DYSOSTOSIS CLEIDOGRANIALIS SHOWING UNIQUE SCAPULq OF PRIMITIVE TYPE*1

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  • 303CLINICAL AND LABORATORY NOTES

    Photographs showing the site of the zoster lesions.

    and buttocks. All the vesicles were in the same phase ofevolution and differed in this respect from the lesionsof varicella. The right axillary and the left inguinallymphatic glands were enlarged.The patients temperature was 100 F. and the pulse-rate

    88. Physical examination revealed no abnormalities.The patient felt well and complained of no subjectivesymptoms except slight burning and irritation. He wastreated in bed and given sodium iodide grs. 10 t.d.s. by themouth. A dusting powder was applied to the vesicles.

    The vesicles rapidly dried up and he developed no post-herpetic pain.Serum collected fourteen days after the onset of the

    eruption gave a positive complement-fixation reactionwith a vesicular antigen from another case of zoster.My thanks are due to Dr. W. J. ODonovan for per-

    mission to publish this case and to Dr. R. T. Brain for theinformation about the complement-fixation reaction.

    DYSOSTOSIS CLEIDOGRANIALIS

    SHOWING UNIQUE SCAPUL OF PRIMITIVETYPE *

    BY VICTOR J. KINSELLA, F.R.C.S. Eng.

    Dysostosis cleidocranialis is a developmental defi-ciency of the skeleton affecting chiefly the claviclesand the bones of the cranial vault. It is comple-mentary to achondroplasia, in which the cartilagebones bear the brunt of the disorder.

    Its interest lies firstly in its rarity. Althoughit was described as long ago as 1766, a careful reviewof the literature in 1908 by Hultkrantz revealed only31 cases described clinically. There are a few othercases in which reference is made to either the clavicleor the skull alone. In the museums of the worldthere are only nine skulls showing the condition, fiveof these being in the institute of pathological anatomyof the University of Vienna. This institute containsalso the skeletons of two cases-I believethe only two in existence. Efforts havebeen made by other institutions toobtain some of this material, but evenin their days of tribulation, the Viennesepreserve their sense of relative valuesand their museums retain their treasures.The second source of interest is the

    light which this condition is said to throwupon the development of the clavicle,the first bone in the human body toossify. In the embryonic clavicle,cartilage cells and afterwards osseoustissue make their appearance in apeculiar precartilaginous condensationof the mesenchyme. Seeing that incleidocranial dysostosis the clavicle suffersalong with the bones of the cranialvault, the clavicle itself has been looked

    * Communicated to the Royal Prince AlfredHospital Medical Officers Association, Sydney.

    upon as being partly a membrane bone. Butas we shall see in the case which I am present-ing, interference with growth in cleidocranial dysos-tosis may be found also in bones developed incartilage-e.g., the scapulae and phalanges of thefoot.A third point of interest is that the possible develop-

    ment of the clavicle in two parts complicates theattempt of morphologists to recognise in the humanshoulder girdle the two ventral elements of theprimitive pectoral girdle, the coracoid and pre-coracoid, which are seen in some of the lower animals,and which correspond with the pubis and ischium ofthe pelvic girdle. In the fruitless controversies overthis question almost every possible combination hasbeen made to represent the primitive elements fromamong the centres of ossification of the coracoidprocess, the clavicles, the metacromion, the supra-sternal bones, and the various ligaments around theclavicle (costoclavicular, coracoclavicular and costo-coracoid and the articular disc). Some morphologists

    FIG. 1.Radiogram of chest showing abnormality of clavicles.

