Holoprosencephaly with clefts : data of 85 patients, treatment and outcome : Part 1 : History, subdivisions, and data on 85 holoprosencephalic cleft patients

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dc.contributor.author Honey, E.M. (Engela)
dc.contributor.author Butow, Kurt-Wilhelm
dc.contributor.author Zwahlen, Roger Arthur
dc.date.accessioned 2020-07-15T05:09:02Z
dc.date.available 2020-07-15T05:09:02Z
dc.date.issued 2019-01
dc.description.abstract Cleft patients with Holoprosencephaly (HPE) constitute a controversy due to a variable facial appearance. HPE appearance varies from only a columella to a prolabium‑premaxilla complex agenesis up to a common unilateral or bilateral cleft lip and palate with a single central incisor, various brain deformities, and/or even normal brain development. It is challenging to designate such various appearances, to understand their etiopathogenesis, and to choose the most appropriate management. Literature was reviewed for diagnostic criteria, pregnancy history, clinical findings, brain development, survival rate, initial perioperative management, and postsurgical midfacial growth in cleft patients with HPE. The findings were compared with a clinical database of 85 cleft patients with HPE at the Department of Maxillofacial and Oral Surgery, University of Pretoria. AIMS OF PART 1: The aim of the study is to overcome disparities widely existing among clinicians regarding definitive diagnostic criteria, especially in cases with a common appearance of a uni‑ or bilateral cleft lip alveolus or cleft lip, alveolus and palate deformity, and cases presenting facial structural agenesis. MATERIALS AND METHOD : A literature search related to diagnostic criteria was compared to results of a cleft HPE database from a single tertiary institution. RESULTS : HPE cleft cases can be allocated to one of the following subdivisions: (1) columella complex agenesis (Ag‑Colum), (2) prolabium‑premaxilla‑columella complex agenesis in cleft lip‑alveolus deformities (Ag‑CLA), (3) prolabium‑premaxilla‑columella agenesis in cases with complete cleft lip alveolus palate (Ag‑CLAP), and (4) standard type (holoprosencephaly in patients with a standard cleft) with uni‑ or bilateral CLA or CLAP, hard and soft palate cleft (hPsP), and atrophic premaxillae, with or without single central incisor. Further, incidence, variation in brain development, and appearances in HPE cleft patients of different races and gender, epilepsy, and early death are discussed. CONCLUSION : This paper adds new data and facts to the existing literature related to cleft lip and palate patients suffering from HPE. en_ZA
dc.description.department Biochemistry en_ZA
dc.description.department Genetics en_ZA
dc.description.department Maxillo-Facial and Oral Surgery en_ZA
dc.description.department Microbiology and Plant Pathology en_ZA
dc.description.librarian am2020 en_ZA
dc.description.sponsorship Dr. EM Honey is supported by the South African Medical Research Council under a Self‑Initiated Research Grant. en_ZA
dc.description.sponsorship The South African Medical Research Council en_ZA
dc.description.uri http://www.amsjournal.com en_ZA
dc.identifier.citation Honey E.M., Bütow K.W., Zwahlen R.A. Holoprosencephaly with clefts: Data of 85 patients, treatment and outcome: Part 1: History, subdivisions, and data on 85 holoprosencephalic cleft patients. Annals of Maxillofacial Surgery 2019;9:140-145. en_ZA
dc.identifier.issn 2231-0746 (print)
dc.identifier.issn 2249-3816 (online)
dc.identifier.other 10.4103/ams.ams_50_19
dc.identifier.uri http://hdl.handle.net/2263/75223
dc.language.iso en en_ZA
dc.publisher Medknow Publications en_ZA
dc.rights © 2019 Annals of Maxillofacial Surgery. This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License. en_ZA
dc.subject Agenesis of cleft lip‑alveolus en_ZA
dc.subject Agenesis of prolabium‑premaxilla en_ZA
dc.subject Holoprosencephalus en_ZA
dc.subject Holoprosencephaly (HPE) en_ZA
dc.subject Cleft lip, alveolus and palate (CLAP) en_ZA
dc.title Holoprosencephaly with clefts : data of 85 patients, treatment and outcome : Part 1 : History, subdivisions, and data on 85 holoprosencephalic cleft patients en_ZA
dc.type Article en_ZA


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