Cleft Lip and Palate 1 Dr Gutmann

Cleft Lip and Palate 1 Dr. Gutmann

Introduction: Cleft Lip and Palate n n n 2 Embryology Epidemiology Etiology Genetics Plastic Surgery n n Dr. Gutmann Hearing and speech Psychology Orthodontics Anthropology

Embryological facial development Frontal View 3 Dr. Gutmann

Embryology(Sperber, 1992 ) 4 th- 8 th week –closure of Primary Palate(Frazer, 1970) 4 th week- Stomodeum(involution of ectomesenchyme) Maxillary and Mandibular processes 5 th week- Primary palate formation- fusion of maxillary and frontonasal process. (Lateral and Medial Nasal Swellings/Nasal pits) 6 th-10 thweek –closure of Secondary Palate(Christensen, 1992) 6 th week- Palatine Shelves/ muscle of soft palate 4 Dr. Gutmann

Cleft lip w/- w/o Palate n n n 5 Cleft anterior to Incisive Foramen(Sperber, 1992) Mesenchymal cells do not penetrate the Nasolacrimal Grooves Cleft lip w/ palate- Primary Palate defect Cleft posterior to Incisive Foramen(Langman, 1981) Palatine Shelves do not fuse. Dr. Gutmann

Embryological palate development Frontal view 6 Dr. Gutmann

Embryological palate development Inferior view 7 Dr. Gutmann

Prevalence of Cleft Lip & Palate Country Years Prevalence Author Finland 1948 -1975 1. 74 Rintala, 1986 Israel 1974 -1988 0. 97 Tolarova, 1998 USA 1983 -1990 1. 30 Druschel, 1996 Japan 1948 -1954 2. 68 Vanderas, 1987 France 1974 -1996 1. 9 Stoll, 2000 China 1980 -1989 1. 2 Cooper, 2000 Nigeria 1976 -1980 0. 37 Iregbulem, 1982 8 Dr. Gutmann

Epidemiology n Cleft lip w/ or w/o Palate: 0. 6 -2 : 1, 000 Births u 9 Japan 1. 7/ Whites- 1. 0 / Blacks- 0. 4 Dr. Gutmann

Epidemiology Males: More Cleft Lip w/- w/o Palate Females: More Cleft Palate-later fusion Palatine plates /longer period/greater exposure time(Burdi, 1969) (Cooper, 2000) n 10 Dr. Gutmann

Etiology of Cleft Lip and Palate n n n Multifocal Midline facial defect (developmental) Growth hormone defect/Teeth development defect Genetic(Wyszynski, 1996) - Mendelian/- Multifactorial Twins study (Keush, 1991)33%- identical/ 7%- non-identical 11 Dr. Gutmann

Etiology of Cleft Lip and Palate (continue) Environmental factors: Humans: Alcohol, smoking, antiepileptic meds Animal experimentation: Cortizol/ Steroids(Richtsmeir, 1994) Bixler, 1991) Anti- Asthmatic/ X-ray (Sa’ad, 1994) 12 Dr. Gutmann

Multifactorial inheritance Siblings- (one sibling) Bilateral cleft lip and palate- 5. 7% Unilateral cleft lip and palate-4. 2% Cleft lip w/o palate- 2. 5% (Bixler, 1981/ Fraser, 1970) 2 siblings affectedadditional (9. 0%) (Wyszynski, 1996) 13 Dr. Gutmann Relatives- (Balgir, 1984) First degree- 4% Uncles- 0. 6% Cousins(First degree)0. 4%

Frequency Unilateral left to Unilateral right to Bilateral 6: 3: 1 CL/P male to female 2: 1 CP female to male 2: 1 CL/P to CL 1. 5 -3: 1 CL to CL/P to CP to 46% to 33%21% Majority of CL/Ps are isolated Majority of CPs are syndromic 14 Dr. Gutmann (Mc. Carthy)

Predicted Occurrence% , CL/P P One sibling affected 4 2 One parent affected 4 6 One sibling and one parent affected 17 15 Two siblings affected 9 1 One sibling plus family history 4 7 (Mc. Carthy) 15 Dr. Gutmann

Examples of Cleft Palate n n Cleft of Back Soft Palate Cleft of Soft and Hard Palate and Cleft Lip 16 Dr. Gutmann

Examples of Cleft lip: Unilateral and Bilateral Normal/ Unilateral/ n 17 Dr. Gutmann

Repair of cleft lip 18 Dr. Gutmann

Repair of cleft palate 19 Dr. Gutmann