 |
 |
 |
 |
 |
 |
Clinical Manifestation of Blepharocheilodontic (BCD) Syndrome: A Case Review (Manifestasi Klinikal Sindrom Blefarokeladontiki (BCD):
Satu Tinjauan Kes)
R. NavasheillaRetna*
& S.N.M.P. Sockalingam
Department of Operative Dentistry, Faculty of
Dentistry, Universiti Kebangsaan Malaysia
Jalan Raja Muda Abdul Aziz, 50300 Kuala Lumpur, Malaysia
Diserahkan: 19 Disember 2011 / Diterima: 18 Mei 2012
ABSTRACT
BCD syndrome is a rare birth
syndrome characterised by cleft lip and palate, euryblepharon with/without limb
anomalies, ectropion of lower eyelids and hypertelorism. The dental anomalies
include cone shaped microdontic teeth and oligodontia. Different combinations
of these signs have been found sporadically in patients. This syndrome is also
known as Elschnig’s syndrome. By at large, patients with this syndrome have no
potentially fatal anomalies. Their growth and development were usually normal.
This case review described the clinical manifestation and oral findings of a
patient with BCD syndrome. The dental need of the patient was
outlined.
Keywords: Ectropion; Elsching syndrome; euryblepharon;
lagophthalmia; oligodontia
ABSTRAK
Sindrom BCD adalah satu sindrom
kelahiran yang jarang dijumpai dan mempunyai ciri-ciri seperti rekahan bibir
dan lelangit, ‘euryblepharon’ dengan/tanpa anomali pada lengan tangan dan kaki,
ektropion kelopak mata bawah dan hipertelorisme. Anomali pergigian termasuk
kehadiran gigi-gigi mikrodontik yang berbentuk kon dan oligodontia. Akan tetapi, kombinasi berlainan daripada ciri-ciri yang dinyatakan
boleh juga dijumpai pada segelintir pesakit secara sporadik. Sindrom ini juga dikenali sebagai sindrom Elschnig. Kebiasaannya, pesakit sindrom ini jarang memiliki anomali serius
yang boleh menyebabkan kematian. Tumbesaran serta
perkembangan mereka juga adalah normal. Tinjauan ini
melaporkan manifestasi klinikal dan penemuan oral pada seorang pesakit sindrom BCD. Keperluan pergigian untuk pesakit ini juga dibincangkan.
Kata kunci: Ectropion; euryblepharon;
lagophthalmia; oligodontia; sindrom Elschnig
RUJUKAN
Allanson, J.E. & McGillivary, B.C. 1985. Familial clefting
syndrome with ectropion and dental anomaly-without limb anomalies. Clin.
Genet. 27: 426-429.
Gorlin, R.J., Zellweger,
H., Curtis, M.W., Wiedemann, H.R., Warburg, M., Majewski, F.,
Gilliessen-Kaesbach, G., Prahl-Andersen, B. & Zackai, E. 1996. Blepharo-cheilo-dontic (BCD)
syndrome. American Journal of Medical Genetics 65: 109-112.
Guion-Almeida, M.L.,
Rodini, E.S.O., Kokitsu-Nakata, N.M. & Bologna-Amantini, D. 1998. Blepharo-cheilo-dontic (BCD) syndrome: Report
on four new patients. American Journal of Medical Genetics 76: 133-136.
Iida, Akihiko., Narai,
Shota., Takagi, R., Ono, K. & Ikeda, N. 2006. Blepharo-cheilo-dontic (BCD)
syndrome: Case report. Cleft Palate-Craniofacial Journal 43: 237-243.
Lopes, V.L.G.d.S., Guion-Almeida, M.L. &
Rodini, E.S.d.O. 2003. Blepharo-cheilo-dontic (BCD)
syndrome: Expanding the phenotype? American Journal of Medical Genetics 121:
266-270.
Martínez, R.B., Monasterio,
A.L., Pinheiro, M., Freire-Maia, N., Opitz, J.M. & Reynolds, J.F. 1987. Cleft lip/palate-oligodontia- syndactyly-hair
alterations, a new syndrome: Review of the conditions combining ecodermal
dysplasia and cleft lip/palate. Am. J. Med. Genet. 27: 23-31.
*Pengarang untuk surat-menyurat; email: sheillaretna@yahoo.com
|
|||
|
