Atypical Hallerman-Streif Syndrome: A Case Report

Abstract

Hallerman Streif syndrome is a rare congenital disorder characterized by dyscephaly, dental anomalies, proportionate nazism ,hypotrichosis, cutaneous atrophy limited to the head ,bilateral congenital cataracts and bilateral microphthalmia .Despite the marked craniofacial characteristics and oral findings, a relative lack of reports in the dental literature has been noted. In this article, a case of a 8 year old boy with dental problems is described.

Craniodentofacial Manifestations In Hallermann-Streiff Syndrome

“…2,3,6,9,16 Our patient showed cardinal ophthalmic features of the syndrome such as ptosis, horizontal nystagmus, blue sclera, and congenital cataracts. It is usually recommended that patients with congenital cataracts at early ages be operated on as early as possible in order to avoid loss of vision.…”

“…The absence of moderate-severe MR, anomalies of the ear, palpebral anomalies, muscular atrophy, nail anomalies support the diagnosis. 2,3,6 Differential diagnosis of HSS includes: progeria and other progeriod syndromes such as DeBarsy, HutchinsonGuilford, Berardinelli-Seip and Cockayne's syndrome, as well as mandibulofacial dysostosis, cleidocranial dysostosis and pyknodysostosis. Rothmund-Thomson syndrome and Bloom syndrome share the features of facial telangiectases and skin atrophy as HSS; however the face of HSS is distinctive.…”

“…Later, Hallermann and Streiff acknowledged this entity as separate from progeria and mandibulofacial dysostosis and the new syndrome was named Hallermann-Streiff Syndrome. [1][2][3][4][5] HSS is a rare congenital disorder characterized by dyscephaly (scaphocephaly or brachycephaly with frontal bossing), short stature, bilateral microphthalmia and cataracts, hypotrichosis, and skin atrophy localized to the head and nose. Additional features of the syndrome include dental anomalies, micrognathia, condylar aplasia, thin pinched nose, and typical faces.…”

“…Additional features of the syndrome include dental anomalies, micrognathia, condylar aplasia, thin pinched nose, and typical faces. 2,3,6 Virtually all cases of HSS have been sporadic. There is no sex predilection.…”

“…A review of the literature reveals that more than 150 HSS cases have been reported to date; however a dental approach has been focused on in very few cases. 3 The aim of this case study is to describe HSS with special consideration of the dentofacial features in a 10-yearold girl.…”