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Hallermann Streiff Syndrome

Important
It is possible that the main title of the report Hallermann Streiff Syndromeis not the name you expected.

Synonyms

  • Francois dyscephaly syndrome
  • Hallermann-Streiff-Francois syndrome
  • Oculomandibulodyscephaly with hypotrichosis
  • HSS
  • Oculomandibulofacial syndrome

Disorder Subdivisions

  • None

General Discussion

Hallermann-Streiff syndrome was first described in the medical literature in 1893. The disorder was named for two investigators who later independently reported cases of the syndrome (Hallermann W, 1948; Streiff EB, 1950), recognizing it as a distinct disease entity.
Hallermann-Streiff syndrome is a rare genetic disorder that is primarily characterized by distinctive malformations of the skull and facial (craniofacial) region; sparse hair (hypotrichosis); eye (ocular) abnormalities; dental defects; degenerative skin changes (atrophy), particularly in the scalp and nasal regions; and/or short stature (i.e., dwarfism). Characteristic craniofacial features include a short, broad head (brachycephaly) with an unusually prominent forehead and/or sides of the skull (frontal and/or parietal bossing); a small, underdeveloped lower jaw (hypoplastic mandible); a narrow, highly arched roof of the mouth (palate); and a thin, pinched, tapering nose. Many affected individuals also have clouding of the lenses of the eyes at birth (congenital cataracts); unusually small eyes (microphthalmia); and/or other ocular abnormalities. Dental defects may include the presence of certain teeth at birth (natal teeth) and absence (hypodontia or partial adontia), malformation, and/or improper alignment of teeth. In almost all cases, Hallermann-Streiff syndrome has appeared to occur randomly for unknown reasons (sporadically) and may be the result of a new change to genetic material (mutation).

Resources

National Foundation for Ectodermal Dysplasias
410 East Main Street
PO Box 114
Mascoutah
IL
62258-0114
Tel: (618)566-2020
Fax: (618)566-4718
maryk@nfed.org
http://www.nfed.org

Children's Craniofacial Association
13140 Coit Road
Suite 517
Dallas
TX
75240
USA
Tel: (214)570-9099
Fax: (214)570-8811
800: (800)535-3643
csmith@ccakids.com
http://www.ccakids.com

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains
NY
10605
Tel: (914)428-7100
Fax: (914)997-4763
800: (888)663-4637
Askus@marchofdimes.com
http://www.marchofdimes.com

FACES: The National Craniofacial Association
P.O. Box 11082
Chattanooga
TN
37401
Tel: (423)266-1632
Fax: (423)267-3124
800: (800)332-2373
faces@faces-cranio.org
http://www.faces-cranio.org

National Association for Parents of Children with Visual Impairments (NAPVI)
P.O. Box 317
Watertown
MA
02472
Tel: (617)972-7441
Fax: (617)972-7444
800: (800)562-6265
napvi@perkins.org
http://www.napvi.org

Little People of America, Inc.
250 El Camino Real
Suite 201
Tustin
CA
92780
Tel: (714)368-3689
800: (888)572-2001
info@lpaonline.org
http://www.lpaonline.org

National Craniofacial Foundation
3100 Carlisle Street
Suite 215
Dallas
TX
75204
800: (800)535-3643NIH/National Eye Institute
Building 31 Rm 6A32
31 Center Dr MSC 2510
Bethesda
MD
20892-2510
United States
Tel: (301)496-5248
Fax: (301)402-1065
2020@nei.nih.gov
http://www.nei.nih.gov/

Institute for Families
P.O. Box 54700
Mailstop #111
Los Angeles
CA
90054-0700
USA
Tel: (323)669-4649
Fax: (323)665-7869
info@instituteforfamilies.org
http://www.instituteforfamilies.org

Craniofacial Foundation of America
975 East Third Street
Chattanooga
TN
37403
Tel: (423)778-9192
Fax: (423)778-8172
800: (800)418-3223
farmertm@erlanger.org
http://www.craniofacialcenter.com

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay
WI
54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
800: (877)336-5333
mums@netnet.net
http://www.netnet.net/mums/

Hallerman-Streiff Syndrome Support Group
3524 Blenheim Road
Phoenix
MD
21131
Tel: (443)318-4461
hmbeam@gmail.com
http://www.hallerman-streiffsupport.org

For a Complete Report:

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  11/12/2008
Copyright  1988, 1990, 1998, 2001, 2002, 2008 National Organization for Rare Disorders, Inc.

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: November 12, 2008
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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