Journal of Japanese Cleft Palate Association
Online ISSN : 2186-5701
Print ISSN : 0386-5185
ISSN-L : 0386-5185
A Case of Wolf-Hirschhorn Syndrome (4p-) with a Bilateral Cleft Lip and Palate, under Long-term Follow-up
Hironobu KOBASHIKoji ISHIHAMAEmiko ISOMURATakeshi HARADATetsuya SEIKAIMikihiko KOGO
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2017 Volume 42 Issue 1 Pages 35-40

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Abstract
Wolf-Hirschhorn Syndrome (WHS) was first described by Cooper and Hirschhorn in 1961, and by Wolf in 1965. The syndrome is caused by terminal deletion of the short arm of chromosome 4 (4p-) and is well known; the patient is usually diagnosed in childhood because of unique facial dysmorphism, cleft lip and palate, congenital heart disease, skeletal anomalies, and seizures.
We report a WHS patient who has a cleft lip and palate, and whom we have been following for a long time. The woman is now 30 years old, and has undergone cheiloplasty for bilateral cleft lip and palatoplasty. We used the von Langenbeck method rather than the pushback method to prevent airway obstruction. We have been following her progress for more than 30 years after the surgery. It is considered that WHS patients cannot reach adulthood if they have a cardiac abnormality, but our patient has a congenital heart malformation. A WHS adult with such a heart malformation is very rare. It is important to follow up their oral hygiene for a long time and to consider the risk of aspiration pneumonia and infective endocarditis.
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© 2017 Japanese Cleft Palate Association
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