A Case of Congenital Deformity (Dr. Treves' Article on Joseph Merrick)

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    The present publication, being the Thirty -sixth Volume ofTransactions, constitutes the Thirty - ninth published AnnualKeport of the Pathological Society's Proceedings.

    The Council think it right to repeat that the exhibitors arealone responsible for the descriptions given of the Specimensexhibited by them, the only change made in the Reports furnishedby the authors being such verbal alterations as were absolutelynecessary.

    53, Beenees Steeet, Oxeoed Steeet;October, 1885.

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    494 MlSCELLANEOtlS SPECIMENS.here to-night. As far as I can ascertain no author makes anyallusion to such bodies, either in normal or pathological conditionsof the thymus. It might reasonably be urged that, inasmuch asthese aj^pearances have been found in such dissimilar cases as pur-pura and haemophilia, they are little likely to have any bearing onthe minute pathology of either disease. To this objection it mightwith equal reason be replied that, inasmuch as both diseases mayin certain cases depend on alterations in the blood itself, no appa-rent alteration in any part of the vascular system or its appendagesshould be neglected in endeavouring to discover the origin of themalady.The second series of specimens is taken from a patient who died,

    aged 14, of a pelvic abscess, which had been preceded by purpura.The appearances in the thymus are almost identical with thosetaken from the case of haemophilia.Whether this condition of the gland is a cause, a consequence,

    or even a usual concomitant of any of the diseases of the vascularorgans experience does not enable me to say. All that is certainis that the appearances are unfamiliar, and no lesion of any othertissue or organ was detected. October \hih, 1884.

    A case of congenital deformity.By Feedekick Treves.

    [With Plate XX.]THE subject of this deformity was a man aged 24, who earned a

    living by exhibiting himself as the Elephant Man. Hewas a little below the average height and was lame by reason of ashortening of the left leg that had followed an attack of hip diseasein childhood. The deformity concerned both the cutaneous andosseous systems. With regard to the skin the affected districtsshowed a twofold abnormality. In the first place over certainregions the subcutaneous tissue was greatly increased in amount.From this it followed that the integument in such regions was raisedconsiderably above the surrounding normal skin. It was also ren-dered remarkably loose, so that it could be freely slid about, and if

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    DESCRIPTION OF PLATE XX.To illustrate Mr. Treves's case of Congeuital Deformity, (Page

    494.)

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    Trans.Paffi.Soc M.XXXVI.PIXX

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    A CASE OF CONGENITAL DEFORMITY. 495grasped it could be drawn away from the deeper parts in immensefolds. In three places the skin so affected had assumed the formof a pendulous flap. Thus from the right pectoral region imme-diately in front of the axilla a great flap of loose flabby skin hungvertically downwards. It measured six inches in vertical heightand about the same in breadth. A smaller, firmer, and much lessconspicuous fold existed also at the posterior aspect of the rightaxilla. The integument over both buttocks was continued down-wards as a huge thick flap which reached almost to the level ofthe middle of the thigh. So solid and extensive was this flap thatit appeared at first to be the buttock itself, and its existence some-what interfered with the act of defsecation.The second species of abnormality met with in the skin took the

    form of a papillomatous condition of its surface. The parts soinvolved presented the appearance of an ordinary congenital papil-loma of the skin when met with in an adult. This condition ofthe surface, when added to the abnormality of the subcutaneoustissue already alluded to, produced a most remarkable appearancein the involved districts. The papillomatous growth was far moreexuberant in some places than in others. Thus over the rightpectoral region the individual abnormal papillae were comjjarativelysmall, and the general surface even. Towards the right claviclethe papillomatous condition passed off into a mere roughening ofthe integument. In other parts, viz. on the front of the abdomen,at the back of the neck, and over the right popliteal space thepapillomatous groAvth was also small, the individual elevationsbeing of no great magnitude. Over the dorsal region, and espe-cially over the gluteal districts, this peculiar growth was met within its most exuberant condition. The individual papillary tumourswere large, the sulci between them deep, and tlie whole mass hereand there broken up into deep fissures and clefts. The colour ofthe skin over the involved districts was normal save over the mostprominent masses and depoudeut parts, where it was dusky orpurplish. From the larger of the growths an exceedingly foulodour arose akin to that met with in smaller papillomata.The whole of the integument of the body was involved in one

