A 24-year-old man with Prader-Willi syndrome presented with a class III malocclusion, featuring maxillary hypoplasia and severe enamel deficiency. Treatment involved orthodontic alignment, surgical advancement of the maxilla and restorative treatment to augment vertical facial height, improve the final occlusion and increase short clinical dental crown heights. The principal features of the syndrome and the management of this case are discussed.
Get full access to this article
View all access options for this article.
References
1.
ButlerM. G. (1990) Prader-Willi Syndrome: current understanding of cause and diagnosis, American Journal of Medical Genetics, 35, 319–332.
2.
FearC. N., MuttonD. E., GerryA. C., HeckmattJ. Z. and DubowitzV. (1985) Chromosome 15 in Prader-Willi Syndrome, Developmental Medicine and Child Neurology, 27, 305–311.
3.
FosterS. C. (1971) Prader-Willi syndrome: report of cases, Journal of the American Dental Associations, 83, 634–638.
4.
HallB. D. and SmithD. W. (1972) Prader-Willi syndrome, Journal of Pediatrics, 81, 286–293.
5.
KrizJ. S. and CloningerB. J. (1981) Management of a patient with Prader-Willi syndrome by a dental-dietary team, Special Care in Dentistry, 1, 179–182.
6.
LauranceB. M. (1985) The Prader-Willi Syndrome, Maternal and Child Health, 10, 106–109.
7.
LauranceB. M., BritoA. and WilkinsonJ. (1981) Prader-Willi syndrome after age 15 years, Archives of Diseases in Childhood, 56181–186.
8.
LedbetterD. H., MascarelloJ. T., RiccardiV. M., HarperV. D., AirhartS. D. and StrobelR. J. (1982) Chromosome abnormalities and the Prader-Willi syndrome: a follow up report of 40 cases, American Journal of Human Genetics, 34, 278–285.
9.
PraderA. (1989) Prader-Willi syndrome, Report of a meeting of forum on mental retardation, Journal of the Royal Society of Medicine, 80, 718–720.
10.
PraderA., LabhartA. and WilliH. (1956) Ein syndrom von Adipositas, Kleinwuchs, Kryptorchismus und oligophrenia nach Myotonieartigem Zuskand im Neugeborenalter, Schweitzerische Medizinische Wochenschrift, 86, 1260–1261.
11.
PraderA, and WilliH. (1963) Das Syndrom von Imbezillitat, Adipositas, Muskelhypotonie, Hypogonadismus und Diabetes Mellitus mit Myotonie,—Anamnese, In: Verhand 2nd International Kongress per Psychiatrie und Entwicklundsstorunger die Kindesalter, Vienna 1961, Part 1, Karger, Basel.
12.
StephenscnJ. B. P. (1980) Prader-Willi Syndrome: neonatal presentation and later developments, Developmental Medicine and Child Neurology, 22, 792–795.
13.
WoodR. E., NortjeC. J. and SchikerlingL. (1987) Dental evidence aids diagnosis of Prader-Willi syndrome, Ontario Dentist, 64, 25–29.
14.
ZellwegerH. and SchneiderH. J. (1968) Syndrome of hypotonia-hypomentia-hypogonadism-obesity (HHHO) or Prader-Willi syndrome, American Journal of Diseases in Children, 115, 588–598.
