748 CLINICAL REPORTS Postgrad Med J: first published as 10.1136/pgmj.69.815.748 on 1 September 1993. Downloaded from Ganglioneuroblastoma may infiltrate locally as Literature regarding the role ofchemotherapy in well as metastasize by way of haematogenous and ganglioneuroblastoma is limited and results are lymphatic channels, as does neuroblastoma. Gan- conflicting. The excellent response obtained in the glioneuroblastomas are slow growing tumours and present case warrants a closer look at chemo- long-term postoperative survivals have been therapy as the first line of treatment. We used a reported in many clinical series.2'3'8 The prognosis is fairly aggressive and broad-spectrum protocol and generally favourable with 2 year survival of 92% it is not clear which drug was the most active. The and 5 year survival of 88% in a large series.' change in drugs after the first three courses of a Spontaneous regression of neuroblastoma is well different protocol were preplanned to reduce the documented and there is evidence in the literature toxicity and to maximize the response. In view of that many of the so-called cures in neuroblastoma the fact that the tumour is so rare we may not have are in fact spontaneous regression. In our patient an answer to these questions for a long time to no mature ganglioneuroma was found on excision come. Our experience is particularly relevant to a and response occurred only after chemotherapy, situation where the tumour is unresectable or too close a coincidence for spontaneous regression. surgery is hazardous. References 1. Adam, A. & Hochholzer, L. Ganglioneuroblastoma of the 5. Kissane, J. & Smith, M. Pathology of infancy and childhood. posterior mediastinum. A clinicopathological review of 80 C.V. Mosby, St Louis, 1967, pp. 706-713. cases. Cancer 1981, 47: 373-381. 6. Ng, T.H.K., Fung, C.F., Goh, W. & Wong, V.C.N. Gang- 2. Johannson, J.H., Rekate, H.L. & Roessmann, U. Gangliomas, lioneuroma of spinal cord. Surg Neurol 1991, 35: 147-151. pathological and clinical correlations. J. Neurosurg 1981, 54: 7. Russell, D.S. & Rubinstein, L.J. Peripheral tumours of the 58-63. neurone series. In: Pathology of Tumours of the Nervous 3. Kalyan-Raman, U.P. & Olivero, W.C. Ganglioglioma: a System. Williams and Wilkins, Baltimore, 1971, pp.305-333. correlative clinicopathological and radiological study of ten 8. Sutton, L.N., Packer, R.J. Rorke, L.B., Bruce, D.A. & Schut,Protected by copyright. surgically treated cases with follow-up. Neurosurgery 1987, 20: I. Cerebral gangliogliomas during childhood. Neurosurgery 428-433. 1983, 13: 124-128. 4. Kilton, L.J., Aschenbrener, C. & Bums, C.P. Ganglioneuro- blastoma in adults. Cancer 1976, 37: 974-983. Postgrad Med J (1993) 69, 748 - 750 ©) The Fellowship of Postgraduate Medicine, 1993 Is clubbing a feature ofthe anti-phospholipid antibody syndrome? A.W. Harris, T.A.C. Harding, M.D. Gaitonde and J.D. Maxwell http://pmj.bmj.com/ Department ofMedicine, St George's Hospital, London SWJ7 OQT, UK Summary: A patient with the anti-phospholipid antibody syndrome and digital clubbing is described. No recognized cause for the clubbing was found. Itis suggested that platelet aggregation and microthrombi formation as a result of anti-phospholipid antibody may be involved in the pathogenesis of the digital on September 30, 2021 by guest. clubbing. This may be a new feature of the anti-phospholipid antibody. Introduction Digital clubbing is a recognized feature of many other recognized cause for his clubbing, and sug- respiratory, cardiac and gastrointestinal diseases. gest that this may be a feature of this condition. We describe a case of clubbing in a man with the anti-phospholipid antibody syndrome, with no Correspondence: A. Harris, B.Sc., M.R.C.P., Ogle Office, Case report Knightsbridge Wing, St George's Hospital, Tooting, London SW17 OQT, UK. A 48 year old retired safe-maker was referred for Accepted: 20 January 1993 investigation of abnormal liver function tests. In CLINICAL REPORTS 749 Postgrad Med J: first published as 10.1136/pgmj.69.815.748 on 1 September 1993. Downloaded from 1984, aged 40, he had a left middle cerebral artery findings, and demonstrated no valvular vegeta- embolic stroke, confirmed by computed tomo- tions. Rigid sigmoidoscopy and biopsy were nor- graphic scan. The following year he was diagnosed mal. A liver biopsy showed features of chronic as having temporal lobe epilepsy and was started venous outflow block, and enzyme induction. on carbamazepine. Four years later, he suffered Immunology revealed negative ANA, ds-DNA and from two transient ischaemic attacks. No further ANCA but strongly positive anti-cardiolipin IgG investigation was undertaken at that time. In 1989 antibody titres (79 units; normal <10). he was seen by a dermatologist for recurrent bilateral, lower leg ulceration. There was no history of hypertension, diabetes mellitus, hyperlipid- Discussion aemia, ischaemic heart disease or deep vein throm- bosis. Recurrent venous and arterial thromboses, throm- General examination revealed finger and toe bocytopenia, heart valve abnormalities, epilepsy clubbing first