The Robin anomalad (Pierre Robin syndrome)--a follow up study.

AJ Williams, MA Williams, CA Walker… - Archives of Disease in …, 1981 - adc.bmj.com
AJ Williams, MA Williams, CA Walker, PG Bush
Archives of Disease in Childhood, 1981adc.bmj.com
During a 10-year period 55 patients with the Robin anomalad were admitted to the Liverpool
Regional Cleft Palate Units. Fourteen (25%) children died. All deaths were within 3 months
of birth. Congenital abnormalities other than mandibular retrognathia and cleft palate were
present in 14 (26%) children. Peripheral limb defects were particularly common. Thirty
children were recalled and reviewed to assess speech, hearing, growth, and educational
achievement. There was a clear association between severe nasal escape of air in speech …
During a 10-year period 55 patients with the Robin anomalad were admitted to the Liverpool Regional Cleft Palate Units. Fourteen (25%) children died. All deaths were within 3 months of birth. Congenital abnormalities other than mandibular retrognathia and cleft palate were present in 14 (26%) children. Peripheral limb defects were particularly common. Thirty children were recalled and reviewed to assess speech, hearing, growth, and educational achievement. There was a clear association between severe nasal escape of air in speech and atypical articulatory patterns. Almost half the children tested had abnormal articulation. Only 4 (13%) of 30 children showed delayed language development. Half the children tested audiometrically showed a binaural handicap but in only one patient was this sufficiently severe to warrant amplification. There was no trend towards abnormalities of growth and only 2 children could be firmly classified as educationally subnormal.
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