心膜液貯留から診断に至った右房自由壁血管肉腫の1例

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先天性心疾患患者の地域連携医療

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左鎖骨下静脈閉塞に対して血管形成術を施行した1例

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不整脈を契機としたうっ血性心不全をきたした心アミロイドーシスの一例

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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

Radial Peripapillary Capillary Network Visualized Using Wide-Field Montage Optical Coherence Tomography Angiography

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.PURPOSE: We quantitatively analyzed the features of a radial peripapillary capillary (RPC) network visualized using wide-field montage optical coherence tomography (OCT) angiography in healthy human eyes. METHODS: Twenty eyes of 20 healthy subjects were recruited. En face 3 × 3-mm OCT angiograms of multiple locations in the posterior pole were acquired using the RTVue XR Avanti, and wide-field montage images of the RPC were created. To evaluate the RPC density, the montage images were binarized and skeletonized. The correlation between the RPC density and the retinal nerve fiber layer (RNFL) thickness measured by an OCT circle scan was investigated. RESULTS: The RPC at the temporal retina was detected as far as 7.6 ± 0.7 mm from the edge of the optic disc but not around the perifoveal area within 0.9 ± 0.1 mm of the fovea. Capillary-free zones beside the first branches of the arterioles were significantly (P < 0.0001) narrower than those beside the second ones. The RPC densities at 0.5, 2.5, and 5 mm from the optic disc edge were 13.6 ± 0.8, 11.9 ± 0.9, and 10.4 ± 0.9 mm-1. The RPC density also was correlated significantly (r = 0.64, P < 0.0001) with the RNFL thickness, with the greatest density in the inferotemporal region. CONCLUSIONS: Montage OCT angiograms can visualize expansion of the RPC network. The RPC is present in the superficial peripapillary retina in proportion to the RNFL thickness, supporting the idea that the RPC may be the vascular network primarily responsible for RNFL nourishment