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Vascular diseases of the extremities V. Raynaud's disease
VASCULAR
DISEASES OF THE EXTREMITIES
V. RAYNAUD’S
DISEASE *
AMOS MAVERICK GRAVES, M.D. NEW ORLEANS, LA.
A
usuaIIy absent, as we11 as paraIysis, aIthough other evidences of disturbed vasomotor innervation, aphasia, hemoglobinuria, and arthropathies may occur.
UGUSTE MAURICE RAYNAUD, in 1862, at the age of thirty presented for his doctor’s degree a thesis entitIed “LocaI Asphyxia and Symmetrica Gangrene of the Extremities.” AIthough the disease which he so masterfuIIy described had been observed by Schrader, in 1629, and Hertius, in 1685, the foIIowing excerpt from his monograph shows that it had remained for him to estabIish the causative factor of this symmetrica mortification.
ETIOLOGY A wide difference of opinion is found to exist among medica authors as to the predisposing and direct causes of Raynaud’s disease. However, it is agreed that the symptomatoIogy is dependent upon a vasomotor disturbance. Raynaud, Monro, Barker, OsIer, Buerger, BarIow, and others have suggested a hereditary factor, but more recent studies seem to prove that Buchanan and Monahan are correct in definiteIy ruIing out any inherited predisposition. The Iatter found a neurotic temperament to exist in onIy haIf the cases reviewed. SyphiIis, malaria, typhoid fever, typhus fever, influenza, and other conditions which give rise to a toxemia have been thought to be etioIogic factors, but now they are mereIv regarded as having occurred in association with the disease. SexuaI excess and excitation were suggested as causes by Raynaud, but these, no doubt, shouId be Iisted as contributory factors, aIong with exposure, trauma, psychic exertion, sudden fright, and acute infections. That exposure to cold precipitates an attack can be demonstrated in the majority of cases. Monahan beIieves that in view of the steriIity of the discharge from diseased tissues one must Iook for the fundamenta1 cause in some constitutiona abnormaIity or subnormaIity, which might produce a chemicophysioIogic toxin.
I propose to show that there exists a variety of dry gangrene affecting the extremities which is impossibIe to expIain upon the basis of vascuIar obliteration; a variety characterized especiaIIy by a remarkabIe tendency to symmetry since rt aIways affects simiIar portions of the body, the two upper extremities, the two lower extremities, or a11 four at the same time: In certain instances the nose and ears are aIso affected. I shaI1 seek to prove that this kind of gangrene has as its cause a disturbance of the innervation of the capiIIary bIood vesseIs. CLINICAL PICTURE CIinicaI characteristics are conciseIy described foIIows :
of a typica case by Cassirer as
Somewhere in the peripheral portions of the body there occurs more or Iess severe pain. This is not confined to distinct nerve territory, but usually affects symmetrica parts. Attacks of vasomotor and trophic disturbances are part of the syndrome, to wit: (I) syncope, asphyxia, or IocaI rubor, and (2) severe trophic disturbances, usuaIIy in the form of gangrene of the parts first affected with symptoms. The course is an intermittent one, for there may be compIeteIy free intervaIs; but in some instances, evidences of disturbed vasomotiIity may persist. The disease may consume itseIf in one attack or several attacks may occur in succession. ObjectiveIy, sensory disturbances are
INCIDENCE most of Age. According to Raynaud, the cases occur between the ages of eighteen to thirty years. Monro gives the average
* Presented before the Surgical FacuIty of T&me University, New OrIeans, March 17, 1930. Previous articles on Vascular Diseases of the Extremities appeared as follows: I. ArterioscIerotic disease, vo1. IZ, p. 32; II. Erythromelalgia, vo1. 12, p. 40; III. Thermic Gangrene, vo1. 12, p. 324; IV, Thromboangiitis obliterans, vol. IZ, p. 489.
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age of onset as 28.9 vears, and Morgan in his 93 cases found It to be 26.6 years. Cassirer cohected 168 cases and found that there were 55 in which the disease occurred in the first and second decades of Iife as compared with 40 in the third decade. Subsequently, in studying 109 cases, he found the greatest age incidence from twenty-one to forty-years of age. Of 67 cases studied by Buchanan at the Mayo Clinic, 36 were in patients from twenty-one to forty years of age. That the disease may frequentIy occur in children is evidenced by the fact that Cassirer coIIected 22 cases occurring in patients less than five years of age. In the more recent Iiterature there are numerous reports of its occurrence in infants and in the newIy born. Not infrequentIy the disease is reported to have been observed in individuaIs of advanced years; but that these are cases of true Raynaud’s disease is to be doubted except in those few in which the symptom-compIex was characteristic. E. P. Henry reported a case in which the phenomena occurred in the ears, nose, and extremities in a woman seventy-seven years oId. Sex. Women are more frequentIy affected. Raynaud found that 80 per cent of his cases were in women; 6.2 per cent of Monro’s cases were femaIes, and this sex predominated in 62.9 per cent of Cassirer’s series and in 59.8 per cent of Buchanan’s series. Adson and Brown beheve that with improvement in diagnosis the incidence of true Raynaud’s disease in the maIe wiI1 drop to Iess than 5 per cent of cases. The racia1 and geographica distribution has been shown to be fairIy widespread. Three cases have been reported in the negro by Lee. That Raynaud’s disease is rare has been shown. Monro reports that it occurs about once to every 3000 other affections and Buchanan found that it comprised Iess than 0.02 per cent of registrations at the Mayo CIinic. At the Johns Hopkins Hospita1 there were 19 cases in 23,000 admissions. Buchanan states that he could not demonstrate that physica or nervous
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strain incident to any one type of occupation or social stratum was a cause of the condition. MODES
OF
ONSET
AND
DURATION
Sixty out of Buchanan’s 67 patients described a sudden onset with portions of the body turning white, blue or bIack. Five described a gradua1 onset consisting of tingIing, numbness, cIumsiness, or indescribabIe discomfort occurring at irreguIar intervaIs, foIIowed at a Iater date by the coIor phenomena. Raynaud described the onset of the disease as sudden and the duration as short. Other earIy observers gave the same conception; but with a more widespread knowIedge of its symptomatoIogy and better methods of diagnosis the chronic and insidious forms of the disease have been recognized and not infrequently reported. a case in which Monahan reports prodroma1 symptoms Iasted for nine months before the acute stage occurred to persist for four years before amputation was resorted to. Many of the miId cases require exposure to coId to produce the characteristic symptoms. These may cIear up and remain especiaIIy if winters are symptom-free, spent in tropical zones. However, most cases remain remittentIy active, although some may never advance to the moderate or severe forms. Buchanan reported that 30 of his cases had had symptoms for more than five years and of these 7 had been affected for from fifteen to thirty years and I for fortythree years. Thus, it is evident that the duration is not aIways short. That the disease may spontaneousIy subside cannot be doubted. Proof of this cannot readiIy be obtained, as recovered cases are usuaIIy Iost before a conchrsive period of observation is ended. CertainIy many miId cases remain undiagnosed or unobserved simpIy because they subside or do not progress to the extent that the cIassica1 picture is evident.
