Report Of The Hague Conference On Human Limb Maldevelopment
Sidney Fishman, Ph.D. Charles H. Frantz, M.D.
An interesting experiment in conference organization was successfully conducted at The Hague, Holland, on September 11-13, 1963. The subject was: "Human Limb Development and Maldevelopment with Special Reference to Experimental Teratogenesis and Medical Management of Limb Deficiencies".
Under the auspices of the Association for the Aid of Crippled Children, approximately 50 invited specialists from eight countries convened to interchange scientific and clinical experiences pertinent to the child born with malformed extremities. The fields of experimental genetics and embryology, teratology, epidemiology, pediatric and orthopedic management and clinico-social psychology were represented by the conferees.
The conference was designed to consolidate the contributions of these various disciplines at a single meeting in order to develop a definitive statement concerning the current status of knowledge relative to problems of human limb maldevelopment. A coordinate purpose was to provide a medium for the mutual education of the participants. It was felt that individuals studying the congenital malformation problem from differing scientific and clinical vantage points would undoubtedly enhance their understanding and progress through a close interchange with persons involved in other aspects of the problem.
The range and diversity of the professional backgrounds represented at the conference reflects the scope of the subjects discussed. In view of this heterogeneity it is likely that a number of the papers presented contained less technical detail than the authors would have preferred. This tendency toward generalized presentations was not necessarily disadvantageous, however, since it obligated each of the speakers to distill his ideas and experiences into communicable language containing a minimum of technical terms peculiar to a specific field of work.
A brief outline of the progressive development of the subject matter under consideration provides an overview of the scientific fields discussed.
The conference opened with a dissertation on the orderly series of anatomic events occurring in the development of the limb buds. The purely anatomic and embryological conceptions were then broadened to include a consideration of the biochemical factors which exert dynamic influences in limb genesis. The influence of induction mechanisms believed to exist between the ectodermal cap and mesenchymal cells and acting to affect differentiation was outlined.
Following discussions of normal limb development, several papers dealing with efforts to produce malformations experimentally were presented. Animal studies in which specific types of malformations were produced on a genetic basis were particularly intriguing. Consideration was then given to the possible teratogenic effects of environmental factors. A review of known teratogenic drugs was presented with specific emphasis on thalidomide, its breakdown products and its specific effects on the embryo.
Great interest was also manifested in the epidemiology of malformations. All of the data reported clearly indicated a shift in incidence rates during 1961-62 in the countries concerned, as a result of the ingestion of thalidomide. The major foci of these papers, however, related to the methodological problems involved in obtaining data on changes in incidence rates rapidly and reliably. Difficulties inherent in data collection procedures were explored and various suggestions offered to improve these processes. A number of special surveys, designed to obtain a more accurate picture of the extent of the thalidomide problem, were also reported upon.
Presentations related to the orthopedic management of these children included concise statements concerning the present statue of surgical and prosthetic restoration procedures. Røentgenographic analyses of a variety of malformations and the management problems associated with them were also presented. Considerable emphasis was placed on externally powered prostheses which appear to offer the most promising approach to the provision of adequate prehensile function for multiply handicapped children. Although the details of prosthetic and surgical practices were necessarily limited because of time considerations, the audience was able to obtain an accurate overview of current patient management problems. The final phases of the meeting dealt with the psychological and social problems faced by the patients and families of malformed children.
Discussion of Nomenclature
In conjunction with the conference, an informal gathering of the International Ad Hoc Committee on Congenital Skeletal Limb Deficiency Nomenclature was held. The goal of this group is the establishment of an internationally acceptable system of nomenclature for describing the variety of malformations which are seen. A degree of progress towards this end was achieved by isolating major differences of opinion concerning nomenclature and providing a channel of communication between interested individuals in several countries. It is likely that a second meeting of this group will be arranged to complete agreements on nomenclature following further exchanges of ideas via correspondence.
Leonard W. Mayo, Executive Director of the Association for the Aid of Crippled Children, acted as General Chairman, and Dr. Chester A. Swinyard, Director of the Children's Service, Institute of Physical Medicine and Rehabilitation, and Staff Associate of the Association for the Aid of Crippled Children, served as Technical Chairman and was responsible for professional planning. Ernest Koller, Staff Associate with the AACC, assisted with planning and arrangements.
It is anticipated that a report containing all of the papers presented at the conference, together with pertinent discussion, will be published by the Association for the Aid of Crippled Children within the next six months.
Sidney Fishman is Project Director, Prosthetic and Orthotic Studies, Research Division, New York University-College of Engineering, New York, New York
Charles Frantz is Medical Co-Director, The Area Child Amputee Center, Grand Rapids, Michigan; and Chairman, Subcommittee on Children's Prosthetics Problems