551
Radiologic Case A 38-Year-Old Man With Painful, Dependent Ankle Edema and Clubbing TED W. GRACE, MD, MPH, San Diego, California
A "Radiologic Case" is published in this format on a regular basis. Physicians interested in contributing to the series are encouraged to send their radiologic cases to the series' editor. JONATHAN M. LEVY, MD Series' Editor
A 38-year-old man presented with mild edema and arthralgias of his ankles at the end of the day. Both conditions improved with elevation of the feet and the use of anti-inflammatory agents. The patient had smoked a pack of cigarettes per day since age 18. He said he had never had arthritis. Roentgenograms were taken of the ankles (Figure 1).
What is the diagnosis? What test would you do next? SEE FOLLOWING PAGE FOR DIAGNOSIS AND DISCUSSION
Figure 1.-Anteroposterior and lateral films of the right ankle show subperiosteal new bone in the distal diaphyseal regions (arrows).
(Grace TW: A 38-year-old man with painful, dependent ankle edema and clubbing. West J Med 1990 Nov; 153:551-552) From the Clinical Services, Graduate School of Public Health, College of Health and Human Services, San Diego State University, San Diego, California. Reprint requests to Ted W. Grace, MD, MPH, Director of Clinical Services, Student Health Services, San Diego State University, San Diego, CA 92182-0405.
552
RADIOLOGIC CASE
DIAGNOSIS: Hypertrophic Osteoarthropathy Syndrome CHEST FILMS WERE TAKEN, and the patient was admitted to the hospital a week later for evaluation of a 2-cm coin lesion of the left lung (Figure 2). A bronchoscopic biopsy of the lesion showed adenocarcinoma, and after exclusion of metastases, a left upper lobectomy was done. Clubbing of the digits resolved within three months after the operation (Figure 3). The hypertrophic osteoarthropathy syndrome is characterized by the presence of periostitis and new bone formation, arthritis, autonomic dysfunction, and clubbing of the digits. This syndrome has also been referred to as hypertrophic pulmonary osteoarthropathy because more than 90% of cases result from disease in the thorax. With the decline of infectious diseases in the antibiotic era, intrathoracic malignant lesions are now the most frequent cause of hypertrophic osteoarthropathy. It occurs in 4% to 12% of patients with primary lung tumors, primarily bronchogenic
Figure 2.-A chest film shows a 2-cm
mass
in the left lung.
b.',
and pleural carcinomas. It is rare with metastatic tumors of the lung. The syndrome also is seen with cardiac disease (cyanotic congenital heart disease and subacute bacterial endocarditis), liver disease (especially hemochromatosis), gastrointestinal diseases producing acute diarrhea, and various other conditions. The primary form of the syndrome, sometimes called pachydermoperiostosis, may be hereditary or idiopathic. This form is characterized by postpubertal clubbing and bone changes, increased sweating of the palms and soles, and a pronounced thickening of the skin of the face, forehead, and scalp. Clubbing of the digits is a prominent feature of the syndrome but is not always present. Because these features can occur independently, different pathogenic mechanisms may exist. Vasodilatation and increased blood flow appear to promote transudation of fluid and fibroblasts into the soft tissues of the terminal tufts, leading to the deposition of collagen. Clubbing can be extremely difficult for patients and physicians alike to recognize, partly because it develops so insidiously. It can precede the clinical appearance of bronchogenic carcinoma by as much as two years, or it can occur much later in the course of the disease. Therefore, this sign is not a reliable indicator for the duration of disease. Clubbing is also neither sensitive nor specific for any particular disease, but its sudden appearance probably heralds a serious disorder. A unique feature of this syndrome is the aggravation of lower extremity edema and bone pain with dependency. The rheumatic symptoms are rapidly controlled with aspirin or other nonsteroidal anti-inflammatory agents, leading to confusion with the diagnosis of rheumatoid arthritis. The differential diagnosis also includes polyarteritis nodosa, thyroid