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Even cases of moderate severity may progress slowly and subside after several attacks which have produced small areas of gangrene. Osler states that the severe cases are exceptional, although every large hospital is likely to have one that returns at intervals for amputations unti1 the victim finally drifts into an aImshouse. SYMPTOMATOLOGY
Three characteristic phenomena of the disease are generahy described. These are: (a) local syncope; (6) IocaI asphyxia; (c) IocaI or symmetrical trophic lesions or gangrene. The term “local syncope ” was used by Raynaud to signify the blanching of a part. It is manifested in the fingers, toes, cheeks, ears or nose and Iess frequently in other parts by a sudden death-like, demarcated paIlor and a decrease in surface temperature of from 15 to 20~. This condition Iasts from a few minutes to a few hours and may rarely result in gangrene. It usuaIIy recurs with or without periodicity and is likely to be precipitated by exposure to cold. Associated with it is a feeling of numbness, tingling or coIdness. The attack may be terminated by a painful reaction. In only 3 of Monro’s 176 cases IocaI syncope existed alone, and Buchanan noted it alone in 15 per cent of his cases. It occurred either concomitantly or associated with local asphyxia in IO per cent of Monro’s and in 42 per cent of Buchanan’s cases. Boyer originated the term “local asphyxia, ” but it was Raynaud who applied it to that phenomena of the disease which usually foIlows, but may rarely precede syncope. As the term implies, there is a IocaI anoxemia which causes the skin to appear bluish white, dusky blue, slate coIor, dark blue, violet gray or purple, or there may be redness with mottling of degrees of blueness. Surface temperature is decreased less than it is in syncope, and there is often a temporary sweIling of the part or a cold
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sweat may appear. If this phenomenon persists, the skin becomes black and mortified, or there may result desquamation of the epidermis. Occasionally an incomplete asphyxia may persist for years to produce a false edema as a resuIt of undernutrition to the part. Local asphyxia is more prone to recurrences and is usually accompanied by more severe pain than is syncope. This phenomenon occurred alone in 16 per cent of Monro’s cases and in 6 per cent of Buchanan’s. When it occurs simultaneousIy with syncope, there is a play of colors with many shadings from cadaveric white to several tints of blue or bhrish red, or the phalanges may be white while proximalIy there is a change to a bluish tint. Pressure over an affected part displaces the color which CharacteristicaIly does not reappear for some time. Following either syncope or asphyxia there may follow a period of hyperemia in which redness is marked and is accompanied by increased pulsations, increased warmth of the part, increased moisture, and a tingling sensation approaching that of pain. With recurrence and persistence of the phenomena discussed gangrene of the eventualIy results. A peripheral parts bleb may form at the tip of the fingers and discharge a blackish or serosanguineous content. The resulting ulcer usuaIly heaIs by cicatrization. Not infrequently a finger tip may merely become thickened and indurated and later desquamate. More commonly there occur sharply demarcated bIackened, symmetrical areas which remain dry for variable periods of time before sloughing. These areas may be massive, causing the Ioss of a leg or forearm or HeaIing by even a whole extremity. granuIation with the formation of a tapering cicatrix is characteristic. Although the gangrene is usualIy of the dry variety, it may at times be moist. Pain in this disease is an important symptom as it almost constantIy occurs though it may be absent in one attack and present in another. It may precede an
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attack; but usuaIIy it coincides with asphyxia or syncope as a painfuI numbness folIowed by a sensation of burning and tinghng. It is exaggerated by pressure or the occurrence of gangrene and may occasionaIIy radiate to unaffected extremities. Heat and coId sensations are not changed, but rarely is there analgesia or diminution in tactile sensation. In a11 phenomena the skin is IikeIy to be covered with a cIammy moisture. Motor disturbances are not observed unIess muscuIar atrophy occurs. Bone changes were first observed by Cassirer. CharacteristicaIIy, one or more or a11 the termina1 phaIanges may become atrophic or disappear without the formation of a sinus. Long bones may aIso become invoIved as manifested by a thinning of the cortex and an increase in the size of the marrow cavity in an occasiona case. PuIsations in the dorsaIis pedis, posterior tibiaI, and radia1 arteries are commonIy thought to be absent during the stage of syncope. This is incorrect as puIsations, though at times diminished, may be eIicited in a11 stages of the disease. BIood pressure determinations, p&e rates, and bIood pictures were found to be within normaI Iimits in a11 cases at the Mayo CIinic. HemogIobinuria was not observed in any case, though it has been reported by many authors. AREAS
INVOLVED
Of the cases reported by Buchanan the Iesions were biIatera1 and symmetrica in 56.7 per cent and asymmetrica in 43.3 per cent. Buerger states that symmetry is occasionaIIy Iacking and reports of isoIated cases in the Iiterature substantiate this. Not infrequentIy the nose, Iips, and sterna1 regions are affected and very rareIy multiple gangrenous areas occur anywhere on the trunk. Monro found the upper extremities were affected in 43 per cent of cases, the Iower extremities in 24 per cent, and both in 22 per cent. Buchanan found the Iower extremities more frequentIy affected, but
Disease he reports quite a diverse distribution in some cases. Nineteen patients had Iesions a11 over the body, one on the side of the tongue, one in the pharynx, one on the Ieft side of the face and one in which both forearms and Iips sIoughed and heaIed by cicatrices. That this disease has an even more widespread anatomica distribution is beIieved by many. Raynaud himself noticed that there were coincident aIterations in the retina1 vesseIs with impairment of vision in 2 of his cases when the characteristic phenomena occurred in the extremities. Many cases with like symptoms have been reported, the most recent being those of ShinkIe and AppeIbaum. Weiss reported a remarkabIe case in which the Ieft cervical sympathetic nerve was invoIved causing the Ieft chest suddenIy to become warm and red, the Ieft eyebalI to retract, hyperhidrosis, and contraction of the pupil. A recession of a11 manifestations except for sIight retraction of the eyebalI was compIete within a week, but recurrences occurred unti1 finaIIy a condition simiIar to a partia1 atrophy of the soft parts of the Ieft side of the face remained. HemogIobinuria in association with Raynaud’s disease was not observed by Raynaud. Hutchinson in 1871 recorded the first case in which this symptom occurred and subsequentIy simiIar cases were recorded by Druitt, WiIks, Southey, Barlow, Dickinson, Abercrombie, Monro, Spencer, and many others. ScIeroderma and ScIerodactylia not infrequentIy occur in association with the typica phenomena of Raynaud’s disease and have been thought by some to be mereIy a complication of Raynaud’s disease. CertainIy at times when either is present, it is impossibIe to ruIe out Raynaud’s disease. Raynaud referred to fibrous ankyIosis of the termina1 phaIangea1 articuIations and to thickening aIong the processes of the paImar fascia and showed how remarkabIy such thickening may subside foIIowing recovery from proIonged IocaI asphyxia.
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Southey reported a case in which there was an effusion in both knee joints. OsIer states that in a large number of mentaI disorders attacks, of Raynaud’s Mania, disease have been observed. meIanchoIia, circuIar epiIepsy, amentia, insanity, and progressive paraIysis of the insane have been reported to occur in association with the disease. Not a few cases exhibiting the characteristic phenomena have deveIoped transient monopIegias, hemiplegias, aphasias, and It seems reasonabIe to even parapIegias. regard these attacks as due to vascuIar changes in the brain of the same character as those which occur in the peripheral vessels. PATHOLOGY
The most frequent Iesion observed in Raynaud’s disease is the gangrene which is usuaIIy of the dry variety. It is sharpIy demarcated and remains so until the sIough comes away. Infection rareIy occurs and healing readiIy takes pIace by granuIation. When a periphera1 part is involved, there resuIts a characteristic tapering cicatrix. BIeb formations with resuIting uIcerations may be observed or there may occur dry dystrophic processes in which a dark scab forms and subsequentIy becomes detached in a scaIe-Iike manner. That the characteristic Iesions are due either to constriction of the capiIIaries or to diIatation and stasis in them has Iong been assumed, but it was not unti1 1925 that Brown and others made observations with the capiIIary microscope which satisfied a11 doubts concerning the type of Iesions. Brown concIuded from a study of 8 cases under various conditions and in different stages of the disease that in IocaI syncope there is a constriction of a11 three vascuIar eIements in the skin and that in IocaI asphyxia there is a refiIIing of diIating capiIIary 100~s from segments of arteria1 or venous bIood or both. In this Iatter stage there is apparentIy a Ioss of coordination between the capiiIaries and arterioIes.
