One thousand single anastomosis (omega loop) gastric bypasses to treat morbid obesity in a 7-year period: outcomes show few complications and good efficacy.

BACKGROUND: A short-term randomized controlled trial shows that the one anastomosis gastric bypass (OAGB) is a safe and effective alternative to the Roux-en-Y gastric bypass (RYGB). OBJECTIVE: The aim of this study is to evaluate the OAGB at our University Hospital between 2006 and 2013. PATIENTS: One thousand patients have undergone an OAGB. Data were collected on all consecutive patients. The mean follow-up period was 31 months (SD, 26.3; range, 12-82.9), and complete follow-up was available in 126 of 175 patients (72 %) at 5 years after surgery. RESULTS: Mortality rate was 0.2 %. Overall morbidity was 5.5 %; 34 required reoperations: i.e., 6 leaks, 5 obstructions, 5 incisional hernias, 7 biliary refluxes, 2 perforated ulcers, 2 bleeds, 2 abscesses, and 1 anastomotic stricture. Four patients were reoperated for weight regain. Overall rate of marginal ulcers was 2 % (n = 20), all in heavy smokers. Conversion from an OAGB to a RYGB was required in nine cases (0.9 %): seven for intractable biliary reflux, two for a marginal ulcer. At 5 years, percent excess body mass index loss was 71.6 ± 27 %. One hundred patients with type-2 diabetes, with a mean preoperative HbA1C of 7.7 ± 1.9 %, were followed for >2 years; the total resolution rate was 85.7 %. CONCLUSION: This study confirms that the OAGB is an effective procedure for morbid obesity with comparable outcomes to RYGB; in addition, it seems to be safer with lower morbidity. Its technical simplicity represents a real advantage and makes it an option that should be considered by all bariatric surgeons.

SDHD immunohistochemistry: a new tool to validate SDHx mutations in pheochromocytoma/paraganglioma.

CONTEXT: Pheochromocytomas (PCC) and paragangliomas (PGL) may be caused by a germline mutation in 12 different predisposing genes. We previously reported that immunohistochemistry is a useful approach to detect patients harboring SDHx mutations. SDHA immunostaining is negative in SDHA-mutated tumors only, while SDHB immunostaining is negative in samples mutated on all SDHx genes. In some cases of SDHD or SDHC-mutated tumors, a weak diffuse SDHB labeling has however been described. OBJECTIVE: Here, we addressed whether the same procedure could be applicable to detect patients with germline SDHD mutations, by testing two new commercially available anti-SDHD antibodies. DESIGN AND METHODS: We performed a retrospective study on 170 PGL/PCC in which we investigated SDHD and SDHB expression by immunohistochemistry. RESULTS: SDHx-mutated PGL/PCC showed a completely negative SDHB staining (23/27) or a weak cytoplasmic background (4/27). Unexpectedly, we observed that SDHD immunohistochemistry was positive in SDHx-deficient tumors and negative in the other samples. Twenty-six of 27 SDHx tumors (including the four weakly stained for SDHB) were positive for SDHD. Among non-SDHx tumors, 138/143 were positive for SDHB and negative for SDHD. Five cases showed a negative immunostaining for SDHB, but were negative for SDHD. CONCLUSION: Our results demonstrate that a positive SDHD immunostaining predicts the presence of an SDHx gene mutation. Because SDHB negative immunostaining is sometimes difficult to interpret in the case of background, the addition of SDHD positive immunohistochemistry will be a very useful tool to predict or validate SDHx gene variants in PGL/PCC.

[Vascular Ehlers-Danlos syndrome]. / Syndrome d'Ehlers-Danlos vasculaire.

The vascular type of Ehlers-Danlos syndrome (EDS) is a rare genetic disease transmitted as an autosomal dominant trait. It is distinguished from other forms of EDS by its unstable acrogeric morphotype and by vascular, gastrointestinal, and obstetrical complications. Diagnosis is based on various clinical signs, noninvasive imaging, and on the identification of a mutation of the COL3A1 gene, which provides diagnostic certainty but has a sensitivity of only 61%. When two major diagnostic criteria are present, a genetic test should be proposed, performed and its result presented in a multidisciplinary group. The precautionary principle requires that preventive measures be implemented when the diagnosis is suspected. All artery puncture, surgery, and gastrointestinal and uterine endoscopy are contraindicated, permissible only in life-threatening emergencies. Straining against a closed glottis and all other situations or drugs likely to raise blood pressure must be avoided. Contraception must be discussed to avoid pregnancy during the diagnostic period. Arterial lesions suggestive of the disease include dissecting aneurysms of the internal carotid and iliac arteries and of the anterior visceral branches of the abdominal aorta, fusiform aneurysms of the splenic artery, and early onset nontraumatic direct carotid-cavernous fistulae. Early-onset varicose veins, spontaneous peritonitis or unusually important perineal lesions after giving birth should also attract the physician's attention. Psychological treatment and support of patients and their families is essential, to help them both to live with their disease and to deal with the information and screening issues. The prognosis of Ehlers-Danlos syndrome, vascular type, is grim but there is wide interindividual variability and life expectancy is best among patients receiving regular follow-up. Management by an experienced multidisciplinary team, implementation of drastic prevention measures and, depending on the results of the BBEST study, the possible prescription of beta-blockers should help to reduce the risk of complications and justify hope for a real improvement in prognosis in the near future.