noticed about 6 months previously and skin ulceration are all well recognized features (Figure 1). There were signs ofmitral and tricuspid ofthe anti-cardiolipin syndrome.'4 The patient we incompetence, a mild expressive dysphasia, spastic have described did not suffer from systemic lupus left hemiparesis and bilateral lower leg venous erythematosus or take any medication associated ulceration. with anti-phospholipid antibodies.5 Therefore a Investigations revealed normal haematology diagnosis of the primary anti-phospholipid anti- other than thrombocytopenia (84 x 109/l) and pro- body syndrome was made. longed activated partial thromboplastic time Binding of the anti-cardiolipin antibody to (APTT) to 97 seconds (normal: 34- 48), with the platelet phospholipid may damage platelets and presence of a lupus anticoagulant. The alkaline increase their adhesiveness leading to aggregation phosphatase and gamma-glutamyl transferase and microthrombi formation.68 Thrombocyto- were elevated, 264 IU/l (normal 30-120) and penia is a common finding in patients with anti- Protected by copyright. 540 IU/l (normal <30) respectively. The remaining cardiolipin antibodies and thrombosis.9 Dickinson liver function tests were consistently normal. and Martin'0 proposed that the release of platelet- Hepatitis B, C and VDRL were negative. Arterial derived growth factor (PDGF) from impacted blood gases and electrocardiogram were normal. platelet clumps in the fingertips leads to increased Chest radiograph showed an enlarged heart with capillary permeability and connective tissue hyper- upper lobe blood diversion. Radiographs of the trophy which may result in clubbing. Support for wrists were normal. Abdominal ultrasound con- this hypothesis was given by Fox et al." They firmed pulsatile hepatomegaly and dilated hepatic studied autopsy samples from patients with un- veins, but no thrombosis. Subsequent venography equivocal digital clubbing and randomly selected of the deep leg veins and inferior vena cava was controls. Numerous platelet microthrombi were normal. Echocardiography confirmed the clinical demonstrated in all the clubbed specimens, and in none of the controls. They concluded that finger clubbing is associated with platelet aggregation in the capillary network of the nail with bed, possible http://pmj.bmj.com/ release of PDGF. Our patient was not congenitally clubbed. There was no evidence of any chronic respiratory condi- tion or bronchial Nz ..... carcinoma, Ti. ...... inflammatory bowel disease or malabsorption, cirrhosis, infective endo- carditis or congenital cardiac anomalies. In the absence of these conditions, we suggest that the finger and toe nail clubbing may be a new feature of on September 30, 2021 by guest. the anti-phospholipid antibody syndrome. Acknowledgements Figure 1 Digital clubbing in a patient with the anti- The authors thank Professor C. Seymour for her help phospholipid antibody syndrome. with the case. References 1. Hughes, G.R.V. Thrombosis, abortion, cerebral disease and 3. Alegre, V.A., Gastineau, D.A. & Winklemann, R.K. Skin the lupus anticoagulant. Br Med J 1983, 287: 1088-1089. lesions associated with circulating lupus anticoagulant. Br J 2. Hughes, G.R.V., Harris, E.N. & Gharavi, A.E. The anticar- Dermatol 1989, 120: 419-429. diolipin syndrome. J Rheumatol 1986, 13: 486-489. 750 CLINICAL REPORTS Postgrad Med J: first published as 10.1136/pgmj.69.815.748 on 1 September 1993. Downloaded from 4. Galve, E., Ordi, J., Barquinero, J., Evangelista, A., Vilardell, 9. Harris, E.N., Asherson, R.A., Gharavi, A.E., Morgan, S.H., M. & Soler-Soler, J. Valvular heart disease in the primary Derve, G. & Hughes, G.R.V. Thrombocytopenia in SLE and antiphospholipid syndrome. Ann Intern Med 1992, 116: related autoimmune disorders: association with anticar- 293-298. diolipin antibodies. Br J Haematol 1985, 59: 227-230. 5. Canoso, R.T. & Sise, H.S. Chlorpromazine induced lupus 10. Dickinson, C.J. & Martin, J.F. Megakaryocytes and platelet anticoagulant and associated immunological abnormalities. clumps as the cause of finger clubbing. Lancet 1987, 573: Am J Hematol 1982, 13: 121-129. 1434-1435. 6. Anon. Lupus anticoagulant. Lancet 1984, 389: 1157-1158. 11. Fox, S.B., Day, C.A. & Gatter, K.C. Association between 7. Elias, M. & Eldor, A. Thromboembolism in patients with the platelet microthrombi and finger clubbing. Lancet 1991, 336: 'lupus'-type circulating anticoagulant. Arch Intern Med 1984, 313-314. 144: 510-515. 8. Weiner, H.N., Vardinon, N. & Yust, I. Platelet antibody binding and spontaneous aggregation in 21 lupus anti- coagulant patients. Vox Sang 1991, 61: 111-121. Postgrad Med J (1993) 69, 750 - 751 © The Fellowship of Postgraduate Medicine, 1993 The development ofspontaneous colo-umbilical fistula John B. Pracyk, Stephen G. Pollard and Sir Roy Y. Calne Department ofSurgery, University ofCambridge, Level 9, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK Protected by copyright. Summary: A patient with colo-umbilical fistula is reported. This presentation is unique because it documents the development of a fistula from a colonic diverticulum.