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6. The possibility of mis-diagnosing an acutemyocardial infarction, when pneumothorax oc-curs in an older patient, is pointed out. Carefulphysical examination is necessary, often aug-mented by special diagnostic procedures, inorder to arrive at the correct diagnosis.

7. Only two cases of this series, or 12.5%occurred in persons suffering from pulmonarytuberculosis; both of these were in the far-advanced and active stage and in one of thesethere was evidence to suggest that the pneumo-thorax resulted from rupture of a large emphy-sematous bulla.

8. One case of recurrent pneumothorax wastreated by induction of a chemical pleurisy, withapparently satisfactory results.

9. One patient who had a persistent hydro-pneumothorax associated with a broncho-pleuralfistula was cured by surgical means.

REEFERENCES1. NIEHAUS, R. F.: Amt. J. Roentgenol. & Radiumn

Therapy, 57: 12, 1947.2. COOPE, R.: Diseases of Chest, E. & S. Livingstone,

1944.3. LEACH, J. E.: Arch. Imtt. Med., 76: 264, 1945.4. SCHNEIDER, L. AND REISSMAN, 1. I.: Radiology, 44:

485, 1945.

PTERYGIUM COLLI AND ALLIEDCONDITIONS

H. 0. Foucar, B.A., M.D., F.I.C.S.

London, Ont.

IN 1931 a theological student aged 19 years pre-sented himself for advice concerning curious

bilateral folds of skin running from the mastoidregion to the point of the shoulder. These werethin, consisting apparently only of skin andsome subcutaneous tissue. They were lax andcould easily be pulled out from the neck as aweb. The ears were large. The hair-line pos-teriorly was low although there was no actualshortening of the neck and no limitation ofmovement. No x-ray study was made. Thechin receded somewhat and there appeared to besome looseness of the skin below the chin.

This was a new anomaly to me and I couldfind no description of it, so called it merely acongenital abnormality of the cervical subcu-taneous tissue and skin. It occurred to me thatthe condition could be improved by ellipticalexcision of skin posteriorly. In 1934 the patientreturned for operation and I found it necessaryto modify the plan. An incision about 3" long

was made in the midline posteriorly and the skinwas undercut. This was not sufficient. A secondincision about 5" long was then made trans-versely at the top of the first incision forming a"T". Each flap of skin was raised and pulledupwards and inwards until the skin folds wereobliterated as in operations for removal ofwrinkles. The excess skin was excised leaving atriangular defect. The skin edges were thensewn together in the form of a "T". It willbe noted that the scar was posterior and wellhidden by the hair or collar. Both folds werecorrected through the one operative field and itwas possible to obtain symmetry easily.

4.~~~~~~~~~~~~~.

Fig. 1.-Ptervgium colli or webbed neck: lateralview. Fig. 2.-Posterior view showing folds of skinand low hair-line, giving the appearanice of shortnessto the neck. Fig. 3.-Posterior view showing laxnessof the skin. Fig. 4.-Lateral view after operationshowing comiplete obliteration of the fold. Note alsothe inconspicuous scar posteriorly.

It was thought that there might be some de-ficiency in elastic, tissue in the skin but histo-logic examination was inconclusive, because thespecimen came from the back of the neck andnot from the sides where the anomaly occurred.

More recently I came across a short descrip-tion in Babcock 's'3 Surgery in which the name

"pterygium colli" was used. This served as a

lead for a review of the literature with specialreference to etiology, pathology and treatment.Some difficulty was experienced because of thedifferent terms used.

