POSTGRAD. MED. J. (1966), 42, 506.

Current Survey

CHRONIC COR PULMONALEM. K. TOWERS, M.A., M.B., M.R.C.P.

Cardiologist, Harefield and Clare Hall HospitalsResearch Assistant, Institute of Diseases of the Chest

DefinitionChronic Car Pulmonale is defined as right

ventricular bypertrophy due to impairment of theventilatory and respiratory function of the lungs.This is a narrower definition than that of theWorld Health Organisation (1961) and excludesprimary diseases of the lung vasculature whichhave a different iphysiological mechanism andclinical course.

Diseases causing Chronic Cor PulmonaleThe diseases causing Chronic Cor Pulmonale

listed Iby the World Health Organisation (1961)are:1. Diseases primarily affecting air passages of the

lung and the alveoli.1. Chronic bronchitis with generalized air-ways

obstruction with or without emphysema.2. Bronchial asthma.3. Emphysema without bronchitis or asthma.4. Pulmonary fibrosis, with or without emphy-

sema, due to:-(a) Tuberculosis(b) Pneumoconiosis(c) Bronchiectasis(d) Other pulmonary infections(e) Radiation(f) ;Muco-viscidosis

5. Pulmonary granulomata and infiltrations(a) Sarcoidosis(b) Chronic diffuse interstitial fibrosis(c) Berylliosis'(d) Bosinophilic granuloma or histio-

cytosis(e) Malignant infiltration(f) Scleroderma(g) Disseminated lupus erythematosus(h) Dermatomyositis(i) Alveolar microlithiasis

6. Pulmonary resection.7. Congenital cystic disease of the lungs.8. High altitude hypoxia.

II. Diseases primarily affecting the movements ofthe thoracic cage

1. Kyphoscoliosis and other thoracic deform-ities

2. Thoracoplasty3. Pleural fibrosis4. Chronic neuromuscular weakness-e.g.,

poliomyelitis5. Obesity with alveolar hypoventilation6. Idiopathic alveolar hypoven'tilation

PathogenesisIn the following discussion and in the account

of the clinical and other findings attention will beconcentrated on bronchitis and emphysema whichare the most important causes of pulmonary heartdisease.Cor pulmonale is always associated with disturb-

ances in the blood gases and a number ofmechanisms are involved. Chronic bronchitis andemphysema are characterised by airways obstruc-tion. In bronchitis it is due, partly at least, toexcessive secretion of mucus and in emphysemato the expiratory collapse of poorlysupportedsmallairways. Obstructed airways limit the rate atwhich air can move in and out of those parts ofthe lungs which they serve; many alveoli areunderventilated or not ventilated at all. There isgrossly uneven matching of ventilation 'to bloodflow in the lung; parts of the lung are wellperfused but poorly ventilated, whilst others arewell ventilated but poorly perfused. The wellventflated areas cannot compensate entirely forthe poorly ventilated; alveolar hypoventilationinvariably results in hypoxia, 'hypercapn'ia andacidos0is, and the overventilated alvecoli cannotsupply a sufficient excess of oxygen to counter-balance the hypoxia.

In 'pulmonary fibrosis and in 'the pulmonarygranulomata, ventilation-perfusion inequalities arethe main cause of blood gas changes. Kypho-scoliosis 'has been studied 'by Fishman (1965).Gross distortion of the chest increases the energycost of breathing, and rapid shallow 'breathing(the most economical pattern) is adopted. Thisresults in good ventilation of the dead space 'butpoor ventilation of the alveoli and leads to blood gaschanges. In the Pickwickian syndrome (obesitywith alveolar hypoventilation) diminished respira-tory drive is 'the most important abnormality.

Thus, a great variety of ventilatory and respira-tory faults can result in 'blood gas disturbances.In a patient with moderately severe bronchitisand emphysema 'the blood gases might be Po255 mm.Hg., Pco2 55 .mm.Hg. and ipH 7.3. (Normalvalues Po2 100, 'Pco2 40, pH 7.4). It is difficultto sort out the separate effects of anoxia,hypercapnia and acidosis on individual organs.

