This paper I wrote while working in the Blood Flow Laboratory of the Vascular Surgical Services of Philip N. Sawyer, M.D., at the State University of New York Downstate Medical Center in Brooklyn, New York. It is published in a book titled, "Noninvasive Cardiovascular Diagnosis: Current Concepts." ref.
CHAPTER 22 Page 205
Diagnosis of Atypical Raynaud's Phenomenon
P.N. Sawyer, M. L. Stark, L. N. Malik, B. Stanczewski, and R. M. Stillman
Vascular Surgical Services and Vascular Surgical Research Laboratory, Departments of Surgery and Surgical Research, Downstate Medical Center, The State University of New York, Brooklyn, New York, USA
Frequently patients present with mild but distressing cold sensitivity or numbness in the digits, but a definitive diagnosis of Raynaud's phenomenon, although strongly suggested, is never authenticated. Detailed laboratory testing including blood flow studies using the Barcroft technique and Doppler blood flow determination seem to provide an objective method of diagnosing these cases of "atypical Raynaud's phenomenon." However, more research in this area is needed.
In his classic monograph in 1863, Maurice Raynaud first described the pathological process, known today as Raynaud's disease, in a series of patients who had blue hands and/or fingertips and, less commonly, blue feet or toes (1). In an attempt to find the cause of the problem he suggested that it was attributable to small vessel vasospastic abnormality mediated via the sympathetic nervous system.
In the century since that time, many attempts have been made to categorize these patients. There has been considerable controversy concerning the etiology (2, 3, 4), pathophysiology (5, 6), and therapy (7) of their symptomatology; it is known that a variety of different diseases begin with a symptom complex that is likely to be labeled "Raynaud's phenomenon" (8). This inherent confusion is further complicated by the need to differentiate between primary or idiopathical Raynaud's disease and the secondary form. Yet, this differential diagnosis bears heavily upon prospective treatment and prognosis.
Originally, changes in the distal arteries observed by arteriography (9) were considered to be objective proof of vasospasm. However, most individuals appear to develop intimal thickening in the digital arteries in the course of aging despite the absence of Raynaud's type symptoms. Objective proof of Raynaud's phenomenon is best obtained by demonstration of vasospasm with its characteristic color changes and/or pain after exposure to cold or to psychological stress. (Page 206) Anatomical occlusion of digital arterioles must be excluded to rule out thromboangitis obliterans (Beurger's disease) (9). Reactive hyperemia, a noninvasive blood flow parameter, seems to present an objective measurement of the competence of vasomotor function to compensate for induced anoxia in the tissues of the forearm and calf.
Despite the application of clinical examination, cold immersion testing with pulsatile wave form analysis, and arteriography, there appear to be a number of patients with mild but distressing cold sensitivity or numbness in the digits for which a definitive diagnosis of Raynaud's phenomenon is strongly suggested but never clearly authenticated. Many of these patients are seen in our Vascular Surgical Clinic following referral from general practitioners, internists, and general surgeons. They often are discouraged and feel they must live in reconciliation with their constant symptoms. We have performed blood flow studies using the classical Barcroft technique, Doppler blood flow determination and thorough laboratory testing in this group of patients and report here our experience with diagnosis and therapy of those patients with "atypical Raynaud's phenomenon."
Plethysmographic studies are modeled after the Barcroft semiocclusive and totally occlusive cuff techniques as refined by Whitney (10). These employ a mercury in Silastic strain gauge to measure changes in limb volume that reflect occlusion of venous and arterial flow. The basic principle of the method is that any circumferential rate of change in a limb segment is directly proportional to
Atypical Raynaud's Phenomenon Page 207
the volumetric rate of change of that segment (11), keeping in mind that the length of the limb segment is essentially constant. Limb expansion can therefore be considered to occur in only two dimensions, giving a volumetric change based on two variables instead of the usual three. The initial size of the "venous well" can be determined, as well as the change in volume with arterial inflow under normal resting flow and induced hyperemic conditions. Thus, the venous well acts as a receptacle for the arterial flow. The Parks model 270 strain gauge plethysmograph coupled with the Jobst intermittent compression unit for the necessary inflation and deflation serves as the strain gauge plethysmograph (Figure 1). Further details of this method have been published elsewhere (12).
