scholarly journals Median facial cleft syndrome: A case report

2021 ◽  
Vol 7 (2) ◽  
pp. 61-63
Author(s):  
Kaushik Bhattacharya ◽  
Aditya Shikar Bhattacharya ◽  
Neela Bhattacharya
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Upper Lip ◽  
Live Births ◽  
Facial Cleft ◽  
The Face ◽  
Median Cleft ◽  
Median Facial Cleft Syndrome ◽  
Congenital Cleft ◽  
Type 3

Congenital Cleft Lip and Palate is a common birth defect with an incidence of 1 in 600 to 800 live births. This defect usually affects either the left, right or in some cases both sides of the lip and is called a Tessier Type 3 cleft. Clefting of the face in the midline is exceedingly rare and such a case of a Median Facial Cleft Syndrome is being reported which occurs in nearly 1 in 1,000,000 live births. This was first described by Bechard in 1823 and can be sporadic or part of an inherited syndrome. The child had a median cleft of the upper lip, nose, and palate of a severe, complete variety, with absent philtrum of the upper lip, premaxilla, columella, nasal septum and vomer.

Median Cleft of the Lip: Its Significance and Surgical Repair

1993 ◽  
Vol 30 (1) ◽  
pp. 94-96 ◽  
Author(s):  
James Apesos ◽  
Gregg M. Anigian
Keyword(s):  
Surgical Technique ◽  
Cleft Lip ◽  
Surgical Repair ◽  
Surgical Reconstruction ◽  
Upper Lip ◽  
Facial Cleft ◽  
Median Cleft ◽  
Median Facial Cleft Syndrome ◽  
Associated Abnormalities

Median cleft lip is a midline vertical cleft through the upper lip in the absence of a prolabial remnant. This may occur as a sporadic event or be part of an inherited sequence of anomalies. A failure of formation or fusion of the medial nasal prominences derived from the frontonasal prominence is ultimately responsible for this aberration. Two categories of dysplasia are associated: (1) frontonasal deformity associated with hypotelorism and (2) median facial cleft syndrome associated with hypertelorism. A patient presents with median cleft lip, mild bifid nose, and hypertelorism. Following surgical reconstruction, a good result is achieved. The embryology, implications for associated abnormalities, and surgical technique for treating these cases are discussed.

scholarly journals THE MAIN DIRECTIONS IN THE COMPLEX REHABILITATION OF CHILDREN WITH CLEFT LIP AND PALATE

2013 ◽  
Vol 1 (1) ◽  
pp. 36-43 ◽  
Author(s):  
Yulia Vladimirovna Stepanova ◽  
Margarita Sergeevna Tsyplakova
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Social Adaptation ◽  
Psychological Help ◽  
Complex Treatment ◽  
Upper Lip ◽  
Orthopedic Treatment ◽  
Professional Psychological Help ◽  
Specialized Center ◽  
Congenital Cleft

Complex treatment of children with cleft lip and palate is complicated and multi-step. Treatment is conducted by high-skilled specialists. The organization and execution of this complex are possible only at the large specialized center. Coordinator of this work is the maxillofacial surgeon. The performance of rehabilitation circuits includes preoperative orthodontic and orthopedic treatment, operative intervention (reconstructive and plastic surgery), orthodontic and orthopedic treatment after operation. Post-operative conservative treatment prevents the development of secondary deformities of the nose and upper lip. Professional psychological help and long supervision promote the achievement of good social adaptation of patients with congenital cleft lip and palate, improvement of their health.

Lobar Holoprosencephaly with a Median Cleft: Case Report

2009 ◽  
Vol 46 (5) ◽  
pp. 549-554 ◽  
Author(s):  
Elżbieta Gawrych ◽  
Joanna Janiszewska-Olszowska ◽  
Anna Walecka ◽  
Maria Syryńska ◽  
Hanna Chojnacka
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Brain Abnormalities ◽  
Facial Deformity ◽  
Unique Case ◽  
Craniofacial Malformations ◽  
Live Births ◽  
Facial Defects ◽  
Median Cleft

Holoprosencephaly is a congenital abnormality of the prosencephalon associated with median facial defects. Its frequency is 1 in 250 pregnancies and 1 in 16,000 live births. The degree of facial deformity usually correlates with the severity of brain malformation. Early mortality is prevalent in severe forms. This report presents a child with lobar holoprosencephaly accompanied by median cleft lip and palate. The treatment and 9 months’ follow-up are presented. This unique case shows that holoprosencephaly may present different manifestations of craniofacial malformations, which are not always parallel to the severity of brain abnormalities. Patients with mild to moderate brain abnormalities may survive into childhood and beyond.

