A surgical case of mitral valve replacement for a patient with Fabry disease complicated with hemodialysis

　Fabry disease is a rare genetic disease, and surgical reports for the patients with Fabry disease are also rarer.　A 58-year-old man presented with chest pain. At the age of 40, he commenced dialysis due to chronic renal failure and at the age of 50, he developed shortness of breath on exertion, and echocardiography showed mitral regurgitation and left ventricular hypertrophy. He was then diagnosed with Fabry disease due to decreased alpha-galactosidase activity. This diagnosis led to enzyme replacement therapy (ERT). The ERT was effective as he had not never experienced further exacerbation of congestive heart failure. While the CHF was put under control, his mitral stenosis gradually worsened, and the patient began to have more chest pain and became hypotensive. He then referred to our section for mitral valve replacement. His mitral annulus was severely calcified and we removed mitral annulus calcification (MAC) at minimum so that we could stich needles and implanted mechanical valve. Paroxysmal atrial fibrillation and bradycardia made his hemodynamics unstable against ERT, which also caused low dialysis efficiency. It took longer than usual to wean him off catecholamines. His hemodynamics became more stable and dialysis efficiency generally improved, so he moved from ICU to ward on postoperative day 11. On day 32, he was transferred back to the referring hospital for his rehabilitation.　We have reported a surgical case of Fabry disease, that are not only rare but have high perioperative risk due to Fabry disease’s specific complications

Case report on a coronary artery bypass graft for a patient with antiphospholipid antibody syndrome associated with systemic lupus erythematosus

　Antiphospholipid antibody syndrome (APS) is an immune disease in which antiphospholipid antibodies cause hypercoagulability and thromboembolic complications. We experienced APS cases associated with systemic lupus erythematosus with three-vessel lesions of the coronary artery. After a below knee amputation on a 60-year-old woman with APS, she complained of chest pain at rest. An electrocardiogram showed an ST depression and a coronary angiography showed complicated three-vessel disease, as a result she was referred to the cardiac surgery department. A coronary artery bypass with arterial grafts was performed along with postoperative anticoagulant and antiplatelet therapy, and the short-term graft patency was good. Case reports of coronary artery bypass grafts for secondary APS are rare, so we report here on our case and our strategy to treat thromboembolic complications

Minimally invasive cardiac surgery via a right mini-thoracotomy

　Minimally invasive surgery, which has become very active outside the cardiovascular field, has recently come to the fore in this area. Then, procedures such as offpump coronary artery bypass grafts without extracorporeal circulation and stent grafts for treating aortic aneurysms have been frequently performed.　In cardiac surgery, as in other surgical fields, more and more surgeries that are less and less invasive have been introduced in recent years. Off-pump coronary artery bypass grafting has contributed to the development of these less invasive surgeries. For example, cardiac surgery utilizing a partial sternotomy was introduced as a way to better access the surgical location. However, minimally invasive cardiac surgery (MICS) through a right mini-thoracotomy, a portaccess cardiac surgery, is said to be trending recently because it avoids a sternotomy and has less bleeding and wound infection. All of these factors not only promote early recovery, but are also expected to have a positive impact on early discharge and the health care economy.　With surgeons and hospitals accumulating experience, MICS is being applied to more complex lesions and has begun to be used to treat the aortic valve in addition to the mitral valve. Off-the-job training and team building are also key factors for implementing a successful program. This type of port-access cardiac surgery is already beginning to be developed into a robotically assisted heart surgery by various facilities around the world

Current Status and Future Potential of Robotic Surgery for Gastrointestinal Cancer

