Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Introduction to Cleft Lip and Palate History Hippocrates (400 BC) and Galen (150 AD) mention cleft lip, but not cleft palate in their writings. For centuries, perforations of the palate were considered to be secondary to syphilis, and cleft palate was not recognized as a congenital disorder until 1556, by Fanco. The first successful closure of a soft palate defect was reported in 1764 by LeMonnier, a French dentist. The first closure of the hard palate was performed in 1834 by Dieffenbach. In the 1930's, Kilner and Wardill independently developed the "pushback" procedure. Embryology The lip and primary palate begin to develop at four to five weeks gestational age. The two medial nasal swellings and the maxillary swellings fuse to form the upper lip. The nasal swellings merge at deeper levels also and form the intermaxillary segment, which becomes the triangular primary palate, so embryologically, clefts of the lip are almost invariably associated with clefts of the primary palate. The secondary palate develops at approximately nine weeks developmental age. It is formed by medial growth of the palatal shelves of the maxilla, which normally fuse together and with the nasal septum as the tongue is pushed down during development. So, the pathogenesis of secondary cleft palate is failure of this fusion to occur. Classification/Anatomy Clefts of the lip are incomplete or complete. Incomplete means only a portion of the lip is involved, and may mean anything from a muscular diastasis only, to a large cleft with only a small band of tissue connecting the two sides. A complete cleft lip involves the entire thickness of the lip. Cleft lips are unilateral or bilateral. The primary palate is anterior to the incisive foramen, and the secondary palate is posterior to the foramen. Clefts of the palate are unilateral if on one side the palatal process of the maxilla is fused with the nasal septum. A bilateral cleft is not attached to the nasal septum, and the septum is visible through the cleft. A complete palatal cleft involves the primary and secondary palate; an incomplete cleft involves the secondary palate only. Incidence Cleft lip and palate are present in one of 1000 live Caucasian births. The incidence of cleft palate alone is one in 500 live births. Considering the cleft deformities of all races grouped together, 50% are cleft lip and palate, 30-35% are palate only, and 15-20% are cleft lip only. The genetics of cleft lip and palate are not classical Mendelian, but siblings of cleft patients are at greater risk. The risk is directly related to the frequency and severity of the clefts. Approximately 10% of patients with a cleft deformity will have other anomalies at birth. Anatomy The blood supply to the palate is derived form the internal maxillary artery: the greater palatine supplies the hard palate, and the lesser palatine the soft palate. The soft palate is also called the velum. The anterior soft palate is amuscular, and the posterior soft palate is muscular. The bony anatomy includes the premaxilla, the maxilla, the palatine bone itself, and the lateral and medial pterygoid plates of the sphenoid--the anterior most portion of the medial pterygoid is called the hamulus. Normally the tensor and levator muscles insert into the midline aponeurosis of the velum. In cleft palate, the levator attaches anteriorly to the hard palate and often is hypoplastic, and the tensor may end at the hamulus, or insert into the lateral velum. Complications Patients with cleft palate may have the following complications: abnormal midface development, velopharyngeal incompetence, speech defects, and abnormal Eustachian tube function. Clefts of the primary palate usually have underdeveloped maxillary alveolar arches, and facial growth is abnormal. Cleft patients have an incompetent velopharyngeal valve, which results in nasal regurgitation while swallowing, as well as "typical cleft palate speech": Nasal escape of air (heard as snorting or grunting), a hypernasal quality, and difficulty with consonants. Normal infants begin to develop consonants in speech at age nine months, which influences the timing of operative repair of cleft palate. Even after palate repair, velopharyngeal function may not return entirely to normal, and speech therapy is often helpful. One aspect of patients with cleft palate which will effect virtually every otolaryngologist is the Eustachian tube dysfunction and middle ear disease. In a landmark article in 1971, Bluestone compared the Eustachian tube