Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.

A 66-year-old, postmenopausal woman was referred to our hospital because of abnormal breast cancer screening results. A tumor was found in the upper outer part of the left breast. Biopsy revealed papillotubular carcinoma, ER (Allred score total score [TS] 3 = proportion score [PS] 2 + intensity score [IS] 1), PgR (Allred score TS 3 = PS 2 + IS 1), HER2 (2+), fluorescent in situ hybridization 1.1 (negative), and Ki-67 labeling index 15%. In diagnostic imaging, the tumor size was 35 mm. The diagnosis was T2N0M0 Stage IIA, luminal B-like breast cancer. First, letrozole 2.5 mg/day was administered as preoperative hormone therapy. After 2 months of treatment with letrozole, the tumor size had increased to 44 mm and preoperative hormone therapy was discontinued. She was started on preoperative chemotherapy (4 courses of epirubicin plus cyclophsphamide followed by 4 courses of triweekly docetaxel). The tumor size decreased, becoming undetectable. After these preoperative treatments, nipple-sparing mastectomy, sentinel lymph node biopsy, and breast reconstruction with a primary latissimus dorsi flap were performed. As of 3 years and 6 months after the operation, there has been no recurrence. At first, preoperative hormone therapy is performed for Luminal B-like breast cancer as in this case, if the response is insufficient, preoperative chemotherapy after hormone therapy may be a therapeutic option.

Here, we report 3 cases of conservatively treated ischemic colitis with hepatic portal venous gas. In Case 1, an 84-year-old man visited the emergency department because of fatigue, vomiting, and upper abdominal pain. In Case 2, a 72-year-old man was hospitalized for rehabilitation in another hospital following cervical spine trauma. He had diarrhea and mesogastric pain for 2 days and was transferred to our hospital. In Case 3, an 89-year-old woman visited the emergency department because of upper abdominal pain and vomiting. In all cases, we diagnosed the illness as ischemic colitis with hepatic portal venous gas based on computed tomography findings, and the vital signs of all patients were stable. They were treated conservatively. They were able to resume oral intake and were discharged or transferred to another hospital. Hepatic portal venous gas has been considered a poor prognostic sign of severe diseases such as intestinal necrosis. However, recently, reports of cases treated conservatively have been increasing. Conservative treatment may be selected for ischemic colitis associated with hepatic portal venous gas taking into account the patient’s general condition.

The patient was a 52-year-old woman who had undergone laparoscopic colectomy with lymph node dissection and inferior mesenteric artery preservation for cancer of the descending colon. Functional end-to-end anastomosis was performed using a linear stapler. Colonoscopy at the 1-year follow-up revealed a type 2 tumor at the anastomotic site. She was diagnosed with anastomotic recurrence. We performed laparoscopic high anterior resection with inferior mesenteric artery dissection. Rectal resection preceded mobilization of the mesocolon because of severe adhesion around the anastomotic site. Laparoscopic surgery for anastomotic site recurrence is an option under the condition that the initial surgery was laparoscopic.

The purpose of this study was to compare the results of open surgery and laparoscopic surgery for acute appendicitis in adults. In 2013, we primarily selected open surgery for acute appendicitis, but in 2017 we opted to select mainly laparoscopic surgery. We compared between 92 patients who had undergone open surgery in 2013 and 88 patients who underwent laparoscopic surgery group in 2017. There was no difference in age, sex, body mass index, white blood cell count, C-reactive protein, or perforation rate on computed tomography between the two groups. The laparoscopic surgery group had significantly longer operation time but significantly lower blood loss and postoperative complication rate. In particular, laparoscopic surgery was advantageous in terms of hemostasis for moderate bleeding. The results suggest that laparoscopic surgery may be more useful in adult appendicitis than open surgery.

