234 JULY 28, 1962 HYDATID DISEASE MEDICLJORNAL

The methods of treatment are discussed and particularreference is made to hydatid disease of the liver. Theadvantages of using black packs at operation areemphasized. It is suspected that the use of solutionsof formalin in concentrations greater than 4% is a causeof delayed healing post-operatively.The mode of spread of the disease among the Bedouins

in relation to their environment is discussed.Kuwait has not previously been recorded as a hydatid

area, and the reasons for the disease being found herenow are purely economic. The influx of previouslyinfected persons into the State is mainly due to thecompletely free health service and to the paucity ofmedical services in the surrounding Arab States. It isalso due to the prosperity in Kuwait, which attractsimmigrants as work is easily obtainable.

It is emphasized that the incidence figures give a falsepicture because the population served by the KuwaitHealth Service is far greater than the population of theState.The method of prevention of hydatid disease in

animals and man is discussed in detail.

We thank all the doctors who were of assistance to usin the examination of the records; Major J. M. Adam,R.A.M.C., for his constructive criticism; and the ChiefMedical Officer, Mr. E. Parry, for permission to publishthe article.

REFERENCESEl Gazzar, A. (1961). Arab Medical Congress, Cairo.W.H.O./F.A.O. (1959). Joint Expert Committee on Zoonoses.

2nd Report. F.A.O. Agricultural Study No. 47.

"In more recent years the great advance [in methods oftreating leprosy] has in many countries begun to win theconfidence of the people. Where formerly the diseaseremained hidden in fear and prejudice, now more and moresufferers in need of treatment come forward to seek forthemselves the benefits which new drugs and new ortho-paedic surgery are said to confer. The matter is indeedblazed abroad, and patients flock in 'from every quarter,'sometimes to the embarrassment of medical workers, whoseresources are often strained to the limit. The record of theMission to Lepers' work in 1961 offers an excellent exampleof this notable increase, the way it tends to develop, andthe effort that is made to meet it. On January 30 therewas opened a new out-patients' treatment centre at a placecalled Barabanki, about sixty miles from the FaizabadLeprosy Hospital in Uttar Pradesh, North India. This is adevelopment of the work of the Faizabad Home, which wasopened in 1938 by H.E. Sir Maurice G. Hallett, at that timeGovernor of the United Provinces and now the HonoraryTreasurer of the Mission. The scheme was launched byMr. A. Donald Miller, then Secretary for India, in responseto an appeal from local authorities whose main object atthat time was to get off the streets the 'leper beggars ' whoinfested Ajodhya, a famous centre for Hindu pilgrims andthe reputed birthplace of Ram. It was not thought thatthere was much leprosy in the Faizabad area. 'Get rid ofthese beggars, who come from other parts of India, and ourproblem is solved,' they said. But when Dr. P. J. Chandybegan to develop his medical work the news spread through-out the whole area and soon patients were coming frommany villages all over the Faizabad civil district, and fromfar beyond its boundaries. Year by year this process wenton, until it became clear that Faizabad itself could no longercope with the greatly increased flood of out-patients. Sothe Barabanki treatment centre was built with help fromlocal sources, from the Central Government and theGovernment of Uttar Pradesh, and from the Mission toLepers." (From Every Quarter, 1961, Mission to Lepers.)

7- HYDATID DISEASE IN THEPOSTERIOR FOSSA

BY

J. D. CARROLL, M.D.Senior Registrar, Department of Neurology, King's College

Hospital, London; formerly Resident Medical Officer,National Hospital, Queen Square, London

ANDR. G. LASCELLES, M.B., M.R.C.P., D.P.M.