FOUCAR: PTERYGIUM COLLI [ Canad. M. A. J.Ssept. 1948, vol. 59

Kobylinskil (1883) was the first to describewebbing of the skin on the lateral aspects of theneck and suggested its possible relationship tocongenital short neck. Sutton2 placed web-likeexpansions in general among the instances ofspurious atavism. He regarded them as spon-

taneous variations or "sports". The term"pterygium colli" was first applied by Funkelin 1902 to this anomaly seen in a 15 year oldgirl. It was associated with abnormalities ofthe breast and nipples and with the absence ofpubic and axillary hair. In the same year

Bussiere7 reported webbing of the neck in a

Hindu boy aged 12 years. Ballantyne8 thoughtthat the web-like structures found passing be-tween various parts of the body might be ofthe same nature as feto-fetal bands or amnioticadhesions. These expansions, to which the namepatagium might be applied, sometimes passedfrom the neck to the shoulder on one or bothsides. He felt that, while the patagium mightiiot be amniotic in origin, it was sometimes foundassociated with malformations which were gen-

erally regarded as such.In 1912 Klippel and FeilWl described another

syndrome which consisted of synostosis of some

or all of the cervical vertebrae associated withcervical spina bifida and in which a shortenedhair-line and limitation of movement producedan appearance not unlike that of pterygium colli.Hutchinson4 actually demonstrated the firstspecimen of this in 1894 and Clarke10 the firstclinical case in 1906.A case was reported by Drachter31 in 1923 in

which webbed neck ("dove neck") was found.The presence of a suboccipital encephalocele,however, suggested a diagnosis of Klippel-Feilsyndrome rather than pterygium colli. Frawley14reported webbed neck in two sisters 12 and 16years of age, in a woman of 28 and in a girl of7 in whom there was also webbing of the axillaryfolds. X-ray examination was negative.

Sections of the folds of the neck obtained atautopsy were studied by De Bruin.15 They re-

vealed muscle and suggested an abnormal dis-tribution of the platysma myoides. The deepfascia and muscles were not involved. Ullrich'6stressed the combination of multiple abnormali-ties. Nageotte-Wilbouchewitz'9 believed the con-

dition to be one of pure atavism and remarkedthat it occurred naturally in the chimpanzee.One case associated with elephantiasis was re-

ported from Ihelsinki by Rantasalo.'0 This was

a female infant with a thick fold of skin fromthe mastoid process to the acromial process onboth sides. One arm was slightly thickened andboth legs and feet were markedly and symmetri-cally thickened. He stated that pterygium collwas rare and belonged to the same class as foldsof the axilla, elbow, fingers and knees. Ele-phantiasis congenita lymphangiectoides was alsovery unusual. Only two cases have been re-

ported in which the two conditions occurred inthe same individual. He also discussed mon-

golism in this connection. Hoffman21 in 1937reported three cases and also stressed the com-

bination with other malformations. He felt thatit bore some relationship to mongolian idiocyand to the Klippel-Feil syndrome. Two more

cases were added by Chandler22 and he referredto the differential diagnosis from Klippel-Feilsyndrome and also from Sprengel's deformity.He discussed the surgical correction by means

of plastic procedures especially those of the"-plasty type and described in detail his

modification of the latter. Marquardt23 de-scribed one case of folds involving the legs andtwo cases involving the neck. Until now thesehad been treated according to the principles ofplastic surgery. He thought, however, that thiscould usually be avoided by bloodless orthoptdicmeasures.

Turner24 in 1938 described a syndrome con-

sisting of the triad, infantilism, webbing of theskin of the neck and deformity of the elbow(cubitus valgus). He reported seven femalepatients all of whom presented osseous andsexual retardation similar to that associated withhypoantuitarism or the Lorain-Levi type ofdwarfism with retardation in growth and sexualdevelopment. The cervical vertebrae were nor-

mal but the neck appeared short because of thewebbing and the low hair-line posteriorly. Hespecifically mentioned that mental retardationand other abnormalities were not present. Treat-ment with pituitary growth hormones was un-

satisfactory. Definite genital development fol-lowed administration of anterior pituitary gona-

dotropic hormone in two cases.

