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. 2021 Jul-Dec;11(2):270-273.
doi: 10.4103/ams.ams_77_21. Epub 2021 Nov 29.

The Influence of Genetic Syndromes on the Algorithm of Cleft Lip and Palate Repair - A Retrospective Study

Kinga Amália Sándor-Bajusz  1 , Teodor Barna Maros  2 , Lajos Olasz  3 , George Kálmán Sándor  4 , Kinga Hadzsiev  5 , Attila Mihály Vástyán  1   6
Affiliations

The Influence of Genetic Syndromes on the Algorithm of Cleft Lip and Palate Repair - A Retrospective Study

Kinga Amália Sándor-Bajusz et al. Ann Maxillofac Surg. 2021 Jul-Dec.

Abstract

Introduction: This study aimed to determine if the treatment algorithm used for nonsyndromic cleft patients required alteration to manage syndromic cleft lip and/or palate patients.

Methods: The records of patients managed by the Pécs Cleft Team between January 1999 and December 2015 were analyzed retrospectively. The sources of the data included clinical and genetic records.

Results: A total of 607 patients were managed by the cleft team during the study. Sixteen patients (2.6%) were noted to be afflicted with a particular identifiable syndrome. Seven different genetic syndromes and one sequence were present in the study. The Pierre Robin sequence occurred most often, comprising 50% of the cohort. The treatment algorithm used in managing nonsyndromic clefts required modification in 13 of the 16 syndromic patients.

Discussion: The presence of a genetic syndrome may notably affect the treatment algorithm in children born with cleft lip and/or palate. The surgical treatment of certain associated anomalies has by necessity, priority over the timing of the reconstruction of the cleft lip and/or cleft palate in syndromic patients.

Keywords: Child; cleft lip; cleft palate; syndrome; treatment timing.

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Conflict of interest statement

Dr. George Kalman Sandor was associated as a section editor of this journal and this manuscript was subject to this journal's standard review procedures, with this peer review handled independently of this section editor and their research group.

Figures

Figure 1
Figure 1
(a) The distribution of the seven genetic syndromes and one sequence present in the cohort. (b) Number of individuals in each group
Figure 2
Figure 2
The distribution of cleft type in syndromic patients
Figure 3
Figure 3
The timing of the cleft repair surgery for syndromic patients. Vertical lines in bold (1 and 2) represent the usual timing of the cleft repair surgeries carried out by the Pécs Cleft Team. Patients 1–16 are grouped according to the types of syndromes
Figure 4
Figure 4
The distribution of additional surgeries for the affected organ system (s) for syndromic patients
Figure 5
Figure 5
Types of secondary ancillary operations carried out on syndromic cleft patients
See this image and copyright information in PMC

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