arthropathy, syphilis, gonococcal arthritis, scurvy, thrombophlebitis, peripheral neuritis, and progressive diaphyseal dysplasia. When clubbing is associated with periostitis of the distal ends of the long bones, the roentgenogram is the key to the correct diagnosis of hypertrophic osteoarthropathy. The ulna and radius are involved in 80% of cases and the tibia and fibula in 74%. Confirming radiographic findings include a symmetric layering of subperiosteal new bone in the distal diaphyseal regions of long bones. Bone scan changes may precede other radiologic alterations by months, and a "parallel-tract" sign from the pericortical deposition of the tracer in the involved areas should help to distinguish the problem from focal metastatic lesions with their central tracer concentration. Although the cause of the syndrome is unknown, both humoral and neurogenic factors have been implicated. Surgical resection of a pulmonary neoplasm is not contraindicated by the presence of this disorder. Success in treating the underlying disease process may cause the clubbing or the hypertrophic osteoarthropathy syndrome to regress, as occurred in this patient. GENERAL REFERENCES
Figure 3.-Top, The finger shows preoperative digital clubbing. Bottom, There is postoperative regression of the clubbing.
Alberts WM: A clinician's guide to clubbing. J Respir Dis 1989; 110:37-46 Armitage KB, Fischer ME: Pitting edema and hypertrophic pulmonary osteoarthropathy. Arch Intern Med 1989; 149:223-224 Hansen-Flaschen J, Nordberg J: Clubbing and hypertrophic osteoarthropathy. Clin Chest Med 1987; 8:287-298 Lopez-Majano V, Sobti P: Early diagnosis of pulmonary osteoarthropathy in neoplastic disease. J Nucl Med Allied ci 1984; 28:69-76 Stenseth JH, Clagett OT, Woolner LB: Hypertrophic pulmonary osteoarthropathy. Dis Chest 1967; 52:62-68
Radiologic Case A 38-Year-Old Man With Painful, Dependent Ankle Edema and Clubbing TED W. GRACE, MD, MPH, San Diego, California
A "Radiologic Case" is published in this format on a regular basis. Physicians interested in contributing to the series are encouraged to send their radiologic cases to the series' editor. JONATHAN M. LEVY, MD Series' Editor
A 38-year-old man presented with mild edema and arthralgias of his ankles at the end of the day. Both conditions improved with elevation of the feet and the use of anti-inflammatory agents. The patient had smoked a pack of cigarettes per day since age 18. He said he had never had arthritis. Roentgenograms were taken of the ankles (Figure 1).
What is the diagnosis? What test would you do next? SEE FOLLOWING PAGE FOR DIAGNOSIS AND DISCUSSION
Figure 1.-Anteroposterior and lateral films of the right ankle show subperiosteal new bone in the distal diaphyseal regions (arrows).
(Grace TW: A 38-year-old man with painful, dependent ankle edema and clubbing. West J Med 1990 Nov; 153:551-552) From the Clinical Services, Graduate School of Public Health, College of Health and Human Services, San Diego State University, San Diego, California. Reprint requests to Ted W. Grace, MD, MPH, Director of Clinical Services, Student Health Services, San Diego State University, San Diego, CA 92182-0405.
552
RADIOLOGIC CASE
DIAGNOSIS: Hypertrophic Osteoarthropathy Syndrome CHEST FILMS WERE TAKEN, and the patient was admitted to the hospital a week later for evaluation of a 2-cm coin lesion of the left lung (Figure 2). A bronchoscopic biopsy of the lesion showed adenocarcinoma, and after exclusion of metastases, a left upper lobectomy was done. Clubbing of the digits resolved within three months after the operation (Figure 3). The hypertrophic osteoarthropathy syndrome is characterized by the presence of periostitis and new bone formation, arthritis, autonomic dysfunction, and clubbing of the digits. This syndrome has also been referred to as hypertrophic pulmonary osteoarthropathy because more than 90% of cases result from disease in the thorax. With the decline of infectious diseases in the antibiotic era, intrathoracic malignant lesions are now the most frequent cause of hypertrophic osteoarthropathy. It occurs in 4% to 12% of patients with primary lung tumors, primarily bronchogenic
Figure 2.-A chest film shows a 2-cm
mass
in the left lung.
