1401MR. S. STEPHENSON: JUVENILE TABES DORSALIS ; NOTES OF FIVE CASES.

JUVENILE TABES DORSALIS: NOTES OFFIVE CASES.

BY SYDNEY STEPHENSON, M.B., C.M. EDIN.,F.R.C.S. EDIN.,

OPHTHALMIC SURGEON TO THE EVELINA HOSPITAL, THE QUEEN’SHOSPITAL FOR CHILDREN, HACKNEY ROAD, LONDON, ETC.

A REMARKABLE communication on juvenile tabes dorsalis by Dr. A. Cantonnet, of the Hôtel Dieu, Paris,1 has onceagain brought this rare and interesting disease to the front.Dr. Cantonnet, after describing a case of his own in a ladaged 15 years, analysed 89 cases of juvenile tabes dorsaliswhich he had collected from the literature, and reachedsome important conclusions with regard to the malady. Forexample, in 57’ 1 per cent. of the cases there was certainevidence of inherited, and in 8’ 5 per cent. of syphilisacquired during the first years of life ; total, 65 ’ 6 per cent.Moreover, in 13’ 90 per cent. of the cases personal stigmatawere found in choroido-retinitis or in interstitial keratitis.The part played by syphilis in the production of the malady,therefore, is as considerable as in adult tabes dorsalis.Direct heredity was not common. In 15 ’ 30 per cent. of thecases analysed by Cantonnet one of the parents was tabetic,and in 12 per cent. one was affected with general paralysis.Juvenile tabes dorsalis, which, on the average, commences at15 years of age, affects females in 62 ’ 6 per cent. as againstmales in 37 ’ 3 per cent., a striking and as yet unexplainedcontrast to the sex-incidence in later life, which is given byGowers 2as "about ten to one." The ocular symptoms ofjuvenile tabes dorsalis include inequality of the pupils, theArgyll Robertson phenomenon (simple or complicated), opticatrophy, paralysis of the extrinsic muscles of the eye, latentocular ataxy, and so forth. According to Cantonnet the

prognosis as regards sight in juvenile tabes dorsalis is un-favourable, seeing how common optic atrophy is. On theother hand, as regards life, it is relatively good, since among12 patients whose history was followed for from 10 to 20years after the beginning of the affection there was not asingle death. It must not be forgotten, however, that (as inthe adult) general paralysis may supervene, and, indeed, foursuch instances are included among the cases collected byCantonnet.That juvenile tabes dorsalis, like adult tabes dorsalis,

may assume various clinical guises is well known. Thus,there is a group of cases mainly characterised by ’’ lightningpains " in the legs or the arms, ataxy of the limbs, and lossor reduction of the knee- and ankle-jerks. It is to be notedthat in about one-quarter of Cantonnet’s cases (25’ 2 percent.) the disease commenced with "lightning pains." Inanother group, which it is the more particular object of thisbrief communication to describe, the prominent symptomsinclude amblyopia due to optic atrophy, together with

changes in the pupillary reflexes, and abolition of the knee-and ankle-jerks. Amblyopia was the first sign noted in14 per cent. of Cantonnet’s series, and in no fewer than43’ 9 per cent. optic atrophy, complete or incomplete, waspresent. Ataxy is often conspicuous by its absence in thisclass of case, thereby bearing out what is sometimes knownas "Benedikt’s law," which is to the effect that ataxicsymptoms disappear or fail to develop on the advent of opticatrophy. Cantonnet’s figures with regard to this point areas follows: among 40 cases of optic atrophy there were70’ 5 per cent. without ataxy, 20 per cent. with slight ataxy,and 9’ 5 per cent. with pronounced ataxy. This variety ofjuvenile tabes dorsalis appears to correspond with thosefamiliar to every ophthalmic surgeon in adults under thename of "pre-ataxic optic atrophy." It is the class that isvery likely to fall, in the first instance at any rate, underthe notice of the ophthalmologist rather than of the generalphysician. It is important that we should be familiar withthe diagnosis of such cases, if only for the purpose of gettingthe patients under suitable medical treatment without loss oftime.Confusion may arise between juvenile tabes dorsalis, on

the one hand, and Friedreich’s ataxy, on the other. In well-marked cases, however, the distinction is not difficult. Thesalient characteristics of Friedreich’s ataxy are (1) nystagmusand (2) associated deformities, such as pes cavus and lateralcurvature of the spinal column. The " lightning pains," the