By 1938 MIacCollum25 found that approxi-mately 20 patients had been described. He ap-

proached the problem from the standpoint ofembryology. During the early weeks of' fetallife the neck was difficult to distinguish fromeither the head or shoulders as the diameters ofall three structures were very nearly the same.

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As the branchial arches grew ventrally to formthe lower face and anterior portion of theneck and as the limb buds begani to developthe neck became a definitely recognizable struc-

ture. Its actual lenigth, however, was almostnegligible as the cervical flexure of the fetusplaced the chin against the thorax and thatportion of the head which would become themastoid process remained almost continuouswith the shoulders. As the fetus continued to

develop the head grew more rapidly than therest of the body so that in the 2nd or 3rdmonth of pregnancy it had become over one-

quarter or one-third wider than the shouldergirdle. Observations made at this period re-

vealed that the neck was quite long on its dorsalsurface because of the cervical flexure. It was

found to be even longer on the ventral surfacebecause of the medial ingrowing of the branchialarches. It is to be particularly noted that therewas very little length to the neck on both itslateral surfaces and that the region of themastoid process was likely to be either lateralto or in direct vertical line with the region ofthe acromial process. If, at this time, there was

a failure of normal development it was possiblefor the skin over the lateral surfaces of theneck to remain shortened. As the body grew

and the shoulders assumed their normal width,the skin would be even more deficient so thattight bands or webs would form between theacromion and mastoid, the two points whichwere so closely approximated in early fetal life.He studied four cases and several fetuses andadvised the "Z "-type plasty but enumeratedseveral precautions.

Bizzarro26 felt that there was a definite rela-tionship between the conge nital high scapula(Sprengel3 1891) and congenital short neck

(Klippel and Feill" 1912). In both conditionsthere may be absence of the vertebree or

anomalies in their number or malformed or

anomalous ribs. Brevicollis, it seems, is an

advanced stage of high scapula. In both con-

ditions the descent of the shoulder girdle is in-

complete. Further, bilateral high scapula mayshow a lowered scalp hair-line and thereforea relatively short neck; it also displays a

webbed wing-like neck as in brevicollis. The

limitation of the facial expression among frog-necked people may be the result of endocrine

dysfunction for in some the expression is

quite cretinoid. He reported the case of a

boy aged 14 years. The main features were

the expressionless mongoloid face, the lowerednape line, the high level of the scapulteand the short neck. Radiograms showed thecervical vertebre normal in number and shapeexcept for the slight cuneiform appearance ofthe third. He had bilateral undescended testesand no pubic hair but no changes in the elbows.He was given gonadotropic hormone, pituitrinand thyroid extract without improvement. Itwas suggested that later a plastic operation(after Gillies) might improve the contour ofthe neck. Bizzarro felt that this case was a

link between two separate conditions. Clini-cally it could be classified either as an element-ary form of Klippel-Feil syndrome or an

elaborate form of congenital high scapula. I

feel that this case is even more interesting.The diagnosis of Klippel-Feil syndrome restsonly on the shape of the third cervical vertebra.I submit that the diagnosis of pterygium colliwith high scapula should be entertained. Inaddition, this patient showed evidence ofinfantilism and only lacked cubitus valgus tobe considered an example of Turner 's syn-

drome. This case exemplifies the merging ofsome features of four clinical entities.Sharpey-Schafer and Schrire28' 29 reported

that urinary creatinine excretion in a case ofTurner's triad was normal. They injectedpituitary thyrotropic extract and caused a risein temperature and pulse rate on the sixth day.The thyroid became enlarged and tender andthe basal metabolic rate rose to 43%o. Sharpey-Schafer30 treated with testosterone propionatea similar patient who had had hot flushes forfour years. Three doses of 50 mgm. each stoppedthe flushes. The dose was raised to 100 mgm.

daily and the flushes remained absent. Syl-vert32 described one case of pterygium colli ina boy of 16 years who also presented an ab-normality of the patella and Thiemann-Fleischner's disease of the proximal epiphysesof the second phalanx of both middle fingers.In 1940 Capurra33 reported the first case ofTurner's syndrome in South America. Haupt-man and Thannhauser35 described an entirelydifferent heredo-familial entity in which the

"webbed neck" was produced by tension of

the shortened trapezius.Gilln our36 advanced the hypothesis that the

Klippel-Feil syndrome is merely a mild form

of the deformity characteristic of iniencephaly.

