙龈增生。该病例强调了牙科护理在维持口腔健康、预防诸如呼吸道感染等并发症以及提高长期机械通气的综合征患者生活质量方面的重要作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/255b/12172574/8976f9843fee/SCD-45-0-g004.jpg

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