1992, The British Journal of Radiology, 65, 722-724 RESNICK, D. & NIWAYAMA, J., 1988. Osteomyelitis, septic
arthritis, and soft tissue infection: the axial skeleton. In Diagnosis of Bone and Joint Disorders. Ed. by D. Resnick and J. Niwayama (W. B. Saunders Co, Philadelphia), pp. 2619-2646.
Case reports SWISCHUK, L. E., 1989. Imaging of the Newborn, Infant, and Young Child. (Williams & Wilkins, Baltimore), pp. 751-7581018-1021.
Patellar metastasis: a rare presentation By R. K. Sur, MD, D. P. Singh, MD, *M. S. Dhillon, MS, B. D. Gupta, MD, FRCR, B. Murali, MB, BS and tR. Sidhu MB, BS Departments of Radiotherapy, tRadiodiagnosis and 'Orthopaedics, Postgraduate Institute of Medical Education and Research, Chandigarh, India {Received 2 May 1991 and in revised form 30 July 1991, accepted 16 August 1991) Keywords: Metastases, Patella, Bronchogenic carcinoma
Primary patellar neoplasms are uncommon and metastasis to the patella is rarer still. Very few reports have been published in the literature (Stoler & Staple, 1969; Keeley, 1973; Gall et al, 1974; Ashby & Dappen, 1976; Hussey, 1976; Kwa & Nade, 1989). We present a case of primary bronchogenic carcinoma presenting as a huge swelling around the knee owing to a metastatic lesion in the patella. Case report A 50-year-old man was seen initially in the orthopaedic clinic complaining of pain in the region of the left knee of 2 months duration. Detailed questioning revealed a history of long-term smoking together with persistent cough and occasional bloodtinged expectoration. Appetite was diminished and there was some loss of weight. Examination revealed 2 cm quadriceps wasting, limitation of knee flexion to 110° and tenderness over the anterior aspect of the patella. There was a significant effusion. Haemoglobin was 9.5 g%, total leucocyte count 11000 x 106/htre with 30% lymphocytes and the erythrocyte sedimentation rate (ESR) was 29 mm/h (Westergren); Mantoux test was positive. A presumptive diagnosis of tuberculosis of the knee was made and the patient was commenced on anti-tubercular therapy consisting of isoniazid, rifampicin and ethambutol. Chest and knee radiographs were advised but the patient did not return for followup for 3 months. During this period the knee swelling had dramatically increased to a size of 10 cm x 12 cm and the knee was fixed in a position of 90° flexion (Fig. 1). A biopsy performed by a private practitioner was reported as squamous cell carcinoma, and he was referred to the Department of Radiotherapy in February 1991. At this time he had severe pain in the knee and the tumour was fungating through the skin. Detailed systemic examination revealed an impaired percussion note and diminished breath sounds at the base of the right lung below the fifth intercostal space. Chest radiographs showed collapse of the right middle
Address correspondence to Dr M. S. Dhillon, Assistant Professor, Department of Orthopaedics, 1241 Officer's Apartments, Sector 24, Chandigarh 160024, India.
722
and lower lobes as well as a small effusion. Knee radiographs showed complete destruction of the patella (Fig. 2). Computed tomographic (CT) scans at the level of the knee and patella showed complete patellar destruction with only tiny residual fragment of bone (Fig. 3). A radionuclide bone scan was not available at that time. Histology of a bronchoscopic biopsy revealed a keratinizing squamous cell carcinoma and a review of the patellar biopsy showed similar appearances, thus confirming a metastasis. The patient was treated by palliative radiation therapy, delivering a dose of 30 Gy/10 fractions/2 weeks to the right lung and to the left patella. He improved symptomatically but developed low back pain on the last day of the external radiotherapy. Lumbar spine radiographs revealed collapse of the third lumbar vertebra; this was also treated by external radiotherapy (800 cGy/single fraction) and the patient was discharged. Discussion
Although the knee is a common site for bone tumours, the patella is not usually involved (Ehara et al,
Figure 1. Photograph showing patellar swelling and flexion deformity of the left knee. Note the involvement of overlying skin and biopsy scar. The British Journal of Radiology, August 1992
Case reports
Figure 3. CT scan at the level of the distal femur and patella showing complete destruction of the patella. Only a part of the edge of the patella is seen.