  • 304 CLINICAL AND LABORATORY NOTES

    deny that the shoulder girdle has anypubic representative.The patient who came under my care is

    a boy of 16 years. He is of slender buildand with more than average intelligence,features noted by Paltauf in his case. Hisparents relate that the anterior fontanelleclosed very late. In many cases a stronghereditary tendency has been noted, butclinical examination of the parents of thisboy and X ray examination of their handsrevealed no abnormality, and no relevantpoint could be elicited in the familyhistory. A large number of the casesdescribed are of French extraction, as waspointed out to me by Dr. Sear, but theparents of my patient knew of no Frenchblood in their families.The patient was almost able to approxi-

    mate his shoulders across the front of hischest. On palpation absence of theacromial end of each clavicle was noted.The sternal ends tapered laterally to a point.rrnQA hnnPC nan hA ennm in t..1Bo. -rgr1;ntTTQYYBin the upper part of the lung field, and their radio-graphic density is much less than that of the ribs.They are no doubt very soft and deficient in limesalts (Fig. 1). From the under surface of the pointedextremities a thin cord passed laterally to the shoulderregions. Whether these cords were attached laterally tothe coracoid or acromion or to some other part could notbe definitely determined, but they probably represent thecoracoclavicular ligaments (or thickened upper edge ofthe costocoracoid membrane). The clavicular parts of thedeltoid and pectoralis major muscles were poorly, if at all,developed. The patient complained of no disabilityassociated with the condition of his shoulder girdle ; onthe contrary, he played such games as tennis with ease.Absence of the acromial end of each clavicle, as describedabove, is the commonest type of clavicular abnormalitymet with in these cases. The next most common arrange-ment is replacement of the middle portion by a fibrousband which unites the acromial and sternal extremities.Fig. 2 shows an apparent absence of the supraspinous

    fossse of the seapulse, the spine and acromion springingdirectly from the upper border, an arrangement rathersimilar to that found in the iguana and platypus. Icannot find reference to such a malformation in anyother case. I am indebted to the director of the patho-logical anatomical institute of the University of Viennafor a personal communication, wherein he informs me thatthere is no such abnormality in the scapulae of the twoskeletons in the institute.The skull in this patient shows the features usually

    described-a large cranial vault, small and narrow base,small facial bones, metopic suture, wormian bones in theneighbourhood of the lambda, high vaulting of the palate,and marked delay and irregularity in the eruption of theteeth. The erupted teeth were stunted and all were milkteeth, except three premolars and one canine. The

    FIG. 3.-Radiogram of the patients toes (left-hand photograph) compared,

    . with those of a normal person.

    FIG. 2.-Radiogram showing abnormality of acapulse.

    remaining second teeth were still unerupted. The dentalsurgeon is at present exercised to provide an efficientmasticatory apparatus, and proposes the removal of theerupted teeth and the use of prostheses. Dr. Sear hasdrawn my attention to an increased density along thesuture lines in these radiograms, a feature already notedin cases seen by him.Radiograms of the toes are illcstrated in Fig. 3 together

    with those of a normal person of about the same size butthree years older than the patient. Note the shorteningof the phalanges (already described by Murk Jansen) andthe more open texture of the cancellous bone and deficientdevelopment of the compact bone. The phalanges of thehand show similar features in lesser degree. The indexmetacarpal is very long and shows an epiphysis at each end.There were also noted a marked scoliosis, with lower

    limbs of equal length, bilateral cervical rib, club-feetcorrected by operation in infancy, and changes in thepelvis. The narrowing of the pelvis, especially of theilium, and the gap in the region of the symphysis pubishave already been noted, as by Paltauf.

    For further accounts of cleidocranial dysostosis, andof the various speculative questions associated there-with, the reader may consult articles by Fitzwilliams 1and Paltauf.2

    PEPTIC ULCER IN A SOLOMON ISLANDNATIVE

    BY CLIFFORD JAMES, M.B.N.Z., F.R.C.S.Edin.,D.T.M. & H.

    IT has been said that peptic ulcers are" confined to men of civilised racesliving in temperate climates," and thefollowing case may therefore be ofinterest.

    I had started medical work in a native-built hospital on Choiseul, the island lyingat the north-western extremity of theBritish Solomons Group, and the mostout-of-the-way of the larger islands. Thelatitude is 7 South, and the climate hotand moist. Organised fighting had ceasedabout ten years before. The wildest part of

    1 Fitzwilliams, D. C. L. : THE LANCET, 1910,ii., 1466.

    2 Paltauf, R.: Verhandl. d. Deut. path.Gesells., 1912, xv., 337.

    3 Ogilvie, W. H.: THE LANCET, 1935. i.,419.

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