Disease definition. Hallermann-Streiff syndrome is a rare genetic syndrome characterized mainly by head and facial abnormalities such as bird-like facies ( with. Hallermann-Streiff syndrome is characterized by a typical skull shape ( brachycephaly with frontal bossing), hypotrichosis, microphthalmia, cataracts, beaked. Hallermann-Streiff syndrome (HSS) is a rare inherited disorder characterized by malformations of the cranium and facial bones, congenital.

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A breathing endotracheal tube is then passed through the mouth down the throat and into the windpipe. Early preventive care protocols, detailed oral hygiene instructions, and regular dental visits are essential for patients with this syndrome.

The typical bird-like facies and parrot-beak nose, sparse hairs, hypertelorism, and strabismus Click here to view. Hallermann-Streiff syndrome was first described in the medical literature in Copyright American Medical Association.

Int J Oral Maxillofac Surg. The content of the website and databases of the National Organization for Rare Disorders NORD is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD.

Other syndromes resembling this disorder are caused by mutations in the RBBP8 gene on chromosome 18q The genetic cause of Hallermann—Streiff syndrome has not been conclusively determined. Other treatment is symptomatic and supportive. Nicholson AD, Menon S. This potential anesthetic risk must be taken into consideration by surgeons, pediatric anesthesiologists, and other health care providers when making decisions concerning surgery.

Purchase access Strwiff to the journal. These signs are 1 dyscephalia and birdlike facies, 2 dental abnormalities, 3 proportionate short stature, 4 atrophy of skin especially on the nose5 hypotrichosis, 6 bilateral microphthalmos, and 7 cataract. For all other comments, please send your remarks via contact us. Forsius and de la Chapelle found normal chromosomes in 2 cases.


Hallermann Streiff Syndrome-The Oral Manifestations in a Child | OMICS International

Radiograph of pelvis shows dysplastic pubic bone and pubic dilatation. Only comments written in English can be processed. The mother reported of the child having cardiac problem ventricular septal defect for which surgery had been performed. There was no major abnormality detected on routine hematological investigations and ultrasonography of abdomen. The parent also gave a history of child having frequent cough and cold for which symptomatic treatment was being given.

Madelung’s deformity Clinodactyly Oligodactyly Polydactyly. Select your language of interest to view the total content in your interested language.

Hallermann Streiff Syndrome-The Oral Manifestations in a Child

The orodental abnormalities in the present case confirmed by radiographic examination were in the form of severely carious hypoplastic teeth, missing permanent teeth, hallegmann deciduous teeth, bilateral hypoplastic condyles and coronoid processes. Congenital malformations and deformations of face and neck Q18 Progeria differs from HSS by having premature atherosclerosis, nail dystrophy, chronic deforming arthritis, acromicria, and normal ocular findings.

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Hallermann-Streiff syndrome and pregnancy. Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more.

This article has been cited by 1 Generalized odontodysplasia in a 5-year-old patient with Stteiff syndrome: As a result of these small deeply-seated eyes, patients may appear syreiff have small, droopy eyelids blepharoptosis. 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.

For information about clinical trials conducted in Europe, contact: The syndrome can be associated with sleep apnea.

Generalized odontodysplasia in a 5-year-old patient with Hallermann-Streiff syndrome: Five negative signs were also described by Halermann as differential diagnostic criteria for HSS. Early measures are based around ensuring proper hallrrmann and intake of nutrients and may include a tracheostomy.


Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account. Robinow also emphasized the risks of problems with upper airway obstruction in HSS, particularly in the neonatal period and in infancy. Get free access to newly published articles. Koliopoulos and Palimeris observed 5 cases in 3 generations with male-to-male transmission.

It is a rare clinical entity of unknown etiology that affects growth, cranial development, hair growth, and dental haolermann [ 4 ]. Pectus excavatum Pectus carinatum. Purchase access Subscribe now. Sign in to make a comment Sign in to your personal account. Steele and Bass reviewed 50 published cases.


For information about clinical trials sponsored by private sources, contact: Treatment of the nasal abnormalities of Hallermann-Streiff syndrome by lipofilling. Cleidocranial dysostosis Sprengel’s deformity Wallis—Zieff—Goldblatt syndrome. Degenerative skin changes atrophy are also often present and largely limited to the scalp and nose.

How to cite this URL: Indirect ophthalmoscopy revealed bilateral macular serous retinal detachments with horizontal retinal folds through the macula, as well as peripapillary and peripheral retinal pigment epithelial mottling Figure 2. Treatment The treatment of Hallermann-Streiff syndrome is directed toward the specific symptoms that are apparent in each individual.

Get free access to newly published articles Create a personal account or sign in to: The palate was high arched and V shaped Figure 6. Radiologic findings in 5 cases and in the literature were reviewed by Christian et al. Hutchinson-Gilford progeria syndrome is due to a de novo heterozygous mutation in the lamin A gene LMNA on chromosome 1q