Hostname: page-component-745bb68f8f-grxwn Total loading time: 0 Render date: 2025-02-11T19:28:40.632Z Has data issue: false hasContentIssue false
  • English
  • Français

Temporal bone histopathological findings in campomelic dysplasia

Published online by Cambridge University Press:  29 June 2007

H. Takahashi ,
I. Sando  and
H. Masutani
H. Takahashi
Affiliation:
Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Eye and Ear Institute of Pittsburgh.
I. Sando*
Affiliation:
Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Eye and Ear Institute of Pittsburgh.
H. Masutani
Affiliation:
Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Eye and Ear Institute of Pittsburgh.
*
Dr I. Sando, Division of Otopathology, Eye & Ear Institute of Pittsburgh, 203 Lothrop Street, Pittsburgh, PA 15213.
Rights & Permissions [Opens in a new window]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

Both temporal bones of a newborn (35 gestational weeks old) with campomelic syndrome were studied histopathologically. This is to our knowledge the second temporal bone report (third case) of this syndrome. The findings included: abnormal cartilagenous and osseous tissues and abnormality in the globuli interossei in the otic capsule; deformities of the vestibule and semicircular canals, probably due to compression by the abnormal cartilaginous tissue; hypoplastic cochleaand semicircular canals; aberrant course of the facial nerve; wide dehiscence of the facial canal in the tympanic portion; slight hypoplasia of the malleus and anomalies in the incus and stapes; and large epitympanic space. These findings closely resembled those of the first report, and suggest that: 1) campomelic dysplasia is a definite disease entity with consistent pathogenesis, and 2) similar otologic manifestations may be expected in the majority of patients with this syndrome.

Keywords

Temporal boneHistologyDysplasia, compomelic
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 106 , Issue 4 , April 1992 , pp. 361 - 365
Copyright
Copyright © JLO (1984) Limited 1992

References

Anson, B. J., Donaldson, J. A. (1981) Surgical anatomy of the temporal bone. 3rd edn. W. B. Saunders Co.: Philadelphia.Google Scholar
Bain, A. D., Barrett, H. S. (1959) Congenital bowing of the long bones: report of a case. Archives of Diseases in Childhood, 34: 516–524.CrossRefGoogle Scholar
Bergsma, D. (1979) Birth defects Compendium, 2nd edn. Alan R. Liss Inc.: New York. p. 161–162.CrossRefGoogle Scholar
Bound, J. P., Finley, H. V. L., Rose, F. C. (1952) Congenital anterior angulation of the tibia. Archives of Diseases in Childhood, 27: 179–184.CrossRefGoogle ScholarPubMed
Gillerot, Y., Vanheck, C. A., Foulon, M., Podevain, A., Koulischer, L. (1989) Campomelic syndrome: manifestations in a 20-week fetus and case history of a five-year-old child. American Journalof Medical Genetics, 34: 589–592.CrossRefGoogle Scholar
Houston, C. S., Opitz, J. M., Spranger, J. W., Macpherson, R. I., Reed, M. H., Gilbert, E. F, Herrmann, J., Schinzel, A. (1983) The campomelic syndrome: review, report of 17 cases, and follow-up on the currently 17-year-old boy first reported by Maroteaux et al. in 1971. American Journal of Medical Genetics, 15: 3–28.CrossRefGoogle ScholarPubMed
Hwang, W. S. (1979) Pathology of cartilage in camptomelic dwarfism. Laboratory Investigation, 40: 305–306.Google Scholar
Lazjuk, G. I., Shved, I. A., Cherstvoy, E. D., Feshchenko, S. P. (1987) Campomelic syndrome: econcepts of the bowing and shortening in the lower limb. Teratology, 35: 1–8.CrossRefGoogle Scholar
Lee, F. A., Isaacs, H., Strauss, J. (1986) The ‘campomelic’ syndrome. American Journal of Diseases of Children, 124: 485–496.CrossRefGoogle Scholar
Nogami, H., Oohira, A., Kuroyanagi, M., Mizutani, A. (1986) Congenital bowing of long bones: clinical and experimental study. Teratology, 33: 1–7.CrossRefGoogle ScholarPubMed
Sando, I., Doyle, W. J., Okuno, H., Takahara, T., Kitajiri, M., Coury, W. J. III (1986) A method for the histopathological analysis of the temporal bone and the Eustachian tube and its accessory structures. Annals of Otology, Rhinology and Laryngology, 95: 267–274.CrossRefGoogle ScholarPubMed
Sando, I., Takagi, A., Takahashi, H. (1989) Computer-aided threedimensional reconsturction andmeasurement of temporal bone structures. Annals of Otology, Rhinology and Laryngology, 98 (supplement 143): 35–38.CrossRefGoogle Scholar
Takagi, A., Sando, I. (1989) Computer-aided three-dimensional reconstruction: a method of measuring temporal bone structures including the length of cochlea. Annals of Otology, Rhinology and Laryngology, 98: 515–522.CrossRefGoogle ScholarPubMed
Tokita, N., Chandra-Sekhar, H. K., Daly, J. R, Becker, M. H., Alek-sic, S. (1979) The campomelic syndrome: temporal bone histo- pathologic features and otolaryngologic manifestations. Archives of Otolaryngology, 105: 449–454.CrossRefGoogle Scholar
Williams, G. H. (1990) Developmental anatomy of the ear. In: Otolaryngology, Vol. 1, Diseases of the Ear and Hearing (English, G. M., ed.). J. B. Lippincott Co.: Philadelphia, pp. 42–48.Google Scholar