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The arteries stiI1 remain contracted, except for an intermittent reIaxation which aIIows smaI1 segments of arteria1 bIood to enter the diIated capiIIary loops. The venuIes apparentIy undergo simiIar reactions since a reflux of bIood takes pIace DiIatation of the into the capiIIaries capiIIaries precedes the cyanosis, and it is this change in caIiber together with a Ioss of tone which causes stasis and a resuIting increase in carbon dioxide content of the blood. PROGNOSIS
Prognosis as to Iife of an affected part has been poor in the past and amputations have been frequentIy performed in order to to aIIeviate pain. It seems reasonabIe assume that for the most part onIy the more advanced or severe cases were observed, and consequentIy the oIder writers have given a gloomy outIook for patients affected with Raynaud’s disease. With an increasing number of earIy cases being recognized and observed for years and with the advent of more satisfactory therapy, we are finding that an affected part may remain usefu1 for many years in not a few cases. DIAGNOSIS
The diagnosis of Raynaud’s disease is made on the tinding of a combination of the characteristic phenomena. The mere observance of ischmeic fingers hardIy warbut the absence of rants a diagnosis; singIe symptoms in a case which has been adequateIy studied and observed for a suffrcientry Iong period shouId onIy rarely render the diagnosis uncertain. Differentiation of Raynaud’s disease from other vasomotor and trophic neuroses is occasionaIIy difflcuIt and may be impossibIe without proIonged observation. Certain cases of scIeroderma, especiaIIy those exhibiting scIerodactyIia, manifest vasomotor symptoms and are believed by some to be caused by Iesions of the sympathetic nervous system. Numerous cases are on record in which Raynaud’s phenomena occurred CoincidentaIIy with,
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preceded, or folIowed the deveIopment of the indurative and atrophic stages of scIeroderma and scIerodactyIia. Since in Raynaud’s disease there may not infrequentIy deveIop induration and thickening of the skin and subcutaneous tissues, a differentiation from scIeroderma may be impossibIe unti1 the skin Iesion spreads to other areas in which manifestations of a vasomotor disturbance are not evident. The absence of pain and paresthesia is of vaIue in eIiminating Raynaud’s disease; but when pain and paresthesia are present, one shouId consider that the two diseases coexist unti1 further observation proves otherwise. ErythromeIaIgia shouId offer no difflculty in differentia1 diagnosis. The attacks are precipitated by heat or dependency of the affected part. The redness, increased temperature, and pain disappear on eIevation of the part and are aIIeviated by the appIication of coId, whereas in Raynaud’s disease the attacks are IikeIy to be initiated by exposure to coId, are not infrequentIy reIieved by appIication of heat, and eIevation of the part does not resuIt in a Iessening of the asphyxia. The acroparesthesia of SchuItze or Nothnagel resembIes Raynaud’s disease in that suddenIy or graduaIIy the fingers or even the hands and arms become cold, numb, white, and painfu1. Disturbances of tactiIe sense and a diminished sensibiIity for needIe pricks, touch, temperature, and eIectrica1 irritants exist. Both hands are affected, though usuaIIy unequaIIy. The differentiation from an earIy stage of Raynaud’s disease is impossibIe unIess Raynaud’s second phenomenon of asphyxia occurs. Acroasphyxia chronica manifests itseIf as a sIowIy deveIoping cyanosis of the periphera1 parts without paroxysms or attacks associated with sensory or trophic disturbances. In certain cases a hypertrophy of the soft parts deveIops, which resembIes acromegaIy. AIthough this condition differs from Raynaud’s disease in its progressive course, absence of paroxysma
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attacks and deveIopment of hypertrophied periphera1 parts, there are cases which seem to be clinica transitions to Raynaud’s compIex. Even in these, however, syncope does not occur and pain is insignificant. The differentiation of Raynaud’s disease from obIiterative vascuIar disease is usuaIIy easy. In arterioscIerosis of the extremities the caIcified vesseIs are paIpabIe as cords and are found not to puIsate, whereas in Raynaud’s disease puIsations are obtainabIe at a11 times. The paroxysma nature of the attacks, the youthfuIness of the patient, the absence of pain of intermittent cIaudication, and the more frequent invoIvement of the upper extremities are important differentia1 points for Raynaud’s disease. Thromboangiitis obIiterans with vasomotor symptoms is at times di&uIt to Buerger especiaIIy since differentiate, warned that in Raynaud’s disease ischemia and rubor may be eIicited. These, however, may be observed onIy during the paroxysms in Raynaud’s disease, whereas they may be eIicited at wiI1 in thromboangiitis obliterans. If this point is borne in mind, no diffIcuIty shouId be encountered in arriving at a diagnosis. In certain cases of Buerger’s disease the inexperienced may be misIed by finding that the dorsaIis pedis pulsation is stiI1 present. In these the thrombosis has not extended beyond the digits, and the rubor and ischemia are found to be more or Iess Iimited to the area of vascuIar obIiteration. If other characteristic symptoms of thromboangiitis obliterans, such as pain of cIaudication, trophic Iesions, and migrating phIebitis are not present, one may have to depend on the sex of the patient and the Iocalization of the disease for differentiation. TREATMENT
Therapy in Raynaud’s disease aims onIy at a symptomatic cure. MiId cases which are more common probabIy do best without any treatment other than the institution of prophyIactic measures. The wearing of warm Ioose gIoves, soft wooIen stockings,
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and adequate cIothing usuaIIy suffice to minimize the frequency of attacks. Remova1 to warm, even cIimates has proved benef’icia1 in not a few cases. Attacks may be aborted or shortened by the application of heat. If these cases do not progress in two years, they probabIy never wil1; but shouId they become more severe, more energetic measures are indicated. Repeated galvanic hand and foot baths of ten minutes’ duration have seemed to heIp some few; but resuIts equaIIy as satisfactory may be expected from the production of hyperemia by Bier’s method or by the use of an eIaborate apparatus designed to produce a negative pressure about the affected part. Cushing advocated the application of a semi-eIastic bandage just tight enough to constrict the venous return from the part. This may prove beneficia1, but pain is intensified. Amy1 nitrite and nitrogIycerin have quite naturaIIy been given a tria1 by many, but there are no reports of either’s ever having been of constant benefit. Various endocrine gIand extracts have been vaunted as of exceptional vaIue, but Iike a11 other conservative measures they have faIIen into disuse in uncompIicated Raynaud’s disease. RecentIy Brown and Adson have reported distinct benefit in some few cases from the production of a febrile reaction by the injection of non-specific proteins. A vasomotor paresis is thought to resuIt from fever. CertainIy conservative treatment as outIined has only occasionaIIy proved satisfactory. Those cases not destined to remain miId have progressed in spite of treatment and, because of this, more radical measures were sought, and these are now fast becoming standardized. Small areas of gangrene shouId be aIIowed to demarcate and sIough, as healing by cicatrix may be expected. Large gangrenous areas are best amputated to save time and free the patient of pain. With the deveIopment of surgica1 procedures designed to interrupt innervation
