OBJECTIVES. To investigate the pattern of malignancies in Chinese patients with neurofibromatosis type 1.

DESIGN. Historical cohort study.

SETTING. Queen Mary Hospital and Duchess of Kent Children’s Hospital in Hong Kong.

PATIENTS. Patients with neurofibromatosis type 1 seen between January 1995 and August 2011.

RESULTS. We identified 123 Chinese patients with neurofibromatosis type 1, diagnosed at a median age of 4.9 years (range, 0.1-16.1 years); 75 (61%) were males. They were followed up for a median of 9.7 years (range, 0.2-27.6 years). Most (80%) of the patients participated in our surveillance programme. Twelve patients developed malignancies at the ages of 0.8 to 41.6 years. These malignancies included: peripheral nerve sheath tumours (n=3), juvenile myelomonocytic leukaemia (n=2), optic nerve glioma (n=1), thalamic pilocytic astrocytoma (n=1), rhabdomyosarcoma (n=1), osteosarcoma (n=1), neuroblastoma (n=1), anaplastic large cell lymphoma (n=1), and breast carcinoma and subsequently carcinoma of the ampulla of Vater (n=1). Among them, three had their tumours (optic glioma, thalamic astrocytoma, sacral malignant peripheral nerve sheath tumour) initially detected by surveillance imaging. Four patients survived without disease progression, three are alive with active disease, the remaining five died (when aged 3 to 56 years) with progressive or relapsed malignancies. The latter patients died from a neuroblastoma, a juvenile myelomonocytic leukaemia, a malignant peripheral nerve sheath tumour, a lymphoma, and a second primary tumour (carcinoma of ampulla of Vater, at the age of 56 years). In neurofibromatosis type 1 patients with malignancy, overall 30-year survival was significantly shorter than in those without malignancy (35% vs 93%, P<0.001).

CONCLUSION. Chinese patients with neurofibromatosis type 1 are susceptible to different malignancies which contribute to mortality. These findings are similar to reports from overseas. Outcomes were unfavourable, except in patients having low-grade gliomas. Surveillance imaging may help early detection of deep-seated malignancies but the benefits accruing from such monitoring warrants prospective evaluation.

Key words:Child; Hong Kong; Neoplasms; Neurofibromatosis 1

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OBJECTIVES. To study patients’ perceptions and knowledge about total joint replacement surgery.

DESIGN. Cross-sectional survey.

SETTING. University teaching hospital, Hong Kong.

PATIENTS. Three hundred consecutive patients with the diagnosis of osteoarthritis or inflammatory arthritis attending the out-patient clinic from June 2010 to May 2011.

MAIN OUTCOME MEASURES. Patients’ knowledge and how they got the knowledge about total joint replacement surgery, and concerns about the outcome of such operations.

RESULTS. Whilst 94% of the patients knew about total joint replacement surgery, 77% obtained such knowledge from their friends and relatives. The three most common concerns related to this type of operation were whether they might: be wheelchair bound after surgery (64%), need to be taken care of by others for more than 3 months (61%), and have post-surgery complications (54%). Most of them recognised the advantages of the surgery, 82% knew about good pain relief after surgery, and 87% realised that total joint replacement surgery could improve their mobility. Patients did not have a realistic idea regarding the survival of the prosthesis; 41% thought the prosthesis might last for less than 10 years and 34% had no idea about its longevity.

CONCLUSION. Patients did recognise the advantages of total joint replacement surgery in treating arthritis. However, they had many concerns about its outcome that warrant clarification. Public education on these aspects is necessary to address concerns, and may be achieved in cooperation with the media.

Key words:Arthroplasty, replacement, knee; Knee joint; Knee prosthesis; Postoperative complications; Treatment outcome

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OBJECTIVES. To investigate the diagnostic performance and safety of endobronchial ultrasound-guided transbronchial needle aspiration in patients presenting with radiological features of lung cancer.

DESIGN. Prospective case series.

SETTING. University teaching hospital, Hong Kong.

PATIENTS. Consecutive patients with mediastinal or hilar abnormalities suspected of or confirmed as having lung cancer underwent endobronchial ultrasound-guided transbronchial needle aspiration and presented between August 2006 and December 2010.

MAIN OUTCOME MEASURES. Diagnostic performance (including sensitivity, specificity, negative predictive value and accuracy), procedural complications, and tissue adequacy for molecular profiling.

