Ch 11 Cleft Lip Cleft Palate November 17 2011 Clefts Craniofacial abnormalities congenital malformations involving the head and face and affecting speech Clefts abnormal openings in anatomical structures necessary for speech in the oral tract usually upper lip and soft palate can also be when something does not close Velopharyngeal inadequacy VPI velopharyngeal mechanism does not close adequately air pressure cannot build up in oral tract lots of articulation issues Also affects swallowing Clefts interfere with basic biological functioning breathing feeding and communication Physical Challenges of Clefts Through the Lifespan Birth no surgery before 3 mos o Obstructed breathing immediate medical attention o Difficulty with food intake No single feeding method is successful for infants with different types of clefts 3 months o Clefts of the lip are surgically closed o Monitored for middle ear infections 9 12 months o Surgeons close clefts of the hard and soft palate has to do with physical development additional growth of the neck and head has to occur before First surgery at first year to minimize the impact of cleft on speech and language development When structures can function properly speech develops properly Second surgery may be necessary later to treat difficulties with velopharyngeal closure 2 years o Correction of dentition Later orthodontic treatment Generally people can lead a fulfilling life after proper treatment Development of the Face and Palate Facial Development o During the embryonic and fetal periods of life humans are at a higher risk of death than any other period of life except extreme old age Half of all conceptions are not recognized clinically and approximately 15 of clinically recognized pregnancies result in miscarriages o Fig 11 1 on page 318 o 5 8 weeks gestation face anterior aspect of mouth o Mandibular processes mandible lower lip develops o 5th week Frontonasal processes Nasomedian processes and lateral nasal processes o Olfactory pits become the right and left nasal cavities Nasal alae flared portion of nostrils o Maxillary processes maxilla and palatal shelves develop o Teratogens chemical or environmental agents that produce congenital abnormalities clefts of the lip are interrupted Development of the secondary palate structures that cover the oral tract bony hard palate and soft palate o 8 12 week gestation processes of hard and soft palates fuse o Palatal shelves wedge shaped tissue masses grow downward from maxillary processes o Nasal septum o Clefts of the palate fusion of the palatal shelf is interrupted Cleft Lip and Palate Classification Systems Contemporary cleft palate classification systems are based on the embryological development of the face and palatal structures Veau System Table 11 1 on page 320 o Quick general reference o Nature and extent of clefts Kernahan s Striped Y o Visual identification system o Based on location o System based by the ACPA focuses on primary and secondary palates Based on anatomy and physiology of physical structures location and which structure s is impacted Clinical Features of Clefts Clefts Commonly Classified as o 1 Unilateral or bilateral cleft of the lip o 2 Unilateral cleft of the lip and palate o 3 Bilateral cleft of the lip and palate o 4 Submucous cleft usually not detected until later tissue covering a hollow hole looks normal from the outside o 5 Bifid uvula causes articulation issues Cleft of the Lip o Involves vermilion of upper lip vertical dent between lip and nose on normal person and can extend to nostril o Incomplete cleft some position of the vermilion is not closing on one side o Complete cleft closure extends all the way up to oral cavity continues through the upper lip into the floor of the nostril o Flattened nose flaring nostril o Columella strip of tissue that connect base of nose to tip of nose short V shaped misaligned o Most commonly on the left side unilateral do not know why o If bilateral usually cleft palate also o Isolated cleft of the lip is rare less than 5 of all cleft cases Unilateral Cleft of the Lip and Palate o Unilateral complete cleft of lip and palate extends lack of fusion for areas that fall behind upper lip between upper gum and upper lip external portion of upper lip through alveolus and through hard and soft palate o Clefts of secondary palate alone vary in severity Bilateral Cleft of the Lip and Palate o Most severe because of lack of tissue nothing is there lip teeth and nose effected o Features Lip and alveolar processes clefted under each nostril Abnormal position of prolabium central portion of lip alveolar process premaxilla o Columella usually absent tip of nose attaches to lip o Nasal septum not attached to palatal shelves Submucous Cleft o Cleft of the muscular region of the soft palate o Thin layer of mucosal tissue covers cleft o May not be detected until later o Bifid uvula uvula split in half 1 in 80 people o Zona pellucida bluish color in the middle of soft palate o Notch on posterior border of the hard palate can be felt o Velopharyngeal incompetence not connecting no proper air flow restriction Etiologies of Clefts Genetic Disorders account for substantial percentage of clefts o Syndromes combination of symptoms Pierre Robin syndrome Robin sequence born with cranial facial abnormalities micrognathia underdeveloped mandible retracted tongue structures heart problems missing toes and fingers a lot of hearing loss Treacher Collins syndrome malar hypoplasia underdeveloped cheekbones undeveloped mandible malformation of the external ear and ear canal conductive hearing loss cleft palate a projection of scalp hair onto the cheek Velocardiofacial syndrome have universal language deficits and learning disabilities in conjunction with palatal cleft Small stature of with broad flattened nose underdeveloped cheekbones and heart problems Apert syndrome craniosynostosis premature closing of the structures of the skull that greatly disfigures forehead and syndactyly webbing of the fingers and toes with bone fusion Chromosomal Aberrations o Trisomy 13 have an extra chromosome 13 60 70 cleft lip with or without cleft palate 1 in every 6 000 births o Multiple congenital abnormalities o Thalidomide caused thousands of birth defects including clefting in the 1960s when the drug was administered to pregnant women As a result the drug was outlawed for over 30 years In 1998 the FDA approved its use for the treatment of certain serious conditions such as leprosy