Registrar, Departments of Neurology and PsychiologicalMedicine, St. Thomas's Hospital, London; formerly

Assistant House-physician, National Hospital,Queen Square, London

[WITH SPECIAL PLATE]

Hydatid disease affecting the brain is uncommon in thiscountry and its presence in the posterior fossa is acomparative rarity. We are therefore reporting twocases from Malta and Cyprus which were admittedrecently to the National Hospital. The diagnosis wasestablished at operation and confirmed by histologicalstudy.One of the first successful posterior fossa craniotomies

was performed in 1889 by Maunsell, of Dunedin, whenhe operated on a subtentorial cyst, at that time thoughtto be a hydatid, in an 18-year-old youth. Since thenmany accounts of hydatid disease have appeared in theliterature, the largest series being those of Ddve (1913)and Barnett (1943). In the series reported by Deve(2,727 cases) the incidence of cerebral involvement was1.4% and in that of Barnett (1,617 cases) it was as lowat 0.5%. This low incidence of involvement of thecentral nervous system can be explained by the fact thatthe larvae have difficulty in passing through the capillaryfilters of the liver and lung. However, in New Zealandit is said that 1 % of all space-occupying intracraniallesions are hydatid cysts, and the incidence may evenbe higher if only those under the age of 21 are con-sidered (Robinson, 1960). In view of this possibilitycerebral hydatid disease is always seriously entertainedif a child presents with symptoms suggestive of such aspace-occupying lesion.

In the brain the commonest sites for hydatid diseasewould appear to be the parieto-occipital and temporalregions (Valentino, 1959). It usually occurs in the formof a single cyst about 5 cm. or more in diameter,multiple cysts being extremely rare. The response ofthe surrounding brain to the presence of the cyst isminimal, and although there is some gliosis a perfectplane of cleavage remains between the cyst and thebrain.

Case 1The patient, a boy of 6, was referred by Professor Zammit

Maempel, of St. Luke's Hospital, Malta. He had been ingood health until March, 1959, when he developed aninfluenza-like illness consisting of a high temperature.malaise, unproductive cough, and a watery discharge fromthe nose. The acute symptoms lasted a week, and duringthe following week there was a complete recovery. OnApril 9 he began to vomit, and this continued for a month,occurring every second or third day and usually on wakingin the mornings. The vomiting was associated with severeretro-orbital headaches, photophobia, and irritability. Inthe early part of May he began to experience obscurationsof vision lasting seconds at a time and not associated withchange of position. The visual disturbances continued fora month. On his admission to hospital in July all his

on 6 August 2020 by guest. P

rotected by copyright.http://w

ww

.bmj.com

/B

r Med J: first published as 10.1136/bm

j.2.5299.234 on 28 July 1962. Dow

nloaded from

JULY 28, 1962 BRITISHMEDICAL JOURNAL

J. D. CARROLL AND R. G. LASCELLES: HYDATID DISEASE IN THE POSTERIOR FOSSA

FIG. 1.-Case 1. Ventriculogram showing dilatation of the FIG. 2.-Case 2. Ventriculogram showing moderate symmetri-lateral and third ventricles without displacement of these struc- cal hydrocephalus involving the lateral and third ventricles.tures. The aqueduct is kinked anteriorly and, with the fourth The fourth ventricle is incompletely filled, but some air hasventricle, is displaced anteriorly and to the right of the midline. passed into the cisterna magna, which is fairly capacious.

S. M. TUCKER AND D. E. SIBSON: FOETAL COMPLICATION OF VACCINATIONIN PREGNANCY

Photograph showing, on larger twin, discrete vaccinial lesions on neck and confluent lesions on body; on smaller twin,a large lesion around the eye.

E..:....

on 6 August 2020 by guest. P

rotected by copyright.http://w

ww

.bmj.com

/B

r Med J: first published as 10.1136/bm

j.2.5299.234 on 28 July 1962. Dow

nloaded from

JUJLY 28. 1962

symptoms had completely subsided and in fact he had no

spontaneous complaints. In his previous medical historyhe had experienced seven convulsive episodes, the last attackoccurring seven months prior to his admission. There was

no history of epilepsy on either side of his family.