1 Archives d’Ophtalmologie, November, 1907.2 A Manual of Diseases of the Nervous System, vol. i., 1892, p. 399.

pupillary signs, and especially the optic atrophy of tabesdorsalis, are absent from the other affection. The bilateralatrophy of the optic discs coming on in young subjects mightlead to some confusion with that peculiar abiotrophy knownas "hereditary optic atrophy," which is liable to attackmore than one member of a family at or about puberty."Leber’s disease," as it is sometimes called, is, however,usually preceded by optic papillitis, and even when theappearances of optic atrophy are present yet the peripheralparts of the visual field retain their functions. The defect of

vision, in other words, is in the nature of a central scotoma.In my opinion, it admits of little doubt that a proportion(perhaps a large one) of those cases in children where thesole obvious findings include amblyopia and optic atrophyare in reality instances of juvenile tabes dorsalis. I cannotdoubt that a more systematic investigation of the nervoussystem than is usually feasible in an ophthalmic out-patientroom would result in a more definite diagnosis of these cases.As matters stand, however, the association in a syphiliticchild of bilateral simple optic atrophy with loss of the knee-jerks, would by itself point to a provisional diagnosis ofjuvenile tabes dorsalis, and suggest the need for systematicinvestigation by a neurological physician. Another suggestivecombination would be paralysis of an ocular muscle with lossof the deep reflexes in a syphilitic subject.

Cases of Juvenile Tubes Dorsalis.CASE 1.-The patient, a boy, aged 13 years, came under

my notice for the first time at the Evelina Hospital for SickChildren, London, on August llth, 1899. His left eye wasstated to have been blind and to have squinted outwardssince the occurrence of a "iit" " when he was a baby. Onadmission the right pupil was two millimetres and the leftpupil five millimetres in transverse diameter. Neither pupilresponded to light or to accommodation. The movementsof the eyes were intact. R.V. = 5/18 and No. 6 Jaeger;punctum proximum, 15 centimetres. L.V. = ? no per-ception of light. There was incomplete optic atrophy, whichwas more pronounced in the left eye. The ophthalmoscopicappearances were those of so-called " simple " atrophy. Thelad’s face rather suggested syphilis. A small epithelialdenudation lay beneath the right angle of the mouth. Therewere no fiae deep vessels in the corneas, such as might indi-cate a former attack of interstitial keratitis. But there wassomewhat coarse pigmentation, together with other changes,over the lower half of the left fundus oculi, and also somechoroidal disturbance about the outer side of the left opticdisc. For more complete examination the patient was

referred to my colleague, Dr. George Carpenter, who waskind enough to furnish me with the following report:I I The only suspicious thing I can find about him is thefact that the fronts of his shins, upper third, are thickerthan natural. The sharp line of the tibia is rounded, as alsothe anterior surface of the bone, both sides, suggests nodes."Jan. 31st, 1902 : R.V. = and No. 1 Jaeger. L.V. = fingersat half a metre. The right pupil was very small (1 to 1 - 5 5millimetres) ; there was no response to light but there wasslight action to accommodation. The left pupil, whichmeasured five millimetres, was devoid of action either tolight or to accommodation. Right field of vision for whitefull-90°, 60°, 65°, and 600 in the chief four meridians.The gait was not ataxic. Romberg’s sign was absent. Therewas no rectal or urinary trouble. The boy gave an accountof having suffered intense periodical pain in the left thighand leg at times since I last saw him. It had been much

, worse than usual in May, 1901, but had been better since. June of that year. The knee-jerks were absent; there was,

no ankle clonus.,

In the foregoing case the diagnosis of juvenile tabes,

dorsalis was come to on the following grounds: (1) thesyphilitic inheritance, as attested by the facies and the

