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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 1  |  Issue : 1  |  Page : 21-27

Neglected multi-articulate chronic tophaeceous cyst: Case reports one review of literature


Department of Surgery, State House Medical Centre, Aso Rock, Abuja

Date of Submission09-Aug-2021
Date of Acceptance26-Nov-2021
Date of Web Publication30-Aug-2022

Correspondence Address:
Dike Chijioke Obalum
Department Of Surgery, State House Medical Centre, Aso Rock
Abuja
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jnam.jnam_8_21

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  Abstract 

Gout is a metabolic disease characterised by acute or chronic arthritis and deposition of monosodium urate crystals around joint, bones, soft tissue and kidneys.
This is the case of a 51 year old man who re-presented with a nineteen year history of joint pains and five year history of multiple joint swellings. This followed his defaulting from follow up after an earlier presentation 16 years previously with acute gouty arthritis.
Clinical evaluation revealed a middle aged man with multiple swellings over the hands, ankles, feet, elbows and knees. Serum uric acid was very elevated and radiographs revealed large tophi involving multiple joints. Abdomino-pelvic ultrasound scan and renal function tests were normal.
He was subsequently treated with medications and had significant improvement with clear regression of the size of the tophi. This case demonstrates that delayed treatment of gout or neglected gout over time can lead to tophaceous gouty arthritis.

Keywords: Gout, Gouty arthritis, Gouty tophi


How to cite this article:
Obalum DC. Neglected multi-articulate chronic tophaeceous cyst: Case reports one review of literature. J Niger Acad Med 2022;1:21-7

How to cite this URL:
Obalum DC. Neglected multi-articulate chronic tophaeceous cyst: Case reports one review of literature. J Niger Acad Med [serial online] 2022 [cited 2022 Nov 27];1:21-7. Available from: http://www.jnam.com/text.asp?2022/1/1/21/354772




  Introduction Top


Gout is a metabolic disease characterised by monosodium urate crystal deposition in joint, bones, and different body tissues including skin and soft tissues.[1],[2] It can manifest as acute or chronic arthritis.[1]

Chronic tophaceous gout frequently occur after 10 years or more of recurrent polyarticular gout. Our case is a rare form of tophaceous gout, which presented with generalised tophi after years of untreated disease. Tophaceous gout is present in approximately 12–35% of patients with gouty arthritis.[3] The presence of tophi have been associated with significant morbidity including poor quality of life and increased healthcare resource use.[3]

The tophi have the potential to cause irreversible joint damage with bony destruction depending on their anatomic location.[4],[5] Tophi can also cause radiculopathy[6] or entrap nerves via compressive neuropathy, such as carpal tunnel syndrome.[7],[8] Superficial tophi are also susceptible to infection, especially if the overlying skin is ulcerated.[2]


  Clinical presentation Top


Usually gout attacks are monoarticular initially. However, polyarthritic attacks can occur.[9] More than 75% of acute gout attacks affect a joint in the lower extremity, especially the first metatarsophallangeal joint.[9]

Patients with recurrent gout have a longer duration of illness and are more likely to have polyarthritic attacks.[9] In addition to the great toe, other areas affected include the heels, ankles, knees, fingers, wrists, and elbows. Acute gouty arthritis has the chief complaint of agonising pain accompanied by signs of inflammation and low grade fever. These attacks are usually worse at the night.[9]

Tophaceous gout

Tophi are nodular masses of monosodium urate crystals deposited in the soft tissues of the body. They are a late complication of hyperuricemia.[9] Rarely tophi can develop without previous acute gouty arthritis.[10] The most common site of deposition of urate are the base of the great toe, and the fingers, wrist, hand, olecranon, bursae, and Achilles tendon.[9]

Tophi occur, on average, approximately 12 years after the initial attack with reported rang of 3 to 42 years after initial symptoms.[1],[11] Complication of tophi include pain, soft tissue damage, deformity, joint destruction and nerve compression syndromes such as carpal tunnel syndromes.[9]

Diagnostic evaluation

Apart from the musculo-skeletal system, patients with gout sometimes also have hypertension and impaired renal function, thus, examination of the renal and cardiovascular system is very essential to detect any associated affectation of this organ by this disease.[9] Baseline laboratory test should include complete blood count, urinalysis, and serum creatinine, blood urea nitrogen and serum uric acid measurements.[9]

Plain radiography is not very useful in diagnosing initial attacks of gouty arthritis since the radiographic findings are generally non`-specific, consisting of soft tissue swelling around the joint.[9] The presence of visible bony abnormalities indicates the presence of chronic gout.[9] The classic radiological features are the presence of tophi, an overhanging edge of cortex and a ‘‘punched out’’ erosion of bone with sclerosed borders.[12] Mineralisation is normal and joint space are preserved. Distribution includes the feet ankles knees, hands and elbow.

