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ORIGINAL ARTICLE - RETROSPECTIVE STUDY
Year : 2019  |  Volume : 9  |  Issue : 1  |  Page : 146-151

Holoprosencephaly with clefts: Data of 85 patients, treatment, and outcome: Part 2: Management, surgical treatment, and unexpected aspects of holoprosencephaly cleft patients


1 Department of Maxillofacial and Oral Surgery, Facial Cleft Deformity Clinic, University of Pretoria; Suite A2-Maxillo-Facial Surgery, The Life Wilgers Hospital, Pretoria, South Africa
2 Private Practice in Oral and Maxillofacial Surgery, Grand-Places 16, Fribourg, Switzerland

Correspondence Address:
Dr. Roger Arthur Zwahlen
Private Practice in Oral and Maxillofacial Surgery, Grand-Places 16, 1700 Fribourg
Switzerland
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DOI: 10.4103/ams.ams_52_19

PMID: 31293944

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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.


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