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TITLE: Cleft Lip and Palate SOURCE: UTMB Dept.

of Otolaryngology Grand Rounds DATE: January 28, 1998 RESIDENT PHYSICIAN: Greg Young, M.D. FACULTY: Ronald Deskin, M.D. SERIES EDITOR: Francis B. Quinn, Jr., M.D., F.A.C.S. |Return to Grand Rounds Index| "This material was prepared by physicians in partial fulfillment of educational requirements established for Continuing Postgraduate Medical Education activities and was not intended for clinical use in its present form. It was prepared for the purpose of stimulating group discussion in a interactive computer mediated conference setting. No warranties, either express or implied, are made with respect to its accuracy, completeness, or timeliness. The material does not necessarily reflect the current or past opinions of subscribers or other professionals and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion."

INTRODUCTION Cleft lip and palate represents the second most frequently occurring congenital deformity (after clubfoot deformity). Cleft lip, cleft palate or both affects approximately 1 in 750 births. Clefting is associated with many problems including cosmetic and dental abnormalities, as well as speech, , hearing and facial growth difficulties. The otolaryngologist is uniquely qualified to identify and manage many of these problems, and holds a key role on the cleft palate team. ANATOMY The palate consists of the hard palate and soft palate, which together form the roof of the mouth and the floor of the nose. The palatine processes of the maxilla and horizontal lamina of the palatine bones form the hard palate. Its blood supply is

The soft palate is a fibromuscular shelf made up of several muscles attached like a sling to the posterior portion of the hard palate. a cleft does not exist (unlike the secondary palate in which cleft formation occurs as a natural stage of development). which passes through the greater palatine foramen. It includes that portion of the alveolar ridge containing the four incisor teeth. Normally during development of the primary palate. The nerve supply is via the anterior palatine and nasopalatine nerves. the levator veli palatini. Clefts of the secondary palate are due to lack of fusion of the palatal shelves. The secondary palate forms during the 6th to 9th weeks of gestation. The soft palate consists of the tensor veli palatini. and palatal component (forms the triangular primary palate). EMBRYOLOGY The primary and secondary palates are delineated according to embryological development. the musculus uvulae. the palatoglossus. thereby contacting Passavants ridge posteriorly. It closes off the nasopharynx by tensing and elevating. The primary palate or premaxilla is a triangular area of the anterior hard palate extending from anterior to the incisive foramen to a point just lateral to the lateral incisor teeth. The secondary palate consists of the remaining hard palate and all of the soft palate. Clefts of the primary palate occur anterior to the incisive foramen. CN V supplies the tensor veli palatini. A cleft lip occurs when an epithelial bridge fails. while CN IX and CN X innervate the others. The levator veli palatini is the primary elevator of the palate. a maxilla component (forms alveolus and 4 incisors). The intermaxillary segment is composed of a labial component (forms the philtrum). patients with clefts have a deficiency of tissue and not merely a displacement of normal tissue. The tongue must migrate away from the shelves in an antero-inferior direction for palatal fusion to occur. as the palatal shelves change from a vertical to horizontal position and fuse.mainly from the greater palatine artery. and palatopharyngeus muscles. The primary palate forms during the 4th to 7th weeks of gestation as the two maxillary swellings merge and the two medial nasal swellings fuse to form the intermaxillary segment. CLEFT FORMATION In general. due to lack of mesodermal delivery and proliferation from the maxillary and nasal processes. and .

and inferiorly displaced. the floor of the nose communicates freely with the oral cavity. Facial clefts are associated with a syndrome 15-60% of the time. The muscles of the orbicularis oris do not form a complete sphincter but instead are directed superiorly to the ala nasi laterally and the base of the columella medially. the risk rate for future offspring is 2% with only one parent affected. . 2% with only one sibling affected. Associated dentition abnormalities include supernumerary teeth (20%). The lower lateral cartilage of the nose is lower on the cleft side. dystrophic teeth (30%). In the bilateral cleft lip. For cleft palate alone. The cleft of the lip occurs earlier and inhibits tongue migration. the maxilla on the cleft side is hypoplastic. and the nasal ala on the cleft side is laterally. GENETICS Nonsyndromic inheritance of facial clefting is multifactorial. For cleft lip with or without cleft palate. Common syndromes with cleft palates include Apert's. its lateral cruz is longer. 9% with two siblings affected. which may then prevent horizontal alignment and fusion of the palatal shelves. and 17% with one parent and one sibling affected. posteriorly. which may explain why isolated clefts of the secondary palate are more common in females. More than 200 recognized syndromes may include a facial cleft as a manifestation. depending on whether a parent or sibling is affected. congenitally missing teeth (50%). In palatal clefts. and 10-17% with one parent and one sibling affected. Van der Woude's and Waardenberg's syndromes are associated with cleft lip with or without cleft palate. The secondary palate closes 1 week later in females. the risk rate for future offspring is 7% with only one parent affected. and malocclusion (almost 100%). and the angle between the medial and lateral cruz in more obtuse. Familial inheritance of both cleft lip and palate occurs with varying frequency.always occur posterior to the incisive foramen. the central portion of the alveolar arch is rotated anteriorly and superiorly. In the unilateral cleft lip. 4% with only one sibling affected. 1% with two siblings affected. A cleft of the lip increases in probability of a cleft palate developing. the columella is displaced to the normal side. the muscles of the soft palate are hypoplastic and insert in the posterior margin of the remaining hard palate rather than the midline raphe. Stickler's and Treacher Collins. The medial or prolabial segment of skin contains no muscle or vermillion. Chromosome aberrations such as trisomy D and E have increased incidence of clefts.

