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解析丹迪-沃克综合征：一例临床症状与治疗见解的病例报告

Unraveling Dandy-Walker Syndrome: A Case Report with Clinical Presentations and Management Insights.

作者信息

Banu Syeda N, Raj Sunil N, Anand Anitha, Nandan N, Vishwanathan Soundarya, Kumar Aarya S

机构信息

Department of Pediatric and Preventive Dentistry, Bangalore Institute of Dental Sciences and Hospital, Bengaluru, Karnataka, India.

Department of Pedodontics and Preventive Dentistry, Bangalore Institute of Dental Sciences, Bengaluru, Karnataka, India.

出版信息

Int J Clin Pediatr Dent. 2025 Mar;18(3):299-304. doi: 10.5005/jp-journals-10005-3098. Epub 2025 Apr 19.

DOI:10.5005/jp-journals-10005-3098
PMID:40415754
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12096882/
Abstract

AIM

This case report presents a unique instance of Dandy-Walker syndrome (DWS) with notable oral manifestations, emphasizing the importance of interdisciplinary management in such complex cases.

BACKGROUND

DWS is a rare congenital cystic malformation of the posterior cranial fossa. Clinical findings include delayed motor development in infancy, macrocephaly and hydrocephalus with bulging occiput, nystagmus, and ataxia. DWS is also associated with hypertelorism, cardiac, skeletal, renal, dental, and vertebral malformations.

CASE DESCRIPTION

A 10-year-old male patient presented with typical neurological symptoms of DWS, including hydrocephalus, developmental delay, and motor skill impairment, alongside distinctive dental anomalies such as a high arched palate, malocclusion, and poor oral hygiene. The interdisciplinary approach involved collaboration between neurologists, pediatricians, and dental specialists to address both the neurological and oral health challenges. Following clinical examination and behavioral assessment, dental treatments were planned and completed under local anesthesia. Dental management included preventive care, root canal treatment (RCT), stainless steel crowns (SSC), and coronal restorations.

CONCLUSION

The patient tolerated the procedure well with no complications during or after the treatment. Dental care for individuals with DWS should be tailored to their needs, with regular monitoring and maintenance. Alternative communication methods and caregiver support are essential.

CLINICAL SIGNIFICANCE

DWS is a rare clinical entity. Understanding the unique challenges associated with DWS can help dental professionals provide better care and improve the overall health and quality of life for these patients.

HOW TO CITE THIS ARTICLE

Banu SN, Raj SN, Anand A, . Unraveling Dandy-Walker Syndrome: A Case Report with Clinical Presentations and Management Insights. Int J Clin Pediatr Dent 2025;18(3):299-304.

摘要

目的

本病例报告展示了一例伴有显著口腔表现的丹迪-沃克综合征（DWS）的独特病例，强调了在这类复杂病例中进行多学科管理的重要性。

背景

DWS是一种罕见的后颅窝先天性囊性畸形。临床症状包括婴儿期运动发育迟缓、巨头畸形和伴有枕部膨隆的脑积水、眼球震颤和共济失调。DWS还与眼距过宽、心脏、骨骼、肾脏、牙齿和脊柱畸形有关。

病例描述

一名10岁男性患者出现了DWS的典型神经症状，包括脑积水、发育迟缓及运动技能受损，同时伴有独特的牙齿异常，如高拱腭、错牙合畸形和口腔卫生差。多学科方法涉及神经科医生、儿科医生和牙科专家之间的合作，以应对神经和口腔健康方面的挑战。经过临床检查和行为评估后，在局部麻醉下计划并完成了牙科治疗。牙科管理包括预防性护理、根管治疗（RCT）、不锈钢冠（SSC）和冠修复。

结论

患者对该治疗耐受良好，治疗期间及治疗后均无并发症。对DWS患者的牙科护理应根据其需求进行定制，并进行定期监测和维护。替代沟通方法和护理人员支持至关重要。

临床意义

DWS是一种罕见的临床病症。了解与DWS相关的独特挑战有助于牙科专业人员提供更好的护理，并改善这些患者的整体健康和生活质量。

如何引用本文

Banu SN, Raj SN, Anand A, . 解读丹迪-沃克综合征：一例临床病例报告及管理见解。《国际临床儿科牙科学杂志》2025年；18(3):299 - 304。

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本文引用的文献

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Prevention of Viridans Group Streptococcal Infective Endocarditis: A Scientific Statement From the American Heart Association.《预防草绿色链球菌感染性心内膜炎：美国心脏协会科学声明》。
Circulation. 2021 May 18;143(20):e963-e978. doi: 10.1161/CIR.0000000000000969. Epub 2021 Apr 15.
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A rare case of Dandy-Walker syndrome in the 12-year-old girl.一名12岁女孩患丹迪-沃克综合征的罕见病例。
J Indian Soc Pedod Prev Dent. 2020 Jan-Mar;38(1):94-96. doi: 10.4103/JISPPD.JISPPD_343_18.
4
[Intravenous Sedation and Repeated "the Same Day General Anesthesia" for a School-age Boy with Dandy-Walker Syndrome and Dentinogenesis].[一名患有丹迪-沃克综合征和牙本质生成异常的学龄男孩的静脉镇静和重复“同日全身麻醉”]
Masui. 2016 Mar;65(3):304-7.
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Oral findings and dental treatment in a patient with Dandy-Walker syndrome: a case report.丹迪-沃克综合征患者的口腔检查结果及牙科治疗：病例报告
Spec Care Dentist. 2014 May-Jun;34(3):151-5. doi: 10.1111/scd.12044. Epub 2013 Jul 16.
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Dandy-Walker malformation: An incidental finding.丹迪-沃克畸形：一项偶然发现。
Indian J Hum Genet. 2010 Jan;16(1):33-5. doi: 10.4103/0971-6866.64936.
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Dandy-Walker malformation: analysis of 19 cases.丹迪-沃克畸形：19例分析
J Child Neurol. 2010 Feb;25(2):188-91. doi: 10.1177/0883073809338410. Epub 2009 Oct 15.
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J Natl Med Assoc. 2009 May;101(5):456-61. doi: 10.1016/s0027-9684(15)30932-9.
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