Cleft Lip & Palate

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Cleft VS cleft lip/palate

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C: Abnormal opening in an anatomical structure, representing a failure of structure to fuse early in embryonic development

CL/P: caused by failure of structures in oral cavity to unite during embryonic development

- results in a split (cleft) in upper lip and base of nose, hard palate, and/or soft palate in some combination

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Embryological period

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Typically developing fetuses: Complex fusion of embryonic processes: mandibular processes, frontonasal process, and maxillary processes. 5th week – mandible and lower lip fuse in the midline. 8 weeks – anterior aspects of facial development is completed.

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What goes wrong in fetal development

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- these defects occur very early in fetal development

- cleft usually occurs when lip or palatal structures don't fuse completely

- occurs when dev. is disrupted at approx. 5-12 weeks of gestation

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Incidence of cleft lip and palate

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Occurs in one in every 750 live births

- clefts of lip occur more often than cleft palates alone

Submucous clefts occur in one in every 1200 live births

- In the US, incidence is highest among people of aborginal decent

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Cleft of the lip may occur:

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Unilateral or bilateral - if unilateral, most likely on left side

- extends from external portion of the lip and may extend to nose

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Unilateral cleft lip and palate

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Extends from external portion of upper lip, through alveolus, hard and soft palate. Nasal septum attaches to larger of the two palatal segments. Occasionally, cleft of the palate (without lip) may occur.

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Bilateral complete cleft of lip and palate

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- Most severe due to severe tissue deficiency

- Lip and alveolus are cleft under both nostrils.

- Free-floating premaxilla: tissue formed by central portion of lip, alveolus, and premaxilla is positioned at the tip of the nasal septum

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Submucous cleft

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Muscular cleft of softpalate

- Cleft coveredby thin layer of mucosal tissue making it hard to see lack of muscle fusion

- May not bediscovered until later in childhood (once hypernasality is evident in the speech)

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What causes cleft lip and palate?

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Genetic disorders

- May occur inover 400 multipleanomaly syndromes

- Pierre RobinSyndrome (Robin Sequence)

- Treacher Collinssyndrome

- 22q11 deletionsyndrome (velocardiofacial syndrome)

- Apert Syndrome

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Chromosomal aberrations

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Characterized by multiple congenital anomalies

- Trisomy 13 – a rare condition resulting from three #13 chromosomes.Results in cleft lip (with or without cleft palate) in 60 – 70% of cases

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Mechanically induced abnormalities

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Mechanical factors that impinge on the normally developing embryo e.g. amniotic rupture, intrauterine crowding (from twin, uterine tumour, or irregularly shaped uterus)

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Environmental teratogens

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Agents that interfere with normal development of a fetus

Includes: Dilantin (anticonvulsant medication); Thalidomide; excessive use of aspirin; excessive use of alcohol, nicotine, caffeineX-rays, certain viruses

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Hypernasal resonance

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Resulting from too much air entering nasal cavity. May be caused by structural issues (opening in palate)May be caused by functional issues (poor movement of soft palate)

May result in: nasal air emission on production of sounds, hypernasal resonance, consonants may be weak and distorted

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Effects on articulation

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Lack of intra-oral pressure buildup impacts production of high pressure sounds (plosives, fricatives, affricates). Articulation also impacted by hearing loss and dental/bite abnormalities. Compensatory articulation behaviors may develop. Glottal stops to substitute for other plosives. Pharyngeal fricative to substitute for fricatives, affricates.

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Effects on voice (phonation)

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Problems such as decreased loudness and as a compensation for hypernasality, which results in a weak and breathy voice

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Children with cleft palate are more likely to have ____ ear problems, with perhaps ___% of them being affected at least temporarily

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- middle

- 80%

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Surgical management

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Surgery for repair of palate ideally should occur between 6 and 18 months of age, dependent on severity of cleft. Primary correction of lip at 3 months. Secondary surgical correction to improve VP competence for speech may also occur prior to 18 months of age