Kennedy, Roger L.J.

PERIODICALLY, it appears to be appropriate and perhaps even necessary to pause for a moment to consider the question, "Where are we going and by what means are we moving along our course?" In the case of the Academy, the answers are to be found in a consideration of the present structure and activities of the components of the group. A very brief, even categorical review of the committees, liaison representatives and official subdivisions will tell us much, and will serve to remind us that we are participants in a great vital program dedicated to the health and welfare of children. Let us first consider the aims and activities of the various committees of the Academy. In the past few years, an increasing awareness of loss of child life through accidents has prompted the Committee on Accident Prevention to survey the causes and to initiate a program of prevention which has received nationwide attention. Unpleasant though it may be to contemplate the prospect of a world engaged in another holocaust of war, the need for preparedness has been recognized at all levels of our civil structure, and in consonance with the emphasis on national alertness, the Academy has established a Committee whose purpose it is to compile information as to the possible needs of children in wartime and the role that pediatricians can play in satisfying those needs, if the occasion demands it. During the lives of most of us, contagious and infectious diseases have become increasingly less common and less severe. In order that the most effective means of controlling, as well as treating, these diseases may be available to all, the Committee on Control of Infectious Diseases has issued and, at intervals, has revised a manual that contains the latest and most reliable information that can be supplied by a panel of experts in the field.

IN 1912 Crouzon presented his classic paper to the Medical Society of the Hospitals of Paris. It was a report on two patients, a 29-year-old mother and her 3-year-old son, with strange malformations of the face and head to which he gave the name "hereditary craniofacial dysostosis." In general Crouzon listed the following factors as characteristic of the disease: 1) cranial malformation that is a combination both of a skull in the shape of a boat (scaphocephaly) and in the shape of a wedge (trigonocephaly); 2) malformation of the face resulting in an arched nose resembling a parrot's beak and marked protrusion of the mandible beyond the maxilla (prognathism); 3) bilateral exophthalmos with external strabismus; and finally 4) a hereditary and familial occurrence of these malformations. In addition to these four main characteristics of the syndrome, the child described in Crouzon's paper also had suffered from epilepsy and urinary incontinence. In discussing the possible etiology of this disorder, the author favored the hypothesis that it represented a hereditary malformation of embryonic origin. Since this original report, Crouzon and others have had the opportunity to see and report on additional cases of this strange malady. Atkinson reviewed the literature in 1937 and found that, to that time, 86 certain cases of this condition had been reported. In 28 of the 86 cases occurrence of the disorder in other members of the afflicted member's family was not mentioned. Vogt described this condition associated with syndactylism, and in 1942 Arce and Arce reviewed the roentgenologic findings in eight cases of pathologic synostosis of the vault, including Crouzon's disease. Parks and Costenbader presented the cases of three patients having Crouzon's disease, one of whom had undergone operation, and Pinkerton and Pinkerton reported four cases of craniofacial dysostosis in a family of seven. Three members of the family reported by the Pinkertons, including the father, were normal. Four others in this family, including the mother, had Crouzon's disease. One of those affected was a 3½-year-old girl with marked visual impairment and ophthalmoscopic evidence of chronic increased intracranial pressure. Cranial surgery was recommended in this instance but was refused.

Presacral teratomas are seen most frequently during infancy and childhood. Eighteen children with presacral teratomas were seen at the Mayo Clinic between 1907 and 1953, inclusive. Seventeen were girls. The clinical and pathologic findings are presented. A mass in the presacral area usually is the only symptom as well as the only abnormal physical finding. Such a mass was seen at birth in 14 of the 18 patients. Occasionally symptoms or signs, or both, of obstruction of the urinary tract and/or the gastrointestinal tract constitute the presenting complain. Roentgenograms aid in diagnosis; other laboratory findings contribute little. Pathologically, the size of the tumor may vary greatly. It may contain almost any type of tissue. There was no orderly arrangement of the tissue in any case in our series. In 4 of the 18 cases malignant changes were found microscopically. This is in close agreement with the recorded incidence of malignant changes in presacral teratomas. It is interesting that in one case 2 types of tissue were involved in malignant metamorphosis. Adenocarcinoma is the commonest type of malignancy found, and this is usually papillary. Metastasis frequently occurs via the blood stream to the lungs. The lymph nodes likewise may be the seat of metastatic lesions. Surgical removal should be undertaken regardless of the size of the tumor on the age of the patient.