.

osseous and choroidal changes ; (2) the absence of the knee-: jerks ; (3) the inequality of the pupils and the Argyll. Robertson phenomenon ; (4) the "lightning pains"; and’

(5) the bilateral optic atrophy.It is of some little importance to notice that on the first

visit (August llth, 1899) the lad presented paresis of accom-.

modation as regards his right eye, since his range of accom-’ modation was only equal to a convex lens of 6 6 D., whereasit should have equalled at his age a lens of at least 12 D.spherical. When examined some two and a half years later this symptom had disappeared. Upon the whole, then, the

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case may be characterised as a fairly typical example ofjuvenile tabes dorsalis.

1402 NR. S. STEFHB NSON : JUVENILE TABES DORSALIS; NOTES OF FIVE CASES.

CASE 2.-The patient, a female, aged 24 years, attendedthe Royal Eye Hospital, Southwark, under the care of Mr.Robert W. Doyne, on Dec. 2nd, 1904, complaining that thesight of the left eye had been failing for about one year. Onadmission she was found to be a healthy but somewhatstupid-looking young woman, whose fades suggested heredi-tary syphilis. Her jaws were ill-developed and the uppercentral incisor teeth, although not typical, conveyed a strong !suspicion of hereditary syphilis. The patient had recentlybecome somewhat deaf. Knee-jerks were absent, even on

reinforcement. Romberg’s sign was not present. There wasno trouble as regards the bowels, but recently the patienthad been compelled to urinate more frequently and to do sothe moment the desire seized her. The right pupil reactedfeebly to light ; the left pupil was motionless. Both pupilsresponded to accommodation. Reflex iridoplegia (ArgyllRobertson phenomenon) was therefore present. Both opticdiscs, especially the left one, were pale and without oph-thalmoscopic evidences of antecedent neuritis. The retinalvessels were of good size. There was no peripheralchoroiditis ; no fine vessels could be found in the corneas;and no ectropion uvea3 was present. R.V. _ . L.V.shadows only. The patient, an orphan, was the eldest ofthree sisters, one of whom, aged 21 years, was examined byme on Sept. 18th, 1905. The knee-jerks were present, thepupils were normal, and the optic discs were of good colour.Vision=g. There was slight hyperopic astigmatism. Thepatient was constipated and occasionally she wetted thebed at night. There were rhagades about the mouth.The third sister, aged 23 years, I had no opportunity ofexamining.CASE 3.3-The patient, a girl, 14 years of age, attended the

out-patient department of the Kensington General Hospitalfor the first time on Feb. 26th, 1908. The personal historyof the case showed that nothing was known to be amiss withthe patient’s sight until she reached the age of ten years,when the school teacher sent to say that the child wasunable to see well. She was sent to school at the age offour years and during her school life, which terminatedrecently, she was a dull child. She reached, however, thefifth school standard. Upon the complaint of bad sight shewas taken on Oct. 14th, 1903, to the Royal London Oph-thalmic Hospital, Moorfields, where spectacles were pre-scribed and these were worn for about a year without anyparticular benefit. On Oct. 20th, 1904, the patient attendedthe Royal Westminster Ophthalmic Hospital, the out-patientletter belonging to which I have inspected. Her sight wasthen less than 6/60 with either eye and a diagnosis of "post-neuritic atrophy was marked on the sheet. Spectacles(plus 1 D. spherical) were prescribed for reading, but,again, they gave little help. The child made three attend-ances at the hospital in question during January last andwas treated with a mixture containing iron and strychnine.When admitted to the Kensington General Hospital, thepatient was found to be a well-grown but rather dull girlwho had not yet begun to menstruate. R.V. = < -e,-.L.V. = < ’6/60. The optic discs were pallid and cupped.They presented all the appearances of so-called simpleatrophy "-that is to say, they were clearly defined, themarkings of the lamina cribrosa could be made out, theretinal vessels were of good size, and the surrounding retinashowed no morbid changes. There was no choroiditis orperivasculitis ; no fine vessels could be found in thecorneas. The visual fields for white (estimated bymeans of a 10 millimetre white square) were verysmall, especially in the left eye, where the field didnot exceed 10&deg; in any direction. In the right eye the limitswere: externally, 180; internally, 15&deg; ; above, 19&deg; ; andbelow 50. The pupils, five millimetres, although sluggishto light, reacted promptly and well to accommodation.In other words, a potential Argyll Robertson pupil waspresent. The girl showed no ataxy, but she easily tired.Romberg’s sign was not present. The bowels were regularwithout recourse to medioine. There was an imperativedesire to urinate. The right knee-jerk could not be elicited,even on reinforcement; an occasional jerk could be obtainedfrom the left knee. The ankle-jerks were absent. There wasno ankle-clonus and Babinski’s sign was absent. No definiteaccount could be got of "lightning pains."The diagnosis in the foregoing case rests mainly upon:

