local' aneesthetic after sedation with 7 to 10 grains of chloral hydrate. I now feel sure that local aneesthesia is better. The ill child will stand the procedure, ...
Section of Pcediatrics
53
1063
Since coming to Sheffield I have abandoned general anaesthesia in favour of infiltration with 0 5 % local' aneesthetic after sedation with 7 to 10 grains of chloral hydrate. I now feel sure that local aneesthesia is better. The ill child will stand the procedure, pre-operative therapy is needed less often, the post-operative course is smoother, and babies are on full feeds and ready for home on the third day. Dr. J. H. Moseley: I would like to emphasize the value of rectal saline in the post-operative care of these babies. They are usually passing few stools and could be easily re-hydrated or have'their chlorides restored in this way. With breast-fed babies I aim at returning them to breast feeding twenty-four hours after operation.
Dr. J. J.-Kempton: There seems to be much to be said for combined responsibility in treatment. *The condition is one of feeding difficulty-with a mechanical factor-and the general surgeon is unlikely to have any special interest in the problems of baby feeding. In such a system, the paediatrician is responsible for diagnosis and pre-operative preparation. The surgeon, if he accepts the diagnosis and thinks the case suitable, operates; and the return to normal feeding in the shortest possible time is again the responsibility of the paediatrician, every effort being made to get the baby home by the fifth post-operative day. Dr. R. E. Bonham Carter: The pharmacology of'Eumydrin is not completely understood. The pylorus is more^responsive to changes of pH than to any other influence and I suggest that Eumydrin really acts indirectly by altering gastric secretion and hence the acidity of gastric juice. I wonder whether the line of treatment suggested by this idea had been explored. Dr. D. MacCarthy: I have often observed visible peristalsis after Ramstedt's operation has been performed, which seems to indicate that the stomach is still acting in an abnormal way. I have had' no experience of what happens on Eumydrin and I should like to ask the experts if visible peristalsis is abolished.
Dr. Harold Waller: I have many times observed typical gastric peristalsis and projectile vomiting in the first fortnight of life, and their disappearance under treatment with Eumydrin.
Mr. Ralph H. Gardiner (Aylesbury): I would draw attention to accidental perforation of the duodenal mucosa when the hypertrophic muscle is divided at operation. Provided that the perforation is recognized at the time and adequately dealt with, no ill-effects should result. Suture of the delicate mucous membrane is difficult, as the stitches tefid to cut out, and I, personally, use a reinforcing stitch through the seromuscular coats. The stitch, although it appears to reunite the muscle, does not in my experience in any way detract from the success of the operation, and is an added safeguard. With regard to the choice of anxesthetic, I always use a local (0-5% novocain), and I think it better for the surgeon to give this himself, as, by so doing, the necessary time-interval to allow the anesthetic to work is assured, gowns and gloves being changed before towelling up and proceeding to the actual operation. Dr. J. H. Burkinshaw: What happens to the breast feeding when cases on medical treatment are retained in hospital for twenty days or more? The somewhat longer period of hospital stay in the Eumydrin cases seems on this account to be a point strongly against medical treatment. Dr. Harold Waller: If the method of expressing the breast milk is correctly taught the supply can be maintained long enough in such cases.
[May 25, 1951]
MEETING
WESTMINSTER CHILDREN'S HOSPITAL, VINCENT SQUARE, S.W. 1 Laurence-Moon-Biedl Syndrome.-J. G. MILLICHAP, M.D., M.R.C.P. (for CHARLES F. HARRIS, AT THE
M.D., F.R.C.P.). S. N., aged 4 years. British. Birth weight 7 lb. 11 oz. Obesity dating from 5 months of age. Night blindness. Spasmodic bronchitis. Family history.-Father and paternal grandfather-diabetes mellitus. Sister died at birth with polydactyly and other abnormalities. Brother aged 11 years is normal. Cousin (child of father's twin brother) mentally defective and has double athetosis. Maternal cousins all obese. Paternal grandparents related.
