Ann Thorac Surg 2009;88:e58-e59. doi:10.1016/j.athoracsur.2009.07.090
© 2009 The Society of Thoracic Surgeons
Case Reports
Uncommon Etiology of an Anterior Chest Wall Mass
Gary S. Schwartz, MDa,*,
Liliana Rios, MDb,
Tracy Zivin-Tutela, MDb,
Faiz Y. Bhora, MDa,
Cliff P. Connery, MDa
a Department of Surgery, Division of Thoracic Surgery, St. Luke's-Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York
b Department of Medicine, Division of Infectious Diseases, St. Luke's-Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, New York, New York
Accepted for publication July 27, 2009.
* Address correspondence to Dr Schwartz, Department of Surgery, St. Luke's-Roosevelt Hospital Center, Columbia University College of Physicians and Surgeons, 1000 10th Ave, Ste 2B, New York, NY 10019 (Email: gschwart{at}chpnet.org).
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Abstract
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A rare but important constellation of musculoskeletal and cutaneous symptoms, including synovitis, acne, pustulosis, hyperostosis, and osteitis, has recently been designated the SAPHO syndrome. The exact etiology is unknown, although various infectious agents have been proposed. The most common site of osteoarticular involvement is the sternoclavicular joint, and therefore, recognition of this syndrome and appropriate workup and management is crucial in the differential diagnosis of an anterior chest wall mass.
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Introduction
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We present a case of an anterior chest wall mass that was ultimately discovered to be part of a rare but important constellation of musculoskeletal and cutaneous symptoms, including synovitis, acne, pustulosis, hyperostosis, and osteitis, known as the SAPHO syndrome. Although SAPHO syndrome is uncommon, knowledge of this constellation of symptoms and the recommended management is necessary for the complete evaluation of an anterior chest wall mass.
A 39-year-old woman with no medical history presented complaining of gradual onset of swelling and dull but constant pain over her left clavicle for the previous 3 months. Upon further questioning, she reported similar symptoms intermittently over 2 years, occasionally associated with right hip pain. She denied any history of trauma to any of these sites, intravenous drug use, recent antibiotic use, or occupational or veterinary exposures, although she did have an extensive travel history. She had no medical or environmental allergies.
The physical examination found she was afebrile with normal vital signs. Her chest examination was significant for a 3 x 5-cm mass overlying the left sternoclavicular joint that was erythematous and warm to the touch. Her lungs were clear to auscultation and there was no cervical, supraclavicular, or axillary lymphadenopathy. Her white blood cell count and differential were within normal ranges, but her erythrocyte sedimentation rate was elevated at 80 mm/h. Results of serologic studies for cryptococcus and histoplasmosis were negative, as was a tuberculosis skin test (purified protein derivative).
The result of a chest roentgenogram was normal, but a computed tomography scan (Fig 1) was significant for costochondritis of the left first costosternal junction, with ossification of the anterior junction and sclerotic changes in the adjacent bones. A technetium-99 bone scan (Fig 2) showed a large focus of uptake in the medial aspect of the left clavicle and the left side of the manubrium and adjacent first rib, as well as a focal area of increased uptake involving the superior aspect of her right sacroiliac joint.
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Fig 1. (A) A computed tomography scan of the chest with (B) 3-dimensional reconstruction shows the sclerotic changes of the left first rib, manubrium, and clavicle.
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Fig 2. Technetium-99 bone scan shows a large focus of uptake at the left sternoclavicular joint and a focal area of increased uptake at the right sacroiliac joint.
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The decision was made to take her to the operating room for débridement of a presumably infectious source. A curvilinear infraclavicular incision was made over the mass. The pectoralis major was elevated off the sternum medially to expose the sternoclavicular joint, which revealed no gross purulence or sinus tracts but irregular, sclerotic bone that was sharply débrided. After multiple specimens were sent for culture and pathologic analysis, the pectoralis major was reapproximated and the skin closed.
Histopathology was significant for reactive and sclerotic bone with adjacent lymphoplasmacytic acute inflammatory infiltrates. No neoplastic processes were evident. Cultures were significant for Propionibacterium acnes from 2 specimens and coagulase-negative Staphylococcus spp in 1 specimen, both isolated from broth media. Mild erythema persisted, and concern for adequate culture data and possible contamination as well as the nonspecific histologic findings prompted a second surgical débridement 3 weeks later. No antibiotics were given during this period.