We describe a rare complication and treatment progression that occurred in a 64-year-old man with an aortic abdominal aneurysm (AAA) that had been treated by endovascular aneurysm repair (EVAR). He had undergone EVAR to treat an infra-renal type AAA 21 months previously and returned to the emergency department with back pain. Contrast-enhanced computed tomography (CT) revealed acute type B aortic dissection, so he was admitted and conservative medical management was started. Acute stomachache and limb pain appeared on hospital day 7, which prevented him from moving his lower limbs. The main body of the stent graft had collapsed, blocking blood flow, and contrast was not found in arteries from the collapsed stent graft portion to the knee level on emergency contrast CT images of the leg. His legs were revascularized by an extra-anatomical right axial-bilateral external iliac bypass. His symptoms disappeared and reperfusion injury was avoided. The collapsed stent graft had retained its original shape at 11 and 18 days after surgery. Furthermore, follow-up CT 4.5 years later showed that the stent graft retained its original form.

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.

BACKGROUND/AIMS: The clinical impact of single-stage endoscopic stone extraction by endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy during the same hospitalization remains elusive. This study aimed to determine the efficacy and safety of single-stage ERCP and cholecystectomy during the same hospitalization in patients with cholangitis. METHODS: We retrospectively reviewed the medical records of 166 patients who underwent ERCP for mild to moderate cholangitis due to choledocholithiasis secondary to cholecystolithiasis from 2012 to 2016. RESULTS: Complete stone extraction was accomplished in 92% of patients (152/166) at the first ERCP. Among 152 patients who underwent complete stone extraction, cholecystectomy was scheduled for 119 patients (78%). Cholecystectomy was performed during the same hospitalization in 89% of patients (106/119). We compared two groups of patients: those who underwent cholecystectomy during the same hospitalization (n=106) and those who underwent cholecystectomy during a subsequent hospitalization (n=13). In the delayed group, cholecystectomy was performed about three months after the first ERCP. There were no significant differences between the groups in terms of operative time, rate of postoperative complications, and interval from cholecystectomy to discharge. CONCLUSIONS: Single-stage endoscopic stone extraction is recommended in patients with mild to moderate acute cholangitis due to choledocholithiasis. The combination of endoscopic stone extraction and cholecystectomy during the same hospitalization is safe and feasible.
Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystolithiasis ; Choledocholithiasis ; Hospitalization ; Humans ; Medical Records ; Operative Time ; Patient Outcome Assessment ; Postoperative Complications ; Retrospective Studies

No abstract available.
Endoscopic Mucosal Resection ; Gastroscopy ; Dissection

Inflammatory bowel disease (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), is a chronic inflammatory disease of the gastrointestinal tract, with increasing prevalence worldwide. IBD Ahead is an international educational program that aims to explore questions commonly raised by clinicians about various areas of IBD care and to consolidate available published evidence and expert opinion into a consensus for the optimization of IBD management. Given differences in the epidemiology, clinical and genetic characteristics, management, and prognosis of IBD between patients in Japan and the rest of the world, this statement was formulated as the result of literature reviews and discussions among Japanese experts as part of the IBD Ahead program to consolidate statements of factors for disease prognosis in IBD. Evidence levels were assigned to summary statements in the following categories: disease progression in CD and UC; surgery, hospitalization, intestinal failure, and permanent stoma in CD; acute severe UC; colectomy in UC; and colorectal carcinoma and dysplasia in IBD. The goal is that this statement can aid in the optimization of the treatment strategy for Japanese patients with IBD and help identify high-risk patients that require early intervention, to provide a better long-term prognosis in these patients.
Asian Continental Ancestry Group ; Colectomy ; Colitis, Ulcerative ; Colorectal Neoplasms ; Consensus ; Crohn Disease ; Disease Management* ; Disease Progression ; Early Intervention (Education) ; Epidemiology ; Expert Testimony ; Gastrointestinal Tract ; Hospitalization ; Humans ; Inflammatory Bowel Diseases* ; Japan* ; Prevalence ; Prognosis