b.',
and pleural carcinomas. It is rare with metastatic tumors of the lung. The syndrome also is seen with cardiac disease (cyanotic congenital heart disease and subacute bacterial endocarditis), liver disease (especially hemochromatosis), gastrointestinal diseases producing acute diarrhea, and various other conditions. The primary form of the syndrome, sometimes called pachydermoperiostosis, may be hereditary or idiopathic. This form is characterized by postpubertal clubbing and bone changes, increased sweating of the palms and soles, and a pronounced thickening of the skin of the face, forehead, and scalp. Clubbing of the digits is a prominent feature of the syndrome but is not always present. Because these features can occur independently, different pathogenic mechanisms may exist. Vasodilatation and increased blood flow appear to promote transudation of fluid and fibroblasts into the soft tissues of the terminal tufts, leading to the deposition of collagen. Clubbing can be extremely difficult for patients and physicians alike to recognize, partly because it develops so insidiously. It can precede the clinical appearance of bronchogenic carcinoma by as much as two years, or it can occur much later in the course of the disease. Therefore, this sign is not a reliable indicator for the duration of disease. Clubbing is also neither sensitive nor specific for any particular disease, but its sudden appearance probably heralds a serious disorder. A unique feature of this syndrome is the aggravation of lower extremity edema and bone pain with dependency. The rheumatic symptoms are rapidly controlled with aspirin or other nonsteroidal anti-inflammatory agents, leading to confusion with the diagnosis of rheumatoid arthritis. The differential diagnosis also includes polyarteritis nodosa, thyroid arthropathy, syphilis, gonococcal arthritis, scurvy, thrombophlebitis, peripheral neuritis, and progressive diaphyseal dysplasia. When clubbing is associated with periostitis of the distal ends of the long bones, the roentgenogram is the key to the correct diagnosis of hypertrophic osteoarthropathy. The ulna and radius are involved in 80% of cases and the tibia and fibula in 74%. Confirming radiographic findings include a symmetric layering of subperiosteal new bone in the distal diaphyseal regions of long bones. Bone scan changes may precede other radiologic alterations by months, and a "parallel-tract" sign from the pericortical deposition of the tracer in the involved areas should help to distinguish the problem from focal metastatic lesions with their central tracer concentration. Although the cause of the syndrome is unknown, both humoral and neurogenic factors have been implicated. Surgical resection of a pulmonary neoplasm is not contraindicated by the presence of this disorder. Success in treating the underlying disease process may cause the clubbing or the hypertrophic osteoarthropathy syndrome to regress, as occurred in this patient. GENERAL REFERENCES
Figure 3.-Top, The finger shows preoperative digital clubbing. Bottom, There is postoperative regression of the clubbing.
Alberts WM: A clinician's guide to clubbing. J Respir Dis 1989; 110:37-46 Armitage KB, Fischer ME: Pitting edema and hypertrophic pulmonary osteoarthropathy. Arch Intern Med 1989; 149:223-224 Hansen-Flaschen J, Nordberg J: Clubbing and hypertrophic osteoarthropathy. Clin Chest Med 1987; 8:287-298 Lopez-Majano V, Sobti P: Early diagnosis of pulmonary osteoarthropathy in neoplastic disease. J Nucl Med Allied ci 1984; 28:69-76 Stenseth JH, Clagett OT, Woolner LB: Hypertrophic pulmonary osteoarthropathy. Dis Chest 1967; 52:62-68
