池田 太郎

Abdominoscrotal hydrocele (以下，ASH) は陰嚢水腫が鼠径管を介し，腹腔内に交通しているまれな疾患であり，その発生機序と治療法は未だ一定の見解は得られていない．今回，我々は ASH を鼠径管アプローチに腹腔鏡を併用することで完全切除した症例を経験した．症例は 11 ヵ月の男児．左陰嚢腫大を主訴に来院した．超音波検査にて腹腔内に連続する陰嚢水腫を認め，ASH を疑い，MRI にて診断した．手術は腹腔鏡を併用して鼠径法による水腫摘出を行った．術後経過は良好で現在まで再発は認めていない．腹腔鏡を併用することは ASH の形態的評価が可能であり，安全かつ確実に切除することが可能であるため非常に有用である．

The local control of neuroblastoma is a very important treatment consideration. We describe a patient who received high-dose rate 60Co remote after loading system treatment for local control of recurrent neuroblastoma and discuss the efficacy of high-dose rate 60Co remote after loading system treatment. (C) 2011 Elsevier Inc. All rights reserved.

In western countries, left-sided diverticular disease (LDC) is very common, but right-sided diverticular disease (RDC) is rare. On the other hand, in Oriental countries including Japan, RDC is more common than LDC. In the pathogenesis of diverticular diseases, RDC has been considered among those diseases with anomaly of colonic motility. It is well known that the genesis of pressure-induced diverticula related to the increased tension which causes blow-out of the mucosa in the colon haustrum located between two successive segments of spastic circular muscle (i.e., colonic segmentation). Peristalsis is very important in the transport of intestinal contents from the mouth to the anus and is mainly controlled by the enteric nervous system (ENS). Physiological, pharmacological and histochemical investigations have indicated that non-adrenergic, non-cholinergic (NANC) excitatory and inhibitory nerves, in addition to adrenergic and cholinergic components, play an important role in the regulation of intestinal peristalsis. However, there have been few reports in regard to the physiologic function of the ENS in patients with diverticular disease. In particular, physiologic functional differences of the ENS between the RDC and the LDC remain unknown. Recently, it has been reported that the ENS contains excitatory and inhibitory nerves and that the former, especially cholinergic nerves, are dominant in both RDC and LDC. Furthermore, there are no functional differences of the ENS between the RCD and LDC. These observations may be related to the high intraluminal pressure due to colonic segmentation in colonic diverticular disease, including RCD and LDC.

Background Focal nodular hyperplasia (FNH) of the liver is a rare benign lesion that may be related to the vascular and hepatic damage induced by completion of tumor therapy and a reaction to localized vascular abnormality. The aim of this study was to analyze the clinical course in pediatric solid tumor patients with FNH. Methods We analyzed thirty-two patients with pediatric solid tumors who received multiagent chemotherapy (15 advanced neuroblastomas, 7 hepatoblastomas, 5 rhabdomyosarcomas, 2 nephroblastomas, 1 rhabdoid tumor of the kidney, 1 clear cell sarcoma of the kidney and 1 pancreatoblastoma). All of them had been previously treated at our hospital, and have been alive for over 3 years without recurrence. Results FNH lesions were discovered in three (9.4%) of 32 patients, and were neuroblastoma (NB) stage 4. All 3 patients received induction chemotherapy and high-dose alkylating agents, and developed grade 3 (National Cancer Institute Common Toxicity Criteria; NCI-CTC) liver dysfunction during completion of tumor therapy without veno-occlusive disease. Two of the 3 patients received the same induction chemotherapy and high doses of alkylating agents with total body irradiation for cytoreductive agents prior to peripheral blood cell transplantation. FNH lesions in both female patients who received estrogen replacement therapy after completion of tumor therapy have expanded and are increasing. Conclusion FNH appears to be a late complication of iatrogenic disease in NB stage 4 patients. The therapeutic agents for NB stage 4 and estrogen replacement therapy should be considered as risk factors for the development of FNH.

急性膵炎にて発症した先天性胆道拡張症の一例を経験したので報告する．症例は 8 歳，女児．腹痛を主訴に来院した．血液検査と画像所見より，急性膵炎および先天性胆道拡張症と診断して治療を開始した．急性膵炎に対しては絶飲食と補液，蛋白分解酵素阻害剤および抗生剤を投与による保存的治療を開始した．腹部症状・膵酵素の改善が認められたのちに，再発予防として成分栄養剤 (ED) による経腸栄養管理を行い，入院 28 日目に先天性胆道拡張症に対して肝外胆道切除・肝管空腸吻合術を施行した．術後経過は良好であり，術後 15 日目に退院となった．小児における急性膵炎では先天性胆道拡張症は主因の一つと考えられ，膵炎で発症した症例では膵炎の再発・再燃を防ぐためにも早期に根治術を行う事が必要であり，急性膵炎に対してはそれらを念頭に管理を行うことが肝要であると考える．

Background: Meconium obstruction without cystic fibrosis in low-birthweight neonates is a distinct clinical entity. We aimed to determine what therapeutic strategies work best in very-low-birthweight neonates with meconium obstruction of the small bowel under varied clinical conditions caused by the associated diseases of prematurity. Methods: Medical records of very-low-birthweight neonates with meconium obstruction of the small bowel treated from 1998 to 2008 were retrospectively reviewed. Pre- and postnatal data, treatments, and clinical outcomes were assessed. Results: Nine patients with perinatal complications were identified. Mean gestational age and birthweight were 26.9 weeks and 863 g, respectively. Abdominal distension developed from 1 to 7 days of life. Five patients were initially treated with Gastrografin enema, three of whom had successful outcomes. Two hemodynamically unstable patients failed to respond to Gastrografin treatment; they ultimately died of sepsis. The remaining four without Gastrografin treatment underwent enterostomy to resolve the obstructions with good results. Conclusions: Gastrografin and surgical treatments should be appropriately selected based on the underlying pathologies of meconium obstruction of the small bowel. Therapeutic Gastrografin enema is effective, safe and repeatable; however, it is not recommended for hemodynamically unstable patients. Surgical intervention is reserved for those who develop rapid abdominal distension that risks perforation.

