Estructura del esqueleto en la osteogenesis imperfectaa propósito de dos casos

  1. S.M. ALMENAR-MEDINA 1
  2. M. GONZALEZ-DEVESA 1
  3. J. J. SANTONJA LUCAS 1
  4. A. LLOMBART BOSCH 1
  1. 1 Universitat de València
    info

    Universitat de València

    Valencia, España

    ROR https://ror.org/043nxc105

Journal:
Revista española de cirugía osteoarticular

ISSN: 0304-5056

Year of publication: 1986

Volume: 21

Issue: 122

Pages: 117-126

Type: Article

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Abstract

A description is made of the pathological findings of two fetuses affected by lethal congenital osteogenesis imperfecta. A review of the latest bibliography is made on the clinico-pathological, biochemical and genetic aspects of the four types of osteogenesis imperfecta (01) described in the classification of SILLENCE et al. The most constant skeletal alterations in lethal congenital 01 (type 11 of SILLENCE) are considered to be: a) Difficulty in the macroscopic identification of membranous bone of the cranial vault; b) Thin cortical regions consiting of reticular bony tissue with multiple chondrogenic fracture callus; c) Metaphyseal fractures with structural collapse; d) Absence of the RANVIER ossiflcation groove; e) Hyperosteocytosis with a decrease in the amount of bony tissue; f) lncrease in the size of the epiphyseal and growth cartilage vasculÍlr channels.

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