function of patients with unrepaired cleft palate and normal-palate patients with a history of serous otitis. Although both groups demonstrated evidence of Eustachian tube dysfunction, the cleft palate patients had a "functional" obstruction of the tube at the nasopharyngeal end--as opposed to the "mechanical" obstruction found in serous otitis patients. Historically, two muscles have been implicated in the Eustachian tube opening--the levator palatini, and the tensor veli palatini. The levator arises from the petrous bone, courses under and parallel to the tube, and inserts into the dorsal surface of the soft palate. The tensor veli palatini originates from the membranous wall of the tube, and converges into a tendon which wraps around the hamulus and inserts into the aponeurosis. Experimental animal studies from Pittsburgh in the 1980s showed that tensor dysfunction was probably responsible for this middle ear disease. However, this point is controversial and there are several authors who feel that abnormalities of the levator cause the ET dysfunction in cleft palate patients. Management The management of the ear problems in cleft palate patients is much less controversial, however. In most reports, virtually 100% of cleft palate patients will have persistent culture-negative serous otitis media throughout infancy, and it should be managed with myringotomy and tube placement as soon as possible--often at the time of first cleft repair--with subsequent close follow-up and replacement of tubes after extrusion. The major complication of tube placement is otorrhea, which occurs frequently--68% of patients in Paradise and Bluestone's study. Although no prospective study of outcome has been performed, most studies show a trend toward improved middle ear function after cleft repair. Timing In view of these complications, the reconstructive surgeon is faced with timing the cleft repair so that hearing and speech are optimized, but effects on midface growth are minimized. Cleft lip repair is usually planned at approximately ten weeks of age, if the baby weighs ten pounds, and the hemoglobin is ten grams. Cleft palate repair is usually scheduled at approximately six to 12 months of age. Submucous Cleft Palate The classic definition is the triad of bifid or notched uvula, notching of the hard palate, and muscular diastasis of the soft palate. Although bifid uvula has been considered a "marker" for submucous cleft palate, some investigators have disputed this. Two recent studies have clarified the issue. In a 1985 screening of almost 10,000 school children from Yugoslavia using the strict definition of submucous cleft, 232 cases of bifid uvula were noted (2.38%), but only five cases (0.05%) of submucous cleft palate were found. The same year, using a different method of examining patients, a smaller group of 25 patients with bifid uvula from Washington, DC were studied: 76% were found to have some degree of diastasis of velar musculature, and 48% had endoscopic evidence of velopharyngeal insufficiency, however only one of 25 patients fulfilled the classic definition of submucous cleft palate. The best conclusion to be drawn from this information is that bifid uvula is a marker for muscular velar diastasis and/or velopharyngeal insufficiency, and the need for adenoidectomy should be evaluated very carefully. In conclusion, cleft lip and palate disorders are complex disorders affecting speech, hearing and cosmesis. They require evaluation and care by a multidisciplinary team including a pediatrician, reconstructive surgeon, otolaryngologist, speech therapist, audiologist, and a prosthodontist. The coordinated effort of all these specialists is important in their management. Case Presentation Bibliography Bagatin M: Submucous cleft palate. J Maxillofac Surg 3:37-38, 1985. Beery QC, Doyle WJ, et al: Eustachian tube function in an American Indian population. Ann Otol Rhinol Laryngol 89:28-33, 1980. Blijdorp P, Muller H: The influence of the age at which the palate is closed on the rhinological and otological condition in the adult cleft patient. J Maxillofac Surg 12:247-54, 1984. Bluestone CD: Eustachian tube obstruction in the infant with cleft palate. Ann Otol Rhinol Laryngol 80:1-30, 1971. Bluestone CD, et al: Eustachian tube ventilatory function in relation to cleft palate. Ann Otol Rhinol Laryngol 84:333-38, 1975. Bluestone CD, Paradise JL, et al: Certain effects of cleft palate repair on Eustachian tube function. Cleft Palate J 9:183-93, 1972. Bumsted RM: Cleft lip and palate, in Cummings CJ, et al, eds. Otolaryngology-Head