A TRIPLET PREGNANCY WITH CRANIOPAGUS TWINS

A TRIPLET PREGNANCY WITH CRANIOPAGUS TWINS

683 Special Articles A TRIPLET PREGNANCY WITH CRANIOPAGUS TWINS A. W. FRANKLIN M.B. Cantab., F.R.C.P. J. S. TOMKINSON M.B. Birm., F.R.C.S., M.R.C.O...

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683

Special Articles A TRIPLET PREGNANCY WITH CRANIOPAGUS TWINS A. W. FRANKLIN M.B. Cantab., F.R.C.P.

J. S. TOMKINSON M.B. Birm., F.R.C.S., M.R.C.O.G.

PÆDIATRICIAN

OBSTETRIC SURGEON

E. ROHAN WILLIAMS Lond., F.R.C.P., F.F.R.

M.D.

RADIOLOGIST

QUEEN CHARLOTTE’S

WE report

some

MATERNITY

HOSPITAL, LONDON, W.6

preliminary details of a

case

of cranio-

pagus twins. Two features believed to be unique are that the mother gave birth to one single normal healthy baby at the same time as the conjoined twins and that one of the conjoined twins has had congenital hypertrophic pyloric stenosis. The last case known to have been born in England was described in The Lancet exactly thirty years ago by Cameron (1928). Craniopagus twins are the rarest of all conjoined twins, estimated at 1 in every 2 to 4 million births by Robertson (1953), who analysed Tartuffi’s figures. Of 117 conjoined twins 86 were joined anteriorly by the thorax (thoracopagus), 22 posteriorly in the sacral area (pygopagus), 7 caudally by the lower abomen (ischiopagus), and only 2 by the skull (craniopagus). The present case is the 42nd to be, recorded in the available literature, but the 6th since 1949, which may reflect no more than the present age’s greater ability to read and write. The usual fate of these babies has been death, sometimes in the first week, nearly always by the end of the first year. One set, the first on record, 2 girls joined by the forehead born in Bierstadt in 1495 (Miinster 1559), lived until the age of ten years, when one died. The dead partner was cut away and the second died shortly after. In Cameron’s case an emergency separation was attempted by Mr. L. Bromley on the twelfth day because one was obviously deteriorating, but both died during the operation. Cameron commented that if the intradural spaces communicate, attempts at separation are useless. Technical progress in neurosurgery, plastic surgery, and anxsthetics has altered the prognosis to some extent.

The first success was reported by Sugar and Grossman in 1953. One baby died one hour after separation, but the other survived and was developing well at eighteen months. Voris et al. (1957) reported successful separation with satisfactory condition of both twins at two years and four months. These three sets resemble in the photographs the set reported here, the main junction being between the parietal bones (c. parietalis). The most recent paper, by Baldwin and Dekaban (1958), concerns craniopagus twins joined mainly at the forehead (c. frontalis), a rarer type in which the actual area of the junction is smaller. The twins were successfully separated in 1956 and both were within the normal range on their first birthday. Separation with survival of both of the twins can be successful only if sufficient tissue is present for two separate individuals. The smaller the area of fusion the better the outlook. The tissues involved are the brain which has been adequate and virtually separate in hitherto reported cases. The covering membranes, especially the dura mater, are usually deficient over some part of the fused area. The arteries are expected to be independent, but the exact state of affairs must be established by angiography. The most probable stumbling-block is large venous communication. Skin to cover the vertex can be prepared without great difficulty. The problem of the replacement of skull bones has not yet been solved. In only two cases so far reported have both of craniopagus

