Early experience of thalidomide therapy for high-grade peripheral and facial arteriovenous malformations.

BACKGROUND: Arteriovenous malformations (AVMs) are developmental defects in the vascular system with abnormal connections between arteries and veins. A minority of AVMs are characterized by aggressive growth and continue to proliferate despite maximal surgical and interventional therapy. We report our outcomes with the use of thalidomide as the only UK specialist center adopting this novel approach for the management of AVMs refractory to conventional therapy. METHODS: This was a retrospective case series which included only complex and proliferative AVM lesions (Schobinger grade III and IV). All patients prescribed thalidomide on a compassionate basis between September 2006 and August 2022 after attempts at embolosclerotherapy without satisfactory response were reviewed. RESULTS: Eleven patients were included in our study. The median total duration of thalidomide use was 10 months. Two thirds of patients with pain (six of nine) reported an improvement, three quarters reported a reduction in swelling (six of eight) and all who presented with bleeding reported improvement in overall volume or frequency (four of four). Over the study period, 45% achieved a non-proliferative state with no further target vessel demonstrable on angiography. Mild, tolerable side effects such as fatigue were common (73%). There was only one major adverse reaction (neutropenia) necessitating cessation of therapy. CONCLUSIONS: We can conclude that thalidomide is able to reduce the symptom burden for patients with complex and proliferative AVMs that were refractory to established treatment modalities. Adverse effects are common, but the benefit achieved from taking thalidomide in otherwise treatment resistant cases outweighs the risks, most of which are manageable.

Management of a complex cholecystocutaneous fistula with laparoscopic retrieval of gallstones embedded in the abdominal wall.

Cholecystocutaneous fistula is a rare surgical entity caused by an abnormal connection between the gallbladder epithelium and the skin. These complex cases have historically required an open surgical approach and are difficult to manage. We present a rare case of a 65-year-old male patient, with chronic lithiasic cholecystitis and cholecystocutaneous fistula. The patient underwent a laparoscopic subtotal cholecystectomy, dissection of the fistula tract, and removal of the impacted stones from the abdominal wall. With appropriate expertize, a completely laparoscopic approach is acceptable and the technical challenges can be predicted and overcome through careful pre-operative planning.

Three-port versus four-port technique for laparoscopic cholecystectomy: systematic review and meta-analysis.

BACKGROUND: The four-port laparoscopic technique is the standard approach for cholecystectomy. A three-port technique has been described, but there is no consensus over the outcomes and efficacy of this approach. The aim was to perform a systematic review and meta-analysis to compare the three- and four-port techniques in laparoscopic cholecystectomy for benign diseases of the gallbladder. METHODS: The review was conducted according to a predefined protocol registered on PROSPERO. Two authors independently conducted an electronic database search of CENTRAL, MEDLINE, Embase, CINAHL, WHO International Clinical Trials Registry, and ClinicalTrials.gov. Outcomes are reported as risk ratios (RR), mean difference (m.d.), or standardized mean difference (s.m.d.) with 95 per cent confidence intervals. RESULTS: Eighteen trials were included with 2085 patients. Length of hospital stay and postoperative analgesia requirement favoured the three-port group (m.d. -0.29, 95 per cent c.i. -0.43 to -0.16 (P < 0.001); and s.m.d. -0.68, 95 per cent c.i. -1.03 to -0.33 (P < 0.001), respectively). There were no differences in length of procedure or success rate between the two groups (m.d. 0.90, 95 per cent c.i. -3.78 to 5.58 (P = 0.71) and RR 0.99, 95 per cent c.i. 0.97 to 1.01 (P = 0.17), respectively). There were no differences in adverse events. The overall quality of evidence was low. CONCLUSION: The three-port technique for laparoscopic cholecystectomy is an option for appropriately trained surgeons who perform it regularly. However, the decision to use three ports should not be at the expense of safe dissection of Calot's triangle.

Mixed histology bladder cancer as a complication of clam ileocystoplasty.

A 58-year-old woman with a previous clam ileocystoplasty was referred to the urology department for the investigation of haematuria. CT urogram showed a large left-sided soft tissue mass arising from the bladder. Histological analysis of the shavings from transurethral resection revealed a G3pT2 transitional cell carcinoma and T4N1Mx adenocarcinoma. The patient was referred to oncology for the discussion of palliative chemotherapy; however, in the interim she deteriorated and was admitted to hospital with a post-renal acute kidney injury. A right-sided nephrostomy was inserted relieving her obstruction and she subsequently made a good recovery. This case report illustrates the difficulties in the long-term follow-up of patients having undergone what is now a rarely performed procedure. In the absence of regular cystoscopic follow-up post ileocystoplasty, malignancy may present late and with complications from advanced disease.