Surgical Correction of Tessier Number 0 Cleft

Abstract

The no. 0-14 cleft involves the midline of the face and cranium. It may include both a true and a false median cleft lip, with or without associated hypotelorism or hypertelorism. The no. 0 cleft is the most common of the craniofacial clefts. The objective of this study was to review the functional outcome and aesthetic results of the different techniques applied for each case. We have conducted a retrospective analysis of our series consisting of 32 cases of Tessier no. 0 cleft, in the period between 1997 and 2007. The patients were divided into 2 groups: those with the true median cleft and those with the false median cleft. The clinical findings, lip malformation, alveolar cleft, nasal appearance, septal involvement, associated deformities, and surgical procedures, were all reviewed. Holoprosencephaly was present in 9 cases, with a false median cleft upper lip and an absence of the premaxilla, septum, and columella (only 1 patient underwent lip and columella reconstruction at 2 years of age). Nine patients had an incomplete median cleft lip. Seven of these cases had associated median alveolar cleft, and 1 had an intranasal tumor, associated with lipoma of corpus callosum, characteristic of the Pai syndrome. Six cases of a bifid nose were seen, 2 of which were associated with an alveolar median cleft and hypertelorism. An isolated median alveolar cleft was present in 7 cases, 2 of them associated with a no. 30 cleft. This article presents a large series of Tessier no. 0 cleft, describing the differences between the false and the true median cleft. The surgical procedures may vary in relation to the type of involvement.

The oculo-auriculo-fronto-nasal syndrome (OAFNS) – Description of a rare and complex craniofacial deformity and its interdisciplinary management before school age

“…Orr and Kawamoto used "craniofrontonasal dysplasia" for their patients affected by vertical facial midline disturbances (Orr et al, 1997;Kawamoto et al, 2007). According to da Silva Freitas the terms "internasal dysplasia", "median cleft face syndrome", "frontonasal dysplasia" are used for similar conditions clinically recognizable as increased interorbital distance, orbital dystopia or nasal dysplasia being caused by median facial malfusion during embryological development (da Silva Freitas et al, 2008).…”

Demographic and clinical profile of craniofacial clefts at Comprehensive Rehabilitation Service in Uganda

“…In spite of the modern-day advent of new imaging and new surgical techniques, little has been written on the management of the rare CFC since Tessier's initial classification and many factors limit their optimal management [5]. The incidence of these atypical clefts is unknown but is estimated at 1.4 -4.9 cases per 100 000 live births, therefore approximately 100 times less frequent than the common clefts [3,4].…”

“…Approximately 1% of these newborns have syndromes or multiple anomalies; craniofacial anomalies are often a component part [1]. CFC are rare congenital malformations of the cranium and face with deficiencies or excesses of tissue along an anatomic line based on embryologic maldevelopment [2].They are among the most disfiguring of all facial anomalies and have a multitude of clinical presentations with different levels of severity [2,3,4].…”

Facial clefts involving the midline in combination with intracranial anomalies: Case studies illustrating surgical treatment and medical substitution

“…These clefts are not only challenges from the reconstructive point of view, but they also cause particular problems because of their possible disturbance of the hormonal systems that are regulated by the pituitary gland. There have been reports about midline clefts, but the hormonal aspects are not always considered [7][8][9]. A median cleft of the lip may be one end of the holoprosencephaly spectrum, the other end being cyclopaedia.…”