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of the affected vesseIs, it became customary to refer to the surgeon a11 cases which showed trophic changes or gangrene. The resuIts in these were at first inconstant, but encouraging enough to warrant further investigation. Various modifications were instituted cIinicaIIy and experimentaIIy, and out of fifteen or more years of untiring effort have come certain standardized s’urgica1 procedures on the sympathetic nervous system which promise such an exceIIent symptomatic cure that a11 but the miId unprogressive cases shouId be given the benefit of this newIy deveIoped therapy. The evolution of surgery of the sympathetic system is an interesting chapter, too lengthy for a detailed review in this paper. In 1913 Leriche performed his first periarteria1 sympathectomy beIieving that he was dividing the motor nerves of the vessels. The resuIt was gratifying and in subsequent procedures on we11 seIected cases the symptomatic reIief warranted his removing the adventitia from vesseIs in over 300 cases manifesting symptoms of various vasomotor disturbances. Other workers, because of fauIty technique and improper seIection of cases, did not obtain comparabIe resuIts and the procedure became the subject of criticism. Leriche observed that his procedure produced biIatera1 vasodiIatation and eventuaIIy concIuded that he was not interrupting vasomotor fibers, but that he was aItering vasomotor activity by dividing pressor or centripeta1 fibers. He reasoned that division of these fibers around one vesse1 stimuIates “Iong distance” reflexes which resuIt in a transitory modification of bIood pressure and bIood flow in a11 four extremities and that IocaI hyperemia is of a more persistent nature because pressor inffuences are freed from dista1 intramura1 centers, which probabIy exist even though they have not been demonstrated anatomically. RegardIess of how a periarteria1 sympathectomy produces its characteristic effects, it is probabIy worthy of tria1 in those who are poor risks for a ganghonectomy, because it is easy to execute and because there is
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cIinica1 evidence to indicate that a symptomatic cure may occasionaIIy be expected. Th e h yperemia obtained by this procedure may last for onIy one or two weeks, but the symptomatic reIief not infrequentIy persists. Leriche in 1928 stated that periarteria1 sympathectomy produces Iess marked and Iess persistent IocaI hyperemia than does sympathectomy upon the trunk. This Iatter procedure apparentIy has not produced the unfavorabIe complications that were predicted for it, and therefore its use in even moderately severe cases is justified. Adson and Brown, quick to recognize the incompIeteness of effects from the Leriche operation, sought more satisfactory resuIts from operations on the sympathetic trunk. In 1924, RoyIe, after sympathetic ramisectomy for spastic paraIysis, observed a capilIary diIatation of the Ieg on the operated side. Soon after this, Adson and Brown began using a modification of this procedure for Raynaud’s disease and found that pain was relieved, abnorma1 coIor reactions disappeared, the feet and Iegs presented a pinkish coIor, were drier and sustained an average increase in temperature of 12’~. They beIieve that the distribution of the gray rami from the Iumbar gangIia is not constant, and, therefore, instead of doing a simpIe ramisectomy as urged by Leriche, they resect the second, third, and fourth Iumbar gangIia with the intervening trunk biIateraIIy, thus making sure that interruption of a11 efferent impuIses beIow the second Iumbar gangIion is compIete. Patients so operated upon have remained symptomaticaIIy cured for as Iong as five years and promise to remain so permanentIy. Davis and KanaveI performed a simiIar gangIionecto&y on one side in a case of erythromeIaIgia and obtained defmite improvement. Thus, for the Iower extremities one has a procedure which gives compIete and satisfactory resuIts. But Raynaud’s disease more frequentIy attacks the upper extremities and cervica1 sympathectomies as done by Adson and Brown, FuIton, Diez, RoyIe, and Davis and KanaveI have given onIy incompIete re-
Disease suits. These workers excised the steIIate gangIion in a11 cases and in some a11 of the cervica1 ganglia were removed. Davis and KanaveI, after observing their result, concluded that remova of the steIIate gangIion aIone wouId suEice. Recognizing that resuIts obtained in the upper extremities were not as satisfactory as those observed in the Iower extremities, Adson and Brown concIuded that a11 efferent vasoconstrictor impuIses to the vesseIs of the upper extremity were not being interrupted. This was verified in 1927 by Kuntz, who showed that the second thoracic gangIion contributes gray fibers to the first thoracic spina nerve, as we11 as to the second thoracic spina nerve, and this, in turn, often contributes to the Iower trunk of the brachia1 plexus. SubsequentIy, Adson and Brown through a posterior approach succeeded in excising the second thoracic gangIion aIong with the steIIate gangIion and the intervening trunk on the right side of a patient with Raynaud’s disease. FolIowing this the right hand became warm and dryness 6f the skin was noted over the entire right arm, axiIIa, right side of the face, and the right anterior surface of the chest down to the sixth interspace and posteriorIy down to the fifth dorsa1 vertebra. An incompIete Horner’s syndrome was present, but it Iater aImost entireIy disappeared. At a Iater date the Ieft side was operated upon with simiIar success, except that the Horner’s syndrome on this side remained. Subsequently other patients subjected to the same procedure were simiIarIy benefited. Adson now advocates resection of the Iast cervica1 together with the first and second dorsa1 ganglia. This change was instituted, because the other procedure did not compIeteIy interrupt sympathetic fibers as evidenced by the incompIeteness of the Horner’s syndrome obtained. It seems reasonabIe to expect muItipIe symptomatic cures in patients manifesting Raynaud’s phenomena in a11 four extremities if these compIete procedures are properIy executed. [For References see p. 128.1
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Gordon-“Bryth
of Mankynde”
BALLANTYNE, J. W. Obst. c? Gynec. Brit. Emp., 12: 175-194; 254-274; 1907. BARLOW, H. M. Personal Communication, London, June 20, 1930. CHOULANT, L. History and Bibliography of Anatomic Illustration. Tr. by Mortimer Frank. Chicago, Univ. of Chicago Press, 1917, p. 105. DENMAN, T. An Introduction to the Practice of Midwifery. London, Johnson, 1794, I : 19. ENGELMANN. G. J. Labor among Primitive Peoples. Ed. 2, St. Louis, J. H. Chambers & Co., 1883. FISHER, G. J. Ann. Anat. @ Surg., 3: 120-132, 1881. FISHER, G. J. Ann. Anat. ti Surg., 3: 2oo-206, 1881. FISHER. G. J. Med. Rec...__ 43: 178, .- 1803. _Gentlemen’s Mag., 22: 78, 1752. HAGGARD, H. W. Devils, Drugs and Doctors. New York, Harper, 1929, pp. 15,29. INGERSLEV,E. J. Obst. e*Gynec. Brit. Emp., 15: I, 1909.
REFERENCES
OF
ABERCROMBIE.Arch. Pediat., 3: 567, 1886. ADSON. Verbal Communication. ADSON. Ann. Clin. Med., 5: 161, 1926. ADSON and BROWN. Surg. Gynec. Obst., 48: 577, 1929. ADSON and BROWN J. A. M. A., 92: 444, 1929. ADSON and BROWN. J. A. M. A., 84: 1908, 1925. APPELBAUM. Am. J. Opbtb., 9: 569, 1926. BAILEY. Brit. J. Surg., 16: 166, 1928. BARKER. Internat. Clin., 4: 72, 1925. BARLOW, System of Medicine. London, MacMillan, 1899, vol. 8. BOYER. Cited by de Forest. BROWN. M. Clin. North America, 8: 1189, 1925. BROWN. Arch. Int. Med., 35: 54, 1925. BROWN. Ann. Clin. Med., 5: 168, 1926. BUCHANAN. Am. J. M. SC., 164: 14, 1922. BUERCER Ciculatory Disturbances of ‘the Extremities. Phila., Saunders, 1924. CASSIRER. Centralb. j. Nervenb. u. Psych., I I : 591, 1900. CASTANA. Pediatric, 31: 1305, 1923. CRICHLOW. New Orleans M. & S. J., 78: 511, 1926. CRICHLOW. New Orleans M. ti S. J., 74: 556, 1922. CUSHING. Cited by Buerger. DAVIS and KANAVEL. Surg. Gynec. Obst., 42: 729, 1926. DE FOREST. J. M. Sot. New Jersey, 17: 181, 1920. DICKINSON. W. H. On Rena1 and Urinarv Affections. Miscellaneous Infections of the Kidneys and Urine. New York, Wood, 1885. DIEz. Prensa. m&d., 12: 377, 1925-26. DRUITT, R. M. Times EdGaz., ApriI 19, 1873. DUPERIE. Paris med., 2: 222, 1924. F&TON. Ann. Surg., 88: 825, 1928. GAND. Atlantic M. J., 26: 740, 1923. GHELFI. RiJorma med., 37: 156, 1921. * Continued
JULY,IosI
INGERSLEV,E. J. Obst. <+ Gynec. Brit. Emp., 15: I, 1909. INGERSLEV, E. J. Obst. e+ Gynec. Brit. Emp., 15: 73, ‘909. LEE, S. Dictionary of Nationat Biography. Lond., Smith Elder, 1896, 47: 347. PEI-~ICREW, T. J. Medical Portrait Gallery. Biographical Memoir of Sir Charles Mansheld Clark. Lond., Fisher, Son & Co., I840, I: 2-4. POWER, SIR D’A. The Birth of b’lankind or The Woman’s Book, a Bibliographical Study. London, 1927. (Reprinted, The Bibliographical Society, with additions from the Library, 4. S., 8: I, 1927.) Personal Communication, May 30, 1930. VAN COUWENBERGH, E. Personal Communication, Louvain, Aug. 6, 1930. WILSON, G. Personal Communication, Manchester, June 19, 1930.