twins survived (Voris et al. 1957, Baldwin and Dekaban 1958); in Sugar’s case (Grossman et al. 1953), where there was a closer fusion, a common sagittal sinus made survival of both impossible, and from the description this case resembles the present one most closely. Case-record Obstetric History The mother attended hospital for her fourth pregnancy after ten weeks’ amenorrhoea. She was a healthy woman with a childhood history of scarlet fever, measles, and mumps; both she and her husband are of European descent. Her first pregnancy had resulted in an abortion. Two years later she was delivered by forceps at the forty-third week of pregnancy of a stillborn female infant weighing 3670 g. (8 lb. 11/2 oz.); postmortem examination showed that the baby was normally formed but had died of a massive aspiration of meconium. After a further two years she was admitted at term for a surgical induction of labour under general anxsthesia. As she was not in labour in forty-eight hours after rupture of the membranes, a lower-segment cxsarean section was performed by J. S. T., delivering a boy weighing 9 lb. 11/2 oz. (4125 g.) in good condition. When first seen in her fourth pregnancy she was a fit woman, aged 36 years, height 5 ft. 5 in. She had slight varicose veins of the right leg and a recent rash on her fingers which she attributed to a wasp sting. At every antenatal attendance the height of the uterine fundus was noted to be greater than the duration of the pregnancy warranted. When Mr. J. D. S. Flew examined her at eighteen to twenty weeks the uterus was the size of a thirty-week gestation. Radiographic studies revealed triplets (fig. 1) and are reported in detail below. At the thirtyfirst week the abdominal girth was 46 in., and at the thirtythird 48 in. Symptoms of pre-eclamptic toxaemia were present for the first time at thirty-five weeks: the blood-pressure was 145/90 mm. Hg and there was a trace of albumin in the urine and slight peripheral oedema, so that the patient was kept on strict bed rest. The next day these abnormal signs had

disappeared. During the morning

of delivery there was a small but definite show of blood per vaginam. The membranes were intact. The patient was well, three foetal hearts were heard, and there were no uterine contractions. It was decided to deliver by csesarean section that day because of the previous history and the known presence of fretal abnormality in a multiple pregnancy. Under general anaesthesia (thiopentone, suxamethonium, gallamine, and cyclopropane) a classical section was performed. The first baby was delivered by the breech at 6.45 P.M.; he cried at once and was in excellent condition. The second amniotic sac was opened and twin boys ininf"CI

at

ttif i-rqninl van1t

Wf"1"f"

Clf"liVf"1"f"CI Rtrmitanponslv

at

Fig. 1-Antenatal radiograph at the 35th week. Three foetuses are present, two conjoined at the crowns of their skulls with good mandibular and facial development and apparently normal trunk development.

684 6.48 P.M. Both cried at once. None of the babies presented any resuscitation problem, but in all mucus was cleared from the air passages. Ergometrine 0-5 mg. was injected intravenously into the mother and the same dose directly into the uterine muscle after the delivery of the babies. The previous lower segment operation scar was palpated and estimated to be 3 mm. thick. The uterine cervix was dilated to the diameter of two fingers’ breadth. Postoperative progress was uneventful, except for pyrexia of 100°F on the first and third days. The placenta (fig. 2) was an intimately fused organ with two amniotic sacs which did not communicate. Antenatal Radiographic Studies The first abdominal radiographic studies at the nineteenth week showed three foetal spines, indicating the probability of triplets. That only two skulls were seen, indicated some foetal anomaly, possibly anencephalus. At the 28th week the radiographs were interpreted as showing one normal foetus and a twin with two separate and a gross cranial fusion. At the 35th week immediately before delivery by csesarean section craniopagus twins were clearly seen with two normal trunks and good mandibular and facial development. The third foetus showed no abnormality (fig. 1).

trunks The

Triplets

The first baby (a male weighing 5 lb. 93 J4 oz., 20 in. long, skull circumference 131/2 in.) has appeared completely normal throughout. He was successfully breastFigs. 3 and 4-Radiographs shortly after delivery, taken in two planes fed and at twelve weeks weighed 13 lb. 12 oz. at right-angles. There is craniopagus with parietal fusion over a wide The craniopagus twins, also males, together weighed area. The long cranial axes of the two heads are approximately at right 9 lb. 12 oz. The vertices have virtually coalesced so that one skull cannot be moved on the other. In radiographs of the conjoined skulls (figs. 3 and 4) no bony They lie in a straight line, their long axes twisted through somewhat more than a right angle. This allows both to lie shelf can be seen between the vertices. Parietal fusion exists on the right or on the back while the face is turned half right. over a wide area. The long cranial axes of the two heads When on the left side the neck is strained and they are not are approximately at right angles to each other. Facial bone comfortable. formation of both appears perfect. General examination reveals no other abnormality. Rectal Discussion temperature readings differ and so do pulse and respiration of Questions aetiology are well discussed in some of rates, which do not synchronise. Simultaneous blood-pressure the we have papers quoted, especially by Robertson (1953), readings show 60 mm. Hg systolic in one and 50 mm. in the add that the presence of pyloric stenosis in We can only other. Both