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from

TOWERS: Chronic Cor Pulmonale

The sum of these effects and their interaction onvarious organs and systems are the manifestationsof cor pulmonale. The lung vessels, the kidney,the haemopolietic system, the. brain, the peripheralcirculation, the liver and the heart are principallyinvolved.There 'has been doubt about the relative im-

portance of organic and functional changes inthe lung vasculature. Widespread loss of thepulmonary vascular bed is an important causeof pulmonary hypertension in some cases ofemphysema. Nevertheless, Fletcher, Hugh-Jones,McNichol 'and Pride (1963), have shown thatcor pulmonale occurs more often with chronicbronchitis than with emphysema. The partialreversibility of cor 1pulmonale also suggests func-tional rather than organic change in the lungvessels and contrasts with inexorable deteriorationin obliterative 'pulmonary hypertension. Vaso-constriction brought about 'by the interaction oflow alveolar oxygen tension and high bloodhydrogen ion concentration upon muscular pul-monary arteries of about 1 mm. in diameter(Harvey, 1965) is the most important cause ofpulmonary hypertension. Of course, as in systemichypertension, continual vasoconstriction can giverise to secondary organic changes in the vessels.

Blood gas disturbances probably cause bothrenal vasoconstriction and alterations in tubularfunction. The renal 'blood flow was low in all thepatients studied by A'ber, Bailey and Bishop,(1963), even when the cardiac outpult was normal.This is probably due to renal vasoconstriction. Inoedematous patients the low 'glomerular filtrationrate alone seemed insufficient 'to account for theexcessive sodium and water retention, so thattubular function is probably altered.Shaw and Simpson (1961) 'have shown that in

emphysema the marrow is stimulated prolbablyby tissue anoxia to produce more red bloodcells. This may lead to polycythaemia with anincreased haematocrit, but more often there is aconcomitant increase in plasma volume so thatthe haem'atocrit is normal even though the red-cellmass is increased.

Elevation of Pco2 dilates 'the cerebral vessels,raises the pressure of -the cerebrospinal fluidcausing headache, drowsiness and in extreme casespapilloedema (Hamilton and Gross, 1963). Asharp rise in Pco2 seems 'to be more importantthan the absolute level, 'but Campbell (1965) haspointed out that the more severe symptoms andreally high Pco2 levels are not found unlessoxygen has 'been given. Hypoxia and not hyper-capnia is the stimulus for the respiratory drive inthese patients. If hypoxia is relieved 'by givinghigh concentrations o oxygen, 'the Pco2 builds uprapidly and caribon dioxide narcosis supervenes.

Peripheral vasodilation causing the full. bound-ing pulse and warm skin characteristic of pul-monary heart 'disease ias attributed to rises in Pco?(Hamilton and Gross, 1963). Thomas, Rees andSaunders (1965) 'have show 'ithat liver function is

disturbed in pulmonary heart disease before theonset of "congestive failure". Hypoxia may bethe cause of the liver dysfunction.The cardiac output in pulmonary heart disease

has been extensively studied. (Wade and Bishop,(1962), reviewing the literature, concluded that thecardiac output is normal and falls only in thelate stages of the disease. Alber and others (1963)'have shown that the cardiac 'output may 'benormal even in the oedematous patient. Bycontrast the cardiac output is low in most otherkinds of "heart failure". This paradox high-lights our -present ignorance of the fundamentalphysiology of "heart failure". The term "conges-tive failure" is here used to describe -a clinicalsyndrome 'without implying 'a cause.A number of adverse factors affect the heart

in cor pulmonale. Right ventricular work is in-creased because the cardiac output is maintainedand there is 'pulmonary hypertension, even thoughthis is moderate (systolic 40 to 100 mm.Hg.)rather than severe. If functional tricuspid in-compeltence supervenes, the right ventricular workis further increased. Raised 'blood viscosity inpolycythemia also increases cardiac work. Theeffects of anoxia on the myocardium are difficultto assess, but must be unfavourable and mayaccount 'for left ventricular 'hypertrophy found insome patients with pulmonary heart disease inwhom there is no evidence of coronary arterydisease or hypertension '(Fluck, Chandrasekar andGardner, 1966).