RESULTS. A total of 269 procedures were performed in 259 patients, with malignancy confirmed in 210 (81%) of them. In the whole cohort with confirmed or suspected lung cancer, the overall sensitivity, specificity, negative predictive value, and accuracy of endobronchial ultrasound-guided transbronchial needle aspiration were 87%, 100%, 74%, and 91%, respectively. Among 42 patients with tumour samples sent for mutation tests (epidermal growth factor receptor and/or anaplastic lymphoma kinase), 40 (95%) were found to be adequate. No complication or mortality ensued from these procedures.

CONCLUSION. Endobronchial ultrasound-guided transbronchial needle aspiration is highly effective in determining the diagnosis and lymph node staging in patients with lung cancer. In combination with its excellent safety profile, it should be considered a frontline diagnostic test for patients presenting with mediastinal abnormalities suspicious of lung cancer.

Key words:Biopsy, fine-needle; Bronchoscopy; Lung neoplasms; Neoplasm staging; Sensitivity and specificity

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OBJECTIVES. To report the results of a modified vaporisation incision technique using a GreenLight High Performance System in the treatment of benign prostatic disease in men receiving anticoagulants.

DESIGN. Case series.

SETTING. Regional hospital, Hong Kong.

PATIENTS. From January 2007 to April 2010, 48 patients with a bleeding tendency or on oral anticoagulants who underwent photoselective vaporisation prostatectomy with a GreenLight High Performance System in the North District Hospital were studied. Data collected prospectively were analysed to determine perioperative and postoperative outcomes, including uroflowmetry parameters, serum prostate-specific antigen level, prostate volume, and complications at 1, 3, 6, and 12 months post-surgery.

RESULTS. The patients' mean age was 76 (standard deviation, 7; range 62-94) years. The mean follow-up period was 13 (standard deviation, 9) months. Thirty-six (75%) patients had urinary retention prior to surgery. Bleeding tendencies were due to receipt of aspirin (n=36), two antiplatelet agents (n=6), warfarin (n=4) and clopidogrel (n=1), and to thrombocytopaenia (n=1). Preoperative transrectal ultrasonography showed a mean prostate size of 58 (standard deviation, 30; range, 18-154) mL. Of the patients, 81% were discharged without a catheter and their mean hospital stay was 3 days. Five patients were readmitted for secondary haemorrhage, two had a drop of more than 10 g/L in their haemoglobin level, but only one received a blood transfusion. Mean uroflowmetry parameters, namely, peak flow rate and residual volume, were 8.7 mL/s and 199 mL preoperatively and 14.7 mL/s and 50 mL 1 year after the operation.

CONCLUSION. With an ageing population in which patients with various co-morbidities receive anticoagulant/antiplatelet therapy, photoselective vaporisation prostatectomy using a GreenLight High Performance System is a safe treatment option.

Key words: Laser therapy; Prostate-specific antigen; Prostatectomy; Prostatic hyperplasia; Treatment outcome

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OBJECTIVES. To report the incidence of endophthalmitis after intravitreal injection of anti-vascular endothelial growth factor and the safety profile of multiple doses of bevacizumab from the same vial reused for multiple patients.

DESIGN. Case series.

SETTING. A private hospital in Hong Kong.

PATIENTS. A systematic retrospective review of consecutive intravitreal anti-vascular endothelial growth factor injections between 5 June 2006 and 17 December 2010 at a single institute was conducted. Patients were identified from prospectively designed audit forms, and each patient's medical record was reviewed for any documented complications. Bevacizumab 1.25 mg/0.05 mL to 2.50 mg/0.1 mL was aspirated from the designated vial, with a maximum of 10 consecutive injections being aspirated from the same vial. The opened vial was then discarded without overnight storage. Ranibizumab was aspirated from the commercially available 1 mg/0.1 mL single-use vial.

RESULTS. A total of 1655 intravitreal anti-vascular endothelial growth factor injections into 392 eyes of 383 patients were evaluated during the study period. There were 1184 bevacizumab injections and 471 ranibizumab injections. There was one case of suspected endophthalmitis after ranibizumab injection, though culture of the vitreous tap was negative. The point prevalence of endophthalmitis was 0.06% (1/1655) for the total number of injections: 0.21% (1/471) after ranibizumab, and 0% after bevacizumab.

CONCLUSION. Although many centres aliquot multiple syringes from a single vial to be kept in a refrigerator for use, the current study shows that so long as proper sterile techniques are implemented, there were no cases of endophthalmitis from using the same vial, which was reused for a maximum of 10 consecutive injections. For intravitreal injection, bevacizumab costs approximately US$50 to US$100 per dose, as opposed to US$2000 per dose for ranibizumab. Sharing multiple doses of bevacizumab from a single vial can substantially reduce the cost of treatment.

Key words: Endophthalmitis; Intravitreal Injections; Ranibizumab; Receptors, Vascular endothelial growth factor; Bevacizumab

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