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Inienieephaly is a deformity found only in still-born anid in infants who die shortly after birth.The majority are female. There is great retro-flexion of the head so that the face looks up-wards and forwards. The head is commonlyenlarged. The neck is absent or only indicatedanterior.ly below the chin. The scalp becomescontinuous with the skin of the lower part ofthe back perhaps as far down as the sacralregion. The skin of the face usually passesdirectly on to that of the chest. The scapulaeare pushed aside by the head anld are laterallysituated. The shoulders become more anteriorthan niormially. The boony abnorml-ity is in theocciput, the cervical columni and a variablelength of the spine below this. Klippel-Feilsyndrome or congenital brevicollis coinsists ofshortness of the neck, a low hair-lii-ne posteri-orly anid limiitation of movemenit associatedwitlh fusioni of the cervical vertebree ancd some-times also of the upper thoracic vertebrT.Since the publication of Klippel and Feil, caseshave beeni described which have the same ana-tomical lesion but differ in being without anyexternal manifestations. Miany additionalfeatures, however, have beeni recorded. Comn-mon among the latter are webbing of the neck(pterygium colli), marked depression at thenape of the neck, congenital high scapula,winged scapula, facial asymmetry, spasm ofthe cervical muscles or torticollis, cervical ribs,absence or fusion of the ribs, scoliosis anddorsal kyphosis.

Sharpey-Schafer37 in 1941 gave the post-mortem findings in a case presenting Turner'striad. She was a woman aged 21 years whohad died of tuberculosis. The web of the neckconsisted simply of a fold of skin withoutmuscle. No functioning ovarian tissue was

distinguishable. The endometrium was de-stroyed by tuberculosis. The pituitary, thy-roid and adrenals were not abnormal.

Flavell38 reported the first case of Turner'ssyndrome in a male aged 21 years. He was ofnormal intelligence and a little below averagein physique. From the posterior aspect of bothauricles a ridge of skin and subcutaneous tissuecurved back to the mastoid process and passedthence in a bold web with a gentle concavitydown to the region of the acromion. The webcould be taken between the fingers and flappedabout. Down the whole length of the rightweb was a linear scar made by exploration

carried out when the patient was three yearsold. The facial skin was drawn taut and theangles of the eyes, nose and mouth were pulleddownwards. The chiu was recediing and alsohad the appearaince of being drawn back bytight skin. By contrast, the skin of the napeof the neck was loose and redundant. Thehair-line was low. There was no real shorten-ing of the neck but the base appeared widerthan is normal. Definite cubitus valgus waspresent, the carrying angle of the forearmsbeing '25 instead of 150. The beard was scanity.There was no hair O1n the chest or axillwc.Pubic hair was of the female distribution andhis hips were wide. The testicles were hypo-plastic but the patient said that he had normalerections. Some kyphoscoliosis was presentand he had third degree bilateral claw feet.X-ray examination showed failure of union ofthe 5th cervical neural arch. The family historywas negative. Chandler 's "Z "-type plasticoperation was performed on the left side. Onthe right side the sear and skin from theposterior edge was excised until the normal neckcontour was restored.

In 1946 Bettman reported four patients hav-ing webbed neck and two with webbing of theaxillary region. He mentioned that the entireear may be pulled down and the lobe webbedor the ear may be otherwise malformed. Theweb may be attached in front of or behind theear or may be in front of one ear and behindthe other.