　Robotic surgery has built on innovations in areas such as medical engineering and optical technology. Laparoscopic surgery has been successfully adapted for gastric, colon, and rectal cancer surgeries over the past two decades with numerous clinical trials showing oncological results comparable to those of open surgery. These trials have also shown that the laparoscopic approach shortens postoperative recovery time and decreases complication rates. Another advantage of minimally invasive techniques for the resection of gastric, colon, and rectal cancers is improved visualization of the surgical field. Despite the near absence of tactile feedback, robotic surgery has overcome many of the challenges inherent in laparoscopic surgery through features such as 3D vision, stable magnification, EndoWrist instruments, physiological tremor filtering, and motion scaling. Robotic surgery is not yet widely used in esophageal cancer surgery or in a pancreaticoduodenectomy for pancreatic cancer due to anatomical difficulties and the lack of a suitable approach.　Comparative studies of robotic and laparoscopic surgery have shown similar results in terms of perioperative management, oncologic evaluation, and functional outcomes. However, it is also true that the high cost and lack of tactile feedback in robotic surgery are major limitations in terms of current robotic technology becoming the worldwide standard for minimally invasive surgery. The future of robotic surgery will require cost reduction, the development of new platforms and technologies, the creation and validation of curricula and virtual simulators, and confirmation through appropriate randomized controlled clinical trials

Appendiceal mucinous neoplasm: a review of eleven surgical cases in our institution

　虫垂原発粘液産生腫瘍は WHO 分類に基づき低異型度虫垂粘液性腫瘍（Low-grade appendiceal mucinous neoplasm，以下 LAMN）と粘液癌に分類される．当科にて2010年４月〜2018年11月までに外科的切除された11症例を集積検討した．　11症例の内訳は年齢が27～88歳（中央値61歳）で男女比は男７人，女４人であった．主訴は腹痛が６人で無症状が５人であった．病理診断での腫瘍最大径は３〜12 cm（平均5.9 cm）であった．術前より LAMN と疑われた症例は７例で，虫垂腺癌の術前診断に至った症例は１例であった．虫垂腫瘍との術前診断に至らなかった３症例のうち，虫垂炎の術前診断で虫垂切除術施行後に病理診断で判明したものが２例，十二指腸潰瘍穿孔で緊急手術を行った際に合併切除した虫垂組織より偶然発見されたものが１例であった．術式は虫垂切除のみが３例，回盲部切除が５例，右半結腸切除が３例であった．予定手術は６例で緊急手術が５例であった．最終病理診断（大腸癌取り扱い規約第９版に準拠）は LAMN が７例で虫垂腺癌が２例，粘液嚢胞が２例であった．術後入院期間は2〜47日（中央値12日）で，虫垂腫瘍切除に関連する術後合併症はなかった．LAMN は比較的稀な疾患であるが，腫瘍破裂により粘液が漏出することで腹膜偽粘液腫をきたす可能性がある．そのため，再発を引き起こさないためには①画像検査などでの術前診断（術中診断を含む），②術中に粘液漏出させない術式選択，③術後病理診断で判明した場合の追加治療の適否，についてその都度慎重に判断する必要がある．　LAMN は低悪性度腫瘍にも関わらず再発の危険性があるため，画像検査で疑った場合は再発防止を念頭においた術前評価と治療方針の策定が必要であり，切除後の厳重フォローも重要である．　Appendiceal mucinous neoplasm (AMN) is composed of mucinous adenocarcinoma and low-grade appendiceal mucinous neoplasm (LAMN) according to the fifth edition World Health Organization classification. Although AMN is relatively rare in clinical practice, we had eleven surgical cases of AMN from April 2010 to November 2018 and retrospectively review them in this report. The eleven cases consisted of seven men and four women, ages 27 - 88 years old (average: 65.5 y.o.). Six patients had abdominal pain upon their initial visit. Preoperative examinations made a presumptive diagnosis of LAMN in 7 cases and cancer of the appendix in 2 cases. In the other two cases, one was diagnosed after surgery for acute appendicitis, and the other was coincidently found in the appendiceal tissue that was resected during an emergent laparotomy for a perforated duodenal ulcer. Emergency operations were performed for 5 cases, whereas laparoscopic surgery was done in 4 of the cases. The following operative procedures were performed; 5 ileocecal resections, 3 right hemicolectomies, and 3 simple appendectomies. Pathological examinations concluded that 7 cases were LAMN, 2 were appendiceal adenocarcinoma, and the remaining 2 were hyperplastic mucocele. The length of hospital stay after surgery varied from 2 to 47 days (median: 12 days), with no apparent complications related to surgery in any cases. The most critical features of AMN are the potential to recur as pseudomyxoma peritonei, when the tumor is ruptured during surgery, or in case of tumor cells remaining at the resection stump. Since LAMN is specifically recognized to be low-grade malignant, several steps should be taken to minimize recurrence in addition to the standard postoperative follow-up on regular basis. These steps include assessing each preoperative state of disease adequately, selecting the most suitable procedure to reduce the risk of mucus leakage, and cautiously reviewing the necessity of additional resection based upon pathological determinations

