SUMMARYChildren with medulloblastoma in Bristol are treated surgically and with craniospinal irradiation, and in some cases chemotherapy. Thirteen medium or long term survivors were investigated to determine their growth and endocrine function. Their rate of growth was considerably reduced through the first year of their illness and after spinal irradiation spinal growth was poor. Nine children developed growth hormone deficiency. They were clinically euthyroid but 7 had raised basal thyroid stimulating hormone values. Gonadal function was abnormal in all but the youngest child. The rate of survival is increasing in children with medulloblastoma but this is associated with appreciable endocrine abnormalities. Some of these problems are present shortly after treatment ends but others may develop later and long term surveillance is therefore essential.Cerebral tumours are the second most common malignancy of childhood and a quarter of these are medulloblastomas

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Objectives-To assess the feasibility of setting up a register of patients with asplenia within a defined geographical area; to ensure that guidelines on best practice were implemented; to obtain information on antibody levels to pneumococcal capsular polysaccharides and Haemophilus influenzae type b capsular polysaccharide, before and after immunisation and annually thereafter; to raise awareness of risks among clinicians and to oVer advice on management. Design-Prospective recruitment using multiple sources of recruitment. Annual follow up reminders sent from Registration Centre. Subjects-Population of (old, pre-1995) Northern Health Region: approximately 3.1 million. Main outcome measures-Data were obtained on reasons for asplenia, duration of asplenia, use of prophylactic antibiotics, Medic-Alert bracelets, immunisations, antibody levels, death. Results-The register was initiated at the beginning of April 1995 and ran to the end of March 1997. After two years of operation, 1111 cases had been registered but the response from some health districts was poor. Major primary causes of asplenia were trauma (264), other surgical (198), lymphoproliferative disease (154), and idiopathic thrombocytopenic purpura (147). There were 664 patients on prophylactic antibiotics, of whom 498 were on continuous antibiotics. Only 18 had any type of warning bracelet. Antibody measurements were carried out at least once on 75% of patients; 306 patients had satisfactory antibody levels on first blood sample in year 1, rising to 405 in year 2; 43 patients failed to make any antibody response to Pneumovax despite multiple immunisations, and three patients failed to respond to Hib vaccine. Sixteen patients with satisfactory antibody levels in year 1 had low levels in year 2 requiring vaccine boosters. Sixteen deaths were reported, two of which were directly attributable to overwhelming sepsis. Conclusions-Registration has been successful and has raised awareness of the management of asplenia. Compliance with antibiotic prophylaxis and immunisation was initially poor. A potential high risk group of vaccine non-responders has been identified and poor persistence of pneumococcal antibodies has been identified which is likely to alter approaches to immunisation in asplenic patients. (J Clin Pathol 1999;52:424-429)

A 4-year-old girl presented with a nasopharyngeal rhabdomyosarcoma and had the characteristic features of the naevoid basal cell carcinoma syndrome. She was treated with a modified standard combination of chemotherapy and radiotherapy. However, radiotherapy had to be interrupted because of severe radiation recall.