Recent Articles Of Interest

(The following abstract from the December 1964 issue of Birth Defects is reprinted with the permission of the National Foundation - March of Dimes.)

The Frequency Of Dysmelia In The Former Province Of Hanover From 1920 To 1963.

(In German) Z. Orthop. 99(2):248-257, Oct. 1964

Peterson, D. (Orthop. Center, Annastift, Hanover-Kleefeld, Germany.)

A total of 834 dysmelias, including amelia, peromelia, ectromelia, phocomelia, perodactyly, and peripheral hypoplasia, were reported hy a provincial orthopedic clinic in 44 years. Despite a uniform increase in regional population from 3 to 5 million, with a consequent increase in number of births from 65,000 in 1920-1929 to 82,000 in 1950-1959, the dysmelia frequency remained relatively stationary with no prominent peaks up to 1960. There was a slight relative increase in the curve between 1928 and 1936. The absolute number actually declined from 0.024% in 1920-1929 to 0.0l8% in 1950-1959, per number of live borns, or a 25% decrease. Then an abrupt and enormou6 increase occurred in 1960, peaked in 196l, fell slightly in 1962, and sank as dramatically to normal in 1963.

In the 44 years there were 24 amelias, equally divided sexually, affecting mainly the upper extremities; 15 were bilateral. Sporadic until 1960, seven were born in 196l and five in 1962 - all of the latter in the first 9 months of 1962-, and none in 1963. Moreover, all 12 (196l-1962)cases involved the upper extremities.

There were 242 peromelias, chiefly involving the forearm, especially on the left. Sexual distribution was approximately equal. These cases were not significantly increased in 1960-1962 over earlier peak periods around 1928, the mid-thirties, and 1958. The lowest incidence is registered for the war and postwar periods.

The distribution of ectromelia was roughly uniform until 1960, with peaks in 1928, 1934, 1937, 1949, and 1958 involving eight or nine cases. In 1960 there were 32 cases, in 1961, 72, and in 1962, 60. Three more in 1963 totalled 326 for the 44 year interval, including 166 male and 160 female. Of the 60 in 1962, only one was born after September 1 of that year. Phocomelia was mainly associated with l96O-1962, especially of the upper extremities; radial aplasia or hypoplasia was the most frequent affliction.

True phocomelia has been rare - 14 cases, four of them in 196l-1962. The incidence of perodactyly has been routinely elevated - with a total of 212 cases equally divided sexually; peaks occurred from 1928 to 1938, 1951, and 1959, with no increase in 196O-1962. The 30 cases of peripheral hypoplasia were distributed sporadically.

The 196O-1962 elevation was only quantitatively distinct from earlier years and repeated the seasonal patterns of little involvement after the first three quarters of each year. In particular, the more severe ectromelias predominated.

Endogenous origins are excluded as explanatory of the recent outbreak. But, except for a possible thalidomide influence -which began already in 1958 - no conspicuous exogenous factors are eligible for consideration. (13 references.)

(The following is an abstract of an article, "Recent Concepts in the Treatment of the Limb-Deficient Child," by Cameron B. Hall, M.D. of the Child Amputee Prosthetics Project, Center for Health Sciences, University of California, Los Angeles 24, California. The article appeared in the Manitoba Medical Review, 44:552-557, November 1964.)

According to this article which discusses the genetic aspects and treatment of limb-deficient children, there is increasing evidence that the great bulk of congenital anomalies apparent at birth have their genesis during the first eight weeks of fetal life, before the embryo is one inch in length.

Experimenters using teratogenic agents in animals have produced statistically significant deformities. The timing of administration within the fetal growth period seems to be as important as the nature of the agent itself.

The significance of the time element is also evident in the "thalidomide" cases. Epidemiological studies have revealed a close relationship between the day drug ingestion was started and the type of anomaly produced. The critical maternal ingestion period appears to have been between the 27th and 30th day of pregnancy, as the most severely deformed children were born to mothers who reported taking thalidomide at that time.

Anomalies may also result from chromosomal aberration within the infant itself or may be due to hereditary factors.

Congenital difficulties can be classified by the 0'Rahilly and Frantz method, based, on roentgenographic appearance of the extremities observable within a day or two after birth. Deformities are classified into two basic types: Terminal where no part exists distal to or in line with the deficient portion; and intercalary where there is an intersegmental loss with portions proximal and distal to the affected segment remaining intact. Within both of these classifications, the deficiencies may be transverse, encompassing the entire girth of the limb; or they may be paraxial. occurring as a longitudinal deficiency in the pre- or postaxial elements essentially, with the uninvolved portions remaining generally intact.