of the twins exemplifies the principle pointed out by " Aird (1954) that conjoined twins are seldom so precisely group 0, rhesus posiidentical as separate monovular twins are ". The future tive : figures action in this case could not be better planned than in forr Hb, accordance with Aird’s further statement that " however white-cell great the risk of the death of one or both children, it count, and would seem that operation should nearly always be underdifferential a leucocyte taken if the children are known each to have full complement of the organs and tissues necessary for separate count are not life ". identical. ElectroIn Cameron’s case emergency operation failed, in cardiograms Barbosa’s (1949) both died three hours after operation, differ in rate in Robertson’s (1953) operation had to be abandoned. and pattern, The three latest reports of partial or complete surgical both having success are quoted above. In this case great difficulty is a physioFig. 2-The placenta. to be encountered and the final separation will not logical degree likely be attempted before the first birthday. To achieve of right preponderance. Electroencephalograms soon after birth, kindly carried out by Dr. G. Pampiglione, showed the separation with the survival of both twins will require not only great prowess on the part of the surgeons but brains to be separate. The twins are two distinct individuals with independent also sufficient tissues for two independent beings. To behaviour and personality. One may cry or feed while the other achieve one survivor will amply repay the great demands sleeps. One is bigger and stronger, but both are full of charm on the skill, the patience, and the emotions of all conand mentally alert, and they do not appear to upset each other. cerned. Fortunately it was possible to avoid publicity in Clinical progress of the smaller twin has been interrupted by this case for more than three months, giving the parents severe vomiting from eight to twelve weeks. Projectile vomit of some time to recover from the immediate emotional curds with visible gastric peristalsis, and a palpable pyloric shock. tumour, supported the diagnosis of congenital hypertrophic Attempts to satisfy public curiosity about previous pyloric stenosis. Symptoms were relieved by a course of conjoined twins put a strain on parents, doctors, and atropine methonitrate (’ Pylostropin ’). are

blood-

one

685

It is hoped that in this case newspapers and their representatives will exercise restraint and allow all those involved to conduct a hazardous undertaking in calm and peace. The authors (and the parents, with whom it has been discussed) feel that no photograph of the conjoined twins should under any circumstances be published during the lifetime of either twin.

nurses.

REFERENCES

Aird, I. (1954) Brit. med. J. i, 831. Baldwin, M., Dekaban, A. (1958) J. Neurol. Psychiat. 21, 195. Barbosa, A. (1949) Rev. bras. Cir. 18, 1047. Cameron, H. C. (1928) Lancet, i, 284. Grossman, H. J., Sugar, O., Greeley, P. W., Sadove, M. S. (1953) J. Amer. med. Ass. 153, 201. Munster, S. (1559) Cosmographiae Universalis, Lib. 3, 625. Robertson, E. G. (1953) Arch. Neurol. Psychiat. 70, 189. Voris, H. C., Slaughter, W. B., Christian, J. R., Cayia, E. R. (1957) J. Neurosurg. 14, 548.

POST-HOSPITAL ADJUSTMENT OF CHRONIC MENTAL PATIENTS G. W. BROWN B.A. Lond. M.B.