(1) the state of the optic discs and of the pupils; (2) the

3 This patient was shown on March 20th, 1908, before the Society forthe Study of Disease in Children.

absence of one knee-jerk and the impairment of the other;and (3) the imperative desire to urinate. It is not a typicalcase, but on any other view than that of juvenile tabes, it isdifficult to account for the bilateral optic atrophy, which,clinically at all events, is now certainly not post-neuritic incharacter, whatever it may once have been.With regard to the family history, the patient’s mother,

aged 44 years, was in good health. Her father, aged 44years, was said to be a strong man in rude health. Themother had had two miscarriages. Seven children had beenborn alive, of whom the patient was the third. Three of thechildren had died-one at the age of one year frompneumonia ; one at two years of age from phthisispulmonalis ; and the third at the age of three months frombronchitis.The case to be next described differs from those

already mentioned, since amblyopia was not its prominentfeature.CASE 4.-The patient, a boy, aged 11 years, was first seen

at the Kensington General Hospital on August lst, 1906.The personal history showed that two years previously thelad had suffered from I I rheumaties affecting the toes, thefingers, the knees, and the elbows, and he was confined tothe house on that acccunt for two and a half months. In

May, 1905, the patient had complained of the right eye,which was observed to water, to be inflamed, and "to havea white look." In the following month the other eyebecame slightly affected. In October, 1905, the right eyewas noticed to squint inwards. In January, 1906, there wa&a second attack of ’’ rheumatic " nature, less severe than thefirst one. No history of symptoms pointing to congenitalsyphilis as a baby could be obtained. The family historyshowed that the patient’s father (33 years of age) and mother(40 years of age) were both alive. The mother had hadthree miscarriages and two living children, of whom thepatient was the elder. The other child, a boy, died from"convulsions " when aged one month. When the patient wagseen at the hospital the facies and the upper central incisorswere suggestive of inherited taint. The molars were decayed.There was some thickening of the joints of the first andsecond fingers of each hand between the metacarpus andthe phalanges. There were interstitial opacities, togetherwith Hirschberg vessels, especially of the right cornea.

R.V. = < 6/60. L.V. == partly. The left pupil (the only onethat could be examined satisfactorily) was three millimetresin transverse diameter, and bad extremely slight action tolight but fair response to accommodation (incompleteArgyll Robertson phenomenon). The left knee-jerk wasabsent ; the right knee-jerk could be obtained only on

reinforcement. There had been, and still was, some slighturinary trouble when in bed at night-" a weak bladder," sothe mother said.