Figure 2. (a) Lateral radiograph of the left knee showing complete destruction of the patella, (b) Skyline view of the knee. There is extensive destruction and swelling of the left patella.
1989). Kransdorf et al (1989) reported 42 patellar neoplasms, but found no metastatic lesions. Both the above series stressed vigilance in cases of anterior knee pain. In those cases where patellar destruction is seen on radiographs, rapid increase in size of the lesion and poorly defined margins suggest a metastatic tumour (Kransdorf et al, 1989). Only a handful of reports of patellar Vol. 65, No. 776
metastasis exist in the literature. Keeley (1973) reported bilateral patellar metastasis from carcinoma of the male breast; subsequent radiographs revealed metastatic disease in other bones. Kwa and Nade (1989) described a case of patellar metastasis in renal adenocarcinoma, which also showed increased uptake in the occiput on radionuclide bone scan. Ashby and Dappen (1976) reported a patellar metastasis from oesophageal carcinoma together with a similar lesion in the scapula, and Benedek (1965) described patellar metastasis from bronchogenic carcinoma associated with a second lesion in the scalp. In our patient although a diagnosis of patellar metastasis was entertained once the lung biopsy showed a locally advanced lesion, a radiographic survey of the whole body was not performed. Only when low back pain appeared were further radiographs carried out, which revealed metastatic deposit in the third lumbar vertebra. Patellar metastasis is usually a feature of generalized carcinomatosis when there are sufficient tumour emboli in the circulation. Further investigations to locate other metastatic lesions are thus mandatory. The patella is a sesamoid bone with a relatively poor vascular supply, consisting of a few nutrient branches 723
Case reports
1992, The British Journal of Radiology, 65, 724-725
from the collateral vessels of the knee; this seldom provides sufficient access to tumour emboli for metastatic lesions to develop (Goodwin, 1961). The management of patellar metastasis depends on the size of the lesion. Early deposits without soft-tissue extension may well be treated by patellectomy, whereas larger lesions with soft-tissue extension require palliative external radiotherapy.
Osteolytic lesions of the patella. American Journal of Roentgenology, 153, 103-106. GALL, E. P.,
DIDIZIAN, N.
A. & PARK, Y.,
1974.
Acute
References
monoarticular arthritis following patellar metastasis. Journal of the American Medical Association, 229, 288-289. GOODWIN, M. A., 1961. Primary osteosarcoma of the patella. Journal of Bone and Joint Surgery, 43, 338-341. HUSSEY, H. H., 1976. The vertebral venous system. Editorial. Journal of the American Medical Association, 235, 2524. KEELEY, C. D., 1973. Bilateral patellar metastases from carcinoma of the male breast. Canadian Journal of Surgery, 16, 328-329.
ASHBY, M. E. & DAPPEN, N., 1976. Esophageal carcinoma
KRANSDORF, M. J., MOSER, R. P., JR, VINH, T. N., AOKI, J. &
metastatic to the patella: a case report. Journal of the American Medical Association, 235, 2519-2520. BENEDEK, G. T., 1965. Lysis of the patella due to metastatic carcinoma. Arthritis and Rheumatism, 8, 560-565.
CALLAGHAN, J. J., 1989. Primary tumours of the patella. A review of 42 cases. Skeletal Radiology, 18, 365-371. KWA, S. & NADE, S., 1989. Metastasis in a patella: a rare site. Australian and New Zealand Journal of Surgery, 59, 351-352.
EHARA, S., KHURANA, J. S., KATTAPURAM, S. V., ROSENBERG, A. E., EL-KHOURY, G. Y. & ROSENTHAL, D. I., 1989.
STOLER, B. & STAPLE, T. W., 1969. Metastasis to the patella.
Radiology, 93, 835-836.