DR.
GRAVES*
HENRY. Cited by OsIer. HERTIUS. Cited-by de Forest. HUTCHINSON.M. Times EdGaz.. 2: 678. 1871. IWAI. Japan M. World, 6: 345,‘I926: ’ ’ KOPF. Miincben. med. Wcbnscbr., 72: 940, 1925. KRAUS. Cecil’s Medicine. PhiIa., Saunders, 1927. KUNTZ. Arch. Surg., 15: 871, 1927. LERICHE. Lyon cbir., IO: 378, 1913. LERICHE. Ann. Surg., 88: 449, 1928. LERICHE. Nelson’s System of Surgery. New York, Nelson, 1927, 3: 779. LERICHE & FONTAINE. Surg. Gynec. Obst., 47: 631, 1928. LINDSAY. Atlantic M. J., 28: 300. 1925. MA?THEWS. S. Clin. Nortb America. 8: 1460. 1028. MENSING. Wisconsin M. J., 26: 30, I927. _ ” _ MONAHAN. Am. J. M. SC., 171: 346, 1926. MORGAN. Cited by Buerger. MONRO: Monograph. GIasgow, 1899. OSLER. Modern Medicine. PhiIa., Lea, 1901, vo1. 6. POULTON.Lancet, 2: 1282, 1926. RAYNAUD. On IocaI Asnhvxia and Svmmetrical Gangrene of the Extremities, Thesis. “I862. ROYLE. Surg. Gynec. Obst., 39: 701, 1924. ROYLE. M. J. Australia, Suppl., I I : 341, 1927. ROYLE. M. J. Australia, 2: 436, 1928. SCHRADER.Cited bv de Forest. SHINKLE. J. A. M.“A., 83: 355, 1924. SOUTHEY. St. Bartb. Hosp. Rep., 16: 15, 1880. SPENCER. Tr. Clin. Soc.London, 23:287, 1892. STOKES and GANNER. J. A. M. A., 93: 438, 1929. TERRYMAN. S. Clin. Nortb America, 3: 1659, 1923. WARD. Med. Rec., 97: 694, 1920. WEISS. Wien. Klinik, 8: 346, 1882. WILKS. M. Times e* Gaz., 2: 207, 1879. from page 90.
DISEASES OF THE EXTREMITIES
V. RAYNAUD’S
DISEASE *
AMOS MAVERICK GRAVES, M.D. NEW ORLEANS, LA.
A
usuaIIy absent, as we11 as paraIysis, aIthough other evidences of disturbed vasomotor innervation, aphasia, hemoglobinuria, and arthropathies may occur.
UGUSTE MAURICE RAYNAUD, in 1862, at the age of thirty presented for his doctor’s degree a thesis entitIed “LocaI Asphyxia and Symmetrica Gangrene of the Extremities.” AIthough the disease which he so masterfuIIy described had been observed by Schrader, in 1629, and Hertius, in 1685, the foIIowing excerpt from his monograph shows that it had remained for him to estabIish the causative factor of this symmetrica mortification.
ETIOLOGY A wide difference of opinion is found to exist among medica authors as to the predisposing and direct causes of Raynaud’s disease. However, it is agreed that the symptomatoIogy is dependent upon a vasomotor disturbance. Raynaud, Monro, Barker, OsIer, Buerger, BarIow, and others have suggested a hereditary factor, but more recent studies seem to prove that Buchanan and Monahan are correct in definiteIy ruIing out any inherited predisposition. The Iatter found a neurotic temperament to exist in onIy haIf the cases reviewed. SyphiIis, malaria, typhoid fever, typhus fever, influenza, and other conditions which give rise to a toxemia have been thought to be etioIogic factors, but now they are mereIv regarded as having occurred in association with the disease. SexuaI excess and excitation were suggested as causes by Raynaud, but these, no doubt, shouId be Iisted as contributory factors, aIong with exposure, trauma, psychic exertion, sudden fright, and acute infections. That exposure to cold precipitates an attack can be demonstrated in the majority of cases. Monahan beIieves that in view of the steriIity of the discharge from diseased tissues one must Iook for the fundamenta1 cause in some constitutiona abnormaIity or subnormaIity, which might produce a chemicophysioIogic toxin.
I propose to show that there exists a variety of dry gangrene affecting the extremities which is impossibIe to expIain upon the basis of vascuIar obliteration; a variety characterized especiaIIy by a remarkabIe tendency to symmetry since rt aIways affects simiIar portions of the body, the two upper extremities, the two lower extremities, or a11 four at the same time: In certain instances the nose and ears are aIso affected. I shaI1 seek to prove that this kind of gangrene has as its cause a disturbance of the innervation of the capiIIary bIood vesseIs. CLINICAL PICTURE CIinicaI characteristics are conciseIy described foIIows :
of a typica case by Cassirer as
Somewhere in the peripheral portions of the body there occurs more or Iess severe pain. This is not confined to distinct nerve territory, but usually affects symmetrica parts. Attacks of vasomotor and trophic disturbances are part of the syndrome, to wit: (I) syncope, asphyxia, or IocaI rubor, and (2) severe trophic disturbances, usuaIIy in the form of gangrene of the parts first affected with symptoms. The course is an intermittent one, for there may be compIeteIy free intervaIs; but in some instances, evidences of disturbed vasomotiIity may persist. The disease may consume itseIf in one attack or several attacks may occur in succession. ObjectiveIy, sensory disturbances are
INCIDENCE most of Age. According to Raynaud, the cases occur between the ages of eighteen to thirty years. Monro gives the average
* Presented before the Surgical FacuIty of T&me University, New OrIeans, March 17, 1930. Previous articles on Vascular Diseases of the Extremities appeared as follows: I. ArterioscIerotic disease, vo1. IZ, p. 32; II. Erythromelalgia, vo1. 12, p. 40; III. Thermic Gangrene, vo1. 12, p. 324; IV, Thromboangiitis obliterans, vol. IZ, p. 489.