In the last case to be mentioned the diagnosis is not quiteso clear as in those already recounted. Indeed, it is

possible, I suppose, that we were dealing not with juveniletabes dorsalis but with an instance of hereditary cerebellarataxy.CASE 5.-The patient, a boy, aged ten years, attended the

out-patient department of the Queen’s Hospital for Children,Hackney-road, London, on June 21st, 1906. He presented aspecific facies, with flattened bridge of the nose andcharacteristic upper central incisor. Abundant pus couldbe squeezed from the left lacrymal sac. R.V. = 5/24.L.V. = 5/60. There were nystagmus, especially on lateralmovement, and simple optic atrophy, especially in the left;eye. The retinal vessels were small. No choroiditis or otherocular stigmata of syphilis could be traced. The pupils were3’ 5 millimetres in diameter and active to light. An occa-sional response only could be obtained from the knees onrepeated stimulation with reinforcement. There was noclonus, Romberg’s sign was absent, and there were no" lightning pains." Beyond a tendency to flat-foot therewere no deformities. The child was an orphan and henceno family or personal history worth mentioning could beobtained.Welbeck-street, W.

IN recognition of his researches into paludismthe Soci&eacute;t&eacute; de Pathologie Exotique of Paris has on the nomina-tion of Professor Laveran, the President, elected Dr. IanMacdonald, Huelva, Spain, a corresponding member. The

membership is limited to 70. Dr. Macdonald is a graduateof the Universities of Edinburgh and Paris.

1403MR. J. P. L. MUMMERY : RESTORATION OF CONTINUITY OF BOWEL, ETC.

A NEW METHOD OF RESTORING THECONTINUITY OF THE BOWEL IN CASESOF EXCISION OF A GROWTH LOW

DOWN IN THE SIGMOIDFLEXURE.

BY J. P. LOCKHART MUMMERY, F.R C.S. ENG.,HONORARY SURGEON TO KING EDWARD VII. HOSPITAL; ASSISTANT

SURGEON TO ST. MARK’S HOSPITAL FOR DISEASES OF THE RECTUMAND TO THE NORTH-EASTERN HOSPITAL FOR CHILDREN.

MOST surgeons have experienced the difficulty there oftenis in restoring the continuity of the bowel after removing thetower part of the sigmoid flexure for cancer or other formsof disease. In many cases the growth is situated in thelowest part of the sigmoid flexure, just above the recto--sigmoidal junction and behind the bladder. In such cases,.after an adequate portion of bowel has been removed, it isextremely difficult to deal with the ends, as the lower stumpof bowel or upper end of the rectum is situated so deep inthe pelvis that any ordinary form of end-to-end anastomosisis out of the question, and the only alternatives left are

either (1) to clooe or pack off the upper end of the rectumand establish an artificial anus with the upper end of thesigmoid flexure; or (2) to proceed to remove the whole rectumby the abdomino-perineal route, and bring the end of thesigmoid flexure (if the mesentery is long enough) down to theanus. The first of these methods has the objection that itleaves the patient with a permanent colotomy, and the secondthat it entails a most severe and prolonged operation, andalso means the removal of a perfectly healthy rectum, whichif the continuity of the gut could be restored would be quiteas useful as before. This being the case, I think the follow-ing method of restoring the continuity of the bowel in such<cases without removing the rectum will be found useful. Ihave not been able to find any record of a similar operationhaving previously been performed. In the case here recordedall but a few inches of the sigmoid flexure had to beremoved, and consequently it would not have been possibleto bring the stump to the anus, and the only alternative towhat was done would have been the establishment of a per-manent artificial anus.

I first saw the patient, a man, aged 53 years, with Mr.V. McMnllen of Brixton, on Oct. 18th, 1907, on account ofhemorrhage from the bowel. His history was that fornearly 12 months he had been passing mucus and occa-sionally blood and had had attacks of pain in the abdomen.For the last few months there had been a watery dischargewhich had been tinged with blood on one or two occasions.About a month previously he had a profuse haemorrhage fromthe bowel and this had occurred again the day before I sawMm, and had been accompanied by severe pain in the.abdomen lasting for about half an hour. There was nohistory of constipation, and except for the present illness hehad always been a healthy man. On examination perTectum nothing abnormal could be felt, but very high upa large resisting mass could be felt through the anteriorMetal wall. Examination of the abdomen revealed nothingabnormal. I advised an examination under an anaestheticand for this purpose he went to St. Mark’s Hospital onOct. 26th. Under ether, by bimanual examination, and.after stretching the sphincters so as to allow of two fingersbeing inserted into the rectum, a growth could be distinctlyfelt in the lower part of the sigmoid flexure. This diagnosiswas confirmed by sigmoidoscopy. It was decided that thegrowth was freely moveable and that if the patient agreedan attempt should be made to remove it.The operation was performed on Nov. 4th. The patient was