On examination his temperature was 98.4° F. (36.9° C.)and pulse 72. He was a well-nourished but nervous child.He co-operated well in the examination and there was no

evidence of any intellectual impairment. The fundi showedbilateral papilloedema of moderate severity and chronic intype without haemorrhages or exudates. The visual acuitywas normal and the visual fields were full to confrontation.The cranial nerves were normal. Tone, power, andco-ordination were normal in the limbs. The reflexes were

normally brisk and equal and both plantar responses were

flexor. Sensation was normal. His gait was steady and hecould walk heel to toe along a straight line. Generalexamination was normal. His blood-pressure was 110/70.X-ray films of the skull were normal. Chest x-ray

examination showed no evidence of pulmonary disease. AnE.E.G. was carried out and was reported by Dr. Cobb inretrospect as follows: "A single E.E.G. was recorded on

July 3, 1959. The child was very apprehensive and, probablyfor this reason. there are long stretches of the record wherealpha activity is absent. When present it is of good ampli-tude and fair regularity at 9 cycles per second, though mixedwith many 3-5 c/s waves, which block with it when theeyes are open. Both these components are of rather greateramplitude on the left side. There is some general slowactivity, not excessive for this age, and generalized low-voltage fast rhythm. On a number of occasions briefepisodes occur of high voltage, generalized, rhythmic 3-4 c/swaves, associated initially with spikes. The general recor.'is only slightly abnormal a normal E.E.G. is to be expectedwith benign intracranial hypertension-and the interest liesin the significance of the episodes of slow waves with spikes.If the boy's attacks antedated the appearance of the cystthen it may be suipposed that the episodes are related solelyto the epilepsy, but it is worth noting that, with a very fewposterior fossa tumours, episodes of this kind occur,

disappearing on removal of the tumour" (see Fig.).

eyes closed IOOMUVlsecond

Electroencephalogram of Case 1.

The Casoni intradermal test was negative. The E.S.R.

was 10 mm. in the first hour. The blood count showed a

haemoglobin of 85%, and the total white count was 22,200/

c.mm., of which 6,216 (28%) were eosinophils.

It was felt clinically that the boy was suffering from

benign intracranial hypertension or that he had a posterior

fossa neoplasm. He was referred to Mr. Wylie McKissock

with a view to ventriculography and subsequent exploration

of the posterior fossa if a neoplasm was suspected. The

ventriculogram had the appearance of a left cerebellar space-

occupying lesion (Special Plate, Fig. 1).

Operation.-At operation, on July 16, a thin-walledbluish cyst was encountered, extradural in position andD

DISEASE BR1TISH 235MEDICAL JOURNAL

extending over the posterior and lateral aspects of the leftcerebellar hemisphere, depressing the dura to a matter of1.5-2 cm. The cyst was unavoidably ruptured and it wasfound to contain a large quantity of absolutely clear fluid.The wall of the cyst was gelatinous and firm, and could bereadily removed in several large pieces. Expansion of thedura began to take place at once and the operation wascompleted without incident. He made a satisfactory post-operative recovery.Histology.-The material submitted for histological study

consisted of a cyst wall. In some regions three layers couldbe distinguished- a thin germinal layer of small cuboidcells, a thick laminated layer, and a thin layer of compressedbrain tissue containing glial cells, small round cells, andsmall blood-vessels. Scolices with hooklets and suckerswere found in the contents. The findings were those of ahydatid cyst.

Post-operative Course.-The patient was symptom-freeafter the operation, and over the course of the followingweeks the papilloedema began to subside in each eye. Hewas discharged from hospital three weeks after his operationand returned home to Malta. He was reported to havemaintained his progress and was free of symptoms; alsothat examination of his optic disk showed some pallor butno papilloedema, haemorrhages, or exudates. A recentblood count showed an eosinophilia of 7%.