Clinical FindingsTypically, 'the patient is a middle aged man with

a long history of Ibronchitis and recurrent chestinfections. An infection or a period of smog orcold weather causing an increase in cough, sputumand breathlessness and perhaps the 'appearance ofankle oedema, bring the patient to the doctor,Cough syncope, headache, drowsiness and palpita-tion are less common symptoms.The patient is cyanosed and 'the warm skin

indicates central and not peripheral cyanosis.There may or may not be clubbing of the fingers.The chest is held in the inspiratory position andis hyper-resonant. Usually there are expiratorywheezes and coarse crepitations. The jugularvenous pressure is normal or raised if 'there is"congestive failure". The 'a' and 'v' waves areequal. The arterial pulse is of full volume andrapid in acute exacerbations of cor pulmonale.Sometimes an overactive right ventricle can befelt and P2 (the sound of pulmonary valve closure)may be palpable. MoTe often the cadiac impulsesare 'impalpable and the heart sounds distantbecause the lungs partly cover the heart. Onauscultation P2 may be increased but often itcannot be heard and a right atrial gallop, bestheard alt the lower end of the sternum, is theonly sign. The gallop is characteristic because itis much louder on inspiration, not 'only becausethe atrial sound 'becomes louder but also because

August 1966 507

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from

POSTGRADUATE MEDICAL JOURNAL

JA191

II

I

VI V2 V3 V4 VS V6

XBB

MAY1963

VIV2 V3 V4 VS V6CHEST LEADS N/2

FIG. 1-Electrocardiograms during and after an acute chest infection in a patientwith Cor pulmonale.

In the upper tracing, sinus tachycardia, P pulmonale, right axis deviation(+ 110), rS pattern across the chest and T wave inversion over the rightchest. After treatment the rate is slower, the right axis deviation less (+95),the T wave is upright over V 2 and 3, and the rS pattern does not extendacross the chest.

it tends to move away from the 4rst sound andis more easily heard. In more wvere oases vithgross oedema -the atrial galkop is replaced (by atricuspid pansystolic murmur and third sound.After treatment the atnal gallop bcomnes soteror disappears and the pedipberal circulationreturns to nonmal.

EletrocardiogramThe electrocardiographic saigns of co pulmonale

are a P pulmonale, right axis deviation of themean QRS frontal vector (or occasionally an Si,S2, S .pattern) and

either an rS pattern in the chest leads (thecommonest pattern). In a less fullydeveloped form there is an Rs pattern

508 August 1966

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from

TOWERS: Chronic Cor Pulmonale

II III VR VL

V3 V4 VSFIG. 2-Classical right ventricular hypertophy pattern

pulmonale, right axis deviation (+110), dominantover the right chest and conspicuous S waves in V6.

over the left chest. The T wave is grapihic patt4usually upright in 'the right chest axis deviatileads 'but may be inverted during conclusive eexacerbations. trophy. Thi

or a classical right ventricular hyper- asthmatic attrophy pattern with a dominant R or or two, soR1 in VI and inverted T waves in the stress ratherright dhest leads. through the

or right 'bundle 'branch iblock. an rS pattera 1long peric

Right axis deviation is due to right ventricular right ventrichypertrophy consequent on pulmonary hyper- indicate moftension 'and the P pulmonale indicates right atrial Co-existinhypentrophy due to increased filling resistance of the electrocthe hypertrophied right ventricle. The rS pattern monale diffiin the chest leads is thought to be the result of QRS frontalchanges in the position of the heart relative to cardiographithe chest electrodes. The heart is rotated about (1964), showits long axis by hypertrophy of the right ventricle be diagnoseand overinflation *of 'the lungs may lower the coronary hediaphragm and heart in relation to the chest Granit '(1952electrodes. lungs may coThe most satisfactory evidence of right ventri- vertical dire

