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伴有腭裂的前脑无裂畸形：85例患者的数据、治疗及结果：第2部分：前脑无裂畸形腭裂患者的管理、外科治疗及意外情况

Holoprosencephaly with Clefts: Data of 85 Patients, Treatment, and Outcome: Part 2: Management, Surgical Treatment, and Unexpected Aspects of Holoprosencephaly Cleft Patients.

作者信息

Butow Kurt W, Zwahlen Roger Arthur

机构信息

Department of Maxillofacial and Oral Surgery, Facial Cleft Deformity Clinic, University of Pretoria, Pretoria, South Africa.

Suite A2-Maxillo-Facial Surgery, The Life Wilgers Hospital, Pretoria, South Africa.

出版信息

Ann Maxillofac Surg. 2019 Jan-Jun;9(1):146-151. doi: 10.4103/ams.ams_52_19.

DOI:10.4103/ams.ams_52_19
PMID:31293944
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6585193/
Abstract

CONTEXT

Cleft patients with holoprosencephaly (HPE) provide a wide clinical spectrum. Besides accessory agenesis of facial tissue structures, spanning from a single central incisor to the columella, up to the entire prolabium-premaxilla complex, brain deformities with various functional deficits may prevail, just like normal brain development. Making a precise diagnosis, just like choosing the most appropriate treatment plan often is challenging. A literature and chart review comprising 85 HPE cleft cases at the Cleft Clinic of the University of Pretoria, South Africa, was performed. It yielded pertinent diagnostic criteria and collected information about pregnancy history, brain development and survival rate as well as the initial perioperative management and the course of postsurgical midfacial growth.

AIMS OF PART 2: The aim is to highlight how the here presented classification system of HPE cleft patients according to their clinical picture may facilitate the most appropriate treatment protocol.

MATERIALS AND METHODS

The classification system elaborated in Part I due to diagnostic criteria facilitated establishing classification related treatment protocol for 85 cleft cases with HPE.

RESULTS

According to diagnostic criteria, HPE cleft cases can be subdivided into (1) columella complex agenesis (Ag-Colum), (2) prolabium-premaxilla-columella complex agenesis in cleft lip-alveolus deformities (Ag-CLA), (3) prolabium-premaxilla-columella complex agenesis in complete hard and soft palate clefts (Ag-CLAP), and (4) "standard" uni-or bilateral CLA or CLAP (HPE-Std-cleft), including cases with an atrophic premaxilla with or without single central incisors. Relevant treatment protocols according to the particular classification are highlighted with figures and intra-operative pictures.

CONCLUSION

This paper addresses the following aspects in cleft patients with HPE: A subdivision into four groups, the 3-in-1 surgical approach, the anteriorly directed midfacial growth and maternal HIV infection.

摘要

背景

患有全前脑畸形（HPE）的腭裂患者临床表现范围广泛。除了面部组织结构的附属器官发育不全，从单个中切牙到鼻小柱，直至整个唇前 - 上颌复合体，还可能存在伴有各种功能缺陷的脑部畸形，就像正常脑发育一样。做出准确诊断，就像选择最合适的治疗方案一样，通常具有挑战性。对南非比勒陀利亚大学腭裂诊所的85例HPE腭裂病例进行了文献和图表回顾。得出了相关诊断标准，并收集了有关妊娠史、脑发育、存活率以及初始围手术期管理和术后面中部生长过程的信息。

第二部分的目的

目的是强调根据临床表现对HPE腭裂患者进行分类的系统如何有助于制定最合适的治疗方案。

材料与方法

第一部分根据诊断标准制定的分类系统有助于为85例HPE腭裂病例建立与分类相关的治疗方案。

结果

根据诊断标准，HPE腭裂病例可细分为：（1）鼻小柱复合体发育不全（Ag - Colum）；（2）唇 - 牙槽畸形中唇前 - 上颌 - 鼻小柱复合体发育不全（Ag - CLA）；（3）完全性硬腭和软腭裂中唇前 - 上颌 - 鼻小柱复合体发育不全（Ag - CLAP）；以及（4）“标准”单侧或双侧唇裂或腭裂（HPE - Std - cleft），包括有或无单个中切牙的萎缩性上颌前部病例。根据特定分类的相关治疗方案通过图表和术中图片进行了重点展示。

结论

本文探讨了HPE腭裂患者的以下方面：分为四组、三合一手术方法、向前引导的面中部生长和母亲的HIV感染。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/40a8/6585193/5860b0b2f7af/AMS-9-146-g006.jpg
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