Otologic examination includes pneumatic otoscopy and tuning forks. nurse. macroglossia. the rate of isolated cleft palate is constant among ethnic groups. speech pathologist. then Orientals and Caucasians. Oral cavity examination will identify any cleft. and prosthodontist. Increased paternal. The otolaryngologist has a pivotal role in the diagnosis and management of all disorders relating to the head and neck. septal abnormalities. and least common in Blacks. glossoptosis. but not maternal age is also associated with clefts. and facial . and choanal atresia. a typical team consists of an otolaryngologist. or lingual thyroid. The head is inspected for symmetry. Isolated cleft palates are twice as common in females. In addition to the reconstructive surgeon. Cleft lips. thalidomide. and at times is the primary reconstructive surgeon as well. Teams may vary depending on available resources and individual interest. Anterior and posterior rhinoscopy will identify clefting. In addition. audiologist. the auricle and external canal for development and location. geneticist. malocclusion. isolated cleft lip (20%) and least common is cleft lip and alveolus (5%). MANAGEMENT A team approach is needed to manage the wide variety of problems common to the patient with cleft lip and palate. followed by isolated cleft palate (30%). psychiatrist. INITIAL HEAD AND NECK EXAMINATION The otolaryngologist performs a complete head and neck examination on new patients in the cleft palate clinic. Conversely. Cleft lip and palate patients are particularly prone to speech disorders and ear disease. and aminopterin. A facial analysis is helpful to identify abnormalities of facial symmetry and harmony. dental arch abnormalities and tongue anomalies such as bifid tongue. are most common in Native Americans. Combined cleft lip and palate is the most common presentation (50%). and may have significant airway abnormalities. with or without cleft palate. The otolaryngologist is uniquely qualified to oversee the management of these disorders.EPIDEMIOLOGY Clefts of the lip and combined lip and palate are twice as common in males. hemifacial hypertrophy or atrophy. social worker. rubella virus. Environmental factors found to cause clefts in humans are ethanol. intranasal masses. dentist. Maternal diabetes mellitus and amniotic band syndrome are associated with clefts.

glides. the incidence of eustachian tube dysfunction decreases. cough. Other errors include stop. EAR DISEASE Patients with an isolated cleft lip have an incidence of hearing loss similar to that in the normal population. With increasing age. Flexible fiberoptic nasopharyngoscopy has the added advantage of direct visualization of palatal motion and pharyngeal wall motion with both single sounds and connected speech. Otologic goals in the cleft palate patient are to provide adequate hearing. Velopharyngeal competence can be estimated by direct examination of the nasopharyngeal depth. Velopharyngeal competence is the most important determinant of articulation performance and listener understanding of speech in cleft palate patients. It is estimated that 75% of patients have velopharyngeal competence following primary cleft palate surgery. and prevent deterioration of the tympanic membrane. maintain ossicular continuity and adequate middle ear space. and in many cases normal eustachian tube function develops by mid adolescence. Eustachian tube dysfunction in these patients is due to an abnormal insertion of the levator and tensor veli palatini muscles into the posterior margin of the hard palate. and palate movement during phonation. Velopharyngeal incompetence is associated with an audible escape of air from the nose during production of pressure sounds and is termed nasal emission or snort. In contrast. Indications for myringotomy and tube insertion include a significant conductive . SPEECH DISORDERS Errors in articulation are common in cleft palate patients. The upper airway tract is evaluated by assessing the adequacy of phonation. palatal length. and muscular and neurologic deficits. the patients also appear to have an increased incidence of cholesteatoma (7%). especially those involving affricates and fricatives. associated hearing loss. and this can be increased to 9095% with directed secondary procedures. and by auscultating and palpating the neck. and deglutition. In addition to middle ear effusion. Others factors include dentition. Patients with eustachian tube dysfunction are evaluated every 3-4 months until it resolves. cleft palate is very often associated with eustachian tube dysfunction and a resulting conductive hearing loss.clefting are documented. and nasal semivowels.

