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DOI: 10.1016/J.JHSB.2004.02.013
Chondrocalcinosis of the WristFrom the Institut Français de Chirurgie de la Main, Paris, France Correspondence: Philippe Saffar, MD, Institut Français de Chirurgie de la Main, 5, rue du Dôme, Paris 75116, France. Tel.: +33-1-53655353; fax: +33-1-53655354; E-mail: psaffar{at}ifcm.org
Calcium pyrophosphate dihydrate deposition (CPDD) disease has characteristic radiographic features including soft tissue calcification, joint space narrowing, bone sclerosis, subchondral cyst formation without osteophyte formation, and large intraosseous geodes. Triangular fibrocartilage calcification is frequently found and isolated scapho-trapezio-trapezoid (STT) arthritis is specific for CPDD. Distal radio-ulnar (DRUJ), isolated midcarpal joint and piso-triquetral joint involvement also occur. 127 patients were reviewed. Seventy-eight had symptomatic STT joint arthritis, for which 36 underwent surgery. Twenty-two patients had a SLAC wrist deformity for which ten underwent surgery. Eight patients had isolated midcarpal arthritis for which three midcarpal arthrodeses, two four-bone arthrodeses and two carpal tunnel releases were performed. Nineteen patients had a generalized arthritis and seven of the patients underwent surgery: fourcorner arthrodesis+scaphoidectomy (one case), carpal tunnel relaease (two cases) extensor synovectomy (two cases) and trigger finger release (two cases).
Key Words: wrist • chondrocalcinosis • scapholunate instability • STTR osteoarthritis
Calcium pyrophosphate dihydrate deposition (CPDD) disease (pseudogout or chondrocalcinosis), gout and hydroxyapatite deposition disease are all crystal deposition arthropathies that affect the wrist. CPDD most frequently affects both the soft tissues and bones of the wrist. The pathologic changes increase with age and are relatively asymptomatic, which might explain why CPDD has attracted little attention in the surgical literature. However CPDD can be disabling and it is not correctly diagnosed in many cases. Its onset, frequently between 40 and 50 years of age, can be silent or cause pain, stiffness, tendon or joint capsule inflammation and dorsal wrist synovitis. CPDD is often associated with osteoarthrosis (OA), gout, rheumatoid arthritis and hyperparathyroidism. It is frequently mistaken for posttraumatic or primary osteoarthrosis.
Radiologists and rheumatologists first described the radiological and clinical symptoms of CPDD in the 1970s (Bensasson et al., 1975; Resnick, 1979; Resnick and Niwayama, 1977; Resnick and Utsinger, 1974; Resnick et al., 1977; Utsinger et al., 1975). Its pathobiological and histological features are also well described (Ivorra et al., 1999; Resnick et al., 1977; Stäbler et al., 1990, 1992; Yang et al., 1995). The disease may occur sporadically, or be inherited in an autosomal dominant fashion with a variable penetrance (Bjelle et al., 1982; Uri and Dalinka, 1996). Several families have been investigated (Balsa et al., 1990; Bjelle et al., 1982; Doherty and Dieppe, 1985; Eschel et al., 1990; Gaudreau et al., 1981; Hamza et al., 1992; Nunez- Roldan et al., 1981; Richardson et al., 1983; Salvini et al., 1987) and the clinical onset in these hereditary cases was early and extensive in all but one family. The hereditary ANK gene is for a protein which spans the outer cell membrane and shuttles inorganic pyrophosphate (PPi), a major inhibitor of physiologic and pathologic calcification, bone mineralization and bone resorption. Excess accumulation of extracellular inorganic pyrophosphate (ePPi) in aged human cartilage is crucial for calcium pyrophosphate dihydrate (CPPD) crystal formation in cartilage matrix. ANK expression contributes to higher ePPi accumulation in CPPD crystal-forming cartilage (Hirose et al., 2002; Nurnberg et al., 2001). The mechanism of crystal deposition and synovial change has been elucidated. Firstly crystals appear in the synovial fluid. These are then deposited in the synovial membrane, the wrist ligaments and fibrocartilaginous structures and then in the articular cartilage. The calcium pyrophosphate crystals localize in and around joints. Involvement of the knee and hip are emphasized, and wrist involvement has only recently been described (Bensasson et al., 1975; Resnick and Utsinger, 1974; Utsinger et al., 1975). Crystal deposition at the wrist can cause pseudotumours which bulge into the carpal canal (Raimbeau et al., 2001; Rate et al., 1992). The classic feature which is most specific for CPDD is calcification of the triangular fibrocartilage. Isolated scaphotrapezialtrapezoidal joint disease is also specific to CPDD (Fig 1). The scapholunate joint is the next most frequently involved joint, followed by the distal radioulnar joint (DRUJ) (Fig 2), the lunotriquetral (LT) joint and the isolated midcarpal joint, radiolunate and pisotriquetral joints. There maybe a pancarpal arthrosis at the end stage.