Tessier type 3 oblique facial cleft with a contralateral complete cleft lip and palate

2010 ◽  
Vol 39 (11) ◽  
pp. 1133-1136 ◽  
Author(s):  
E. Gawrych ◽  
J. Janiszewska-Olszowska ◽  
H. Chojnacka
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Facial Cleft ◽  
Type 3

scholarly journals Congenital cleft of the lip and palate in children: pathogenetical significance of matrix metalloproteinases

2019 ◽  
Vol 19 (2) ◽  
pp. 106-113
Author(s):  
Ayaz E. Mardanov ◽  
I. E. Smirnov ◽  
A. A. Mamedov
Keyword(s):  
Matrix Metalloproteinases ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Chromosomal Abnormalities ◽  
Maxillofacial Region ◽  
Diagnostic Significance ◽  
Live Births ◽  
New Methods ◽  
Congenital Cleft ◽  
Tissue Matrix

There are presented data about the prevalence offormation of congenital cleft lip and palate (CCLP) - one of the most common birth defects of the maxillofacial region in children. According to WHO the occurrence of children born with CCLP in the world is 0,6-1,6 cases per 1,000 live births, and varies greatly from the region to region and ethnic group residing there. At the same time, in 63,1% of patients there were established chromosomal abnormalities, and in 36,9% - non-chromosomal syndromes. In Russia, every year there are occurred from 3,5 to 5 thousand children with such defects that requires the development of new methods ofprevention of these malformations. There are reported modern views on the pathogenetical and potent diagnostic significance of the evaluation of the content of tissue matrix metalloproteinases (MMPs) and their inhibitors in mechanisms of the formation of CCLP, shown the probable associations of changes in the expression or activity of MMP and TIMP with various forms of malformations of the palate and upper jaw.

scholarly journals ISSUE OF DETAILING THE CLASSIFICATIONS OF CONGENITAL CLEFT UPPER LIP AND PALATE

2020 ◽  
Vol 15 (4) ◽  
pp. 162-169 ◽  
Author(s):  
Yulia Rogozhina ◽  
Svetlana Blohina ◽  
Evgeniya Bimbas
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
International Standards ◽  
Individual Treatment ◽  
Upper Lip ◽  
Maxillofacial Region ◽  
Functional Changes ◽  
Anatomical Classification ◽  
Congenital Cleft

Subject. Congenital cleft of the upper lip and palate is one of the most common malformations of the maxillofacial region which percentage is 86-88 %. This defect causes a complex set of anatomical and functional changes in the structures of the face and palate, it negatively affects the development of the child's body, therefore, it requires competent definition and extensive professional knowledge on its systematization and early elimination. The purpose of research — study of the incidence of asymmetric cleft lip and palate on children and complement the clinical and anatomical classification of clefts options asymmetry of the affected tissues. Methodology. There was carried out a retrospective statistical analysis of case histories of 687 patients with congenital cleft of the upper lip and / or palate, who received treatment on the basis of State Autonomic Health Institution of the Sverdlovsk Region Multiprofile Clinical Medical Center «Bonum» for the period 2015—2019 with the determination of the variety of types of lesions. Results. Asymmetry of the affected tissues occurred in 8.59 % of cases of the total number of children (687 patients) who was born with maxillofacial pathology of the upper lip and palate which required clarification of the clinical anatomical classification and the choice of individual treatment tactics. A clinical and anatomical classification that takes into account asymmetric clefts of the upper lip and palate was proposed. Conclusions. The allocation of asymmetric clefts of the upper lip and palate into certain groups of lesions and an assessment of their frequency made it possible to present a classification of this type of defect, which expanded the idea of the variety of anomalies of the maxillofacial region. The classification we proposed takes into account in more detail the clinical features of the congenital cleft of the upper lip and palate and, thus, more fully meets the clinical requirements and international standards, which allowed us to develop new methods of surgical treatment of congenital cleft of the upper lip and palate, as well as to build management tactics for such patients together with other specialists.