cular hypertrophy 'is an increase in the weight of mediastinuumthe right ventricle when it has been dissected devi.ation. 1from the heart. There is good correlat-ion lbetween many of th4right ventricular weight and right axis deviation group.and P pulinonale in the electrocardiogram re- A patientcorded before death (Millard, 1966), but the the first timzcause of the rS pattern remains iobscure. pulmonary 4The diagnosis of cor pulmonale can be made pulmonale i

with confidence if the clinical circumstances are the P 'waveappropriate and one of the classical electrocardio- RS pattern

in Cor pulmonale. PR in VI, inverted T's

;erns is found. A combination of righton and a P pullmonale alone is notwvidence of right ventricular hyper-is pattern can occur in very severettacks and resolve completely in a daythat it can indicate right ventricularthan hypertrophy. Many ,patients goclinical course of their disease with

rn in the chest leads. In others, overAd, there is a transition to a classical:ular hypertrophy pattern which mayre severe pulmonary hypertension.Ig ischaemic heart disease may malke,ardiographic diagnosis of cor pul-icult, especially by 'altering the meanI vector. Using conventional electro-ic criteria Rees, Thomas and Rositerved that pulmonary heart disease coulded accurately in 72% of patients,,art disease in 84% and both in 55%.7) suggests that overinflation of theause electrical forces to run in a moreection through the better-conductingn and thus give rise to 'left axisrfhis idea is unproven and, of course,tese patients are in the coronary age

with cor pulmonale !may tbe seen fore in a state of vasomotor collapse andembolism has to 'be considered. A Pis moTe often seen in cor pulmonale;axis tends to be +70° or more, an

-is more often seen in the left sided

I VF

V6VI V2

.1.14 ....... I 1 5.644". . .

I'llilial LjjjLJLJLJLAILJam ..............I......

.... .......................................I... 1. - .... -.. I........

. I. . . . . . . . . . .I - - - ff i i 2 i i ff a i 2 i o i N i i iff 11+14;444...

I I I I I I I A a I I I I I b I I I I a.LJLLJ I I I I LLLLA I a a I 164 1 A LLA.U I i I I I

.......... 4.1 .... 11.1 . . A. . ....... . i .. . .. ..--l I. .- di i u I .1 . .16,- ...- . ff L"h-Ah

509Augitst 1966

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from

POSTGRADUATE MEDICAL JOURNAL

FIG. 3-Cor pulmonale with chest infection. Largeheart, prominence of pulmonary artery and of rightmain branch. Infective shadow at right base.Right dome of diaphragm at level of sixth rib.

chest leads and the T wave tends to be uprightin the right chest leads (Caird and Stanfield, 1962).In the presence of cor pulmonale the electro-cardiographic diagnosis of pulmonary embolismis almost impossible.

Disturbances of rhythm are not uncommon incor 'pulmonale. Atrial ectopic beats are often seenand sometimes prolongation of the P-R interval.Atrial 'fibrillation is rare but paroxysmal atrialtachycardia with block occurs (Corazza andPastor, 1958) usually in patients who are very ill.The possibility of digitalis-induced arrhythmiashould be kept in mind.