    way or another, with the exception of the following parts : theears, the eyelids, portions of the face, the whole of the loft upperextremity, a patch below the right scapula, and another of smallersize over the left buttock, nearlv the whole of the front of the

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    496 MISCELLANEOUS SPECIMENS.abdomen, and of the right thigh, the front of the left thigh andleft leg, and the back of the right leg. The integument of bothfeet was grossly defoimed.

    It is remarkable that the skin of the penis and scrotum wasperfectly normal in every respect. In no part was there anyneevoid growth. In some places, as for example over the frontof the abdomen and the right pectoral region, the papillomaexisted without any other abnormality, without the thickening ofthe subcutaneous tissue that was so marked in other districts. Onthe other hand there were regions where the chief or only deformitydepended uj^on extreme thickening of the connective tissue underthe skin, the integument itself being approximately normal. Thiscondition was met with in the right forearm and in parts of theright hand and the feet, and upon the scalp. There were papillarygrowths over the greater part of the face, but none upon any portionof the hairy scalp. The growths upon the skin followed no distinctareas of distribution. They appeared to be scattered about casuallyor accidentally, and to be influenced by no specific anatomicalarrangement.The deformities of the osseous system were limited to the skull,

    the right upper extremity, and the feet. The proportions of thehead were enormously increased and its general outline that of ahydrocephalic skull. The surface of the skull was so irregular asto render any detailed description very difficult. It was covered byhuge rounded exostoses, the chief of which were larger than a largeTangerine orange. The most consj)icuous of these bossy masses ofbone were placed upon the frontal bone, the posterior parts of theparietals, and the upper part of the occipital bone. Apart fromthese large exostoses the right side of the cranium just above andbehind the ear appeared to bulge outwards with the result that theupper part of the pinna was folded downwards. In the intervalsbetween the more cousj)icuous exostoses the surface of the skullwas not regular, but presented ridges and irregular upheavals ofbone that disregarded sutures and were disposed in a perfectlychaotic manner. Indeed, the whole head was ostentatiously un-symmetrical. From the upper part of the right side of the frontalbone a very large ridge took origin and ran backwards almost hori-zontally across the vertex until it became lost among the elevationsover the hinder part of the parietal bone. Neither the orbit northe meatus had been encroached upon on either side. There was

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    A CASE OF CONGENITAL DEFORMITY. 497no paralysis of any cranial nerve, and the patient had presented noevidences of epilepsy or other cerebral disturbance. He neversuffered from headache and his intelligence was by no means of alow order. The chief exostoses, and especially those in theoccipital region, were covered with scalp tissue that presented agreat thickening of its subcutaneous layers, and by this hypertrophythe prominence of the tumour was much increased. Over thefrontal excrescences was thickened integument covered by a pa|jillarygrowth. The right superior maxillary bone was greatly and irre-gularly enlarged. The right side of the hard palate and the rightupper teeth occupied a lower level than the corresponding parts ofthe left side. By reason of this growth of the bone the nose hadbeen turned to the left side and the lips rendered unduly prominent.From the front part of the right uj^per jaw a connective-tissuegrowth had developed, which, projecting beyond the mouth, hadprevented him from closing his lips and had interfered with masti-cation and articulation. This had been removed at the LeicesterInfirmary two years ago. The lower jaw and the other facial bonesappeared normal.

