Pierre Maroteaux BONE DISEASES OF CHILDREN Philadelphia: J. B. Lippincott Company, 1979, 435 pp., $39.
I do not believe that this volume will help the pediatrician or the orthopedic surgeon who wishes to have a fundamental understanding of skeletal disorders in children - an understanding that will enable them to recognize and classify in a way that will bring to mind the prognosis, what the parents might expect, and where the treatment may lie.
For example some of the labeling given primarily on the basis of radiographic appearance is perpetrated in the grouping of entities without inclusion of a fundamental understanding that this area cries for - thus an entity in which very little bone is seen is labeled "achondrogenesis." Yet an outstanding feature of the disease is the great number of large cartilage cells when the pathology is seen.
Similarly, when achondroplasia is discussed there is little to hang on to in order to make it clear what should be included under this designation. Again pathology seems to be the weakness, for no mention is made of the fact that the proximal long bones are more heavily involved than the distal ones. No mention is made of the fact that when bone is laid down it consists of normal bone trabeculae even though the shape is markedly abnormal. It is recognized that there is a deficiency in the height of the cartilage cell columns.
Part of the author's problem is his tendency to describe case studies because of some variation in anatomic involvement. The inadvisability of attempting to group entities on the basis of a fundamental defect (which could then lead to a certain type of skeletal change with growth) is due to the fact that the underlying reason for these abnormalities is at present unknown.
The Kniest syndrome is better understood by the author - yet the statement is made that it should not be confused with spondyloepiphyseal dysplasia "because of the entirely different appearance of the spine and pelvis." Statements like this are made without a clear explanation. The book contains very extensive illustrations, yet the legends are telegraphic and fail to educate the reader. A radiograph of the pelvis of a sixmonth-old boy, for example, clearly shows deformed acetabular roofs and widened pelvis with broad femoral necks but is noted only as having delayed ossification of the ischiopubic segments. The author does better with a more recognizable entity, such as the Ellis- Van Creveld syndrome. But when the author states that there is a "characteristic appearance of the iliac bones" on the radiograph, the reader would like to know what x-ray signs make the appearance characteristic.
This book could be markedly improved by an additional descriptive paragraph for each legend. Nowhere is the term chondrodystrophy understood ro express the underlying change giving rise to the abnormally shaped bones in spondyloepiphyseal syndromes. The slightest variation in anatomic distribution seems to rate the description of another named syndrome or eponym even though the underlying process is clearly the same.
That the author had access to an enormous amount of material is clear - that it overwhelmed him is also clear. Closely following the diseases in which the cartilage cell cannot grow in organized fashion and grouped with them are such diseases as fibrous dysplasia and osteogenesis imperfecta.
The dysostoses (involvement of one area} made a convenient term for grouping entities which did not have a general involvement. Here an illustration (Figure 4-35) shows congenital bowing of the tibia and fibula alleged to be due to von Recklinghausen's disease. The structure is termed "markedly abnormal." Yet both bones are bowed symmetrically, the cortex is widened on the pressure side only, and the medullary canal remains open in bones that could be of normal length - changes quite characteristic of intrauterine packing.
The book by Pierre Maroteaux would work well as a kind of encyclopedic listing from which one could readily procure a reference in order to read further. It does not enhance understanding of this field or give the reader a method by which to accurately recognize these syndromes. It mirrors the state of knowledge in the field and the fact that the participants in this area are still groping without enough knowledge to bring it all together.