Physical and occupational therapy should be started as soon as the youngster begins to show interest in his environment. For example, if the youngster is placed in the prone position, his back will be strengthened as he lifts his head in curiosity. With upper-extremity amelia, even the most rudimentary digits arising from the malformed shoulders should be stimulated and strengthened for eventual prosthesis control. The ability to grasp and touch must be learned; lack of opportunity will result in atrophy and disuse of valuable extremities. Flexibility of the legs and feet should be encouraged in the armless. Children with both upper- and lower-amelias must be encouraged to feel with their mouth and their lips and to hold objects between their chin and shoulders.

Early fittings of prostheses result in complete patient and family acceptance. Arms should be fitted at about six months of age and legs at about nine months of age.

Fitting a child with upper-extremities amelia or short phocomelias starts when the child has obtained good sitting balance. Those children whose deformity is such that they cannot achieve sitting stability unaided should be provided with appropriate trunk support for upright posture.

Initially the arm prosthesis is fitted for passive control. It is either operated by the parents, or by the child's good hand if he is a unilateral amputee. If this type of fitting is accomplished during the first six or eight months of life, active control motions can be carried out by the time the child is between sixteen and twenty-four months of age.

The fitting of a patient with bilateral upper-extremity amelia or phocomelia is a difficult problem. In Germany such a child is fitted at about six to eight months of age. At first bilateral shoulder and thorax sockets are fitted. Patients are taught a controlled shoulder (abduction) motion which brings the prosthetic hands into apposition in a clapping motion. This allows holding of large balls, dolls and other toys. Release is accomplished by reversing shoulder action (adduction). As maturation and dexterity develop, pneumatic controls for the opening and closing of the terminal hook, pro-nating and supinating the forearm, and for flexion and extension of the elbows can be added in amputees of approximately four years of age. Placement of the control valve for phocomelic digit operation or shoulder motion allows a "normal" neuromuscular pattern to develop. The bilateral phocomelic five-year old can open and close the hook on his ipsilateral side by digit function, rotate his wrist by contralateral digit operation, and flex and extend his elbow by nudging a strategically placed valve with his chin.

In surgical procedures caution should be exercised. What may first appear as a useless and grotesque appendage can frequently mature into a useful limb capable of stabilizing a prosthesis or providing control operations in a power system. However, in certain deformities early amputation at the proper level becomes the procedure of choice, as it allows for optimal prosthetic fitting.

In our clinic, surgery for terminal skeletal overgrowth is often necessary. Good results have been achieved in syndactylism by surgical separation of the digits and in biceps cineplasties.

Shirlee Kempner

(The following abstract from the August 1964 issue of Birth Defects is reprinted with the permission of the National Foundation- March of Dimes.)

EVALUATION OF THE TERATOGENICITY OF DRUGS.

Clin. Pharmacol. Ther. 5(4): 480-5l4, July-August 1964.

Cahen, R.L. (Lafon Res. Center, Maisons-Alfort, France.)

An historical and bibliographical survey of teratogenic agents, the experimental production of congenital malformations, and experimental techniques for screening teratogenic potentiality in new drugs are discussed.

Lists of agents strongly suspected of affecting human embryos, with or without experimental animal confirmation, those teratogenic for animals but nontherapeutic for humans, those indicted for human defects on insufficient evidence, those responsible for authentic human malformation without animal verification, and those which cross the placental barrier but are non-teratogenic are given. Other topics include mechanisms of teratogenic activity, relation to chemical structure, and selectivity of effects.

Choice and number of species, strain variations, and various dose requirements, including ratio of effective and teratogenic dose, significance of small doses, duration and route of administration, optimal number of animals and relevant assay, reproduction studies on chronically treated rats, effect of maternal agents on early embryonic disc formation, and clinical testing are also reviewed.

Finally, methods for producing and verifying gestation, administering test agents, confirming teratogenesis, interpreting results, and choosing tenative reference standards in screening procedures are developed. (262 references)

(The following is an abstract of an article, "Research in a Changing Society," by William M. Usdane, Ph.D., Chief of the Division of Research Grants and Demonstrations, Vocational Rehabilitation Administration which appeared in the July-August I.965 issue of Rehabilitation Record. Although written with the handicapped adult in mind, it has obvious implications for juvenile amputee research and treatment.)