Ultrasound –specific diagnostic features include a hyperechoic, irregular band over the superficial margin of the articular cartilage described as a double contour sign in 92%of gouty joint.[13] Thus may serve as a non-invasive means to diagnose gout.[13]

Magnetic resonance imaging (MRI) is not routinely used in the evaluation of gout. Rarely, a gouty tophus may mimic an infectious or neoplastic process. In this instance, MRI evaluation is necessary.[9]

Joint aspiration -The diagnosis of gout is confirmed by the presence of polymorphonuclear leukocytes and intracellular monosodium urate crystals in the synovial fluid aspirated from the inflamed joint. Examination of the aspirated joint fluid can also rule out other disorders that mimic gout, such as septic arthritis and pseudogout.

Treatment

Serum uric acid concentration may be reduced with non-pharmacologic therapy. Useful dietary and lifestyle changes include weight reduction, decreased alcohol consumption, decreased consumption of foods with high purine content and control of hyperlipidemia and hypertension. When this non-pharmaologic therapy is used alone, however, the serum uric acid levels is normalised, which is the treatment goal of acute gout attacks.

For symptomatic acute gouty attacks, three treatments are currently available the reduce symptom. These include non-steroidal anti-inflammatory drugs (NSAIDs), colchicine, corticosteroid.[9] Other medications that can be used include allopurinol, indomethacin, chloroquine and even anti-neoplastic agents in gout resistant to conventional agenta. NSAIDs are rapid-acting drugs and currently the most favoured treatment in acute gout failure.[9] Indomethacin is generally the drug of choice but others can be used.[9] However, the gastrointestinal side effect of the NSAIDs should be kept in mind while administering the medication. It should also be used with caution in patient with history of peptic ulcer disease, congestive heart and chronic renal.

Colchicine is an agent that is an effective alternative to NSAID in the treatment of acute gouty arthritis. It is most beneficial when it is given in the first 12 to 36 hours of the attack. It has nil analgesic properties but only anti-inflammatory properties and is given orally or parenterally.[9]

Corticosteroids may be administered to mono-arthritic gout and are may be given orally or intra-articularly when other medications are not effective or are contraindicated, systemic parenteral route may be used.

Urate lowering therapy (ULT) is widely accepted as the initial treatment for tophaceous gout.[14] ULT common goal is a target serum urate of less than 6 mg/dL as this level of uric acid suppression has been associated with increased functionality of joints, a decrease in the number and size of tophi, and improved overall control of symptoms.[15],[16] The aim of lowering levels of serum urate is to prevent recurrent attacks of gouty arthritis and for tophus resolution.[14] Drugs used include uricosuric drugs such as probenecid, sulfinpyrazone and the xanthine oxidase inhibitor such as allopurinol.

Allopurinol is the drug of choice in patient with severe tophaceous deposit. It is a xanthine oxidase inhibitor that impairs the conversion of hypoxanthine to xanthine and the conversion of xanthine to uric acid. The effect of drugs depends on the dosage (dose dependent effect). Allopurinol in a dosage of 300 mg per day has been reported to reduce serum urate concentration to less than 7 mg per dl in 70% of patients.[17] Febuxostat can also be used.[2] For refractory or tophaceous gout (for example, persistently elevated serum urate and symptoms despite optimised management with oral ULTs), the recombinant uricase enzyme pegloticase is now an option.[14]

The surgical treatment of gout is an ancient remedy, and before effective medical treatment, surgery was frequently performed. It was most commonly recommended for cosmetic reasons or for removal of large deposits of sodium urate crystal, tophi. This procedure is described as “debulking”.[18]

Case report

A 51 year old man, a public servant presented six months ago with a 15 year history of multiple joint swellings. He was earlier successfully treated over 15 years ago for acute gouty arthritis that started as severe right big toe pain. He however defaulted from clinic appointment till now in 2021, presenting with multiple articular pains and swellings. He has been on native treatment at traditional bone setters care during this default period but was not satisfied with disease progression necessitating presentation in this hospital. There was no family history of gout and he is a muslim who does not drink alcohol, but consumes a diet rich in beef and purine.

Physical examination revealed swollen tender joints with limitation of movement. Specifically affected are elbows, wrists, fingers, knees, patella tendons, Achilles tendons, ankles and feet. On further clinical evaluation, he was noted to have multiple hard swellings (which had developed in the last 15 years) over the hands, feet, elbows and knees. These swellings are shown on the clinical pictures below.

Further diagnostic assessment revealed leucocytosis, elevated C-reactive protein and an elevated serum uric acid value, but normal renal function test. Representation of the serum uric acid levels over time in mg/dL are as follows: 1n year 2002: 11.7, February 2021: 12.0, April 2021: 10.8, June 2021: 5.8. Intra-articular fluid showed birefringent crystals.