Case 2A woman aged 29 was referred by Dr. V. V. Kalbian,

of the General Hospital, Nicosia, Cyprus. She had beenin good health until two years before admission to hospital,when she began to complain of headaches. They occurredprincipally on waking in the mornings and were occipitalin position. For some months they had increased in severityand had been accompanied by nausea and occasional attacksof vomiting. In the previous year she had complained offrequent short-lived attacks of vertigo which were accom-panied by a high-pitched tinnitus in both ears. For abouttwo months she had become aware of double vision onlooking to the left and also had experienced a progressiveloss of sensation over the right side of her face. She alsocomplained of a nagging pain in the upper right quadrantof her abdomen which had become more persistent in thepreceding few days.On examination her temperature was 98.6° F. (37' C.)

and pulse 82. She was alert and co-operative and there wasno evidence of any intellectual impairment. The fundishowed papilloedema of moderate degree and chronic intype without haemorrhages or exudates. The visual acuitywas normal and the visual fields were full to confrontation.She had nystagmus, which was coarse in character on lookingto the right and fine on looking to the left. There wasimpairment of all modalities of sensation over the distribu-tion of the right trigeminal nerve, and the corneal reflexon that side was grossly diminished. There was a rightfacial weakness of lower-motor-neurone type. Tone, power,and co-ordination were normal in the limbs. The reflexeswere normally brisk and symme- ical and both plantarresponses were flexor. Sensation was normal. Her gaitwas steady and she coul(d walk heel to toe along a straightline. General examination was normal except that the liverwasctilarged two fingerbreadths and was tender on palpation.The blood-pressure was 105/70.

X-ray examination of the skull revealed some thinningof the dorsum sellae suggestive of increased intracranialpressure. The chest x-ray picture gave no evidence ofpulmonary disease. A straight x-ray film of the abdomenshowed no abnormality. The E.E.G. showed minimalgeneralized and non-specific abnormalities consisting ofirregularity of the alpha rhythm with a little diffuse fastactivity. The urine was normal, haemoglobin 900%, andthe white cells 5,800/c.mm., of which 420 (7%) were

eosinophils. The E.S.R. was 26 mm. in the first hour.The stools were negative for parasites, cysts, and ova. The

HIYDATID on 6 A

ugust 2020 by guest. Protected by copyright.

http://ww

w.bm

j.com/

Br M

ed J: first published as 10.1136/bmj.2.5299.234 on 28 July 1962. D

ownloaded from

BRITISH236 JULY 28, 1962 HYDATID DISEASE MEDICAL JOURNAL

blood Wassermann reaction was also negative. Liver-function tests were normal. The Casoni intradermal testgave a weak and delayed positive response.

It was felt clinically that the patient had a right lateralrecess space-occupying lesion, and she was referred to Mr.Wylie McKissock with a view to ventriculography and sub-sequent exploration of the posterior fossa. The ventriculo-gram, which entailed the introduction of myodil for moreaccurate assessment, gave the appearance of a space-occupying lesion in the medial part of the cerebellopontine-angle cistern (Special Plate, Fig. 2).

Operationi.-The cerebellar tonsils were widely separatedand in the space between them lay some whitish-walledamorphous-looking material associated with a series of smallclear-walled cysts containing clear fluid. The amorphousmaterial was picked out with a number of cysts adherentto it. There was a considerable amount of thickening ofthe arachnoid over the cisterna magna. No further operativetreatment was carried out in her case. She made a satis-factory post-operative recovery.Histology.-The tissue submitted for histological study

consisted of a papilliferous membrane, part of which hadundergone coagulative necrosis. The viable membrane wascovered with high columnar cells with a deeper eosinophiliccytoplasm and a basal nucleus. External to this was aloose meshwork of round cells. Scolices and daughter cystswere not seen, but the appearances were highly suggestiveof a hydatid cyst.

Post-operative Course.-The patient improved symptoma-tically and the papilloedema began to subside in each eye.The facial weakness improved, but there was no changein the facial anaesthesia. Two weeks after the operationa cystic swelling developed over the operative site. Lumbarpuncture was performed and examination of the fluid showedthe presence of 10 lymphocytes, 160 mg. of protein per100 ml., and a Lange 5555544333. Culture of the fluidgave no growth. The swelling persisted and was reducedin size temporarily by lumbar puncture, indicating that itwas a collection of cerebrospinal fluid which developed asa result of impaired absorption. The blood count wasnormal. The E.S.R. was 55 mm. in the first hour. TheCasoni intradermal test gave an immediate positive response.