Renal ultrasonographic appearances at presentation in an infant with Lesch-Nyhan syndrome By C. N. Ludman, MB, BS, MRCP, *C. Dicks-Mireaux, MRCP, FRCR and A. J. Saunders, FRCP, FRCR Department of Radiology, Guy's Hospital, St Thomas Street, London SW1 9RT and 'Department of Radiology, The Hospital for Sick Children, Great Ormond Street, London WC1 3JH, UK (Received 8 November 1991, accepted 16 January 1992) Keywords: Lesch-Nyhan syndrome, Ultrasound, Hyperechoic cortex, Crystal nephropathy
Lesch-Nyhan syndrome, first described in 1964, is an X-linked recessive disorder of purine metabolism. A cellular deficiency or absence of the purine salvage enzyme hypoguanine phosphoribosyl transferase causes purine overproduction and subsequent hyperuricaemia and gout. Clinically the syndrome manifests as neurological disorders, behavioural disturbances characterized by self mutilation and variable mental retardation. In addition, hyperuricaemia results in the clinical features of gout, with gouty arthritis, tophi, urate and uric acid nephropathies. The previously described renal ultrasonic appearances of Lesch-Nyhan syndrome have referred to patients after they have received allopurinol therapy. We describe a different appearance in a patient with this syndrome, prior to treatment. Allopurinol is a xanthine oxidase inhibitor and leads to an accumulation of the intermediate metabolites xanthine and hypoxanthine. Treated patients develop calculi which appear to be predominantly formed from xanthine secondary to this therapy. We report on the case of an infant who presented at 3 weeks of age with congenital renal failure, who shortly afterwards developed the features of acute tophaceous 724
gout. The renal ultrasonic appearances at presentation were striking and unusual. Case report A 3-week-old male infant in renal failure was transferred to Guy's Hospital. The pregnancy had been unremarkable and the appearance of the kidneys on routine obstetric scanning was reported as normal. Shortly after birth, he developed recurrent vomiting with weight loss and was admitted to his local hospital. He was found to be in renal failure, and was transferred. On admission to Guy's Hospital, the child's plasma urate was 2.2 mmol/1 (n 0.2-0.43) and creatinine was 804 /xmol/1. No detectable activity of hypoxanthine guanine phosphoribosyl transferase was found in intact or lysed erythrocytes, thus establishing the diagnosis of Lesch-Nyhan syndrome. On admission an abdominal radiograph was normal with no evidence of renal tract calcification. The renal ultrasonic appearances showed bilateral markedly echogenic cortices. The medullary pyramids were echo poor, but contained streaks of increased echogenicity (Fig. 1).
Discussion
The previously described ultrasonic findings in Lesch-Nyhan syndrome have been seen following long The British Journal of Radiology, August 1992
arthritis, and soft tissue infection: the axial skeleton. In Diagnosis of Bone and Joint Disorders. Ed. by D. Resnick and J. Niwayama (W. B. Saunders Co, Philadelphia), pp. 2619-2646.
Case reports SWISCHUK, L. E., 1989. Imaging of the Newborn, Infant, and Young Child. (Williams & Wilkins, Baltimore), pp. 751-7581018-1021.
Patellar metastasis: a rare presentation By R. K. Sur, MD, D. P. Singh, MD, *M. S. Dhillon, MS, B. D. Gupta, MD, FRCR, B. Murali, MB, BS and tR. Sidhu MB, BS Departments of Radiotherapy, tRadiodiagnosis and 'Orthopaedics, Postgraduate Institute of Medical Education and Research, Chandigarh, India {Received 2 May 1991 and in revised form 30 July 1991, accepted 16 August 1991) Keywords: Metastases, Patella, Bronchogenic carcinoma
Primary patellar neoplasms are uncommon and metastasis to the patella is rarer still. Very few reports have been published in the literature (Stoler & Staple, 1969; Keeley, 1973; Gall et al, 1974; Ashby & Dappen, 1976; Hussey, 1976; Kwa & Nade, 1989). We present a case of primary bronchogenic carcinoma presenting as a huge swelling around the knee owing to a metastatic lesion in the patella. Case report A 50-year-old man was seen initially in the orthopaedic clinic complaining of pain in the region of the left knee of 2 months duration. Detailed questioning revealed a history of long-term smoking together with persistent cough and occasional bloodtinged expectoration. Appetite was diminished and there was some loss of weight. Examination revealed 2 cm quadriceps wasting, limitation of knee flexion to 110° and tenderness over the anterior aspect of the patella. There was a significant effusion. Haemoglobin was 9.5 g%, total leucocyte count 11000 x 106/htre with 30% lymphocytes and the erythrocyte sedimentation rate (ESR) was 29 mm/h (Westergren); Mantoux test was positive. A presumptive diagnosis of tuberculosis of the knee was made and the patient was commenced on anti-tubercular therapy consisting of isoniazid, rifampicin and ethambutol. Chest and knee radiographs were advised but the patient did not return for followup for 3 months. During this period the knee swelling had dramatically increased to a size of 10 cm x 12 cm and the knee was fixed in a position of 90° flexion (Fig. 1). A biopsy performed by a private practitioner was reported as squamous cell carcinoma, and he was referred to the Department of Radiotherapy in February 1991. At this time he had severe pain in the knee and the tumour was fungating through the skin. Detailed systemic examination revealed an impaired percussion note and diminished breath sounds at the base of the right lung below the fifth intercostal space. Chest radiographs showed collapse of the right middle
Address correspondence to Dr M. S. Dhillon, Assistant Professor, Department of Orthopaedics, 1241 Officer's Apartments, Sector 24, Chandigarh 160024, India.