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age of onset as 28.9 vears, and Morgan in his 93 cases found It to be 26.6 years. Cassirer cohected 168 cases and found that there were 55 in which the disease occurred in the first and second decades of Iife as compared with 40 in the third decade. Subsequently, in studying 109 cases, he found the greatest age incidence from twenty-one to forty-years of age. Of 67 cases studied by Buchanan at the Mayo Clinic, 36 were in patients from twenty-one to forty years of age. That the disease may frequentIy occur in children is evidenced by the fact that Cassirer coIIected 22 cases occurring in patients less than five years of age. In the more recent Iiterature there are numerous reports of its occurrence in infants and in the newIy born. Not infrequentIy the disease is reported to have been observed in individuaIs of advanced years; but that these are cases of true Raynaud’s disease is to be doubted except in those few in which the symptom-compIex was characteristic. E. P. Henry reported a case in which the phenomena occurred in the ears, nose, and extremities in a woman seventy-seven years oId. Sex. Women are more frequentIy affected. Raynaud found that 80 per cent of his cases were in women; 6.2 per cent of Monro’s cases were femaIes, and this sex predominated in 62.9 per cent of Cassirer’s series and in 59.8 per cent of Buchanan’s series. Adson and Brown beheve that with improvement in diagnosis the incidence of true Raynaud’s disease in the maIe wiI1 drop to Iess than 5 per cent of cases. The racia1 and geographica distribution has been shown to be fairIy widespread. Three cases have been reported in the negro by Lee. That Raynaud’s disease is rare has been shown. Monro reports that it occurs about once to every 3000 other affections and Buchanan found that it comprised Iess than 0.02 per cent of registrations at the Mayo CIinic. At the Johns Hopkins Hospita1 there were 19 cases in 23,000 admissions. Buchanan states that he could not demonstrate that physica or nervous
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strain incident to any one type of occupation or social stratum was a cause of the condition. MODES
OF
ONSET
AND
DURATION
Sixty out of Buchanan’s 67 patients described a sudden onset with portions of the body turning white, blue or bIack. Five described a gradua1 onset consisting of tingIing, numbness, cIumsiness, or indescribabIe discomfort occurring at irreguIar intervaIs, foIIowed at a Iater date by the coIor phenomena. Raynaud described the onset of the disease as sudden and the duration as short. Other earIy observers gave the same conception; but with a more widespread knowIedge of its symptomatoIogy and better methods of diagnosis the chronic and insidious forms of the disease have been recognized and not infrequently reported. a case in which Monahan reports prodroma1 symptoms Iasted for nine months before the acute stage occurred to persist for four years before amputation was resorted to. Many of the miId cases require exposure to coId to produce the characteristic symptoms. These may cIear up and remain especiaIIy if winters are symptom-free, spent in tropical zones. However, most cases remain remittentIy active, although some may never advance to the moderate or severe forms. Buchanan reported that 30 of his cases had had symptoms for more than five years and of these 7 had been affected for from fifteen to thirty years and I for fortythree years. Thus, it is evident that the duration is not aIways short. That the disease may spontaneousIy subside cannot be doubted. Proof of this cannot readiIy be obtained, as recovered cases are usuaIIy Iost before a conchrsive period of observation is ended. CertainIy many miId cases remain undiagnosed or unobserved simpIy because they subside or do not progress to the extent that the cIassica1 picture is evident.
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Even cases of moderate severity may progress slowly and subside after several attacks which have produced small areas of gangrene. Osler states that the severe cases are exceptional, although every large hospital is likely to have one that returns at intervals for amputations unti1 the victim finally drifts into an aImshouse. SYMPTOMATOLOGY
Three characteristic phenomena of the disease are generahy described. These are: (a) local syncope; (6) IocaI asphyxia; (c) IocaI or symmetrical trophic lesions or gangrene. The term “local syncope ” was used by Raynaud to signify the blanching of a part. It is manifested in the fingers, toes, cheeks, ears or nose and Iess frequently in other parts by a sudden death-like, demarcated paIlor and a decrease in surface temperature of from 15 to 20~. This condition Iasts from a few minutes to a few hours and may rarely result in gangrene. It usuaIIy recurs with or without periodicity and is likely to be precipitated by exposure to cold. Associated with it is a feeling of numbness, tingling or coIdness. The attack may be terminated by a painful reaction. In only 3 of Monro’s 176 cases IocaI syncope existed alone, and Buchanan noted it alone in 15 per cent of his cases. It occurred either concomitantly or associated with local asphyxia in IO per cent of Monro’s and in 42 per cent of Buchanan’s cases. Boyer originated the term “local asphyxia, ” but it was Raynaud who applied it to that phenomena of the disease which usually foIlows, but may rarely precede syncope. As the term implies, there is a IocaI anoxemia which causes the skin to appear bluish white, dusky blue, slate coIor, dark blue, violet gray or purple, or there may be redness with mottling of degrees of blueness. Surface temperature is decreased less than it is in syncope, and there is often a temporary sweIling of the part or a cold
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sweat may appear. If this phenomenon persists, the skin becomes black and mortified, or there may result desquamation of the epidermis. Occasionally an incomplete asphyxia may persist for years to produce a false edema as a resuIt of undernutrition to the part. Local asphyxia is more prone to recurrences and is usually accompanied by more severe pain than is syncope. This phenomenon occurred alone in 16 per cent of Monro’s cases and in 6 per cent of Buchanan’s. When it occurs simultaneousIy with syncope, there is a play of colors with many shadings from cadaveric white to several tints of blue or bhrish red, or the phalanges may be white while proximalIy there is a change to a bluish tint. Pressure over an affected part displaces the color which CharacteristicaIly does not reappear for some time. Following either syncope or asphyxia there may follow a period of hyperemia in which redness is marked and is accompanied by increased pulsations, increased warmth of the part, increased moisture, and a tingling sensation approaching that of pain. With recurrence and persistence of the phenomena discussed gangrene of the eventualIy results. A peripheral parts bleb may form at the tip of the fingers and discharge a blackish or serosanguineous content. The resulting ulcer usuaIly heaIs by cicatrization. Not infrequently a finger tip may merely become thickened and indurated and later desquamate. More commonly there occur sharply demarcated bIackened, symmetrical areas which remain dry for variable periods of time before sloughing. These areas may be massive, causing the Ioss of a leg or forearm or HeaIing by even a whole extremity. granuIation with the formation of a tapering cicatrix is characteristic. Although the gangrene is usualIy of the dry variety, it may at times be moist. Pain in this disease is an important symptom as it almost constantIy occurs though it may be absent in one attack and present in another. It may precede an
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attack; but usuaIIy it coincides with asphyxia or syncope as a painfuI numbness folIowed by a sensation of burning and tinghng. It is exaggerated by pressure or the occurrence of gangrene and may occasionaIIy radiate to unaffected extremities. Heat and coId sensations are not changed, but rarely is there analgesia or diminution in tactile sensation. In a11 phenomena the skin is IikeIy to be covered with a cIammy moisture. Motor disturbances are not observed unIess muscuIar atrophy occurs. Bone changes were first observed by Cassirer. CharacteristicaIIy, one or more or a11 the termina1 phaIanges may become atrophic or disappear without the formation of a sinus. Long bones may aIso become invoIved as manifested by a thinning of the cortex and an increase in the size of the marrow cavity in an occasiona case. PuIsations in the dorsaIis pedis, posterior tibiaI, and radia1 arteries are commonIy thought to be absent during the stage of syncope. This is incorrect as puIsations, though at times diminished, may be eIicited in a11 stages of the disease. BIood pressure determinations, p&e rates, and bIood pictures were found to be within normaI Iimits in a11 cases at the Mayo CIinic. HemogIobinuria was not observed in any case, though it has been reported by many authors. AREAS
INVOLVED
Of the cases reported by Buchanan the Iesions were biIatera1 and symmetrica in 56.7 per cent and asymmetrica in 43.3 per cent. Buerger states that symmetry is occasionaIIy Iacking and reports of isoIated cases in the Iiterature substantiate this. Not infrequentIy the nose, Iips, and sterna1 regions are affected and very rareIy multiple gangrenous areas occur anywhere on the trunk. Monro found the upper extremities were affected in 43 per cent of cases, the Iower extremities in 24 per cent, and both in 22 per cent. Buchanan found the Iower extremities more frequentIy affected, but
Disease he reports quite a diverse distribution in some cases. Nineteen patients had Iesions a11 over the body, one on the side of the tongue, one in the pharynx, one on the Ieft side of the face and one in which both forearms and Iips sIoughed and heaIed by cicatrices. That this disease has an even more widespread anatomica distribution is beIieved by many. Raynaud himself noticed that there were coincident aIterations in the retina1 vesseIs with impairment of vision in 2 of his cases when the characteristic phenomena occurred in the extremities. Many cases with like symptoms have been reported, the most recent being those of ShinkIe and AppeIbaum. Weiss reported a remarkabIe case in which the Ieft cervical sympathetic nerve was invoIved causing the Ieft chest suddenIy to become warm and red, the Ieft eyebalI to retract, hyperhidrosis, and contraction of the pupil. A recession of a11 manifestations except for sIight retraction of the eyebalI was compIete within a week, but recurrences occurred unti1 finaIIy a condition simiIar to a partia1 atrophy of the soft parts of the Ieft side of the face remained. HemogIobinuria in association with Raynaud’s disease was not observed by Raynaud. Hutchinson in 1871 recorded the first case in which this symptom occurred and subsequentIy simiIar cases were recorded by Druitt, WiIks, Southey, Barlow, Dickinson, Abercrombie, Monro, Spencer, and many others. ScIeroderma and ScIerodactylia not infrequentIy occur in association with the typica phenomena of Raynaud’s disease and have been thought by some to be mereIy a complication of Raynaud’s disease. CertainIy at times when either is present, it is impossibIe to ruIe out Raynaud’s disease. Raynaud referred to fibrous ankyIosis of the termina1 phaIangea1 articuIations and to thickening aIong the processes of the paImar fascia and showed how remarkabIy such thickening may subside foIIowing recovery from proIonged IocaI asphyxia.
