Sains Malaysiana 42(1)(2013): 85–88

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

Received:19 December 2011 / Accepted: 18  May 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

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

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*Corresponding author; email: sheillaretna@yahoo.com