722
and lower lobes as well as a small effusion. Knee radiographs showed complete destruction of the patella (Fig. 2). Computed tomographic (CT) scans at the level of the knee and patella showed complete patellar destruction with only tiny residual fragment of bone (Fig. 3). A radionuclide bone scan was not available at that time. Histology of a bronchoscopic biopsy revealed a keratinizing squamous cell carcinoma and a review of the patellar biopsy showed similar appearances, thus confirming a metastasis. The patient was treated by palliative radiation therapy, delivering a dose of 30 Gy/10 fractions/2 weeks to the right lung and to the left patella. He improved symptomatically but developed low back pain on the last day of the external radiotherapy. Lumbar spine radiographs revealed collapse of the third lumbar vertebra; this was also treated by external radiotherapy (800 cGy/single fraction) and the patient was discharged. Discussion
Although the knee is a common site for bone tumours, the patella is not usually involved (Ehara et al,
Figure 1. Photograph showing patellar swelling and flexion deformity of the left knee. Note the involvement of overlying skin and biopsy scar. The British Journal of Radiology, August 1992
Case reports
Figure 3. CT scan at the level of the distal femur and patella showing complete destruction of the patella. Only a part of the edge of the patella is seen.
Figure 2. (a) Lateral radiograph of the left knee showing complete destruction of the patella, (b) Skyline view of the knee. There is extensive destruction and swelling of the left patella.
1989). Kransdorf et al (1989) reported 42 patellar neoplasms, but found no metastatic lesions. Both the above series stressed vigilance in cases of anterior knee pain. In those cases where patellar destruction is seen on radiographs, rapid increase in size of the lesion and poorly defined margins suggest a metastatic tumour (Kransdorf et al, 1989). Only a handful of reports of patellar Vol. 65, No. 776
metastasis exist in the literature. Keeley (1973) reported bilateral patellar metastasis from carcinoma of the male breast; subsequent radiographs revealed metastatic disease in other bones. Kwa and Nade (1989) described a case of patellar metastasis in renal adenocarcinoma, which also showed increased uptake in the occiput on radionuclide bone scan. Ashby and Dappen (1976) reported a patellar metastasis from oesophageal carcinoma together with a similar lesion in the scapula, and Benedek (1965) described patellar metastasis from bronchogenic carcinoma associated with a second lesion in the scalp. In our patient although a diagnosis of patellar metastasis was entertained once the lung biopsy showed a locally advanced lesion, a radiographic survey of the whole body was not performed. Only when low back pain appeared were further radiographs carried out, which revealed metastatic deposit in the third lumbar vertebra. Patellar metastasis is usually a feature of generalized carcinomatosis when there are sufficient tumour emboli in the circulation. Further investigations to locate other metastatic lesions are thus mandatory. The patella is a sesamoid bone with a relatively poor vascular supply, consisting of a few nutrient branches 723
Case reports
1992, The British Journal of Radiology, 65, 724-725
from the collateral vessels of the knee; this seldom provides sufficient access to tumour emboli for metastatic lesions to develop (Goodwin, 1961). The management of patellar metastasis depends on the size of the lesion. Early deposits without soft-tissue extension may well be treated by patellectomy, whereas larger lesions with soft-tissue extension require palliative external radiotherapy.
Osteolytic lesions of the patella. American Journal of Roentgenology, 153, 103-106. GALL, E. P.,
DIDIZIAN, N.
A. & PARK, Y.,
1974.
