Reflex Sympathetic Dystrophy

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RSD is not rare. It is a rheumatic disorder of clinical importance and academic interest. Involvement is usually regional and diffuse, but can be segmental and small in rare cases (Helms et al. 1980). The condition is also referred to as causalgia, Sudeck's atrophy, posttraumatic osteoporosis and angiospasm, reflex neurovascular dystrophy, and the shoulder-hand syndrome. Common symptoms include pain, swelling, stiffness, tenderness, vasomotor and sensory disturbances, hyperesthesia, disability, and skin atrophy, and other trophic skin alterations such as hypertrichosis and hyperhidrosis. The pathogenesis is yet not clarified, although the theory of internuncial pool proposed by Lorente (1938) is widely supported. The theory assumes that painful impulses created by a peripheral injury travel via the afferent pathways to the spinal cord, where a series of reflexes originate. Reflex-

es then spread through the interconnecting pool of neurons to stimulate the lateral and anterior tracts, provoking the efferent pathways that travel to the peripheral nerves and finally causing neurovascular and bone-periosteal alterations of RSD. The identification of "sympathetic vasoactive intestinal peptide-containing nerve fibers" innervated at the cortical bone and bone-periosteal junction has provided a biochemical basis for the theory (Hohmann et al. 1986). Va-soactive intestinal peptide released from such sympathetic nerve fibers has been shown to cause hyperemia and dramatic bone resorption as in RSD. As described below, our recent pinhole SPECT study demonstrated that the spotty uptake in RSD is peculiarly localized to the peripheries of the tarsal bones. The areas appear to correspond to patchy bone erosions shown ra-diographically in the corticoperiosteal junctions (Bahk et al. 1998).

The diagnosis of RSD largely depends upon symptoms and radiographic and scintigraphic findings. Radiographic manifestations include confluent patchy osteopenia, subperiosteal bone resorption, and subcortical erosions of the carpal bones (Genant et al. 1975) (Figs. 14.10A and 14.12A) or tarsal bones (Figs. 14.13B and 14.14B). Band-like porosis is seen in the distal ends of the radius and ulna, and also the meta-carpal bases when the wrist is involved (Figs. 14.10A and 14.12A). The osteoporosis in RSD may be diffuse (Fig. 14.10A) or peripheral and focal (Fig. 14.12A). Nuclear angiography is useful for the study of RSD, and shows hyper-perfusion (Kozin et al. 1981) (Fig. 14.10B). Planar pinhole scanning shows mottled and band-

Fig. 14.10A-C Reflex sympathetic dystrophy syndrome (RSDS). A Dorsoventral radiograph of the left wrist in a 31-year-old man with severe wrist pain and skin discoloration shows marked porosis in the entire wrist bones. Minimal erosions are noted in the distal radius. B Scintiangiogram reveals increased blood pool in the wrist (arrowheads). C Pinhole scintigraph shows discrete linear and mottled tracer uptake in the radial end and the carpal bones. Note that tracer uptake is peripheral in location and does not necessarily correspond to the radiographic porosis

Rsd Reflex Sympathetic Dystrophy PhotosLower Limb Molttled

Fig. 14.11 Speckled and coarsely granular tracer uptake in disuse osteoporosis. Anterior pinholes scintigraph of the left knee in a 23-year-old woman with a disused left lower limb due to foot pain shows coarsely granular and irregularly mottled tracer uptake in the bones about the knee. More intense tracer uptake tends to occur in the peripheries (arrowheads)

Fig. 14.11 Speckled and coarsely granular tracer uptake in disuse osteoporosis. Anterior pinholes scintigraph of the left knee in a 23-year-old woman with a disused left lower limb due to foot pain shows coarsely granular and irregularly mottled tracer uptake in the bones about the knee. More intense tracer uptake tends to occur in the peripheries (arrowheads)

like areas of intense uptake that is characteristically situated in the carpal bone peripheries, the distal ends of the radius and ulna, and the metatarsal bases when the wrist is involved (Figs. 14.10C and 14.12B). Similar tracer uptake may occur in the tarsal bones when the ankle is affected (Figs. 14.13A and 14.14A). There is the impression that patchy uptake in RSD does not correlate with radiographic osteoporosis. Such a discordance, which is typically observed in the distal ends of the radius and ulna in our limited cases, would imply that not all radiographic porosis is accompanied by active bone turnover. Interestingly, using pinhole SPECT, we have been able to localize spotty "hot" areas to the ligamentous and tendinous insertions (Figs. 14.13A and 14.14A). Such a peculiar localization is interpreted to point the sites where "the dramatic bone resorption mediated by the vasoactive intestinal peptide released from the sympathetic nerve fibers in RSD" (Hohmann et al. 1986) is seen most typically (Bahk et al. 1998; Kim et al. 2003).