Chest X-rayPulmonary heart disease causes enlargement of

the right heart and pulmonary artery. In emphy-sema the heart shadow may ibe abnormally smalland early enlargement is only discernible if aprevious film is available for coparison. In thebronchitic patient with cor pulmonale an acutechest infection may cause considerable enlarge-ment of the heart. When the infection is con-trolled the heart shadow may return almost to itsoriginal size.The radioloyv of emphysema 'has 'been reviewed

'by Simon (1964). The signs are (i) Excess of airin the lungs shown (by a low flat diaphragm (thecentral portion of the right dome being on

FIG. 4-Same case after treatment. Heart smaller.Right dome of diaphragm below level of seventhrib. Hypertranslucent area at left base.

inspiration at or 'below the seventh rib anteriorly),and a large retrosternal translucent area seen inthe lateral film. (2) Cardiovascular changes. Theheart is narrow and vertical. Slight prominenceof the main pulmonary artery is due sometimesto partial rotation of the heart, and sometimes todilatation of the artery from pulmonary hyper-tension or surges in pressure on effort. There isa combination of large hilar vessels and smallmid-lung vessels. (3) The presence of bullae orbullous areas. A patient with emphysema severeenough to cause pulmonary heart disease willshow two or all three of the X-ray features.When a patient with emphysema develops oedemathe heart enlarges and -the diaphragm rises so 'thatthe radiological diagnosis of emphysema may not'be obvious 'but it can readily be made from-previous films if they are available.There are no specific X-ray changes in bron-

chitis. Broncthograms if available may showenlarged ducts of mucus -glands, peripheral non-fillin~g and dilatation of the terminal bronchioles.Other diseases causing pulmonary heart diseasemay present more specific appearances.

TreatmentCor pullmonale is a late stage in the natural

history of chest disease, usually broncbitis lindemphysema. Though dramatic improvements may

510 August 1966

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from

August 1966 TOWERS: Chronic Cor Pulmojwk 511

be achieved in exacerbations, the disease is (pro-gressive and incurable. Effort should therefore beconcentrated on arresting bronchitis at an earlierstage. The excessive secretion of mucus character-istic of bronchitis is maintained largely at least,by bronchial irritation. Smog, smoke, industrialand traffic fumes are important irritants andshould be avoided, but for the majority of patientscigarette smoking is much the most potent andconstant bronchial irritant. The patient shouldunderstand clearly the danger of smoking. Heshould be encouraged to give it up and wherepossible attendance at an anti-smoking clinic mayhelp (Ball, Kiilby and Bogen, 1965). Chest in-fections should be treated promptly and H.influenzae, the pneumococcus and occasionallythe staphylococcus are usually involved '(May1965). Amapicillin and tetracycline are the mostuseful drugs, and should be continued indefinitelyif the sputumbecomes purulent when treatment isstopped. Physiotherapy may be helpful especiallywhen it can 'be arranged for groups of bronchiticpatients.The patient presenting with a chest infection,

cor pulmonale and oedena, requires treatment forrespiratory failure and for congestive failure.The detailed treatment of respiratory failure isoutside the scope of this article. The most im-portant measures are the administration ofoxygen )by Venturi mask (limiting the concentra-tion to 25-29% of oxygen *to avoid carbondioxide narcosis), appropriate antibiotics, respira-tory stimulants such as nikethamide intravenously,bronchodilators, steroids, physiotherapy and ifnecessary tracheostomy and assisted respiration.Digitalis and diuretics are igiven as for congestivefailure. Digitalis is of marginal value but isprobably worthwhile giving for its inotropiceffect. Great care should 'be taken to avoiddigitalis toxicity to which the elderly patients areespecially liable (Schott 1964). If major disturb-ances of atrial rhythm occur in the undigitalisedpatient, digitalis should be given. Strenuousattempts to restore normal rhythm with drugs orD.C. shock are often unsuccessful. When theyare successful the improvement in the patient isapt to 'be disappointing. If digitalis is suspectedas the cause of 'the arrhythmia propranolol is thedrug of choice.

Complications and PrognosisTwo important complications of therapy, carbon

dioxide narcosis and digitalis intoxication, havebeen discussed. A third, cerebral oedema, thoughdirectly due to cerebral hypoxia may be 'broughtabout by 'too rapid a lowering of the Pco,(Hamilton and Gross, 1963). Cerebral vasodila-tion produced 'by hycapnia prdtects the brain,partly at least, from hypoxia. The rapid abolitionof this vasodilatation leaves the brain especiallyvulnerable to hypoxia and irreversible coma orhemiplegia may follow the cessation of oxygentreatment. A further unusual complication of

pulmonary heart disease described by Hamiltonand Gross (1963) is pulmonary artery thrombosisin situ, probably the result of pulmonary vasculardisease from longstanding pulmonary hyper-tension. It should be suspected if a patient withpulmonary heart disease develops intractable con-gestive failure in spite of improving respiratoryfunction.