There have been many follow-up studies of former mental patients in general, and of patients with particular diagnoses or who have been exposed to particular forms of treatment (in most of which a stay in hospital of more than two years is claimed to carry a relatively unfavourable prognosis); but we have been able to trace only two studies which dealt specifically with the fate of long-stay patients after discharge from hospital: Smith (1946) reports the outcome of 56 females who had been in hospital at least four years before discharge from St. Hans Hospital, Denmark. 54 of these still had symptoms;

required considerable help from social agencies. During follow-up period (ranging from nine months to three years) 6 of these patients were readmitted and 1 committed suicide. Of the 49 who remained in the community only 6 were

most

the

self-supporting. Stringham (1952) reported the outcome of 33 patients- discharged after an average stay in hospital of twelve years. 15 returned to hospital, but 6 of these were subsequently discharged again. Of the 24 still in the community about two years later only half were self-supporting. Stringham suggested that opposition on the part of the patient’s family was the principal obstacle to their remaining in the community, and their ability to obtain gainful employment was the strongest positive factor

G. M. CARSTAIRS Edin., M.R.C.P.E., D.P.M.

in those who succeeded.

GILLIAN TOPPING

come

M.A. Lond. From the Medical Research Council Social Psychiatry Research Institute of Psychiatry, Maudsley Hospital, London, S.E.5

Unit,

recently, once a patient had stayed for two years continuously in a mental hospital, his chances of discharge were low (Kramer et al. 1955, Carstairs et al. 1955) and would become progressively lower until, by the eleventh year of stay, they would be about 1 in 100 (Registrar General 1955). This situation may now be changing. The Royal Commission on the Law Relating to Mental Illness (1957) stated a new viewpoint: UNTIL

" There is increasing medical emphasis on forms of treatwhich can be given without bringing patients into hospital as in-patients or which make it possible to discharge them from hospital sooner than was usual in the past. It is not now generally considered in the best interests of patients who are fit to live in the general community that they should be in large or remote institutions such as the present mental or mental deficiency hospitals ... No patient should be retained as a hospital in-patient when he has reached the stage at which he could return home if he had a reasonably good home to go to ".

ment

Brill and Patton (1957) and Kramer and Pollack (1958) show that in the United States in 1955-56, the year in which the use of tranquillising drugs became widespread in that country, the number of patients resident in State Mental hospitals declined, in reversal of a long-established trend. Brill and Patton showed that in New York State this could largely be ascribed to an increase in the number of discharges, especially affecting patients who had been more than two years in hospital. Neither Brill and Patton nor Kramer and Pollack (1958) indicated the level at which patients were functioning after their discharge from hospital. Usually a psychotic patient discharged after a prolonged stay in hospital is not completely recovered but: carries at least some residual disability. His remaining inL the community will depend partly on the nature and degree of this psychiatric handicap and partly on the. social environment to which he returns.

The present study was designed to ascertain the outof long-stay patients discharged from mental hospital and to look for factors in their history and in their subsequent social experiences which were associated with their level of adjustment in the community. Method of

Investigation

Selection of Cases The population consisted of all those male patients who were discharged by any means from seven mental hospitals in or near London between July, 1949, and June, 1956, and who fulfilled the following criteria:

(1) Subjects of the United Kingdom by birth. (2) Aged 20-65 on discharge. (3) Had been in hospital two years or more before discharge. (4) Not known to have gone to an address outside the Greater London Area.

Patients who died in the first year of discharge were excluded from the sample. There were 3 such deaths during the period, all of them natural. Patients who were out on trial for more than three months consecutively were included as from the end of the three-month period. Selection of Informants Whenever possible the primary informant was the housekeeper of the domestic group to which the patient went on leaving hospital-typically his mother, wife, or sibling, but in some cases his landlady. Any patient living alone was himself interviewed. Follow-up Interview An interview schedule was prepared covering 160 items, selected on the assumption that they might have a bearing The schedule was usually on the patient’s outcome. completed for each patient after a study of the hospital case-notes and an interview with the primary informant lasting an hour to an hour and a half. Sometimes more than one person was interviewed. The conduct of the interview was not formalised, but interviewers used a check-list to ensure that all the items had been covered. Each of us took part in interviewing, with weekly meetings for discussion of possible ambiguities in the recording of particular items; in this way it was hoped to ensure uniformity in the recording of data from the interview.