Wright et al. (1994) found near normal radiographs in 62 cadavers, though there were abnormalities including central communicating defects in the SL, LT and TFCC ligaments. These findings confirm Resnick’s belief that "cartilage calcification, or chondrocalcinosis is an important radiographic sign of CPDD, but it need not be evident in an involved joint or, for that matter, in any articulation of the body" (Resnick and Utsinger, 1974). Yang et al. (1995) studied 316 wrist radiographs and demonstrated frequent involvement of the medial wrist compartment: a 28% at the DRUJ, 74% at the TFCC, 77% at the LT ligament and 53% at the LT cartilage, thus LT involvement maybe as common as TFCC involvement (Wright et al., 1994). Berger and Buckwalter (1990) found a relationship between calcium pyrophosphate deposits and degenerative lesions of the TFCC.
Chondrocalcinosis often begins between 50 and 59 years of age and maybe asymptomatic. Women are more affected than men. McCarty (1997) has classified the different clinical presentations (Table 1). When symptomatic, the clinical presentation maybe pain and swelling in the wrist or thumb, decreased strength and range of motion, dorsal wrist synovitis and wrist stiffness. Occasionally, ganglia at the base of the thumb may be present.
CPPD may become symptomatic after wrist trauma, having been previously asymptomatic, though a physical examination might reveal a decreased range of motion and grip weakness. Deformities may also be found at presentation, despite the absence of a past history of wrist disease or any previous trauma or precipitating event. Wrist chondrocalcinosis may also spontaneously (no trauma) cause pain and swelling of the wrist or thumb. This may occur as an acute inflammatory episode that maybe mistaken for a joint infection or an attack of gout. Carpal tunnel syndrome (CTS) is the initial presentation in 14% of the cases. This maybe acute (Gerster et al., 1980; Pattrick et al., 1988; Rate et al., 1992) and the combined presence of CTS and dorsal wrist synovitis should raise suspicions of CPDD and lead to a wrist X-ray. Some special features may help the diagnosis: both wrists are affected in 66% of the cases and the patient is usually about 70 years old. CPDD may be associated with osteoarthritis, gout, rheumatoid arthritis, hyperparathyroidism, hypothyroidism and haemochromatosis, and this can confuse the clinical picture.
The characteristic radiographic features include soft tissue calcification, joint space narrowing, articular surface sclerosis and subchondral cyst formation without osteophyte formation or bone growth (Fig 3). Large intraosseous geodes sometimes occur (20%) (Fig 4) (Bensasson et al., 1975). TFCC calcification is the earliest, most common and specific site of calcification, but isolated STT involvement is also specific to CPDD. DRUJ, isolated midcarpal joint and pisotriquetral joint involvement are other possible locations.
