Letter: Classification and nomenclature of malformations.
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Biomedical subjects
Publications and source records attributed to R B Lowry.
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Submucous cleft palate refers to a situation where the soft palate is largely composed of mucosa with little or no muscle. The defect is often not obvious on inspection of the mouth and pharynx. There is considerable clinical variation, with speech ranging from normal or minimal nasality to severe nasality and defective articulation. Many patients who have latent submucous cleft palate have the condition unmasked by an adenoidectomy because the adenoid pad had served as a compensatory factor in effecting palatopharyngeal closure. All physicians who perform tonsillectomy and adenoidectomy should be aware of the signs and symptoms which may suggest the diagnosis.
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The incidence of PKU in British Columbia in the 1950-1971 period is 1/18,750 which corresponds to that found in two other Canadian studies.(2, 3) Evidence is presented which shows a trend toward a decline in incidence; however, this is not statistically significant. There is a preponderance of male cases in all age groups.
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Cleft lip with or without cleft palate is twice as frequent among Indians of British Columbia as among non-Indians, although the reverse is the case whenever we consider isolated cleft palate. A family is described with 12 affected males. Close examination of this family revealed that the most likely explanation for the cleft palate was an X-linked recessive gene. It is concluded that isolated cleft palate among the Indians of British Columbia is an extremely rare anomaly.
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