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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The case reported here was found to be mute who has not earlier been reported in this syndrome although no correlation to this syndrome could be established.
It is named after German ophthalmologist Wilhelm Hallermann — and Italian-Swiss ophthalmologist Enrico Bernardo Streiff —   who first described the syndrome in and respectively. The greatest anesthetic challenge lies in the maintenance of an appropriate airway due to upper airway deformities which make mask ventilation, laryngeal exposure and tracheal intubation difficult.
About News Events Contact. As a result of these small deeply-seated eyes, patients may appear to have small, droopy eyelids blepharoptosis.
Hallermann Streiff Syndrome – NORD (National Organization for Rare Disorders)
A, The right fundus showed a hyperemic and hypervascular optic nerve with engorged retinal vessels. The underdevelopment of the jaw and nose may result in upper airway obstruction and breathing difficulties in young children. Johns Hopkins Press pub. Core manifestations of this syndrome include marked pre-natal and severe post-natal growth retardation, an wtreiff 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.
Am J Med Genet. Early detection and management of retinal detachment in other patients having Hallermann-Streiff syndrome offer synsrome chance of improved visual function. Glossoptosis refers to downward displacement or retraction of the tongue that may occur secondary to abnormal smallness of the lower jaw micrognathia. The most common ocular finding is clouding opacity of the lenses of both eyes at birth congenital bilateral cataracts.
In all but 3 of these, the affected persons were sibs.
Acheiropodia Ectromelia Phocomelia Amelia Hemimelia. For information about clinical trials sponsored by private sources, contact: Mandibulofacial dysostosis usually has ear anomalies and lower eyelid colobomas [ 13 ]. No negative signs were seen in our patient.
Dental features were discussed by Caspersen and Warburg B, The left fundus had a similar appearance with less vascular engorgement and possible pigment epithelial detachment along the superotemporal vascular arcade. Examination of the face revealed frontal bossing, small and thin face, beaked nose with atrophy of skin near nose Figure 3,4.
Most individuals with HSS have ocular abnormalities. The orthopantommograph revealed multiple carious deciduous teeth, missing permanent teeth, hypoplastic coronoid and condylar processes Figure 7. Affected Populations Hallermann-Streiff syndrome appears to affect males and females in relatively equal numbers. Hallermann-Streiff syndrome is frequently characterized by dental abnormalities.
The patient was pre term 34 weeks low birth weight 2. Wiedemann-Rautenstrauch syndrome also known hallermnn neonatal progeroid syndrome is an extremely rare genetic disorder characterized by an aged appearance at birth neonatal progeroid appearance ; growth delays before and after birth prenatal and postnatal growth deficiency ; and deficient or absent fatty tissue under the skin subcutaneous lipoatrophycausing the skin to appear abnormally thin, fragile, and wrinkled.
J Clin Pediatr Dent.
This page was last edited on 26 Novemberat A typical Hallermann-Streiff syndrome in a 3 year old child. Madelung’s deformity Clinodactyly Oligodactyly Polydactyly.
Looking For More References? Two cases in which the chromosomes were studied. Sclerectomy has been advocated for primary uveal effusion syndrome, but its efficacy in congenital uveal effusion in microphthalmic eyes is unknown.
It may be difficult to perform root canal treatment and other therapies to preserve a tooth with underdeveloped roots, and therefore these patients need appropriate, frequent pediatric dental evaluations. General Discussion Summary Hallermann-Streiff syndrome HSS is a rare disorder that is primarily characterized by distinctive malformations of the skull and facial craniofacial region; sparse hair hypotrichosis ; eye abnormalities; dental defects; degenerative skin changes atrophyparticularly in the scalp and nasal regions; and proportionate short stature.
Also, because individuals with Hallermann-Streiff syndrome have malformed teeth with abnormal roots and enamel hypoplasia, they are sreiff to developing severe dental caries making it imperative to ensure good dental hygiene.
Sur deux cas de syndrome dyscephalique a tete d’oiseau. Neonatal teeth may be present. Views Read Edit View history. Lethal syndrome of slender bones, intrauterine fractures, characteristic facial appearance, and cataracts, resembling Hallermann-Streiff syndrome in two sibs. To call this Francois syndrome Battin et al. The orodental abnormalities in the present case confirmed by radiographic examination were in the form of ztreiff carious hypoplastic teeth, missing permanent teeth, persistent deciduous teeth, bilateral hypoplastic condyles and coronoid processes.
The palate was high arched and V shaped Figure 6.