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Hallermann-Streiff Syndrome

  • Reference work entry
Atlas of Genetic Diagnosis and Counseling

  • 212 Accesses

Hallermann-Streiff syndrome was independently described by Hallermann in 1948 and Streiff in 1950. The syndrome is characterized by proportionate short stature, craniofacial dysostoses consisting of skeletal, ophthalmologic, and cutaneous defects.

Synonyms and Related Disorders

Francois dyscephalic syndrome

Genetics/Basic Defects

  1. 1.

    Sporadic in virtually all cases

  2. 2.

    Inheritance pattern unknown

Clinical Features

  1. 1.

    Characteristic craniofacial features

    1. a.

      Dyscephaly (malformation of the cranium and bones of the face) (89–90%)

      1. i.

        Calvarium

        1. a)

          Brachycephaly

        2. b)

          Thin calvarium

        3. c)

          Delayed closure of fontanelles

        4. d)

          Wide cranial sutures

      2. ii.

        Cranial base

        1. a)

          Platybasia

        2. b)

          Depressed sella

        3. c)

          Elevated anterior cranial fossa

      3. iii.

        Parrotlike face

        1. a)

          A beaked nose

        2. b)

          Hypoplastic mandible

    2. b.

      Hypotrichosis (80–82%)

      1. i.

        Alopecia

        1. a)

          Characteristic sutural alopecia (hair loss following the lines of the cranial sutures)

        2. b)

          Frontal alopecia

          ...

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References

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Editor information

Editors and Affiliations

  1. Perinatal and Clinical Genetics, Department of Pediatrics, LSU Health Sciences Center, 1501 Kings Highway, Shreveport, LA, 71130, USA

    Harold Chen

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© 2012 Springer Science+Business Media, LLC

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(2012). Hallermann-Streiff Syndrome. In: Chen, H. (eds) Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-1037-9_112

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