anaesthetised by Mr. H. Bellamy Gardner with ether by theopen method. The patient having been placed in theTrendelenburg position, an incision was made through theouter part of the rectal sheath on the left side of theabdomen and extending right down to the pubes. Onopening the abdomen I found that the lower part of thegrowth extended down to within one and a half inches of therecto-sigmoidal junction and that a loop of the sigmoid1lexure above the growth had become adherent to, and wasinvolved in, it. This necessitated the removal of all but afew inches of the sigmoid flexure if the growth was to be’removed. The sigmoid flexure was divided above the involvedloop and the meso-sigmoid was stripped up from the posteriorpelvic wall and the vessels clamped as they were divided.

This procedure was rendered necessary owing to the factthat some of the glands in the meso-sigmoid were involved.As a result of the stripping up of the peritoneum the greatiliac vessels were laid bare. The sigmoid flexure at the recto-sigmoidal junction was now divided an inch below the growthand the adherent loops were removed. The peritoneum wasbrought together by stitches over the posterior pelvic walland all bleeding points were ligated. At this stage, owing tothe rectum not having been properly emptied previously tothe operation, some soiling of the pelvic peritoneum un-fortunately took place. Any of the ordinary methods of end-to-end anastomoses were quite impossible owing to the depth

S, Sigmoid. R, Retum. G, Glass tube to the upper end ofwhich the sigmoid is tied. B, Piece of rubber tube toprevent glass tube from slipping up into rectum.

of the wound and to the fact that there was no stump ofbowel below but merely a hole in the pelvic floor. I tied a

glass Keith’s tube into the upper end of the sigmoid flexureand passed the free end of this tube, to the end of which apiece of large-bore rubber tubing had previously beenattached, down into the rectum from the abdominal cavity.An assistant then caught this with forceps introduced peranum and drew it out of the anus. By drawing on theKeith’s tube the upper end of the sigmoid flexure was invagi-nated into the upper end of the rectum so as to form a kindof intussusception. Two or three silk stitches were thenput in to prevent the invagination from coming undone. Adrainage-tube was then inserted and the abdominal woundwas cloed in separate layers. The operation, which hadtaken some time, was well borne and there was no shock.The patient was kept on a diet of albumin for the first

week and sufficient fat "in the form of cream was addedto the feeds to keep the stools quite fluid. The bowels werefreely opened with calomel on the third day through thetube. The tube separated and came away on the seventhday. After the tube came away a fsecal fistula formed alongthe track of the drainage-tube and there was some dischargefor a time, but the bowels continued to act by the rectum.The patient made a good recovery without any bad sym-ptoms and although the fsecal fistula was a troublesomecomplication it soon began to close and in the course of acouple of weeks only allowed a little flatus occasionally toescape and at the time he left the hospital was almosthealed. He had complete control over the action of thebowels and defalcation was quite normal.

It was not possible with a finger in the rectum to feel theline of union, but an examination with the sigmoidoscopebefore he left the hospital enabled us to see it. There wasno appreciable narrowing at the point of union and beyonda faint white line nothing could be seen.

I have since heard that the slight sinus which he hadwhen he left the hospital has quite healed up and that he isnow well.The condition produced was practically a short artificial

intussusception, the two peritoneal coats being in appositionand the ends of the mucous coats close together, though notnecessarily touching each other. Owing to the glass tubetied into the upper portion of the bowel no leakage couldoccur and the ends of the bowel had about a week in whichto become united to each other before there was any possi-bility of any strain being thrown upon the line of union ;while the tube was still in position the bowels could befreely opened without any risk of leakage and this was a verygreat advantage in the case of an anastomosis so near therectum.

I think this method of restoring the continuity of the