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

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.