According to Goodwin Watson in "Psychology of Social Change," innovation requires three kinds of people - originators, who discover and promote a new idea; evaluators, who scientifically test the effectiveness of the new procedure; and disseminators, who make the new knowledge widely available. And with these three types of people, we must have both basic and applied research.

Basic research - an original idea, the testing of a hypothesis, and the development of a body of principles or theories - is necessary before applied research can develop. Moreover, it usually takes five to ten years before a basic research project in human interaction in medicine, or in bioengineering can be brought to the applications phase. However, it has been found at the Vocational Rehabilitation Administration that the number of applications for basic research projects has been limited.

Meanwhile the disabled are becoming impatient with the stereotyped role in which they have been placed. After a decade of research and demonstration programs the stereotype of disabled persons has been actively remodeled by giving them a chance to test their capacities, to upgrade themselves, to achieve, thus refuting our low expectations for them.

Despite these advances, however, more emphasis should be placed on the transition of the disabled person into the home, community and job. He should be given help in coping with the problems that are sure to arise. At present there is a shortage of personnel to handle this follow up - a paucity of placement counsellors, job analysis technicians, job finders, etc. To date research and demonstration projects have tended to stress counselling toward placement rather than placement per se.

Dr. Reuben Margolin, Northeastern University, Boston, Massachusetts, Director of the Rehabilitation Research Institute for that region, wrote in the Rehabilitation Record in 1962.

"The failure of therapeutic programs to prepare the patient for coldblooded reality is sheer folly.... In fact, discharge plans for patients are often set up in such a way that we set the patient up for failure. A tremendous investment reflecting all kinds of cooperative efforts and intense therapeutic skills is made to move the patient along to that point on the continuum of social recovery where he can leave the hospital. Then we rip away the supportive strength ... tell the patient to fend for himself."

We need to move into community adjustment of the disabled through Job placement, job testing, pre-placement and "job ladder" approaches for the physically disabled as well as the emotionally disadvantaged youth and older worker. Research has clearly indicated what can be done when a project director and his staff have high expectations for the disabled. And the disabled are more motivated and less frustrated when their expectations are geared toward life in the community than when they remain in a milieu that has lost its transitional focus.

Regina Crimmins

Hew Process Will Help Amputee To Control Limb With Thought

From the August 16th, 1965, New York Times

By James Feron (Special to The New York Times)

1965 by The New York Times Company. Reprinted by permission.

TEL AVIV, Aug. 15- A team of American doctors and scientists displayed today a development in the field of artificial limbs that enables an amputee to flex an elbow and control arm movement simply by thinking about it.

A film depicting the process was shown for the first time at an international orthopedic seminar. The audience of about 100 experts received it enthusiastically.

Dr. Melvin J. Glimcher of Harvard University, who developed the process with Dr. Thomas DeLorme, chief medical consultant to the Liberty Mutual Research Center, said that the work was still in the experimental stage and its practical use still a few years away.

The artificial limb has not yet been attached physically to the amputee. They are connected through the use of electrodes, however, for purposes of the experiment.

The orthopedic specialists watching the film saw a middle-aged man, an amputee for 26 years who had worn the conventional harness-operated arm, move the new device by "thinking the lifting process," as Dr. Glimcher put it.

Electrodes attached to the man's upper arm were connected to a huge computer that, in turn, translated the signals into the flexing movements and stress control.

Dr. Glimcher, who is professor of orthopedic studies at Harvard Medical School and director of the orthopedic research laboratory at Massachusetts General Hospital, explained that the ability to translate electric signals into mechanical force was not, by itself, a breakthrough. English and Russian scientists have done as much.

However, the English and Russian devices were for forearm amputees and operated on the familiar claw or clamp-like device. In addition, their operation was only an open-and-close one, without refinement for stress. In other words, the same force was applied in grasping an egg as in grasping an iron bar.

A team from Harvard and Massachusetts Institute of Technology, in four years of experiments, sorted out the signals that told the arm when to use the biceps and triceps muscles and when to apply stress.

This was done by studying the signals registered through electrodes of a normal person flexing his arm and lifting weights, feeding them into a computer and studying the results conceptually on a television screen. An arm that would translate this information into action was then built.