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Southey reported a case in which there was an effusion in both knee joints. OsIer states that in a large number of mentaI disorders attacks, of Raynaud’s Mania, disease have been observed. meIanchoIia, circuIar epiIepsy, amentia, insanity, and progressive paraIysis of the insane have been reported to occur in association with the disease. Not a few cases exhibiting the characteristic phenomena have deveIoped transient monopIegias, hemiplegias, aphasias, and It seems reasonabIe to even parapIegias. regard these attacks as due to vascuIar changes in the brain of the same character as those which occur in the peripheral vessels. PATHOLOGY
The most frequent Iesion observed in Raynaud’s disease is the gangrene which is usuaIIy of the dry variety. It is sharpIy demarcated and remains so until the sIough comes away. Infection rareIy occurs and healing readiIy takes pIace by granuIation. When a periphera1 part is involved, there resuIts a characteristic tapering cicatrix. BIeb formations with resuIting uIcerations may be observed or there may occur dry dystrophic processes in which a dark scab forms and subsequentIy becomes detached in a scaIe-Iike manner. That the characteristic Iesions are due either to constriction of the capiIIaries or to diIatation and stasis in them has Iong been assumed, but it was not unti1 1925 that Brown and others made observations with the capiIIary microscope which satisfied a11 doubts concerning the type of Iesions. Brown concIuded from a study of 8 cases under various conditions and in different stages of the disease that in IocaI syncope there is a constriction of a11 three vascuIar eIements in the skin and that in IocaI asphyxia there is a refiIIing of diIating capiIIary 100~s from segments of arteria1 or venous bIood or both. In this Iatter stage there is apparentIy a Ioss of coordination between the capiiIaries and arterioIes.
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The arteries stiI1 remain contracted, except for an intermittent reIaxation which aIIows smaI1 segments of arteria1 bIood to enter the diIated capiIIary loops. The venuIes apparentIy undergo simiIar reactions since a reflux of bIood takes pIace DiIatation of the into the capiIIaries capiIIaries precedes the cyanosis, and it is this change in caIiber together with a Ioss of tone which causes stasis and a resuIting increase in carbon dioxide content of the blood. PROGNOSIS
Prognosis as to Iife of an affected part has been poor in the past and amputations have been frequentIy performed in order to to aIIeviate pain. It seems reasonabIe assume that for the most part onIy the more advanced or severe cases were observed, and consequentIy the oIder writers have given a gloomy outIook for patients affected with Raynaud’s disease. With an increasing number of earIy cases being recognized and observed for years and with the advent of more satisfactory therapy, we are finding that an affected part may remain usefu1 for many years in not a few cases. DIAGNOSIS
The diagnosis of Raynaud’s disease is made on the tinding of a combination of the characteristic phenomena. The mere observance of ischmeic fingers hardIy warbut the absence of rants a diagnosis; singIe symptoms in a case which has been adequateIy studied and observed for a suffrcientry Iong period shouId onIy rarely render the diagnosis uncertain. Differentiation of Raynaud’s disease from other vasomotor and trophic neuroses is occasionaIIy difflcuIt and may be impossibIe without proIonged observation. Certain cases of scIeroderma, especiaIIy those exhibiting scIerodactyIia, manifest vasomotor symptoms and are believed by some to be caused by Iesions of the sympathetic nervous system. Numerous cases are on record in which Raynaud’s phenomena occurred CoincidentaIIy with,
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preceded, or folIowed the deveIopment of the indurative and atrophic stages of scIeroderma and scIerodactyIia. Since in Raynaud’s disease there may not infrequentIy deveIop induration and thickening of the skin and subcutaneous tissues, a differentiation from scIeroderma may be impossibIe unti1 the skin Iesion spreads to other areas in which manifestations of a vasomotor disturbance are not evident. The absence of pain and paresthesia is of vaIue in eIiminating Raynaud’s disease; but when pain and paresthesia are present, one shouId consider that the two diseases coexist unti1 further observation proves otherwise. ErythromeIaIgia shouId offer no difflculty in differentia1 diagnosis. The attacks are precipitated by heat or dependency of the affected part. The redness, increased temperature, and pain disappear on eIevation of the part and are aIIeviated by the appIication of coId, whereas in Raynaud’s disease the attacks are IikeIy to be initiated by exposure to coId, are not infrequentIy reIieved by appIication of heat, and eIevation of the part does not resuIt in a Iessening of the asphyxia. The acroparesthesia of SchuItze or Nothnagel resembIes Raynaud’s disease in that suddenIy or graduaIIy the fingers or even the hands and arms become cold, numb, white, and painfu1. Disturbances of tactiIe sense and a diminished sensibiIity for needIe pricks, touch, temperature, and eIectrica1 irritants exist. Both hands are affected, though usuaIIy unequaIIy. The differentiation from an earIy stage of Raynaud’s disease is impossibIe unIess Raynaud’s second phenomenon of asphyxia occurs. Acroasphyxia chronica manifests itseIf as a sIowIy deveIoping cyanosis of the periphera1 parts without paroxysms or attacks associated with sensory or trophic disturbances. In certain cases a hypertrophy of the soft parts deveIops, which resembIes acromegaIy. AIthough this condition differs from Raynaud’s disease in its progressive course, absence of paroxysma
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attacks and deveIopment of hypertrophied periphera1 parts, there are cases which seem to be clinica transitions to Raynaud’s compIex. Even in these, however, syncope does not occur and pain is insignificant. The differentiation of Raynaud’s disease from obIiterative vascuIar disease is usuaIIy easy. In arterioscIerosis of the extremities the caIcified vesseIs are paIpabIe as cords and are found not to puIsate, whereas in Raynaud’s disease puIsations are obtainabIe at a11 times. The paroxysma nature of the attacks, the youthfuIness of the patient, the absence of pain of intermittent cIaudication, and the more frequent invoIvement of the upper extremities are important differentia1 points for Raynaud’s disease. Thromboangiitis obIiterans with vasomotor symptoms is at times di&uIt to Buerger especiaIIy since differentiate, warned that in Raynaud’s disease ischemia and rubor may be eIicited. These, however, may be observed onIy during the paroxysms in Raynaud’s disease, whereas they may be eIicited at wiI1 in thromboangiitis obliterans. If this point is borne in mind, no diffIcuIty shouId be encountered in arriving at a diagnosis. In certain cases of Buerger’s disease the inexperienced may be misIed by finding that the dorsaIis pedis pulsation is stiI1 present. In these the thrombosis has not extended beyond the digits, and the rubor and ischemia are found to be more or Iess Iimited to the area of vascuIar obIiteration. If other characteristic symptoms of thromboangiitis obliterans, such as pain of cIaudication, trophic Iesions, and migrating phIebitis are not present, one may have to depend on the sex of the patient and the Iocalization of the disease for differentiation. TREATMENT
Therapy in Raynaud’s disease aims onIy at a symptomatic cure. MiId cases which are more common probabIy do best without any treatment other than the institution of prophyIactic measures. The wearing of warm Ioose gIoves, soft wooIen stockings,
