Journal of Japanese Cleft Palate Association
Online ISSN : 2186-5701
Print ISSN : 0386-5185
ISSN-L : 0386-5185
A Case of a Cleft Palate Infant with Treacher Collins Syndrome and Pierre Robin Sequence whose Feeding Dysfunction was Improved by Our Modified Pacifier
Takahiro GOTOKeiichi ARAKAKIJoji NAKAMATaku ISHIKAWANao SUNAKAWAAyako FUJIIHirotaka KATASHIMAHajime SUNAKAWA
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2010 Volume 35 Issue 3 Pages 254-257

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Abstract
Treacher Collins Syndrome (TCS) is an autosomal dominant disorder of craniofacial development. The major features of the disease include midface hypoplasia, micrognathia, microtia, conductive hearing loss and cleft palate. Birth prevalence has been estimated at 1:50,000 newborns and approximately 60% are caused by new mutations.
The Pierre Robin Sequence (PRS) is a relatively rare symptom complex characterized by glossoptosis, micrognathia and respiratory obstruction. The pathogenesis of PRS is multifactorial and syndromic in nearly half of the patients including Stickler Syndrome, Velocardiofacial Syndrome, and Treacher Collins Syndrome.
We report a case of TCS and PRS with feeding dysfunction in a 2-month-old girl. She has underwent tracheotomy soon after birth, and respiratory distress was improved. She required nasogastric feeding as do most infants with PRS. For feeding dysfunction, we used the modified pacifier with tube and syringe installed by us. This pacifier enabled the volume of milk to be measured and made desensitization short. Our modified pacifier may be effective for patients with feeding dysfunction.
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© 2010 Japanese Cleft Palate Association
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