Reflex Sympathetic Nerve Dystrophy

Fig. 14.12A, B The characteristic peripheral tracer distribution in reflex sympathetic dystrophy syndrome (RSDS). A Dorsoventral radiograph of the right wrist in a 34-year-old man with RSDS showing blotchy and mottled porosis in some of the wrist bones (arrows). There are no bone alterations in the distal radius or ulna. B Dorsal pinhole scan showing blotchy tracer uptake characteristically in the peripheries of the navicular and pisiform and in the distal radius and ulna (arrowheads). Note that tracer uptake does not necessarily correspond to radiographic porosis as in Fig. 14.10

Fig. 14.12A, B The characteristic peripheral tracer distribution in reflex sympathetic dystrophy syndrome (RSDS). A Dorsoventral radiograph of the right wrist in a 34-year-old man with RSDS showing blotchy and mottled porosis in some of the wrist bones (arrows). There are no bone alterations in the distal radius or ulna. B Dorsal pinhole scan showing blotchy tracer uptake characteristically in the peripheries of the navicular and pisiform and in the distal radius and ulna (arrowheads). Note that tracer uptake does not necessarily correspond to radiographic porosis as in Fig. 14.10

Ligaments Neck Area

Fig. 14.13A, B Pinhole SPECT findings of reflex sympathetic dystrophy syndrome (RSDS). A Sagittal pinhole SPECT images of the right ankle in a 29-year-old man with posttraumatic RSDS shows spotty hot areas at the ligamentous and tendinous insertions of the bones in the ankle and hindfoot (n talar neck, troc trochlea, stj subtalar joint, tnl talonavicular ligament, iol interosseous ligament, pp posterior process of the talus, tfl talofibular ligament, ttl tibiotalar ligament, ct calcanean tendon). B Lateral radiograph shows subcortical bone erosions at ligamentous and tendinous insertions, including the talar neck (open arrow), posterior talar process (solid arrow), and insertions of talotibial ligament (lig), subtalar joint (stj), and calcanean ligament (ct)

Fig. 14.13A, B Pinhole SPECT findings of reflex sympathetic dystrophy syndrome (RSDS). A Sagittal pinhole SPECT images of the right ankle in a 29-year-old man with posttraumatic RSDS shows spotty hot areas at the ligamentous and tendinous insertions of the bones in the ankle and hindfoot (n talar neck, troc trochlea, stj subtalar joint, tnl talonavicular ligament, iol interosseous ligament, pp posterior process of the talus, tfl talofibular ligament, ttl tibiotalar ligament, ct calcanean tendon). B Lateral radiograph shows subcortical bone erosions at ligamentous and tendinous insertions, including the talar neck (open arrow), posterior talar process (solid arrow), and insertions of talotibial ligament (lig), subtalar joint (stj), and calcanean ligament (ct)

Fig. 14.14A, B Pinhole SPECT findings of reflex sympathetic dystrophy syndrome (RSDS). A Sagittal pinhole SPECT images of the left ankle in a 59-year-old woman with posttraumatic RSDS shows characteristic spotty hot areas at the insertions of the deltoid ligament (dl), tibio-calcaneal ligament in the subtalar joint (stj), posterior tibiofibular ligament (pfl), talonavicular ligament (tnl), bifurcated ligament (bl), and dorsal cuneonavicular ligaments (dcnl). B Lateral radiograph shows blotchy subcortical bone resorption at the insertions of ligaments in the peripheries of the malleoli and tarsal bones (open arrows) (ts talar sulcus, c calcaneus)

Calcaneal Sulcus

Fig. 14.15A, B Homogeneous tracer uptake in regional osteoporosis of the hip. A Anteroposterior radiograph of the painful left hip in a 30-year-old woman shows minimal osteopenia with trabecular coarsening in the femoral head (arrow). B Anterior pinhole scintigraph demonstrates extremely intense tracer uptake in the entire femoral head (arrows). Note well-preserved joint space and intact acetabulum

Fig. 14.15A, B Homogeneous tracer uptake in regional osteoporosis of the hip. A Anteroposterior radiograph of the painful left hip in a 30-year-old woman shows minimal osteopenia with trabecular coarsening in the femoral head (arrow). B Anterior pinhole scintigraph demonstrates extremely intense tracer uptake in the entire femoral head (arrows). Note well-preserved joint space and intact acetabulum

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