Minimally invasive cardiac surgery via a right mini-thoracotomy

Reviewjournal articl

Current Status and Future Potential of Robotic Surgery for Gastrointestinal Cancer

Reviewjournal articl

Two cases of perforated anastomotic ulcer at the site of gastrojejunostomy after subtotal stomach-preserving pancreatoduodenectomy

　当 院 で 経 験 し た 亜 全 胃 温 存 膵 頭 十 二 指 腸 切 除 術（sub-total stomach-preserving pancreatoduodenectomy, 以下 SSPPD）後の胃空腸吻合部に生じた吻合部潰瘍穿孔の２治療例を 報告する．症例１は十二指腸乳頭部癌に対し SSPPD を施行された51歳女性．下腹部痛を主訴に 救急外来を受診，腹部 CT にて free air を指摘された．穿孔性腹膜炎と診断し，緊急開腹手術を施行した．症例２は膵頭体部癌に対し SSPPD を施行された後，肝転移に対し化学療法中であった 69歳男性．左側腹部痛を主訴に救急搬送され腹部CTでfree airを認めるも保存的加療にて軽快した． 　　２例とも SSPPD 後の吻合部潰瘍穿孔であったため術後の胃酸分泌能が術前と同等に維持されていた可能性がある．症例１はプロトンポンプ阻害薬を内服中にも関わらず発症した．症例２は腰痛 に対する非ステロイド消炎鎮痛剤を常用していたことも発症の一助と推察される．SSPPD 術後の合併症として吻合部潰瘍の可能性を念頭におき予防に努める必要がある．症例に応じては保存的加 療でも改善が見込める場合がある．　We herein report on two cases that were treated for a perforated ulcer at the gastrojejunostomy anastomosis site after a subtotal stomach-preserving pancreatoduodenectomy (SSPPD). The first case was a 51-year old female who received an SSPPD for cancer of the duodenal papilla. Six months after the surgery, the patient suddenly experienced lower abdominal pain and a CT scan detected free air in the peritoneal cavity. During the emergent laparotomy, a perforation was found at the site of the gastrojejunostomy. The perforation was sutured, and the postoperative course was uneventful. The second case was a 69-year old male who was receiving chemotherapy for metastatic liver cancers after receiving an SSPPD for pancreatic cancer. He was hospitalized due to left abdominal pain and a CT scan revealed intraperitoneal free air around the gastrojejunostomy anastomosis site. The patient did not require surgery because his abdominal pain was controllable and the peritonitis was localized. He recovered with antibiotics and a proton-pump inhibitor. We presumed that postoperative gastric-acid secretion would be preserved to function as well as it did preoperatively, since both cases received an SSPPD. However, and surprisingly, the first case developed an anastomotic perforation despite regularly taking a proton-pump inhibitor. In the second case, the regular usage of nonsteroidal anti-inflammatory drugs for lumbar pain might have caused the perforation. From these clinical experiences, we learned the necessity of some prophylactic treatments for patients receiving an SSPPD. It is noteworthy that nonsurgical treatments can be applicable for some selected perforated anastomotic ulcers, though in most cases an emergent laparotomy should also be considered