EDITOR: Gaucher's disease, the most common lysosomal storage disorder, is an enzymatic defect with consequent accumulation of undegraded glucocerebroside in cells of the monocyte–macrophage system [1]. Most patients present with enlargement of the spleen and liver resulting in hypersplenism, with thrombocytopaenia generally more significant than anaemia. Skeletal involvement, particularly osteonecrosis of large joints and pathological fractures, invariably is the most significant cause of morbidity and decreased quality of life. In spite of enzyme replacement therapy, osteopaenia and osteonecrosis continue to be clinically important. The need for total hip replacement (THR), and subsequent revision, is relatively common in these patients at a relatively young age. With regard to the anaesthetic management for orthopaedic surgery in patients with Gaucher’s disease, only one case report appears in the literature of an adult with a sub-capital hip fracture who underwent subarachnoid anaesthesia [2]. We wish to report the anaesthetic management of all cases seen in our institution for THR or revision from 1990 to 2005. Patient characteristics and perioperative data are summarized from the clinical records and presented in Table 1.Table 1: Patients with Gaucher’s disease: patient characteristics and perioperative data expressed as mean ± SD (range) or number (%) of patients.There were 14 patients, 10 males and 4 females, who underwent THR. Five of these underwent a revision. Six patients (43%) were homozygous for the mild N370S (1226) mutation; eight patients (57%) had been splenectomized; and six patients (43%) had pulmonary hypertension at the time of surgery. Fifty-three percentage of patients underwent general anaesthesia. Five of the operations (21%) were performed with preoperative platelet counts <80 × 103 mm−3. Perioperative blood product transfusion was required in 68% of the operations. All patients with general anaesthesia were orally intubated utilizing direct laryngoscopy and ventilated with tidal volume 6–8 mL kg−1 and respiratory rate of 10–16 min−1. There were no difficulties in airway management. All patients were given preoperative antibiotic prophylaxis mainly with cefamezine; nonetheless, 37% experienced postoperative wound infections presenting significantly higher rates compared to similar patients in our institution (2%) and the rate reported in medical literature (0.3–2%) [3]. A wound infection was defined as one of the following: wound redness, excessive pain, tissue necrosis, local oedema, purulent discharge from the operation wound and maximal temperature >38.5 and/or white cell count >10 000. Other complications included one inadvertent spinal, one failed spinal and one patient with severe postoperative thrombocytopaenia (21 × 103 mm−3). Joint replacement for patients with Gaucher's disease is an important therapeutic intervention that dramatically reduces pain, improves functionality and increases quality of life. For patients with Gaucher's disease, the preoperative evaluation of haematological status is of primary concern. Many have very low platelet counts with or without abnormal platelet function [4]. Thrombocytopaenia may be sufficiently severe as to preclude regional anaesthesia [5]. In minor procedures (e.g. dental extractions) we have successfully used platelet transfusions immediately prior to and directly after surgery. Coagulation factor deficiencies are also common in this population and may occur independently of platelet abnormalities. A relatively large percentage (44%) in this cohort had pulmonary hypertension at surgery. This complication reflects disease severity but we have also posited a possible connection with enzyme replacement therapy [6], so that a history of enzyme treatment does not preclude abnormal lung function. In these most severe cases, regional anaesthesia may be preferred because of pulmonary hypertension. In conclusion, patients with Gaucher’s disease undergoing THR may have associated medical conditions such as pulmonary hypertension and coagulopathy that complicate their anaesthetic management and postoperative course. Further, they are at high risk for development of postoperative infection. We strongly recommend a multi-disciplinary approach that includes internal medicine, anaesthesiology and orthopaedic surgery when planning of major orthopaedic procedures in patients with Gaucher’s disease.