R.S. Lachman. St. Louis: Elsevier-Mosby; 2007, 1408 pages, 2160 illustrations, $279.00.
The value of a book devoted to an alphabetic listing and description of syndromes, metabolic disorders, and skeletal dysplasias can be debated. To this reviewer, there is some benefit in such a compilation because it can serve as a quick review of well-known diseases or it can serve to let you know that there are diseases out there that you will never encounter.
In the fifth edition of this text (first edition published in 1975), there are roughly 1000 different entries that are either only briefly mentioned or are described in more detail and illustrated with imaging, as appropriate. The book has 3 sections (Section 1, “Syndromes and Metabolic Disorders”; Section 2, “Skeletal Dysplasias”; Section 3, “Gamuts and References”) and 2 appendices (“Classification of Genetic Bone Disorders” and “A Teaching Approach to Skeletal Dysplasias”).
Starting with the A's, you encounter Aarskog syndrome in Section 1 and end with the Yunis-Varon syndrome as the last entry in Section 2 (with the Kabuki make-up syndrome along the way); you quickly get the idea that there are zebras galore in the book. Then you stagger into 167 pages of gamuts. Did you know, for example, that there are 34 syndromes associated with diaphragmatic hernias (more important, do you care?), or from a neuroradiology standpoint, there are about 150 syndromes/diseases associated with external ear malformations, including the Antley-Bixler syndrome and the Bloom syndrome? I suppose it is worthwhile that someone has catalogued all these syndromes and dysplasias, but I do not figure one is going to learn much radiology here. Concerning the imaging, the quality in general is poor, with MR images in particular looking, in some cases, like they came from one of the world's first MR imaging units. To be fair, the authors are dealing in many instances with raris avis, so they were undoubtedly limited in the imaging they could display. On the other hand as just 2 examples, a case of diabetes insipidus is windowed so poorly that the reader has no way of knowing whether there was or was not absence of the posterior pituitary bright spot and a case of Dandy-Walker malformation is from at least 20 years ago. Neither of these 2 diseases is so unusual that the authors could not have gotten modern images. An even more absurd example is that of dermatomyositis; it is shown as basically a big white blob. This problem of very poor image quality abounds and detracts from an otherwise entertaining and at times a curious book.
In summary, this publication could serve as a quick reference for many of medicine's more bizarre and rare syndromes. It may have a place in a departmental library.
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