In the patient with "pure" bronchitis congestivefailure is usually precipitated by a respiratoryinfection. The patient responds well to treatmentand may -be 'able to return to work. Severalepisodes of congestive failure may occur over aperiod of years, the patient being slightly worseafter each one. In contrast, -the patient -with "pureemphysema" often develops congestive failureafter a long period of slow deterioration andresponds poorly to treatment (Fletcher andothers, 1963). Presumably this is because of thelarger irreversible element of pulmonary vasculardamage in the emphysematous patient.

REFERENCES

ABER, G. M., BAILEY, T. J., and BISHOP, J. M. (1963):Inter-relationships between Renal and CardiacFunction and Respiratory Gas Exchange in Obstruc-tive Airways Disease, Clin. Sci., 25, 159.

BALL, K. P., KIRBY, B. J., and BOGEN, C. (1965): FirstYear's Experience in an Anti-smoking Clinic, Brit.med. J., i, 1651.

CAIRD, F. I., and STANFIELD, C. A. (1962): The Electro-cardiogram in Asphyxial and in Acute Embolic CorPulmonale, Brit. Heart J., 24, 313.

CAMPBELL, E. J. M. (1965): Respiratory Failure, Brit.med. J., i, 1451.

CORRAZZA, L. J., and PASTOR, B. H. (1958): CardiacArrhythmia in Chronic Cor Pulmonale, New Engi.J. Med., 259, 862.

FISHMAN, A. P. (1965): Pulmonary Aspects of Scoliosis.Proceedings of a Symposium on Scoliosis (Ed. P. A.Zorab), p. 79. London: National Fund for Researchinto Poliomyelitis and other Crippling Diseases.

FLETCHER, C. M., HUGH-JONES, P., MCNICHOL, M. W.,and PRIDE, N. B. (1963): The Diagnosis of PulmonaryEmphysema in the Presence of Chronic Bronchitis,Quart. J. Med., 32, 33.

FLUCK, D. C., CHANDRASEKAR, R. G., and GARDNER,F. V. (1966): Left Ventricular Hypertrophy in ChronicBronchitis, Brit. Heart J., 28, 92.

GRANT, R. P. (1957): Clinical Electrocardiography:The Spatial Vector Approach, p. 134. New York:McGraw-Hill Book Co.

GROSS, N. J., and HAMILTON, J. D. (1963): Correlationbetween the Physical Signs of Hypercapnia and theMixed Venous Pc02, Brit. med. J., ii, 1096.

HAMILTON, J. D., and GROSS, N. J. (1963): UnusualNeurological and Cardiovascular Complications ofRespiratory Failure, Brit. med. J., ii, 1092.

HARVEY, R. M. (1965): The Influence of HydrogenIon in the Control of Pulmonary Artery Pressurein Patients with Obstructive Disease of the Lungs.Progress in Research in Emphysema and ChronicBronchitis (Ed. R. S. Mitchell), p. 108. Basel(Switzerland): S. Karger.

MAY, J. R. (1965): The Bacteriology and Chemotherapyof Chronic Bronchitis, Brit. J. dis. Chest, 59, 57.

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from

512 POSTGRADUATE MEDICAL JOURNAL August 1966

MILLARD, F. J. C. (1966): Brit. Heart J., to be published.REES, H. A., THOMAS, A. J., and RossITER, C. (1964):The Recognition of Coronary Heart Disease in thePresence of Pulmonary Disease, Brit. Heart J., 26,233.