and Neck Surgery, St. Louis, Mosby & Co., 1129-67, 1986. Cantekin EI, Bluestone CD, Parkin LP: Eustachian tube ventilatory function in children. Ann Otol Laryngol Rhinol 85:171-77, 1976. Cantekin EI, Phillips DC, et al: Effect of alterations of the tensor veli palatini muscle on Eustachian tube function. Ann Otol Rhinol Laryngol 89:47-53, 1980. Casselbrandt ML, Doyle WJ, et al: Eustachian tube function in the Rhesus monkey model of cleft palate. Cleft Palate J 22:185-91, 1985. Cole RM, et al: Eustachian tube function in cleft lip and palate patients. Arch Otolaryngol 99:337-41, 1974. Dickson DR: Normal and cleft palate anatomy. Cleft Palate J 9:280-90, 1972. Dickson DR: Anatomy of the normal and cleft palate eustachian tube. Ann Otol Rhinol Laryngol 85(Suppl 25):25-29, 1976. Dorf DS, Curtin JW: Early cleft palate repair and speech outcome. Plast Reconstr Surg 70:74, 1982. Dorrance GM, Bransfield JW: The push-back operation for repair of cleft palate. Plast Reconstr Surg 1:145-69, 1946. Doyle WJ, Cantekin EI, Bluestone CD: Eustachian tube function in cleft palate children. Ann Otol Rhinol Laryngol 89:34-40, 1980. Doyle WJ, Cantekin EI, et al: Nonhuman primate model of cleft palate and its implications for middle ear pathology. Ann Otol Rhinol Laryngol 89:41-46, 1980. Doyle WJ, Reilly S, et al: Effect of palatoplasty on the function of the Eustachian tube in children with cleft palate. Cleft Palate J 23:63-68, 1986. Doyle WJ, Rood SR: Comparison of the anatomy of the eustachian tube in the Rhesus monkey and man. Ann Otol Laryngol Rhinol 89:49-57, 1980. Dreyer, TM, Trier WC: A comparison of palatoplasty techniques. Cleft Palate J 21:251-53, 1984. Falk B, Magnuson B: Eustachian tube closing failure: Occurrence in patients with cleft palate and middle ear disease. Arch Otolaryngol 110:10-14, 1984. Finkelstein Y, Talmi YP, et al: Levator veli palatini muscle and Eustachian tube function. Plast Reconstr Surg 85:684-92, 1990. Fischler RS, et al: Lack of association of cleft uvula with otitis media in Apache Indian children. Am J Dis Child 141:866-67, 1987. Furlow LT: Cleft palate repair by double opposing Z-plasty. Plast Reconstr Surg 78:724-36, 1986. Gould HJ: Hearing loss and cleft palate: The perspective of time. Cleft Palate J 27:36-39, 1990. Hayashi M, Takahashi H, et al: Clearance of the Eustachian tube under negative middle ear pressure. Am J Otolaryngol 4:399-401, 1986. Hodges PL, Salyer KE: Cleft palate and velopharyngeal function. Selected Readings in Plastic Surgery 4:1-23, 1987. Holborow C: Eustachian tubal function. Arch Otolaryngol 92:624-26, 1970. Holborow C: Eustachian tubal function: Changes throughout childhood and neuro-muscular control J Laryngol Otol 89:47-55, 1975. Ingelstedt S: Physiology of the eustachian tube. Ann Otol Rhinol Laryngol 85:156-60, 1976. Jackson IT, et al: Primary veloplasty or primary palatoplasty: Some preliminary findings. Plast Reconstr Surg 72:153-57, 1983. Krause CJ, et al: A comparative study of results of the Von Langenbeck and the V-Y pushback palatoplasties. Cleft Palate J 13:11-19, 1976. McEvitt WG: The incidence of persistent rhinolalia following cleft palate repair. Plast Reconstr Surg 47:258-61,1971. Misurya VK: Functional veloplasty in bilateral palatal palsy. Arch Otolaryngol 102:436-38, 1976. Paradise, JL: Management of middle ear effusions in infants with cleft palate. Ann Otol Rhinol Laryngol 85:285-88, 1976. Paradise, JL: Otitis media in infants and children. Pediatrics 65:917-43, 1980. Paradise JL, Bluestone CD: Early treatment of the universal otitis media of infants with cleft palate. Pediatrics 53:48-54, 1974. Ruding R: Cleft palate: Anatomic and surgical considerations. Plast Reconstr Surg 33:132-47, 1964. Sataloff J, Fraser M: Hearing loss in children with cleft palates. Arch Otolaryngol 55:61-64, 1952. Seif S, Dellon AL: Anatomic relationships between the human levator and tensor veli palatini and the Eustachian tube. Cleft Palate J 15:329-336, 1978. Shibihara Y, Sando I: Histopathologic study of Eustachian tube in cleft palate patients. Ann Otol Rhinol Laryngol 97:40308, 1988. Shprintzen RJ, et al: Morphologic significance of bifid uvula. Pediatrics 75:553-61, 1985. Tasaka Y, et al: Eustachian tube function in OME patients with cleft palate. Acta Otolaryngol Suppl 471:5-8, 1990. Taylor GD: The bifid uvula. Laryngoscope 82:771-78, 1972. Wada T, Miyazaki T: Growth and changes in maxillary arch form in complete unilateral cleft lip and cleft palate children. Cleft Palate J 12:115-30, 1975.
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