or tympanic membrane retraction. Adhesions may interfere with definitive lip repair and are less often needed in recent years due to the wider variety of specialty feeding nipples available. In contrast. . The rotation advancement method of cleft lip repair is the most commonly used in The United States. 2) The non-cleft side high point of lateral cupid's bow. 3) The low point of cupid's bow. They convert a complete cleft to an incomplete cleft. These are 1) the non-cleft side nasal ala base. AIRWAY PROBLEMS Airway problems may arise in children with cleft palates. nine landmarks on the lip are identified with a vital dye. Pierre-Robin sequence is the combination of micrognathia.hearing loss or persistent middle ear effusion. especially those with concomitant structural or functional anomalies. cleft palate. and serve as a temporizing measure for those infants with certain feeding problems. For example. Initially. almost all patients with cleft palate will require multiple sets of tubes from 3-4 months of age until the beginning of the second decade of life. recurrent otitis media. SURGICAL REPAIR Cleft Lip Cleft lips are repaired at about 10 weeks of age at most institutions. Lip adhesions can be performed at 2 weeks of age. Affected patients may develop airway distress from their tongue becoming lodged in the palatal defect. and glossoptosis. patients with an isolated cleft lip do not have a significantly increased rate of myringotomy and tubes. In practice.

4) The non-cleft side high point of medial cupid's bow (equal to distance between #2 and #3). allowing # 4 to drop down with # 2. The cleft side advancement flap is now made. 5) the cleft side high point of cupid's bow (placed where white roll attenuates along vermillion-cutaneous junction). The remaining sutures are then placed to complete the repair. with distance #5 to #6 corresponding to distance #8 to #4 in its newly rotated position. the cleft and non. Next. The current trend is toward procedures that involve less denuding and . Bilateral cleft repair. (The small remaining triangular tissue attached to the columella is Millard's C flap). Through and through cuts are made for the non-cleft side rotation flap. 8) And 9) the superior extent of the rotation incision. The cleft side nasal ala incision is then made (#6 to #7). the tip of the advancement flap (#6) is brought to point #9(base of the columella). although more complex. is performed using similar principles. 6) superior extent of advancement flap(length of #5 to #6 equal height of non-cleft lip(#4 to #8).cleft lip segments are separated in a supraperiosteal plane from the maxilla. and the external and vestibular skin is separated from the cleft side lower lateral cartilage. Next. Cleft Palate Several techniques for cleft palate repair have been developed over the past 3 decades. 7) point on cleft side alar crease such that length of #5 to #7 equals length of #1 to #2.

In the Bardach repair. 1988. Cleft lip and palate. many authors believe there is a relationship between scar formation of the palatal repair and the impaired mid-facial growth that is observed in most patients with cleft palate. Morris HL. and less tension on the soft palate. The mucoperiosteal flaps are elevated. and lateral incisions are made at the junction of the palate and alveolar ridge.eds. Bumsted RM. medial incisions are made which separate the oral and nasal mucosa. The palate is then closed in three layers. stating that feeding. Cleft palate patients at high surgical risk or who refuse surgical treatment may use a dental obturator to obtain velopharyngeal competence. Others advocate an earlier repair. Louis: CV Mosby. because facial growth is less affected when surgery is delayed until 18-24 months of age. Disadvantages of dental obturators include the necessity of wearing a prosthesis and the need for modification of the prosthesis as the patient grows.scarring of the hard palate. speech and socialization are improved if the surgery is performed by the 1st year of life. . Otolaryngology-Head and Neck Surgery. midface retrusion. such as those described by Bardach or Furlow are used commonly in the United States today. 1st nasal mucosa. In: Cummings CW. eds. 1990. The two-flap techniques. The lateral palate incisions are closed loosely. and that facial growth problems can be minimized with less traumatic palate repairs. Facial growth may also be affected by the age when the repair is performed. then velar muscle. In this repair. BIBLIOGRAPHY 1. Philadephia: WB Saunders. and malocclusion. Some surgeons advocate later repair. St. Next. and finally oral mucosa. Multidisciplinary management of cleft lip and palate. taking care to identify and preserve the neurovascular bundle containing the greater palatine artery. the velar muscle is detached from its attachment to the posterior border of the palatal shelves. a single posteriorly based mucoperiosteal flap is developed over each palatal shelf.. Several methods of cleft palate repair have been described. Bony abnormalities that develop include collapse of the alveolar arches. Bilateral cleft palate repairs are performed in similar fashion. Bardach J. et al. Although controversial. 2.

Holt GR and Watson MJ.. In: Healy GB. 5. Seibert RW. et al. and Bumsted RM. 1993. Philadelphia: Lippincott Co.Otolaryngology. ed. Otolaryngol Head Neck Surg 92:406. 6. Velopharyngeal incompetence. The otolaryngologist's role in the craniofacial anomalies team. 1990. 1983. Seibert RW. Crockett DM. Lip adhesion in bilateral cleft lip. Arch Otol Head Neck Surg 109:434. . Chicago: Year Book. Common problems in pediatric otolaryngology. In: Bailey BJ. Head and Neck Surgery. 4.3. Cleft lip and palate: the primary deformity. Crockett DM. eds. 1984.