1064
Proceedings of the Royal Society of Medicine
54
Examination.-Obesity of uniform distribution (Fig. 1). Weight 72 lb. (normal 35 lb.). Height 43 inches (normal for age 38 inches). Short hands and feet. Extra digit on each hand. Syndactyly of second and third toes of each foot. Genu valgum and pes planus. Small penis. Descended testes. Urine normal. Optic fundi (Mr. A. Seymour Philps).-The discs are pale and show congenital defects. In addition there is an absence of choroidal pigment, and the.retinal arteries are very fine. The typical appearance of retinal pigment agglomeration is not present. Investigations.-X-ray of skull, pituitary fossa, and bone age are normal. Serum W.R. and Kahn negative. Glucose tolerance curve normal. No glycosuria. Urinary excretion of 17-ketosteroids 0 45 mg. per day, 20ketosteroids 1I0 mg. per day. Fluid intake 48 fl. oz., urinary output 23 fl. oz., in twenty-four hours. Rhesus grouping (Dr. W. Brewer): Patient, OR; Mother, OR; Father, Or. Psychologist's report. Unco-operative and in the backward group. I.Q. difficult to assess (Miss M. Holt).
Comment.-The earliest reference to this condition appears in the 2nd book of Samuel, Chap. 21, Verse 20, in which there is mention of "a man of great stature, that had on every hand six fingers, and on every foot six toes, four and twenty in number". Although the Hebrew word describing the stature is generally accepted to signify tall, the Aramaic translation uses a word mashach, which means oily or FIG. 1.-Showing uniform distribution of fat. fat. Pliny, in his Natural History, also refers to a baby with six fingers and six toes. The cardinal signs of the syndrome, in order of their frequency, are obesity, retinitis pigmentosa, mental deficiency, genital dsytrophy, familial incidence and polydactyly. In discussing the retinal appearances Laurence and Moon (1866) admit that by describing them as retinitis pigmentosa they have been guided rather by usage than by the intimate nature of the cases. In only 15 % of cases (Clay, 1933) do the retinal changes conform with the classical picture of retinitis pigmentosa and these appear in the later stages of the condition. Lyle (1946) describes the early changes as non-pigmented retinal degeneration with loss of central vision. The peripheral pigmentation of the fundi in this case is minimal, but there is optic atrophy with fine retinal vessels and absence of choroidal pigment. The patient is of particular interest in that his sister, who died a few minutes after birth,.was the subject of a communication by Dr. F. Parkes Weber and Dr. M. Scholtz (1939 and 1949). I wish to thank the authors for verifying this relationship and also for supplying me with a detailed account of the case. The bizarre combination of abnormalities in this infant included a congenital vesico-vaginal fistula with vaginal atresia, brachycephaly, polydactyly, hydrops foetalis and erythroblastosis. Extreme himopoiesis in an enlarged liver of a non-syphilitic infant, and examination of the blood appeared to justify the diagnosis of erythroblastosis. At this time the rhesus factor was, of course, unknown. The occurrence of a rhesus or A.B. incompatibility as a possible itiological factor in the present case has been investigated and excluded; indeed, owing to the mother being rhesus positive and both parents Group 0, it would appear most unlikely if any infant born to them could have erythroblastosis foetalis. I have discussed these findings with Dr. Parkes Weber. He agreed that, in the light of present evidence, the infant who died at birth was an example of the Laurence-Moon-Biedl syndrome, in which the occurrence of stillbirth or early death is by no means uncommon. REFERENCES CLAY, G. E. (1933) Trans. Amer. ophthal. Soc., 31, 274. LAURENCE, J. Z., and MOON, R. C. (1866) Ophthal. Rev., 2, 32. LYLE, D. J. (1946) Amer. J. Ophthal., 29, 939. PLnnr. Secundus (Caius). Historik naturalis. Lib. Xl, 43. WEBER, F. P., and SCHOLTZ, M. (1939) Brit. J. Child. Dis., 36, 131. ~~ (1949) Further rare diseases. London, p. 162. ,
The following cases were also shown: Congenital Heart Disease-Central Cyanosis, Dextrocardia, Pulmonary Stenosis, Septal Defect. Dr. R. V. GIBSON (for Dr. C. J. GAVEY). (1) Complete Transposition of Viscera with Dextrorotation of the Heart and Transposition of Great Vessels. (2) Tricuspid Atresia.-Dr. M. W. ARTHURTON (for Dr. I. M. ANDERSON). Lead Poisoning.-Dr. G. D. REES (for Dr. CHARLES HARRIS). Case of Neuroblastoma and Case of Acute Leukemia. Comparison of Each.-Dr. K. LLEWELLIN (for Dr. CHARLES HARRIS). (1) Infected Congenital Cyst of the Right Lung. (2) Infected Congenital Cyst of the Left Lung. (3) Paratracheal Cyst. (4) Parabronchial Cyst.-Mr. D. H. WOODHEAD (for Mr. C. PRICE THOMAS). Fibrosarcoma.-Mr. T. M. PROSSOR (for Sir STANFORD CADE). Lymphosarcoma.-Mr. T. M. PROSSOR. (1) Simple Palate Cleft (Group II). (2) Unilateral Cleft of Lip and Palate (Group III, i). (3) Bilateral Cleft of Lip and Palate (Group m, iii). Demonstration of Speech Results.-Mr. J. P. REIDY. (1) Urinary Incontinence Treated by Nerve Anastomosis. (2) Osteoporosis.-Dr. G. BUNTON (for Mr. DAVID LEVI).