Reactive hyperemia conditions are induced by cuff inflation above systolic blood pressure for a three - to - five minute period. Sudden deflation permits accelerated blood flow into an ischemic, hypoxic, low pH vascular bed, visualized as a hyperacute inflow curve. In individuals with "normal" vasomotor tone, all subsequent curves show a decreasing slope.
Caption of Figure 2 above: A normal volunteer undergoing plethysmography and Doppler waveform analysis in the blood flow laboratory.
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Doppler pulse pressure measurements (13, 14, 15) are taken in brachial, radial, ulnar, volar, digital, popliteal, and pedal areas to evaluate large vessel function. The output of the Parks Electronics Laboratories model 806 directional Doppler ultrasound device is recorded in dual beam Tektronix storage oscilloscope (#3A3 dual trace differential amplifier). Pulse pressure waveforms are recorded in various stages and photographed using Polaroid film.
Figure 2 is a schematic of the equipment arrangement. This requires one technician for recording and analyzing the data, schematically depicted in Figure
Atypical Raynaud's Phenomenon Page 209
Atypical Raynaud's Phenomenon Page 210
3, showing the systematized checklist used which permits transcription of the data onto key cards for computer processing.
In the last five years, approximately 2,500 examinations have been performed on patients in our blood flow laboratory. One hundred seventy-seven patients were considered to have a vasospastic condition of some sort. Table 1 compares plethysmographic data in three groups of patients: 1) 64 patients with clinically typical Raynaud's phenomenon as determined by classical symptomatology and exclusion of anatomical arterial disease as revealed by normal pulses (this category includes 11 most severe cases which might be considered idiopathical Raynaud's disease); 2) 69 patients with cold extremities and vasospastic symptoms who did not have easily documented Raynaud's phenomenon (we have termed this latter "atypical"); and 3) 40 normal volunteers who constituted the control group.
The most significant changes are found in reactive hyperemia, which serves as a measure of vasomotor tone. This method of producing maximal vasodilation
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Atypical Raynaud's Phenomenon Page 212
in the limb is physiological even though produced by external, noninvasive means (which most likely render the analysis of such vasodilation significant). Normal vasomotor tone reveals a maximal first curve in the succession of hyperemic inflow trace curves. Inflow into the ischemic limb shows a steady decrease with each ejection thereafter. Increasing hyperemic inflow rates are considered abnormal and reveal initial inflow that is not sufficient to immediately compensate for induced tissue ischemia. Because reactive hyperemia time is defined as the amount of time necessary to attain maximal flow, a prolonged reactive hyperemia time is indicative of an abnormal lack of elasticity in the anoxic vascular bed. Figure 4 depicts the ranges of successive hyperemic flow values with trend variables as seen in atients with vasospastic disease and in normal controls.
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The plethysmographic method was used in this study to detect inferior vasomotor tome in the forearm and calf, whereas the actual symptoms being treated were localized in the hand and foot. According to Wilkins and Eichna (16), the blood flow to muscular limb segments is less variable in response to temperature changes than is the blood flow to the tips of the extremities. Figure 5 shows the reactive hyperemia times found in typical Raynaud's patients, atypical Raynaud's patients, and normal volunteers. Variations between the two groups are statistically significant at the 0.005 to 0.01 level in spite of large clinical variations. In those patients with what appears to be clinical Raynaud's with relatively quick reactive hyperemia times the vasomotor dysfunction may be found solely in the extremity tips. here, plethysmography must be applied at the digital level. Observed abnormality in a muscular limb segment can generally be assumed to be a sign of early pathology.