She was sent home to Cyprus five weeks after the opera-tion and up to the time of writing there had been littlechange in her condition as compared with her immediatepost-operative state. She required frequent aspiration ofthe swelling in the back of her neck; this brought temporaryrelief of her headache, dizziness, and vomiting. She wasunable to walk on her own because of the severity of herataxia.

DiscussionAn accurate pre-operative diagnosis is of great

importance in cerebral hydatid disease, as surgerywithout prior knowledge can lead to cyst rupture andwide dissemination of the daughter cysts. The diagnosisis by no means easy, and of the available tests theCasoni intradermal and hydatid complement-fixationare disappointing in cerebral cases (Robinson, 1960).In both our patients an eosinophilia was found in theblood, and it is interesting to note that some authoritiesclaim it is the only test of value in these cases. Thepresence of a hydatid cyst elsewhere in the body doesnot necessarily prove that the lesion in the centralnervous system is of the same nature (Schroeder, 1947).Ventriculography is often undertaken as a means oflocating the site of the lesion more precisely. This canbe a dangerous procedure on account of accidentalrupture of the cyst ; consequently, if this diagnosis isconsidered, then the ventricle on the opposite side tothe suspected site of the lesion should be injectedwith air.

We consider that the diagnosis of cerebral hydatiddisease should be entertained in any patient who comesfrom an area where the disease is prevalent and whopresents with symptoms of increased intracranialpressure together with focal signs pointing to a lesionin the posterior part of either hemisphere or in theposterior fossa.

SummaryTwo cases of hydatid disease occurring in the posterior

fossa, a comparatively rare location, are presented.Both patients came from abroad, the diagnosis in

each case being established at operation and confirmedby histological study.The importance of an accurate pre-operative diagnosis

is stressed, and in this connexion the finding of aneosinophilia in the blood is thought to be of greatersignificance than both the Casoni intradermal and thehydatid complement-fixation tests.The danger of accidental rupture of the cyst on

ventriculography is noted.In conclusion, the diagnosis of cerebral hydatid disease

should be entertained in any patient who comes froman area where this disease is prevalent and who presentswith signs of raised intracranial pressure.

Our grateful thanks are due to Dr. J. St. C. Elkington andDr. John Marshall for allowing us to use these patientsfor presentation, and to Mr. Wylie McKissock for permissionto use the surgical notes. We also thank Mr. Prickett, ofthe photographic department, for the photographs of thex-ray films, and the Medical Committee of the NationalHospital, Queen Square, for permission to publish this paper.

REFERENCESBarnett, L. E. (1943). Aust. N.Z. J. Surg., 12, 240.Devd, F. (1913). Quoted by Barnett (1943).Maunsell, H. W. (1889). N.Z. med. J., 2, 151.Robinson, R. G. (1960). Ann. roy. Coll. Surg. Engl., 26, 145.Schroeder, A. (1947). Arch. int. Hidatid., 7, 195.Valentino, V. (1959). Radiology, 73, 250.

THALIDOMIDE DISASTERCONSIDERED AS AN EXPERIMENT IN

MAMMALIAN TERATOLOGYBY

D. H. M. WOOLLAM, M.A., M.D., M.R.C.P.Departrnent of Anatomy, Cainbridge

During the past ten years experimental work onlaboratory animals has revealed that a large number ofchemical substances have the power when administeredto the pregnant female of producing congenitalmalformations in the young. The unfortunateexperience with thalidomide parallels these observationsin all but two respects-in this case man is theexperimental animal, and the choice of animal was madenot deliberately but accidentally. From the numerousexperiments which have been carried out in thelaboratory there have emerged certain general principlesof mammalian teratogenesis. These principles, whichare summarized below, are highly relevant not only tothe understanding of the mode of action of thalidomidebut also in suggesting how similar tragedies are to beavoided in the future.

Significance of Timing and of DosageThe most important single factor that has to be

considered by the experimental teratologist is when the

on 6 August 2020 by guest. P

rotected by copyright.http://w

ww

.bmj.com

/B

r Med J: first published as 10.1136/bm

j.2.5299.234 on 28 July 1962. Dow

nloaded from