Hallermann-Streiff syndrome (HSS) is a rare disorder that is primarily characterized by distinctive malformations of the skull and facial (craniofacial) region;. Hallermann-Streiff syndrome (HSS) is a rare genetic disorder that is primarily characterized by distinctive malformations of the skull and facial region, sparse hair. Hallermann-Streiff syndrome is characterized by a typical skull shape ( brachycephaly with frontal bossing), hypotrichosis, microphthalmia, cataracts, beaked.
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Hallermann Streiff Syndrome-The Oral Manifestations in a Child
The differential diagnoses to be considered are progeria Hutchinson- Gilfordmandibulofacial dysostosis and cleidocranial dysostosis, pyknodysostosis, Franceschetti mandibulofacial dysostosis, ectodermal aplasia and dysplasia, and occulodentoosseus dysplasia [ 12 ].
Radiological findings in Hallermann-Streiff syndrome: Progeria differs from HSS by having premature atherosclerosis, nail dystrophy, chronic deforming arthritis, acromicria, and normal ocular findings.
Early intervention is important to ensure that children with Hallermann-Streiff syndrome reach their potential. The options available to circumvent the problems during difficult intubation are, awake intubation, intubation over a hallemrann bronchoscope and intubation under inhalational anesthesia.
The palate was high arched and V shaped Figure 6. Corneal opacities in the Hallermann-Streiff syndrome.
Hallermann–Streiff syndrome – Wikipedia
Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts. Koliopoulos and Palimeris observed 5 cases in 3 generations with male-to-male transmission.
Autosomal dominant inheritance has been suggested in some cases. Vogelgesicht und Cataracta congenita. Report of a Case. The edges of the eyelids may appear to turn inwards, particularly on the lower side lower lid entropion so that the eyelashes rub against the eye surface cornea leading to irritation, erosions and corneal opacities.
Some current clinical trials also are posted on the following page on the NORD website: Unfortunately, it is not free to produce. Individuals with the disorder typically have normal intelligence. Ophthalmologica, Basel, Agri and Aquaculture Journals Dr.
OMIM Entry – % – HALLERMANN-STREIFF SYNDROME; HSS
Warburg emphasized that the diagnosis is doubtful srteiff the absence of cataract or microphthalmia. In many cases, additional abnormalities are also present. Stfeiff N, et al.
A typical Hallermann-Streiff syndrome in a 3 year old child. Core manifestations of this syndrome include marked pre-natal and severe post-natal growth retardation, an unusual face triangular shape, sparse hair, small mouth, pointed chindental anomalies natal teeth; hypodontiageneralized lipodystrophy with localized fat masses, and-in some cases-progressive ataxia and tremor.
Preauricular sinus and cyst. J Clin Pediatr Dent. From Wikipedia, the free encyclopedia. Two patients had reproduced: Some affected infants may also have vitiligo, a condition characterized by irregular patches of skin that lack pigmentation. Indeed, a histopathologic case series of 8 eyes with uveal effusion included a case of Hallermann-Streiff syndrome that demonstrated abnormal scleral collagen.
Rare Disease Database
Am J Med Genet A. Familial cases were reported by Bueno-Sanchez who found this syndrome in 2 of 3 sibs from a consanguineous marriage, supporting autosomal recessive inheritance.
She was small for her week gestational age. HSS is characterized by seven essential signs, as described by Francois: September 07, Citation: Sign haller,ann to customize your interests Sign in to your personal account. Congenital heart defect in a patient with the Hallermann-Streiff syndrome.