    In the right upper extremity every bone was found hypertro-phied with the exception of the clavicle and scapula.The whole limb appeared two or three times the size of its

    fellow. The hypertrophy in the bones was quite regular, and noexostoses could be found. The fingers were grossly misshaped.All the phalangeal bones were enlarged and covered by greatlythickened soft parts and rugose hypertrophied skin. All the nailswere perfect, but some of the phalangeal joints had become partiallydislocated by reason of the irregular character of the hypertrophy.The member presented no trace of cedoma. He had good use ofthe right shoulder and elbow, pronation and supination were per-formed with difficulty, and the movements at the wrist and in thefingers were so imperfect that the hand was almost useless. Hecould, however, dress and feed himself without assistance. Bothfeet were in almost the same condition as the right hand, thebones being enlarged and the toes misshaped and of enormoussize.The man was a native of Leicester. He had no brothers or

    sisters, and there was no evidence of similar deformities in anyof his relatives. He gave an elaborate story of a fright his mother

    3-:

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    498 MISCELLANEOUS SPECIMENS.had received shortly before his birth from having been knockeddown by an elephant in a circus.From his own account it would appear that his head, his right

    arm, and his feet have always been grossly deformed. He states,however, that when a child his skin was simply thickened, loose,and rough, and that the main papillary growths were not present.There is distinct evidence to show that these latter growths are atpresent extending rapidly. Moreover, the hypertrophy of thesoft tissues of the fingers has so increased of late as to greatlydiminish the already limited movements of the hand. The manenjoys good health, has suffered from no serious illnesses, andpossesses a fair degree of muscular strength.

    March 17th, 1885.

    8. A neiu and simple ether microtome.By Leopold Hudson.

    THIS little instrument has been invented by Mr. V. H. W.Wingrave, of the I^Iiddlesex Hospital. The following aresome of its advantages

    1. Simplicity of its construction.2. Ease and rapidity in manipulation.A specimen can be frozen

    and cut in five minutes.3. Cleanliness. Ether being the freezing agent, the incon-

    veniences of ice are removed.4. Economy.A specimen can be frozen at an average cost of

    one farthing. There is practically no waste, since the spray isminimised and any surplus ether is collected.

    5. Compactness and portability.6. Roughening of knife edge prevented by the circular plate.7. Moderate cost.It is made by Mr. Hawksley, of Oxford Street,

    at a cost of 20s. (about). December 2nd, 1884,

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    XL DISEASES, ETC, OF THE LOWER ANIMALS.1 . Diseases of the reproductive organs in animals.

    By J. Bland Sutton.THE object of the paper is to place on record some examples ofthe more important affections to which the reproductiveorgans of animals are liable. In this communication the femaleorgans will be chiefly considered. The observations were originallyundertaken with the specific purpose of investigating the peculiartendency fuuctionless ducts have to become cystic.

    In the course of inquiry I was led to obsei-ve closely the internalreproductive organs of a very large number of animals, and foundthat they frequently exhibited some remarkable and importantdiseases. This induced me to extend my researches to the repro-ductive organs generally.

    In addition to the large number of wild animals coming undernotice in the Prosector's room at the Zoological Gardens, I havebeen able to extend my observations to horses, slaughtered therefor the purpose of feeding the camivora. During the summer of1883 I followed up the work by inquiring into the conditions ofthese structures in a very large number of frogs and toads, andhave collected many interesting facts regarding fishes. This paperwill be simply an abstract of my work, commencing with fish andembracing amphibians, i-eptilians, birds, and mammals, up to andincluding quadrumana.The subject, for convenience of description, will be subdi\-idedas follows(1) The utenis and vagina : Their malformations, displacements,

    including flexions, pyoniofra, t\imours, and nij>ture.(2) The Fallopian tubes : Pyosalpinx.(3) The ovaries: Their nialfonnatious, atroj'liy. fibroid aud

    fatty degenerations, cysts and tumours.(4) The parovarium with Gartner's duct : Their cystic dila-

    tations.