Radiological investigation showed peri-articular soft tissue swellings and destruction of the articular surfaces of some of the involved joints as shown on the radiographs below. Other radiological investigations were essentially normal [Figure 1][Figure 2][Figure 3][Figure 4][Figure 5][Figure 6][Figure 7][Figure 8].
Figure 1: Pictorial view of both feet showing tophi on the Achilles tendons, prominent on the right foot

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Figure 2: Anterior-posterior and lateral radiographic views of the ankle joint demonstrating soft tissue prominence (indicated by the arrows) overlying the Achilles tendon, tophi and sclerotic changes at the tibiofibular joint bilaterally

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Figure 3: Anterior views of the knee joint showing tophi with ulceration

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Figure 4: Lateral and medial veiw of the knee joint showing several tophi

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Figure 5: An Anterior-posterior radiographic views of the knee joints showing tophi on the quadriceps, patellar and gastrocnemius tendons and in the popliteal fossa. Erosions on the medial condyle of the right tibia and the head of the fibula

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Figure 6: Picture of both feet showing several tophi with the most prominent on the 1st metatarsophalangeal joint of the left big toe where the pathology started initially in 2002

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Figure 7: An anterior-posterior and lateral view of both feet showing tophi on the 1st metatarsophalangeal joint, Achilles tendon

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Figure 8: An Anterior-posterior and lateral radiographic views of both elbow joint showing erosions caused by tophi on the olecranon process (indicated by the arrows) bilaterally

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Patient was subsequently treated with Colchicine (1 mg/day) and NSAIDs initially during the acute phase. Later w\he was placed on allopurinol 100 mg twice daily for one week, then 200 mg twice daily for six weeks. He also followed dietary modifications of reduced protein and purine consumption. He clinically improved and after four weeks he had significant improvement in symptoms (visual analogue pain score improved from 8/10 to 4/10). The swellings have also started reducing in size.


  Discussion Top


Gout is a disorder of purine metabolism and result from long standing hyperuricaemia and urate crystal deposition in various tissues.[15] In the first stage, it usually affects the first metatarsophalangeal joint and less commonly other joints such as mid tarsi, knees, hand, feet, ankle and arm.[15] The risk factor include older age, male sex, post-menopausal state and black race as well as use of certain drugs such as diuretics, cyclosporine, low dose of aspirin.[19]

Tophi can occur in soft tissue, osseous tissues, ligament and different organs and either in presence or absence of gouty arthritis. Typical location for tophi include the helix of the ears, fingers, toes, wrist, knees, on olecranon bursae, on achilles tendons, and also rarely in the sclera and subconjuctivally.[20]

Our case had multiple large tophi which are not unusual in chronic gout, he had the disease for over fifteen years. Over time, poorly controlled gout may progress to a chronic phase, characterised by polyarticular attacks, painful symptoms between acute flares and monosodium urate crystal deposition (tophi) in soft tissues or joints.[21] Differential diagnosis for subcutaneous or articular nodules includes septic arthritis, synovial cysts, nodal osteoarthritis, rheumatoid arthritis, sarcoidosis, lymphoma or neoplasms.[22] Synovial fluid or tophus aspiration permits diagnosis through demonstration of negatively birefringent monosodium urate crystals.[21] This was done in our patient, and it was positively diagnostic in conjunction with the radiological appearance of tophi and the elevated urate levels in blood.

Current treatment options for acute gouty attacks include dietary and lifestyle modifications, NSAIDs, colchicine, oral or topical steroids and corticotropin (ACTH). Interleukin-1 (IL-1) antagonists, such as anakinra, a human recombinant IL-1 receptor antagonist and canakinumab, a monoclonal antibody against IL-1β, have also shown promising results in the treatment of refractory cases or cases intolerant to classical therapy.[21] Our patient was treated with NSAIDs, colchicine and allopurinol with good results.

Even without treatment, acute attacks may resolve spontaneously within seven to 10 days. Normalising hyperuricemia is of cardinal significance for the control of recurrent attacks and for the regression of tophi. This is achieved with drugs, which either enhance uric acid excretion (probenecid), convert uric acid into soluble allantoin (pegloticase), or inhibit uric acid production (allopurinol, febuxostat)[15]

Lowering of serum urate level with xanthine oxidase inhibitors and uricosuric agents prevents acute flares and tophi development.[23] These agents are therefore good for long term control of serum urate levels. Allopurinol was prescribed for our patient at initial presentation, but he failed to comply. He rather went for native treatment, only to represent 13 years later with multiple gouty tophi. Interestingly, he is currently doing well on allopurinol, with significant reduction in serum urate levels and reduction in the size of the multiple gouty tophi.