X-ray crystallography may detect crystals in the tissues or synovial fluid. Polarized light microscopic examination will show birefringent biaxial crystals in the joint fluid, but not all crystals are birefringent (Ivorra et al., 1999). Ordinarylight microscopy allows better crystal detection and should proceed observation under polarized light. Peroperative direct observation may not detect the crystalline deposits, which are extremely subtle, especially if there is no acute synovitis.
Some presentations are particularly common, but chondrocalcinosis maybe asymptomatic and simply diagnosed as a chance finding on radiographs performed for another reason (Table 2).
1. STT isolated osteoarthritis (Peter et al., 2001) In isolated STT osteoarthritis there is no associated trapeziometacarpal joint involvement, though radiographs may reveal other calcium deposits, particularly in the TFCC. Pain is the main complaint, and this is very precisely located over the scaphotrapezial joint. With involvement of this joint, the first presentation of the disease may not be pain but (a) a posterolateral or palmar ganglia (a wrist radiograph should be performed with these types of ganglia to exclude CPDD); (b) carpal tunnel syndrome (since the STT joint is the radial wall of the carpal tunnel); (c) abductor pollicis brevis muscle wasting; (d) flexor carpi radialis tendonitis (the tendon lies in contact with the STT joint and wear and tear is frequent; (e) De Quervain’s disease. Definite STT osteoarthritis identified CPDD with a sensitivity of 83% when studied with a concomitant polyarthritis of the finger joints (Stucki et al., 1999). It is also very found in association (50% of cases) with trapezio-metacarpal osteoarthritis, but when there is isolated involvement of the STT joint, it is pathognomonic of CPDD. 2. Scapholunate joint (Bensasson et al., 1975; Chen et al., 1990; Doherty and Lovallo, 1993; Helfgott and Skoff, 1992; Resnick and Utsinger, 1974; Resnick and Niwayama, 1977; Resnick, 1985; Stäbler et al., 1990; Taniguchi et al., 1997). In the initial stage there is a scapholunate dissociation, characterized by a scapholunate gap, without scaphoid rotatory subluxation. In the final stage, there is a SLAC deformity, added to which the proximal pole of the scaphoid has eroded into the scaphoid fossa of the distal radius; this is a characteristic feature of this disease. Scapholunate involvement often occurs in combination with CPDD at other sites. Initially it is often asymptomatic and it typically develops after the age 50. Romano (2003) has called this Scaphoid Chondrocalcinosis Advanced Collapse (SCAC-Wrist). SCAC wrist has been classified as follows: SCAC stage 1: there is a scapholunate gap without scaphoid rotation, little or no calcification, and subchondral sclerosis of the radioscaphoid joint line (Fig 5). This corresponds to what Wright et al. (1994) found in his dissections. Pain and swelling maybe present and areas of calcification and ligament tears are found during surgery.
SCAC stage 2: there is a scapholunate dissociation, with an extended scaphoid and lunocapitate joint narrowing. SCAC stage 3: there is a scapholunate dissociation with an extended scaphoid. The proximal pole of the scaphoid has eroded into the scaphoid fossa of the distal radius. There are few or no osteophytes (Fig 6a and b).
SCAC stage 4: there is total wrist destruction with large bone defects in 20% of cases (Fig 7).
There are two types scapholunate joint involvement: Type 1, without scaphoid rotation (SCAC wrist). The scaphoid is extended and tends to erode into the scaphoid fossa. This maybe explained by stiffness of the STT joint preventing scaphoid rotation. The SL gap is more or less significant. Type 2, the scaphoid is rotated (flexed) and the SL gap is increased (SLAC wrist). This corresponds to initial destruction of the scapholunate ligament by the CCPD deposits followed by scaphoid rotation (Fig 8).
