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.