Surgical treatment is seldom required. It is usually reserved for cases of recurrent attack with deformities, severe pain, infection, and joint destruction.[24] It may also be used when tophi are unsightly, painful or when it interferes with tendon function or causes skin necrosis and ulceration or if it encroaches upon nerves causing symptoms of compression.[25] Amputation is always a valid option for untreatable and infected ulcerations.[26] Especially when associated with some form of gangrene. Fortunately our patient has not reached this stage.[27]


  Conclusion Top


Early treatment is key to avoiding progression of acute gout to the chronic tophaceous form. In this 21st year of the 21st century, the case presented is a rare multi-articular gouty tophi complicating untreated gout.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Aradoini N, Talbi S, Berrada K, Abourazzak FZ, Harzy T Chronic tophaceous gout with unusual large tophi: Case report. Pan Afr Med J 2015;22:132.  Back to cited text no. 1
    
2.
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Khanna PP, Nuki G, Bardin T, Tausche AK, Forsythe A, Goren A, et al. Tophi and frequent gout flares are associated with impairments to quality of life, productivity, and increased healthcare resource use: Results from a cross-sectional survey. Health Qual Life Outcomes 2012;10:117.  Back to cited text no. 3
    
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Lee SJ, Nam KI, Jin HM, Cho YN, Lee SE, Kim TJ, et al. Bone destruction by receptor activator of nuclear factor κb ligand-expressing T cells in chronic gouty arthritis. Arthritis Res Ther 2011;13:R164.  Back to cited text no. 4
    
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Rich JT, Bush DC, Lincoski CJ, Harrington TM Carpal tunnel syndrome due to tophaceous gout. Orthopedics 2004;27:862-3.  Back to cited text no. 8
    
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14.
Khanna D, Fitzgerald JD, Khanna PP, Bae S, Singh MK, Neogi T, et al; American College of Rheumatology. 2012 american college of rheumatology guidelines for management of gout. Part 1: Systematic nonpharmacologic and pharmacologic therapeutic approaches to hyperuricemia. Arthritis Care Res (Hoboken) 2012;64:1431-46.  Back to cited text no. 14
    
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Perez-Ruiz F, Calabozo M, Pijoan JI, Herrero-Beites AM, Ruibal A Effect of urate-lowering therapy on the velocity of size reduction of tophi in chronic gout. Arthritis Rheum 2002;47:356-60.  Back to cited text no. 15
    
16.
Becker MA, Schumacher HR Jr, Wortmann RL, MacDonald PA, Eustace D, Palo WA, et al. Febuxostat compared with allopurinol in patients with hyperuricemia and gout. N Engl J Med 2005;353:2450-61.  Back to cited text no. 16
    
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Yu Gutman AB Effect of allopurinol on serum and urinary uric acid in primary and secondary gout. Am J Med 1564;37:885-98.  Back to cited text no. 17
    
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Casangrade P Surgery for tophaeceous gout. Semin Arthritis Rheum 1571;1:262-73.  Back to cited text no. 18
    
19.
Wortmann RL, Kelley WN Gout and hyperuricemia. In: Harris ED, Budd RC, Genovese MC, Firestein GS, Sargent JS, Sledge CB, editors. Kelley’s Textbook of Rheumatology. 7th ed. Philadelphia: Elsevier Saunders; 2005. p. 1402-29.  Back to cited text no. 19
    
20.
Sarma P, Das D, Deka P, Deka AC Subconjunctival urate crystals: A case report. Cornea 2010;29:830-2.  Back to cited text no. 20
    
21.
Iacobellis G, Iacobellis G A rare and asymptomatic case of mitral valve tophus associated with severe gouty tophaceous arthritis. J Endocrinol Invest 2004;27:965-6.  Back to cited text no. 21
    
22.
Evangelos F, Efstathios R, Vasiliki-Kalliopi B, Stavros M, Emmanouil P, Constantinos V Multi articular chronic tophaceous gout with severe and multiple ulcerations: A case report. J Medical Case Rep 2011;5:397.  Back to cited text no. 22
    
23.
William LA, James KF The surgical management of chronic tophaceous gout. J Bone Joint Surg Am 1558;40:743-72.  Back to cited text no. 23
    
24.
Neogi T Clinical practice gout. N Engl J Med 2011;364:443-452.  Back to cited text no. 24
    
25.
Khandpur S, Minz AK, Sharma VK Chronic tophaceous gout with severe deforming arthritis. Indian J Dermatol Venereol Leprol 2010;76:69-71.  Back to cited text no. 25
    
26.
Ertuğrul Sener E, Güzel VB, Takka S Surgical management of tophaceous gout in the hand. Arch Orthop Trauma Surg 2000;120:482-3.  Back to cited text no. 26
    
27.
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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