Acute
References
monoarticular arthritis following patellar metastasis. Journal of the American Medical Association, 229, 288-289. GOODWIN, M. A., 1961. Primary osteosarcoma of the patella. Journal of Bone and Joint Surgery, 43, 338-341. HUSSEY, H. H., 1976. The vertebral venous system. Editorial. Journal of the American Medical Association, 235, 2524. KEELEY, C. D., 1973. Bilateral patellar metastases from carcinoma of the male breast. Canadian Journal of Surgery, 16, 328-329.
ASHBY, M. E. & DAPPEN, N., 1976. Esophageal carcinoma
KRANSDORF, M. J., MOSER, R. P., JR, VINH, T. N., AOKI, J. &
metastatic to the patella: a case report. Journal of the American Medical Association, 235, 2519-2520. BENEDEK, G. T., 1965. Lysis of the patella due to metastatic carcinoma. Arthritis and Rheumatism, 8, 560-565.
CALLAGHAN, J. J., 1989. Primary tumours of the patella. A review of 42 cases. Skeletal Radiology, 18, 365-371. KWA, S. & NADE, S., 1989. Metastasis in a patella: a rare site. Australian and New Zealand Journal of Surgery, 59, 351-352.
EHARA, S., KHURANA, J. S., KATTAPURAM, S. V., ROSENBERG, A. E., EL-KHOURY, G. Y. & ROSENTHAL, D. I., 1989.
STOLER, B. & STAPLE, T. W., 1969. Metastasis to the patella.
Radiology, 93, 835-836.
Renal ultrasonographic appearances at presentation in an infant with Lesch-Nyhan syndrome By C. N. Ludman, MB, BS, MRCP, *C. Dicks-Mireaux, MRCP, FRCR and A. J. Saunders, FRCP, FRCR Department of Radiology, Guy's Hospital, St Thomas Street, London SW1 9RT and 'Department of Radiology, The Hospital for Sick Children, Great Ormond Street, London WC1 3JH, UK (Received 8 November 1991, accepted 16 January 1992) Keywords: Lesch-Nyhan syndrome, Ultrasound, Hyperechoic cortex, Crystal nephropathy
Lesch-Nyhan syndrome, first described in 1964, is an X-linked recessive disorder of purine metabolism. A cellular deficiency or absence of the purine salvage enzyme hypoguanine phosphoribosyl transferase causes purine overproduction and subsequent hyperuricaemia and gout. Clinically the syndrome manifests as neurological disorders, behavioural disturbances characterized by self mutilation and variable mental retardation. In addition, hyperuricaemia results in the clinical features of gout, with gouty arthritis, tophi, urate and uric acid nephropathies. The previously described renal ultrasonic appearances of Lesch-Nyhan syndrome have referred to patients after they have received allopurinol therapy. We describe a different appearance in a patient with this syndrome, prior to treatment. Allopurinol is a xanthine oxidase inhibitor and leads to an accumulation of the intermediate metabolites xanthine and hypoxanthine. Treated patients develop calculi which appear to be predominantly formed from xanthine secondary to this therapy. We report on the case of an infant who presented at 3 weeks of age with congenital renal failure, who shortly afterwards developed the features of acute tophaceous 724
gout. The renal ultrasonic appearances at presentation were striking and unusual. Case report A 3-week-old male infant in renal failure was transferred to Guy's Hospital. The pregnancy had been unremarkable and the appearance of the kidneys on routine obstetric scanning was reported as normal. Shortly after birth, he developed recurrent vomiting with weight loss and was admitted to his local hospital. He was found to be in renal failure, and was transferred. On admission to Guy's Hospital, the child's plasma urate was 2.2 mmol/1 (n 0.2-0.43) and creatinine was 804 /xmol/1. No detectable activity of hypoxanthine guanine phosphoribosyl transferase was found in intact or lysed erythrocytes, thus establishing the diagnosis of Lesch-Nyhan syndrome. On admission an abdominal radiograph was normal with no evidence of renal tract calcification. The renal ultrasonic appearances showed bilateral markedly echogenic cortices. The medullary pyramids were echo poor, but contained streaks of increased echogenicity (Fig. 1).
Discussion
The previously described ultrasonic findings in Lesch-Nyhan syndrome have been seen following long The British Journal of Radiology, August 1992
