Hallermann-Streiff Syndrome

Reference work entry

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

           
        3. c)

          Alopecia at the scalp margins

           

Keywords

Osteoporosis Brittle Glaucoma Cataract Hypothyroidism 

References

  1. Aracena, T., & Sangueza, P. (1977). Hallermann-Streiff-Francois syndrome. Journal of Pediatric Ophthalmology, 14, 373–378.PubMedGoogle Scholar
  2. Christian, C. L., Lachman, R. S., Aylsworth, A. S., et al. (1991). Radiological findings in Hallermann-Streiff syndrome: Report of five cases and a review of the literature. American Journal of Medical Genetics, 41, 508–514.PubMedCrossRefGoogle Scholar
  3. Cohen, M. M., Jr. (1991). Hallermann-Streiff syndrome: A review. American Journal of Medical Genetics, 41, 488–499.PubMedCrossRefGoogle Scholar
  4. Colomb, R. S., & Porter, P. S. (1975). A distinct hair shaft abnormality in the Hallermann-Streiff syndrome. Cutis, 16, 122–128.Google Scholar
  5. David, L. R., Finlon, M., Genecov, D., et al. (1999). Hallermann-Streiff syndrome: Experience with 15 patients and review of the literature. The Journal of Craniofacial Surgery, 10, 160–168.PubMedCrossRefGoogle Scholar
  6. Dinwiddie, R., Gewitz, M., & Taylor, J. F. N. (1978). Cardiac defects in the Hallermann-Streiff syndrome. Journal of Pediatrics, 92, 77–78.PubMedCrossRefGoogle Scholar
  7. François, J. (1982). Francois dysencephalic syndrome. Birth Defects, 18(6), 595–619.PubMedGoogle Scholar
  8. François, J., & Pierard, J. (1971). The Francois dysencephalic syndrome and skin manifestations. American Journal of Ophthalmology, 71, 1241–1250.PubMedGoogle Scholar
  9. Friede, H., Lopata, M., Fisher, E., et al. (1985). Cardiorespiratory disease associated with Hallermann-Streiff syndrome: Analysis of craniofacial morphology by cephalometric roentgenograms. Journal of Craniofacial Genetics and Developmental Biology. Supplement, 1, 189–198.PubMedGoogle Scholar
  10. Golomb, R. S., & Porter, P. S. (1975). A distinct hair shaft abnormality in the Hallermann-Streiff syndrome. Cutis, 16, 122–128.Google Scholar
  11. Haberman, H., & Clement, P. A. (1979). The value of anthropometrical measurements in a case of Hallermann-Streiff syndrome. Rhinology, 17, 179–184.PubMedGoogle Scholar
  12. Haque, M., Goldenberg, D. T., Walsh, M. K., et al. (2009). Retinal detachments involving the posterior pole in Hallermann-Streiff syndrome. Retinal Cases & Brief Reports, X, 1–3.Google Scholar
  13. Hendrix, S. L., & Sauer, H. J. (1991). Successful pregnancy in a patient with Hallermann-Streiff syndrome. American Journal of Obstetrics and Gynecology, 164, 1102–1104.PubMedGoogle Scholar
  14. Hutchinson, D. (1971). Oral manifestations of oculomandibulodyscephaly with hypotrichosis (Hallermann-Streiff syndrome). Oral Surgery, Oral Medicine, and Oral Pathology, 31, 234–244.PubMedCrossRefGoogle Scholar
  15. Malde, A. D., Jagtap, S. R., & Pantvaidya, S. H. (1994). Hallermann-Streiff syndrome: Airway problems during anaesthesia. Journal of Postgraduate Medicine, 40, 216–218.PubMedGoogle Scholar
  16. Nevin, N. C., Scally, B. G., Thomas, P., et al. (1974). The Hallermann-Streiff syndrome. Journal of Mental Deficiency Research, 18, 145–151.PubMedGoogle Scholar
  17. Ohishi, M., Murakami, E., Haita, T., et al. (1986). Hallermann-Streiff syndrome and its oral implications. ASDC Journal of Dentistry for Children, 53, 32–37.PubMedGoogle Scholar
  18. Patterson, G. T., Braun, T. W., & Sotereanos, G. C. (1982). Surgical correction of the dentofacial abnormality in Hallermann-Streiff syndrome. Journal of Oral and Maxillofacial Surgery, 40, 380–384.PubMedCrossRefGoogle Scholar
  19. Ryan, C. F., Lowe, A. A., & Fleetham, J. A. (1990). Nasal continuous positive airway pressure (CPAP) therapy for obstructive sleep apnea in Hallermann-Streiff syndrome. Clinical Pediatrics (Philadelphia), 29, 122–124.CrossRefGoogle Scholar
  20. Salbert, B. A., Stevens, C. A., & Spence, J. E. (1991). Tracheomalacia in Hallermann-Streiff syndrome. American Journal of Medical Genetics, 41, 521–523.PubMedCrossRefGoogle Scholar
  21. Sataloff, R. T., & Roberts, B. R. (1984). Airway management in Hallermann-Streiff syndrome. American Journal of Otolaryngology, 5, 64–67.PubMedCrossRefGoogle Scholar
  22. Scheuerle, A. (1999). Commentary on Hallermann-Streiff Syndrome: Experience with 15 patients and review of the literature. The Journal of Craniofacial Surgery, 10, 225.PubMedCrossRefGoogle Scholar
  23. Sclaroff, A., & Eppley, B. L. (1987). Evaluation and surgical correction of the facial skeletal deformity in Hallermann-Streiff syndrome. International Journal of Oral and Maxillofacial Surgery, 16, 738–744.PubMedCrossRefGoogle Scholar
  24. Slootweg, P. J., & Huber, J. (1984). Dento-alveolar abnormalities in oculomandibulodyscephaly (Hallermann-Streiff syndrome). Journal of Oral Pathology, 13, 147–154.PubMedCrossRefGoogle Scholar
  25. Spaepen, A., Schrander-Stumpel, C., Fryns, J. P., et al. (1991). Hallermann-Streiff syndrome: Clinical and psychological findings in children. Nosologic overlap with oculodentodigital dysplasia? American Journal of Medical Genetics, 41, 517–520.PubMedCrossRefGoogle Scholar
  26. Steele, R. W., & Bass, J. W. (1970). Hallermann-Streiff syndrome. Clinical and prognostic considerations. American Journal of Diseases of Children, 120, 462–465.PubMedGoogle Scholar
  27. Sugar, A., Bigger, J. F., & Podos, S. M. (1971). Hallermann-Streiff-Francois syndrome. Journal of Pediatric Ophthalmology, 8, 234–238.Google Scholar
  28. Suzuki, Y., Fujii, T., & Fukuyama, Y. (1970). Hallermann-Streiff syndrome. Developmental Medicine and Child Neurology, 12, 496–506.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Personalised recommendations