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and adequate cIothing usuaIIy suffice to minimize the frequency of attacks. Remova1 to warm, even cIimates has proved benef’icia1 in not a few cases. Attacks may be aborted or shortened by the application of heat. If these cases do not progress in two years, they probabIy never wil1; but shouId they become more severe, more energetic measures are indicated. Repeated galvanic hand and foot baths of ten minutes’ duration have seemed to heIp some few; but resuIts equaIIy as satisfactory may be expected from the production of hyperemia by Bier’s method or by the use of an eIaborate apparatus designed to produce a negative pressure about the affected part. Cushing advocated the application of a semi-eIastic bandage just tight enough to constrict the venous return from the part. This may prove beneficia1, but pain is intensified. Amy1 nitrite and nitrogIycerin have quite naturaIIy been given a tria1 by many, but there are no reports of either’s ever having been of constant benefit. Various endocrine gIand extracts have been vaunted as of exceptional vaIue, but Iike a11 other conservative measures they have faIIen into disuse in uncompIicated Raynaud’s disease. RecentIy Brown and Adson have reported distinct benefit in some few cases from the production of a febrile reaction by the injection of non-specific proteins. A vasomotor paresis is thought to resuIt from fever. CertainIy conservative treatment as outIined has only occasionaIIy proved satisfactory. Those cases not destined to remain miId have progressed in spite of treatment and, because of this, more radical measures were sought, and these are now fast becoming standardized. Small areas of gangrene shouId be aIIowed to demarcate and sIough, as healing by cicatrix may be expected. Large gangrenous areas are best amputated to save time and free the patient of pain. With the deveIopment of surgica1 procedures designed to interrupt innervation
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of the affected vesseIs, it became customary to refer to the surgeon a11 cases which showed trophic changes or gangrene. The resuIts in these were at first inconstant, but encouraging enough to warrant further investigation. Various modifications were instituted cIinicaIIy and experimentaIIy, and out of fifteen or more years of untiring effort have come certain standardized s’urgica1 procedures on the sympathetic nervous system which promise such an exceIIent symptomatic cure that a11 but the miId unprogressive cases shouId be given the benefit of this newIy deveIoped therapy. The evolution of surgery of the sympathetic system is an interesting chapter, too lengthy for a detailed review in this paper. In 1913 Leriche performed his first periarteria1 sympathectomy beIieving that he was dividing the motor nerves of the vessels. The resuIt was gratifying and in subsequent procedures on we11 seIected cases the symptomatic reIief warranted his removing the adventitia from vesseIs in over 300 cases manifesting symptoms of various vasomotor disturbances. Other workers, because of fauIty technique and improper seIection of cases, did not obtain comparabIe resuIts and the procedure became the subject of criticism. Leriche observed that his procedure produced biIatera1 vasodiIatation and eventuaIIy concIuded that he was not interrupting vasomotor fibers, but that he was aItering vasomotor activity by dividing pressor or centripeta1 fibers. He reasoned that division of these fibers around one vesse1 stimuIates “Iong distance” reflexes which resuIt in a transitory modification of bIood pressure and bIood flow in a11 four extremities and that IocaI hyperemia is of a more persistent nature because pressor inffuences are freed from dista1 intramura1 centers, which probabIy exist even though they have not been demonstrated anatomically. RegardIess of how a periarteria1 sympathectomy produces its characteristic effects, it is probabIy worthy of tria1 in those who are poor risks for a ganghonectomy, because it is easy to execute and because there is
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cIinica1 evidence to indicate that a symptomatic cure may occasionaIIy be expected. Th e h yperemia obtained by this procedure may last for onIy one or two weeks, but the symptomatic reIief not infrequentIy persists. Leriche in 1928 stated that periarteria1 sympathectomy produces Iess marked and Iess persistent IocaI hyperemia than does sympathectomy upon the trunk. This Iatter procedure apparentIy has not produced the unfavorabIe complications that were predicted for it, and therefore its use in even moderately severe cases is justified. Adson and Brown, quick to recognize the incompIeteness of effects from the Leriche operation, sought more satisfactory resuIts from operations on the sympathetic trunk. In 1924, RoyIe, after sympathetic ramisectomy for spastic paraIysis, observed a capilIary diIatation of the Ieg on the operated side. Soon after this, Adson and Brown began using a modification of this procedure for Raynaud’s disease and found that pain was relieved, abnorma1 coIor reactions disappeared, the feet and Iegs presented a pinkish coIor, were drier and sustained an average increase in temperature of 12’~. They beIieve that the distribution of the gray rami from the Iumbar gangIia is not constant, and, therefore, instead of doing a simpIe ramisectomy as urged by Leriche, they resect the second, third, and fourth Iumbar gangIia with the intervening trunk biIateraIIy, thus making sure that interruption of a11 efferent impuIses beIow the second Iumbar gangIion is compIete. Patients so operated upon have remained symptomaticaIIy cured for as Iong as five years and promise to remain so permanentIy. Davis and KanaveI performed a simiIar gangIionecto&y on one side in a case of erythromeIaIgia and obtained defmite improvement. Thus, for the Iower extremities one has a procedure which gives compIete and satisfactory resuIts. But Raynaud’s disease more frequentIy attacks the upper extremities and cervica1 sympathectomies as done by Adson and Brown, FuIton, Diez, RoyIe, and Davis and KanaveI have given onIy incompIete re-
Disease suits. These workers excised the steIIate gangIion in a11 cases and in some a11 of the cervica1 ganglia were removed. Davis and KanaveI, after observing their result, concluded that remova of the steIIate gangIion aIone wouId suEice. Recognizing that resuIts obtained in the upper extremities were not as satisfactory as those observed in the Iower extremities, Adson and Brown concIuded that a11 efferent vasoconstrictor impuIses to the vesseIs of the upper extremity were not being interrupted. This was verified in 1927 by Kuntz, who showed that the second thoracic gangIion contributes gray fibers to the first thoracic spina nerve, as we11 as to the second thoracic spina nerve, and this, in turn, often contributes to the Iower trunk of the brachia1 plexus. SubsequentIy, Adson and Brown through a posterior approach succeeded in excising the second thoracic gangIion aIong with the steIIate gangIion and the intervening trunk on the right side of a patient with Raynaud’s disease. FolIowing this the right hand became warm and dryness 6f the skin was noted over the entire right arm, axiIIa, right side of the face, and the right anterior surface of the chest down to the sixth interspace and posteriorIy down to the fifth dorsa1 vertebra. An incompIete Horner’s syndrome was present, but it Iater aImost entireIy disappeared. At a Iater date the Ieft side was operated upon with simiIar success, except that the Horner’s syndrome on this side remained. Subsequently other patients subjected to the same procedure were simiIarIy benefited. Adson now advocates resection of the Iast cervica1 together with the first and second dorsa1 ganglia. This change was instituted, because the other procedure did not compIeteIy interrupt sympathetic fibers as evidenced by the incompIeteness of the Horner’s syndrome obtained. It seems reasonabIe to expect muItipIe symptomatic cures in patients manifesting Raynaud’s phenomena in a11 four extremities if these compIete procedures are properIy executed. [For References see p. 128.1
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BALLANTYNE, J. W. Obst. c? Gynec. Brit. Emp., 12: 175-194; 254-274; 1907. BARLOW, H. M. Personal Communication, London, June 20, 1930. CHOULANT, L. History and Bibliography of Anatomic Illustration. Tr. by Mortimer Frank. Chicago, Univ. of Chicago Press, 1917, p. 105. DENMAN, T. An Introduction to the Practice of Midwifery. London, Johnson, 1794, I : 19. ENGELMANN. G. J. Labor among Primitive Peoples. Ed. 2, St. Louis, J. H. Chambers & Co., 1883. FISHER, G. J. Ann. Anat. @ Surg., 3: 120-132, 1881. FISHER, G. J. Ann. Anat. ti Surg., 3: 2oo-206, 1881. FISHER. G. J. Med. Rec...__ 43: 178, .- 1803. _Gentlemen’s Mag., 22: 78, 1752. HAGGARD, H. W. Devils, Drugs and Doctors. New York, Harper, 1929, pp. 15,29. INGERSLEV,E. J. Obst. e*Gynec. Brit. Emp., 15: I, 1909.
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