A new case of multiple sulphatase deficiency with onset at birth is described. The patient had many dysmorphic features and hydrocephalus, similar to one other case with early onset described in the literature. The new patient differed from the other case in having chondrocalci‐ficans congenita, heart abnormalities and an abnormal fold of tissue present between the laryngeal inlet and the oesophagus. Excessive mucopolysacchariduria was present and there was profound deficiency of all sulphatases examined in plasma, leucocytes and cultured skin fibroblasts.

BackgroundThere is currently conflicting evidence surrounding the effects of obesity on postoperative outcomes. Previous studies have found obesity to be associated with adverse events, but others have found no association. The aim of this study was to determine whether increasing body mass index (BMI) is an independent risk factor for development of major postoperative complications.MethodsThis was a multicentre prospective cohort study across the UK and Republic of Ireland. Consecutive patients undergoing elective or emergency gastrointestinal surgery over a 4‐month interval (October–December 2014) were eligible for inclusion. The primary outcome was the 30‐day major complication rate (Clavien–Dindo grade III–V). BMI was grouped according to the World Health Organization classification. Multilevel logistic regression models were used to adjust for patient, operative and hospital‐level effects, creating odds ratios (ORs) and 95 per cent confidence intervals (c.i.).ResultsOf 7965 patients, 2545 (32·0 per cent) were of normal weight, 2673 (33·6 per cent) were overweight and 2747 (34·5 per cent) were obese. Overall, 4925 (61·8 per cent) underwent elective and 3038 (38·1 per cent) emergency operations. The 30‐day major complication rate was 11·4 per cent (908 of 7965). In adjusted models, a significant interaction was found between BMI and diagnosis, with an association seen between BMI and major complications for patients with malignancy (overweight: OR 1·59, 95 per cent c.i. 1·12 to 2·29, P = 0·008; obese: OR 1·91, 1·31 to 2·83, P = 0·002; compared with normal weight) but not benign disease (overweight: OR 0·89, 0·71 to 1·12, P = 0·329; obese: OR 0·84, 0·66 to 1·06, P = 0·147).ConclusionOverweight and obese patients undergoing surgery for gastrointestinal malignancy are at increased risk of major postoperative complications compared with those of normal weight.

BackgroundLateral neck radiographs are commonly used in the investigation and management of patients presenting with suspected fish bone impaction. The effectiveness of these is questioned, as many fish do not have radio-opaque bones.ObjectiveThis study evaluated the utility of lateral neck radiographs in the management of patients presenting with fish bones retained in the upper aerodigestive tract, with the creation of a treatment algorithm to guide further management.MethodsAn audit of practice was undertaken at the University Hospital of Southampton, identifying all patients admitted with potential fish bone impaction in the upper aerodigestive tract. Following analysis, a treatment algorithm was constructed for use by junior doctors.ResultsIn total, 34 per cent of patients with a normal radiograph were subsequently found to have a fish bone present under local or general anaesthetic assessment. The sensitivity of radiographs in the detection of fish bones was found to be 51.6 per cent.ConclusionLateral neck radiographs have limited value in the management of suspected fish bone impaction, and should only be used following detailed clinical examination of the upper aerodigestive tract.

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