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Hallermann-streiff syndrome (dyscephalic-mandibulo-oculo-facial syndrome; oculo-mandibulo-dyscephaly; ullrich-fremery-dohna syndrome; francois dyscephalic syndrome; mandibulo-oculo- facial dyscephaly syndrome; francois-hallermann-streiff syndrome; hallermann-streiff-francois syndrome; audry i syndrome; dohna syndrome; francois syndrome (1); dyscephaly- teeth abnormality-dwarfism; dyscephalia oculomandibularis-hypotrichosis; mandibulo-ocular dyscephalia hypotrichosis; fremery-dohna syndrome; oculo-mandibulo-facial dyscephaly)


Hallermann-Streiff Syndrome (Dyscephalic-Mandibulo-Oculo-Facial Syndrome; Oculo-Mandibulo-Dyscephaly; Ullrich-Fremery-Dohna Syndrome; Francois Dyscephalic Syndrome; Mandibulo-Oculo-
Facial Dyscephaly Syndrome; Francois-Hallermann-Streiff Syndrome; Hallermann-Streiff-Francois Syndrome; Audry I Syndrome; Dohna Syndrome; Francois Syndrome (1); Dyscephaly- Teeth
Abnormality-Dwarfism; Dyscephalia Oculomandibularis-Hypotrichosis; Mandibulo-Ocular
Dyscephalia Hypotrichosis; Fremery-Dohna Syndrome; Oculo-Mandibulo-Facial Dyscephaly)

General: Rare; familial occurrence and consanguinity; males and females equally affected.

Ocular: Microphthalmos (bilateral); proptosis; nystagmus; strabismus; cataracts; bilateral optic atrophy; coloboma of optic disk, choroid, and iris; keratoglobus; microcornea; antimongoloid slant; iris atrophy; uveitis; blue sclera; persistent pupillary membrane; secondary glaucoma.

Clinical: Malformations of skull (brachycephaly), facial skeleton, and jaws; erupted teeth at birth; diminished hair growth; hyperextensibility of joints; short stature; skin atrophy; mental deficiency; predisposition to upper airway compromise; obstructive sleep apnea.

Francois J, Victoria-Troncoso V. Francois' dyscephalic syndrome and skin manifestations. Ophthalmologica 1981; 183:63-67.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Hallermann W. Vogelgesicht und Cataracta Congenita. Klin Monatsbl Augenheilkd 1948; 113:315.

Ronen S, et al. The early management of baby with Hallermann-Streiff-Francois syndrome. J Pediatr Ophthalmol Strabismus 1979; 16:119-l21.

Spaepen A, et al. Hallermann-Streiff syndrome: Clinical and psychological findings in children. Nosologic overlap with oculodentodigital dysplasia? Am J Med Genet 1991; 41:517-520.

Streiff EB. Dysmorphic Mandibulo-faciale (Tete d'Oiseau) et Alterations Oculaires. Ophthalmologica (Basel) 1950; 120:79.




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