Ultrasonic wave forms of the pulse sites were recorded both at room temperature and after ice immersion in 0degrees C ice-water bath. Dampened wave form is also indicative of a decreased vascular elasticity with resultant delayed hyperemic response to cold, certainly vasospastic phenomenon (Figure 6). At the brachial artery level, overt changes occurred, both vasodilatation and vasoconstriction, with a slight preponderance of the latter. Popliteal artery perfusion appeared to be less affected by immersion of feet in ice-water (Table 2).
Finally, observation of the vasculature of the conjunctiva or sclera (ie.e., sclera scan) usually reveals irregular, sluggish blood flow, sludging with aggregated red blood cells in conjunctival arterioles, and, often missing capillary flow (Figure 7 C and D) compared with controls (Figure 7 A and B).
We believe we have demonstrated that a group of patients who comprise a category between normal individuals and those with overt Raynaud's phenomenon can usually be documented by clinical criteria alone. Detailed laboratory investigations seem to provide an objective method permitting diagnosis of the atypical vasospastic with greater accuracy when clinical assessment is questionable. We consider these patients to have what we have termed "atypical Raynaud's phenomenon." When the symptoms are sufficient to severely disable
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or to inconvenience the patients, oral treatments with methyldopa (Aldomet) or propranolol (Inderal) is begun in an attempt to relieve the symptoms. An analysis of the utility of these agents is in progress. Intraarterial reserpine and sympathectomy have been reserved for advanced Raynaud's patients with threatened digital tissue loss. It is apparent that more work in this area is needed.
1. Raynaud, M. 1863. Del'Asphyxie Locale et de la Gangrene Symmetrique des Extremites Regnoux. Paris.
2. DeTakats, G., and Fowler, E. F. 1962. The Neurogenic Factor in Raynaud's Phenomenon. Surgery 51:9.
3. Halpern, A., and Shaftel, E. 1962. Raynaud's disease, Raynaud's phenomenon, and serotonin. Angiopatias 2:241.
4. DeTakats, G., and Fowler, G. F. 1962. Raynaud's phenomenon. JAMA 179:1.
5. Jameison, M. B., Ludbrook, M. B., and Wilson, A. 1971. Cold hypersensitivity in Raynaud's phenomenon. Circulation 44:254.
6. Gifford, R. W. 1963. Arteriospastic disorders of the extremities. Circulation 27:970.
7. Kontos, H. A., and Wasserman, A. J. 1969. Effect of reserpine in Raynaud's phenomenon. Circulation 39:259.
8. Bauer, M., Ioannovich, J., and Schwammberger, K. Die Klinische Bedeutung Venenverschluss plethysmographie am Finger bei akralen Durchblutungsstorungen. Zeitschrift fur Kardiologie, Band 62, Heft 5.
9. Allen, E. V., Barker, N. W., and Hines, E. A., Jr. 1962. Peripheral Vascular Diseases. W. B. Saunders Company, Philadelphia.
10. Whitney, R. J. 1953. The measurement of volume changes in human limbs. J. Physiol. 121:1-27.
11. Clarke, R. S. J., and Hellon, R. F. 1957. Venous collection in forearm and hand measured by the strain-gauge and volume plethysmograph. Clin. Sci. 16:103.
12. Eagan, C. J. 1960. The physics of the mercury strain gauge and of its use in digital plethysmography. In AAL Technical Note 60-17. Arctic Aeromedical Lab, Ladd AFB, Alaska. In press.
13. Baker, D. W., and Stegell, G. F. A sonic transcutaneous bloodflow meter. Proc. 17th Ann. Conf. Eng. Med. Biol. p. 76.
14. Morri, R. L. 1973. The resolution of the ultrasound pulsed Doppler for blood velocity. J. Biomech. 6:701-710.
15. McLeod, F. D., Jr. A directional Doppler flowmeter. In B. Jacobsen (ed.), Digest 7th Int. Conf. Med. Biol. Eng., pp. 13-14. Stockholm.
16. Wilkins, R. W., and Eichna, L.W. 1941. Blood flow to the forearm and calf. Bull. John Hopkins Hosp. 68:425.
Noninvasive Cardiovascular Diagnosis
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Edited by Edward B. Diethrich
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