It is of historical interest to note that Watson and Ballet (1984) first described the SLAC deformity in 1984 and Resnick (1985) pointed out in 1985 that scapholunate dissociation might have a non-traumatic cause. 3. Medial wrist compartment involvement (Bensasson et al., 1975; Berger and Buckwalter, 1990; Yang et al., 1995): In this there is TFCC calcification and triquetrolunate dissociation with ligament and cartilage involvement. 4. Radiolunate joint involvement is not present in posttraumatic cases, but is an end stage finding. 5. Isolated midcarpal involvement is rare but pathognomonic of the disease (Fig 9).
6. Generalized destruction of all carpal joints can sometimes occur.
Conservative and surgical treatments are indicated to relieve pain and wrist deformities. Conservative treatment for acute attacks includes splintage, steroid injections and anti-inflammatory drugs. These measures are efficient in two-third of the cases. Surgical treatment is indicated when conservative treatment measures fail. The specific choice of operation depends on the location of the disease. In my series of 127 patients who were treated between 1978 and 2001, there were 78 cases with STT localization and 49 cases with wrist disease at other localizations: the average age of the cases was 65 years and there were 44 men and 83 women. Of the 78 with symptomatic STT joint arthritis, 43 underwent surgery. This was to the STT joint in 36 cases, with seven undergoing a silastic interposition, 10 an STT fusion and 19 a STT resection arthroplasty (Garcia-Elias et al., 1999) (Fig 10). The other seven underwent a carpal tunnel release.
Twenty-two patients presented with a SLAC deformity (SCAC) of which 10 were symptomatic and operated on: four had a four-corner arthrodesis with scaphoidectomy, two had a lunocapitate arthrodesis with scaphoidectomy, three a proximal row carpectomy and one a radial styloidectomy. Eight patients had isolated midcarpal arthritis which was treated in three cases with a midcarpal arthrodesis and in two cases with a four-corner arthrodesis with scaphoidectomy. Two others required carpal tunnel release and one did not need surgery. The other 19 patients had a generalized arthritis (Fig 11). In one case this was treated with a four-corner arthrodesis with scaphoidectomy, three others underwent carpal tunnel releases, two underwent extensor synovectomies and another two trigger finger releases: the other 11 cases were treated conservatively.
The differential diagnosis of CPPD includes osteoarthrosis. Chondrocalcinosis looks like osteoarthrosis, but is more severe and progressive, and the bones maybe fragmented. It may also be mistaken for other crystal deposition arthropathies at the wrist such as gout and hydroxyapatite deposition disease (HADD). However, gout (Bardin and Fritz, 1992; Helfgott and Skoff, 1992; Raimbeau et al., 2001) rarely affects the wrist and in its chronic form is characterized byunilateral involvement in an adult. There is commonly a history of previous episodes of gout affecting other joints and radiographs demonstrate osteolysis and large bone defects, "punched-out" bony erosions, joint space narrowing, limited or total carpal bone necrosis, and severe scapholunate dissociation (Bardin and Fritz, 1992; Helfgott and Skoff, 1992). HADD is extremelyrare at the wrist, but may involve ligaments and tendons (flexor carpi ulnaris) and may also be responsible for intra-articular pathology. It is often difficult to state that a wrist pathologyis due to CPPD deposits and the diagnosis is made on the clinical presentation (age of onset, no history of trauma in the distant past, bilateral wrist involvement, CTS and sometimes ulnar nerve compression at the wrist; knee, shoulder or other joint involvement); and its radiological features (several locations in the wrist or the special features already cited). Recent trauma can make an asymptomatic wrist become painful, probably because CPDD deposits have infiltrated and weakened the ligaments and cartilage. CPDD is probably the most frequently found wrist pathology but it has not attracted much attention mainly because it affects elderly people and is often asymptomatic. However it can affect a variety of areas of the wrist joint (Table 3). It is usually easily diagnosed by its radiological appearance and specific localizations, but it can mimic other arthritides. Treatment is necessary mostly for CTS, STT or SLAC patterns.
Received for publication September 9, 2003. Accepted for publication February 9, 2004.
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Abstract
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