He described an operation which he performed underlocal anaesthesia. The head was pushed to the oppositeside and an incision was made along the length of theprominent ridge down to the muscular layer. A secondincision one inch and a half or more was made at rightangles to the first on the tenser side of the wound anda third on the opposite side. Usually these two right-angled incisions were made in the region of the one-third and two-third points of the length of the primaryincision, one anteriorly and one posteriorly. The skinwas undermined and the head abducted further to theopposite side. The point of one flap of skin automati-cally fell into the angle of the opposite incision.

SUMMARYPterygium colli, while rare, is widely dis-

tributed geographically. It does not appear tobe hereditary but has been reported in twosisters. Possibly the retardation in sexual de-velopment noted in some cases cuts short theline and the appearance of advanced cases doesnot make them very attractive as mates. In itssimplest form it consists of a bilateral fold ofskin arising from the mastoid region but occa-

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Canad. M. A. J. RIPSTEIN: FRACTURES OF CLAVICLE 255Sept. 1948, vol. 59 i

sionally from in front of the ear and extendingto the shoulder. Usually it is thin but may bethick and can be picked up and stretched fromthe side of the neck. It consists of skin andsubcutaneous tissue although it has been sug-gested that there may be some abnormality ofthe platysma myoides. The deep fascia andmuscles are not affected. It is possible that theremay be some deficiency in elastic tissue butthere is no histologic evidence for this. Thehair-line is low posteriorly, giving the appear-ance of a 'short wide neck. The cervical vertebraeare normal and there is no impairment in move-ment. In most instances there is no mentalretardation but in some cases the conditionsuggests mongolism.

Rarely, however, does it occur as simply asthis but is associated with a wide variety ofmialformations. Because of its association withthese anomalies it is suggested that it is theresult of faulty development of the neck and isthus related to iniencephaly, Klippel-Feil syn-drome and Sprengel's deformity and that thesigns of these may overlap. Turner described aspecial subvariety exhibiting the triad, infantil-ism, webbing of the neck and cubitus valgus oc-curring in women. It has also been reported ina man. Endocrine deficiency, when present,may be regarded as secondary to the underlyingcongenital defect and not as a primary causeof the condition. Endocrine therapy has notbenefited growth but has caused some im-provement in the genital symptoms. In mostreports there is no mention of treatment of thedefect. One author suggested a bloodless ortho-pawdic procedure. The only surgical techniquesactually described were modifications of the"Z "-type plasty. General reference was madeto other plastic methods without elaboration.A technique is now described which is simple

and has the advantage of controlling both sidesthrough a single operative field and leaving thescar in an inconspicuous place. While it is ad-mitted that this case was not as advanced as

many, it is suggested from a study of the pub-lished illustrations that this technique will givebetter results than the "Z"-type plasty. Thelengths of the incisions naturally will vary andin extreme cases it might be necessary to repeatthe procedure, if sufficient improvement couldnot be obtained at the first operation, on thesame principle as the so-called French method ofscar removal.

REFERENCES1. KOBYLINSKI, 0.: Arch. f. Anthrop., 14: 343, 1883.2. SUTTON, J. B.: Evolution and Disease, Walter Scott,

London, 1890.3. SPRENGEL, 0. G. C.: Arch. f. klin. Chir., 42: 545, 1891.4. HUTCHINSON, J.: Quoted by Rechtman, A. M., and

Horwitz, M. T., Am. J. Roentgenol., 43: 66, 1940.5. LEWIS, H. F.: Anm. J. Obst. & Gyn., 35: 11, 1897.6. FUNKE: Deutsche. Ztschr. f. Chbir., 63: 162, 1902.7. BUSSlIERE, J. A.: Ann. d'hyg. et de med., Colon., 5: 686,

1902.8. BALLANTYNE, J. W.: Manual of Antenatal Pathology

and Hygiene-The Embryo, Wm. Green & Sons,Edinburgh, 1904.