scholarly journals Association between Subjective and Objective Measures of Lip Form and Function: An Exploratory Analysis

2003 ◽  
Vol 40 (3) ◽  
pp. 241-248 ◽  
Author(s):  
Carroll-Ann Trotman ◽  
Ceib Phillips ◽  
Julian J. Faraway ◽  
Kelly Ritter
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Objective Measures ◽  
Upper Lip ◽  
Form And Function ◽  
Subjective Assessments ◽  
The Face ◽  
Lip Movement ◽  
Analysis System ◽  
And Function

Objective In patients with cleft lip and palate, the aims of this study were to generate objective measures of different attributes of lip movement and to explore the utility of these objective measures by examining the association between examiners’ subjective assessments with the objective measures. Patients and Participants Thirteen patients with unilateral cleft lip and palate with varying degrees of cleft scar severity were selected. All patients had a previously repaired complete unilateral cleft lip and palate. Interventions Photographs and videotape recordings were made of the patients with cleft at rest and during smiling. Measurements of lip movement were obtained by means of a motion analysis system. Main Outcome Measures The study sought to obtain rankings of cleft scar severity and impairment on a 6-point Likert scale by a lay and professional panel and measurements of displacement, asymmetry, speed, and velocity of upper lip during smiling. Results Displacement was the most consistent and valid objective measurement. An objective analysis of the entire upper lip provided the most information. In general, there was a decrease in the objective measures of upper lip movement as examiners’ perceptions of facial appearance or disfigurement at rest and impairment during movement became worse. This relationship was stronger for the at-rest perceptions, implying that subjective assessments should be made with the face at rest. Conclusions Objective measures provided the promise for differentiation of the components of movement and should be used to supplement subjective evaluations of lip appearance at rest and during movement.

Aspects of orthodontic protocol in cleft lip and palate patients

2020 ◽  
Vol 20 (2) ◽  
pp. 137-142
Author(s):  
O. V. Dudnik ◽  
Ad. A. Mamedov ◽  
O. I. Admakin ◽  
A. A. Skakodub ◽  
Y. O. Volkov ◽  
...  
Keyword(s):  
Oral Hygiene ◽  
Orthodontic Treatment ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Comprehensive Approach ◽  
Maxillofacial Region ◽  
Indirect Methods ◽  
Indirect Bonding ◽  
The Face ◽  
Direct And Indirect Methods

Relevance. Cleft lip and palate is one of the severe malformations of the face and jaw, requiring a comprehensive approach to the rehabilitation of the patients, including doctors of various specialties, one of which is orthodontists. A feature of orthodontic treatment is difficulty of fixing bracket systems, as well as lowering the level of oral hygiene, caused by deformation and displacement of fragments of the maxillofacial region.Purpose. Improving the effectiveness of orthodontic treatment and hygiene of the oral caviti in patients with cleft lip and palate in permanent bite period.Materials and methods. A comparison was made of the effetctiveness of fixing brackets systmes with direct and indirect bonding techniques and the effectiveness of oral hygiene during orthodontic treatment using irrigators.Results. The results of the study showed a difference in the effectiveness of using direct and indirect methods of fixing bracket systems in patients with cleft and palate. The use of irrigators as additional means of oral hygiene has demonstrated a positive dynamic of hygiene indices.Conclusions. Results of the study demonstrate the advantages of fixation the brackets by indirect bonding and use additional hygiene products irrigator for improving of oral hygiene.

Repair of Giant Anterior Skull Base Encephalocele Containing Intralesional Eloquent Brain: Technical Note

2021 ◽  
Author(s):  
Andrew J Kobets ◽  
Richard J Redett ◽  
Jonathan M Walsh ◽  
Joseph Lopez ◽  
Melike Guryildirim ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Skull Base ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Neural Tissue ◽  
Anterior Skull Base ◽  
Csf Rhinorrhea ◽  
Hernia Sac ◽  
Live Births ◽  
Anterior Cranial Base

Abstract BACKGROUND Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis. OBJECTIVE To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue. METHODS A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac. RESULTS We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula. CONCLUSION The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.

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