55
Section of Puediatrics
1065
A Specimen of a Rhabdomyosarcoma of Uterus in a Child.-Mr. RONALD W. RAVEN. (1) Three Cases of Hydrocephalus. (2) Two Cases of Myelomeningocele. (3) Thoracic Meningocele. (4) Lumbar Meningocele. (5) Hydrocephalus, Myelomeningocele.-Mr. GEORGE MACNAB. Demonstration: A New Technique in the Diagnosis of Pertussis.-Dr. ANDREW BOGDAN. The technique of supra-laryngeal swabs in the diagnosis of pertussis entails the patient coughing on to a swab held just above the opening of the larynx. This procedure simplifies effective repetition as no resentment is caused, and adds to diagnostic accuracy especially with swabs cultured on selective medium (Lacey, B. W. (1951) J. gen. Microbiol., 5, vi). [June 23, 1951]
MEETING AT FARNBOROUGH HOSPITAL, FARNBOROUGH, KEN r Preliminary Report on a Study of the Social Background of Healthy Children.-JOHN LANDON, M.R.C.S., D.P.H. THE Kent Pediatric Society are conducting an investigation into the Incidence and Causation of Health in 10-11-year-old school children (1,100) in the Borough of Bexley in conjunction with the fnstitute of Social Medicine, Oxford. Health is taken to mean soundness in body, mind and personality. We had (a) to find the healthy children and (b) to evolve a method of studying Causation. The first involved investigation into physical, mental and personality qualities. Physical status.-A medical examination with X-ray was given and a percentage mark allocated based on a specially devised marking form. Children scoring less than 90% were excluded from category of "Health". Mental status.-All children given intelligence test, those with I.Q. below 95 were excluded as above. Personality status.-The Rorschach Ink Blot Test was adopted-group technique being used. Clinical examination by psychiatrist used to confirm conclusions whenever necessary. Failure in personality test excluded from category of "Health". Up-grading.-To find the healthy children up-grading marks to a maximum of 25 were awarded for each of the following: Physical status.-Endurance tests by hanging bar and dynamometer applied and marks awarded in accordance with "pulling-up time" on basis of distribution curve for the group. Mental status.-On basis of I.Q. level. Personality status.-Method of scoring devised on basis of seven positive factors of personality health and seven factors of mental pathology. The "honours" children were those with the highest marks out of a possible 175. A pass group and failed group were also ascertained. In regard to Causation, this was approached through application of a questionnaire including environmental, economic, social, genetic and cultural factors and applied by doctors at personal interviews with both parents and child in all three groups (200). The results transcribed for treatment by Power Samas technique at Institute of Social Medicine, Oxford. A number of collateral investigations are also being pursued. A report will be issued in due course.
Practical Problems in the Management of Erythroblastosis.-RUFUS C. THOMAS, F.R.C.S.Ed., F.R.C.O.G., and D. G. LEYS, B.A., D.M., F.R.C.P. Mr. Rufus C. Thomas described the arrangements at Farnborough Hospital for dealing with erythroblastosis. The hospital receives cases from other large maternity units in the area and expects to deal with about 30 cases of erythroblastosis a year. The policy as regards induction and treatment of the cases is settled by consultation between Obstetric and Pldiatric staffs and the Pathologist, each case being considered on its merits from the thirtieth week. In general the policy has been to avoid induction for the first affected child unless a steep rise in the albumin antibody, and especially a subsequent fall, seems to indicate a severely affected case. Although the time interval between induction and delivery was sometimes as much as a week; he did not think that casarean section was justifiable. He agreed with P. L. Mollison's view that sensitization to the Rhesus factor was not proper grounds for sterilization. Dr. D. G. Leys said that present policy in the Unit was exchange transfusion for every case shown to be Coombs positive, if the cord heemoglobin was less than 100 %. He drew attention to two deaths experienced by the Unit from heemorrhage, both following exchange transfusion, and suggested that since banked blood had been used, the deaths were probably due to prothrombin deficiency. Twitching of face or limbs at the time of birth, or during an exchange transfusion, was not necessarily an indication of kernicterus.