SCHOTT, A. (1964): Observations on Digitalis Toxicity--a Plea, Postgrad. med. J., 40, 628.

SHAW, D. B., and SIMPSON, T. (1964): Polycythemia inEmphysema, Quart. J. Med., 30, 135.

SIMON, G. (1964): Radiology and Emphysema, Clin.

Radiol., 15, 293.THOMAS, A. J., REES, H. A., and SAUNDERS, R.A. (1965):

Liver Function in Pulmonary Heart Disease, Brit.Heart J., 27, 791.

WADE, 0. L., and BISHOP, J. M. (1962): Cardiac Outputand Regional Blood Flow. Oxford: BlackwellScientific Publications.

WORLD HEALTH ORGANISATION TECHNICAL REPORTSERIES No. 213. (1961): Chronic Cor Pulmonale.Geneva: W.H.O.

TRIGGER THUMB IN INFANCYA. P. PATEL, F.R.C.S.E.

Department of Orthopaedics, The West Hill Hospital, Dartford, Kent

STENOSING tenovaginitis of the flexor tendons of thehand causing trigger or snapping finger in adultsis a well recognised condition, but in young childrenis often misdiagnosed.Tubby (1912) considered it to be congenital,

Whitman (1927) described it as "not infrequentlyseen in infancy," and it has been described by Jahss(1936), Zadek (1942), Rose (1946), Spreecher (1949),Fahey and Bollinger (1954).

Clinical featuresTen young children with trigger thumbs were

seen at The West Hill Hospital, Dartford, rangingin age from six months to four years, and of these,two were bilateral and eight were unilateral. Ahistory of trauma was often given by the parentsbut in this series it was thought that the injury wasincidental and had merely drawn attention to analready existing deformity. In one child with boththumbs affected, the deformity was noticed by themother from birth. In the rest, it was present fromsix to twelve weeks prior to the parents seekingmedical advice.

All the children presented with a flexion de-formity of 15 to 50 degrees on the interphalangealjoint of the thumb, and attempts to correct it byforce only caused pain. The other constant findingwas the presence of a distinct palpable nodule overthe volar surface of the neck of the first metacarpal.In none of the children could snapping or triggeringbe obtained, (Fukase, Fukada and Yamaguchi,1961).

PathologyNormally, the tendon sheath of the digit at the

level of the metacarpophalangeal joint is slightly

thicker and acts as a pulley. In trigger thumb ofadults, this pulley is constricted and thickened andproduces enlargement of the tendon on each side.The pathological lesion seems to be different inchildren, and in five children operated on in the lasttwelve months, no such undue thickening of thepulley was found. Instead, a large bulbous nodulein the tendon was met with at this level. Distal tothis nodule, the pulley appeared to be constrictedbut this was rather relative to the size of the tendon.

Biopsy of the tendon nodule showed normaltendon tissue infiltrated with lymphocytes and mono-cytes (Spreecher, 1949), and evidence of colliquativedegeneration (Fahey and Bollinger, 1954). This isconsistent with a post-traumatic reaction of thetendon.

TreatmentConservative measures are unlikely to succeed.

Passive manipulation of the flexed thumb is painfuland the condition returns at a later date. In one,boy of five, such manipulation resulted in lockingof the thumb in extension and operation was neces-sary to relieve it. The condition when left untreatedresults in permanent flexion contracture, and awoman of 45 was seen with such a deformity givinga clear history of the condition existing since earlyinfancy.

Operative treatment is, therefore, advised.Through a small transverse incision, the nodule isexposed and the digital nerves on each side of thetendon are protected. The constricting pulley distalto the nodule is incised and the interphalangealjoint is extended fully. It has been found unneces-sary to interfere with the nodule in the tendon. Onlythe skin is sutured.

copyright. on 4 A

pril 2019 by guest. Protected by

http://pmj.bm

j.com/

Postgrad M

ed J: first published as 10.1136/pgmj.42.490.506 on 1 A

ugust 1966. Dow

nloaded from