The previous incision was opened and extended laterally, and the pectoralis major was again mobilized. The clavicle was noted to be persistently sclerotic, as was the first rib; both were sharply débrided to healthy bone and sent for culture and pathology. Again, no gross purulence was encountered. The wound was copiously irrigated and closed primarily. The specimens were again negative for malignancy with nonspecific inflammatory changes, but multiple cultures were again positive for P acnes.
Upon further questioning, she complained of a significant acne outbreak in the postoperative period after the first débridement. She also reported a remote (9 months prior) scalding rash on both hands, which she attributed to a detergent allergy. Given the findings of sternoclavicular synovitis, acne, palmar pustulosis, and osteitis of the sternum, clavicle, rib, and hip, the diagnosis of SAPHO syndrome was proposed.
The patient was treated with 6 weeks of oral doxycycline for the P acnes, after which she was asymptomatic, with a normal erythrocyte sedimentation rate. The anterior chest had trace residual edema with no erythema and a well-healed incision. Her hip and rib pain resolved with no further episodes of palmar pustulosis or acne.
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Comment
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SAPHO syndrome is a rare clinical entity defined as synovitis, acne, pustulosis, hyperostosis, and osteitis. Although it is considered idiopathic, some attribute an infectious etiology, namely P acnes and S aureus. Musculoskeletal lesions include synovitis, hyperostosis, and aseptic osteitis most commonly affecting the anterior chest wall; however, the sacroiliac joints, vertebral bodies, and mandible have been reported with variable incidence. Cutaneous lesions tend to precede osteoarticular symptoms and include palmoplantar pustulosis, severe acne, and hidradenitis suppurativa [1].
The course SAPHO syndrome is chronic, often with self-limited exacerbations. The various symptoms may occur simultaneously but are often metachronous and can even be decades apart [2]. Although diagnosis is only possible after exclusion of infectious arthritis and osteomyelitis, some include the syndrome among the seronegative spondyloarthropathies associated with inflammatory bowel disease, reactive arthritis, and psoriatic arthritis.
Because of the unknown etiology and limited incidence of this SAPHO syndrome, current treatment is controversial. Antibiotics are uniformly administered to treat a proposed infectious etiology, however, rarely alone lead to remission [3]. As a result, surgical débridement is pursued, at the minimum to rule out an underlying malignant process and obtain tissue for culture [4]. Débridement may require resection of the medial clavicle or ipsilateral manubrium, or both, with reconstruction using a pectoralis flap [5, 6]. Of note, the previous reports of surgical débridement of sternoclavicular joint infections were considered spontaneous or secondary to trauma. Nonsteroidal anti-inflammatory agents satisfactorily control symptoms in many patients, but are not considered definitive treatment. Bisphosphonates are helpful in regenerating sclerotic bone but are incapable of treating an underlying infectious pathogen.
In conclusion, SAPHO syndrome is a rare and poorly understood constellation of symptoms that can include an anterior chest wall mass. The precise etiology is unknown, although an infectious component is likely. Current recommendations for treatment are controversial, but should include prompt surgical débridement with possible resection of the sternoclavicular joint and adequate tissue and bone culture data for antibiotic selection. This entity should be considered in the differential diagnosis of an anterior chest wall mass and appropriately evaluated for concurrent symptoms as well as previous episodes because SAPHO syndrome tends to be chronic with acute exacerbations.
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References
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- Govoni M, Colina M, Massara A, Trotta F. SAPHO syndrome and infections Autoimmun Rev 2009;8:256-259.[Medline]
- Touma Z, Arayssi T. Long-delayed onset of chest wall pain defining a patient with SAPHO syndrome J Clin Rheumatol 2007;13:338-340.[Medline]
- Rozin AP, Nahir AM. Is SAPHO syndrome a target for antibiotic therapy? Clin Rheum 2007;26:817-820.[Medline]
- Haddad M, Maziak DE, Shamji FM. Spontaneous sternoclavicular joint infections Ann Thorac Surg 2002;74:1225-1227.[Abstract/Free Full Text]
- Song HK, Guy TS, Kaiser LR, Shrager JB. Current presentation and optimal surgical management of sternoclavicular joint infections Ann Thorac Surg 2002;73:427-431.[Abstract/Free Full Text]
- Burkhart HM, Deschamps C, Allen MS, Nichols FC, Miller DL, Pairolero PC. Surgical management of sternoclavicular joint infections J Thorac Cardiovasc Surg 2003;125:945-949.[Abstract/Free Full Text]
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Abstract
Introduction
