Cleft Lip and Palate

The earliest documented history of cleft lip (CL) and cleft palate (CP) was described by the Romans and Spartans and was thought to be evidence of evil spirits in the affected child.
The first CL surgery was performed in 390 BC by a Chinese physician named Wey Young-Chi on an 18-year-old.
CW Tennison in 1953 and Peter Randall in 1958 made important modifications of the triangular flap repair.
In 1955, Peter Millard introduced the rotation-advancement flap, which remains the most commonly used technique for unilateral repair.¹

Closure of the lip requires fusion of the lateral nasal, medial nasal, and maxillary process at 35 days postconception.
Failure of closure of any 1 or all of the above 3 processes may produce unilateral or bilateral lip clefting, which may extend into the palate.
Conversely, isolated CP results from failure of primary fusion of the palates, which may occur after closure of the lip because palatal fusion occurs 56 days postconception.

ETIOLOGY AND EPIDEMIOLOGY

The most common craniofacial defect is the cleft lip with or without cleft palate (CL/P) and isolated CP.
These 2 defects vary in etiology, epidemiology, and genetic predisposition.

Incidence: In the United States for all orofacial clefts is 1 of 690; about one-third of those have CP alone and two-thirds have CL/P.²

Rates of CP alone are consistent across races. CL/P is most highly associated with Asian populations. Caucasian and
Hispanics have an intermediate risk, and the lowest rates are among African Americans.
About 50% of CP and 30% of CL/P are associated with syndromes.²
The male to female ratio for CL/P is 2:1 and 1:2 for CP.

Figure 40.1 The spectrum of cleft lip deformities. A, Microform cleft lip. B, Unilateral incomplete cleft lip. C, Unilateral complete cleft lip. D, Bilateral complete cleft lip. E, Bilateral incomplete cleft lip. (Reprinted with permission from Thorne CH, Grabb WC, Smith JW, eds. Grabb and Smith’s Plastic Surgery. 6th ed. Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2007.)

Etiology: CL and CP have different etiologies; genetic and environmental factors affect the development of CP more than CL.

Phenytoin, smoking in the first trimester, folate deficiency, and maternal age <20 or >39 years are associated with CL/P.
There is a 3% to 5% chance that an affected parent will have a child with CL/P.

CLINICAL PRESENTATION

CL and CP may present with obvious craniofacial deformity; however, some cases of isolated CP may be more occult (Figure 40.2).
Signs and symptoms of CL and/or CP include difficulty with feedings, difficulty swallowing with possibility of liquids to come out the nose, nasal voice, and chronic ear infections.
The incidence of otitis media in patients with CP is 97%, likely due to abnormal eustachian tube anatomy and insertion of the pharyngeal musculature.²
Regarding feeding, infants with isolated CL can usually be fed by breast or bottle. Those with CP have difficulty creating adequate suction and benefit from modified nipples.

Figure 40.2 Classification of cleft lip and cleft palate. Class 1: Cleft of the tip of the uvula; Class 2: cleft of the uvula (bifid uvula); Class 3: cleft of the soft palate; Class 4: cleft of the soft and hard palates; Class 5: cleft of the soft and hard palates that continues through the alveolar ridge on one side of the premaxilla, usually associated with cleft lip of the same side; Class 6: cleft of the soft and hard palates that continues through the alveolar ridge on both sides, leaving a free premaxilla, usually associated with bilateral cleft lip; Class 7: submucous cleft in which the muscle union is imperfect across the soft palate. The palate is short, the uvula is often bifid, a groove is situated at the midline of the soft palate, and the closure to the pharynx is incompetent. (Reprinted with permission from Wilkins EM. Clinical Practice of the Dental Hygienist. 12th ed. Philadelphia, PA: Wolters Kluwer; 2016.)

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