Obesity surgery and Ehlers-Danlos syndrome: challenges and considerations based on a case report.

Ehlers-Danlos syndrome is a hereditary connective tissue disorder that has gastrointestinal manifestations in over 50% of its cases. We present the first case of bariatric surgery in a patient with Ehlers-Danlos syndrome and outline management challenges in the context of the relevant literature. A 56-year-old man with type IV Ehlers-Danlos syndrome and a body mass index of 41.8 kg/m2 was referred to the bariatric centre of the Churchill Hospital, Oxford, for consideration of surgery for morbid obesity. His comorbidity included type 2 diabetes, hypertension, dyslipidaemia and obstructive sleep apnoea. He underwent a laparoscopic Roux-en-Y gastric bypass. His initial recovery was uneventful and he was discharged on the first postoperative day. Six weeks later, he presented with 43.9% excess weight loss and improved glycaemic control. Three months postoperatively, however, he complained of dysphagia, regurgitation and postprandial pain. A barium meal and gastroscopy suggested the presence of a gastric diverticulum. A surgical exploration was planned. Intraoperative gastroscopy demonstrated an asymmetrical gastric pouch dilatation and the pouch was therefore refashioned laparoscopically. Despite the initial symptomatic relief, two months later he experienced retrosternal pain with progressive dysphagia. Since then, multiple endoscopic dilatations of the gastro-oesophageal junction have been performed for recurrence of symptoms. Finally, a laparoscopic hiatus hernia repair and adhesiolysis was performed resulting in complete relief of patient's symptoms. Bariatric management of patients with Ehlers-Danlos syndrome can prove challenging. The bariatric team must implement a careful management plan including a detailed consent process, a tailored surgical intervention and a follow-up focused on potential gastrointestinal manifestations.

Outcomes of renal transplantation in patients with major lower limb amputation.

INTRODUCTION: The impact of severe peripheral vascular disease on graft survival in patients undergoing renal transplantation is poorly defined. The aim of our study is to establish outcomes in renal transplant recipients who have severe peripheral vascular disease necessitating major lower limb amputation. METHODS: Data for patients undergoing renal transplantation from January 2001-December 2010 was extracted from a regional transplantation database. Patients undergoing lower limb amputation pre- and post-transplantation were identified and outcome measures including delayed graft function, biopsy-proven acute rejection, serum creatinine level at 1 year, and graft loss and recipient survival at 1 year and long-term were compared with patients who did not undergo amputation. Student t and Pearson's chi-squared tests were used to compare patients with and without amputation and Kaplan-Meier curves were used for survival analysis. A P value < .05 is considered statistically significant. RESULTS: A total of 762 patients underwent renal transplantation. Four (0.5%) patients had an amputation before transplantation and 16 (2.1%) underwent amputation after transplantation. Serum creatinine levels at 1 year were significantly higher in patients who had amputation after transplantation (308.5 ± 60.8 µmol/l vs 177.6 ± 6.4 µmol/l; P = .03). During longer follow-up (mean: 2053.1 ± 58.3 days), patients who underwent amputation after transplantation had a higher rate of graft loss (P < .01) and higher death rate (P < .01). CONCLUSION: The requirement for amputation after renal transplantation is associated with poor long-term graft and patient survival and higher serum creatinine levels at 1 year. Patients at increased risk of severe peripheral vascular disease should be identified and measures taken to reduce the long-term risk.

A unique case of penile necrotizing fasciitis secondary to spontaneous corpus cavernosal abscess.

Corpus cavernosal abscess and necrotizing fasciitis occur rarely, and precipitating factors can usually be elicited with careful history and examination. Whilst both conditions share common risk factors such as diabetes mellitus, this is the first reported case of penile necrotizing fasciitis secondary to spontaneous corpus cavernosal abscess in an otherwise healthy patient. A 32-year-old man presented with 4-day history of swollen, painful penis, with ultrasound confirming corpus cavernosal abscess. Biopsies were taken and the cavity aspirated, but, despite intravenous antibiotics, he developed penile necrotizing fasciitis necessitating open cavernostomy and debridement. The overlying skin defect healed by secondary intention, but the patient experienced persistent postoperative erectile dysfunction, so he was referred for penile prosthesis insertion.

Proximal humeral fractures with a severe varus deformity treated by fixation with a locking plate.

We treated 47 patients with a mean age of 57 years (22 to 88) who had a proximal humeral fracture in which there was a severe varus deformity, using a standard operative protocol of anatomical reduction, fixation with a locking plate and supplementation by structural allografts in unstable fractures. The functional and radiological outcomes were reviewed. At two years after operation the median Constant score was 86 points and the median Disabilities of the Arm, Shoulder and Hand score 17 points. Seven of the patients underwent further surgery, two for failure of fixation, three for dysfunction of the rotator cuff, and two for shoulder stiffness. The two cases of failure of fixation were attributable to violation of the operative protocol. In the 46 patients who retained their humeral head, all the fractures healed within the first year, with no sign of collapse or narrowing of the joint space. Longer follow-up will be required to confirm whether these initially satisfactory results are maintained.