9. ABBOTT, M. E. LOCKHART, F. A. L.: J. Obst, & Gyn.Brit. Emp., 8: 236, 1905.

10. CLARKE, J. J.: The Lazncet, 2: 1350, 1906.11. KLIPPEL, M. AND FEIL, A.: Nouv. Iconogr. de la

Salpetriere, 25: 223, 1912.12. Idem: Bull. et m6m. Soc. Anat. de Paris, 6s, 14: 185,

1912.13. DitACaT1TER, R.: Kli. Wchnschr., 2: 664, 1923.14. FRAWLEY, J. M.: Am. J. Dts. Child., 29: 799, 1925.15. DE BRUlN, M.: Am. J. Dis. Child., 36: 333, 1928.16. ULLRICH, 0.: Ztschr. f. Kinderh., 49: 271, 1930.17. BAUMAN, G. L.: J. Am. M. Ass., 98: 129, 1932.18. ROEDERER, C.: Bull. Soc. de pediat. de Paris, 31: 255,

1933.19. NAGEOTTE-WILBOUCHEWITCH, M.: Bull. Soc. de pediat.

de Paris, 32: 683, 1934.20. RANTASALO, V.: Acta. Pediat., 18: 368, 1936.21. HOFFMAN, II.: Arch. f. Kinderh., 110: 1, 1937.22. CHANDLER, F. A.: Amb. J. Dis. Child., 53: 798, 1937.23. MARQUARDT, W.: Ztschr. f. Orthop., 67: 379, 1938.24. TURNER, H. H.: Endocinology, 23: 566, 1938.25. MACCOLLUM, D. W.: New England J. Med., 219: 251,

1938.26. BIzZARRo, A. H.: The Lancet, 235: 828, 1938.27. TURNER, E. L., SHOULDER, H. S. AND SCOTT, L. D.:

Am. J. Roentgenol., 40: 43, 1938.28. SHARPEY-SCHAFER, E. P. AND SCHRIRE, I.: Clin. Sc., 4:

185, 1939.29. Idem: Quart. J. Med., 8: 195, 1939.30. SHARPEY-SCHAFER, E. P.: The Last,cet, 1: 161, 1940.31. RECHTMAN, A. M. AND HORWITZ, M. T.: Am. J. Roent-

genol., 43: 66, 1940.32. SYLVEST, 0.: Ugesk. F. Laeger., 102: 1270, 1940.33. CAPURRA, A. M.: Tesis de la Facultad de Ciencias

m6dicos de Buenos Aires, 1940.34. BELGRANO, C. AND CAPURRA, A. M.: Semacna Med., 1:

644, 1941.35. HAUPTMANN, A. AND THANNHAUSER, S. J.: Arch.

Neurol. & Psychiat., 46: 654, 1941.36. GILMOUR, J. R.: J. Path. 6 Bacteriol., 53: 117, 1941.37. SHARPEY-SCHAFER, E. P.: The Lancet, 241: 559, 1941.38. FLAVELL, G.: Brit. J. Surg., 31: 150, 1943.39. BABCOCK, W. W.: Principles and Practice of Surgery,

Lea and Febiger, Philadelphia, 1944.40. BETTMAN, A. G.: Plastic & Reconstructive Surg., 1:

205, 1946.

KIRSCHNER WIRE FIXATION INFRACTURES OF THE CLAVICLE*

Chas. B. Ripstein, M.D., C.M., F.R.C.S.[C.]Montreal, Que.

FRACTURES of the clavicle are extremely com-mon, but there is no method of treatment

which has found universal acceptance. Thissuggests that none is wholly satisfactory. Themajority of cases are treated conservativelyand despite inadequate reduction and immobi-lization firm bony union usually occurs.

There is, however, a small group in whichopen reduction and fixation is necessary for agood result. The indications for operation maybe listed as follows: (1) Oblique fractures of

* From the Department of Surgery, Royal VictoriaHospital, Montreal, P.Q.