【目的】小児外鼠径ヘルニアに関する対側発症症例を中心とした統計学的検討から,対側発症の危険因子を抽出し,対側発症の危険度をスコア化によって評価する方法を考案することを目的とした.【対象と方法】平成13年4月から5年間に経験した小児外鼠径ヘルニア手術症例662例を対象とし,対側発症症例を中心に,性別,初回手術時年齢,患側,出生体重,初回片側発症から対側発症までの期間について調査し,後方視的に統計学的検討を行った.【結果】全症例における男女比は1.3:1で,手術時年齢は1歳未満が30.2%で最も多かった.患側は右側が52.7%,左側が40.2%,両側は7.1%であり,男児で右側,女児で左側に多かった.片側手術症例における対側発症率は8.8%で,1歳未満が38.9%で最も多く,女児の初回手術側が左側である症例の対側発症率が11.4%で最も高かった.対側発症までの期間は,対側発症症例全例では1年以内が53.7%であったのに対し,低出生体重児例のみでは77.0%と有意差がみられた.対側発症症例および両側手術症例について,片側手術のみの症例と比較すると,低年齢および低出生体重児例の割合が有意に高かった.【結論】対側発症症例および両側症例における臨床的特徴は,これまでの報告と同様の傾向であった.片側手術のみの症例との検定結果より,年齢および出生体重において有意差がみられた.われわれは今回の結果を基に,対側発症の危険度をスコア化によって評価する方法を考案した.現在,これを運用し対側発症の予知・対策について前方視的調査に取り組んでいる.

Background/Aims: There are few reports of the segmental colonic transit time (SCTT) in healthy men. To clarify the SCTT for healthy men, the author measured the SCTT of healty men using radiopaque markers (RM). Methodology: The author analyzed the SCTT of 26 healthy men, who were aged from 24 to 60 years and had a mean age of 48.8 years, with RM (20 radiopaque polyvinyl chloride 0-rings). The criteria for participation included a usual stool frequency of between three per week and three per day, no history of gastrointestinal disease, and no use of medications known to affect gastrointestinal motility. Results: The overall gastrointestinal transit time was 36.2±5.1 hours, and the transit time from the mouth to the cecum was 6.8±1.4 hours. The half-dose transit times of the ileocecal valve, hepatic flexure, splenic flexure, descending-sigmoid line, and evacuation were 6.9±1.6, 15.9±1.7, 18.8±1.7, 24.3±4.4 and 36.4±5.3 hours, respectively. The ascending (9.5±2.3 hours) and descending colon (5.5±4.1 hours) had shorter transit times than the sigmoid-rectum section (12.7±3.5 hours) (p&lt 0.001, p&lt 0.01, respectively), and the transverse colon (4.2±2.1 hours) had a shorter transit time than the ascending colon (p&lt 0.05). There were no significant differences in transit time between the descending and transverse colon. Conclusions: Measuring the SCTT using RM may be effective for detecting the SCTT of specific sections of the bowel. This method is simple and can be easily performed at any radiology department. © H.G.E. Update Medical Publishing S.A.

症例は11歳女児.排尿困難を主訴に近医受診.導尿にて改善するも再び同様の症状を認めたため,当院泌尿器科に紹介となった.骨盤内腫瘤を認め,CA19-9,CA125が高値であったため,卵巣腫瘍を疑い当科紹介となる.下腹部全体に軽度の膨隆を認め,超音波検査では8×8×12cm大の骨盤内腫瘤を,CT・MRI検査では子宮・腟内腔の拡張を認めた.外陰部の診察の結果,処女膜閉鎖症による子宮・腟溜血腫と診断した.治療は処女膜十字切開と貯留液のドレナージを行った.貯留液は月経血様で450mlであった.処女膜は上辺縁を切除し,切開縁を結節縫合し,癒着・再閉鎖の予防とした.術後経過は良好で,CA19-9,CA125の値は術後速やかに減少,術後1か月目には月経が発来した.処女膜閉鎖症は比較的稀な疾患とされるが,外陰部の診察により容易に診断が可能で,月経未発来児の下腹部腫瘤では本症も念頭においた診察が重要と思われた.

男児。超低出生体重児(ELBWI)で重症新生児仮死のため救急処置後にNICUへ入室した。日齢3に少量の胎便排泄のみで十分な排泄はなく、X線でガス像は下部消化管に届かず、胎便関連性腸閉塞(MOSB)と診断した。ガストログラフィン治療を続けたが、日齢24より静脈栄養に伴う胆汁鬱滞によると思われる直接ビリルビン値(直ビ値)の上昇を認め、日齢27に外科的治療を行った。回腸末端より65cm口側にcaliber changeを認め、肛門側の狭小な腸管内に充満した非常に粘稠な胎便を除去し、術中X線でガストログラフィンが到達していることを確認して双孔分離式回腸瘻を造設した。経腸栄養を開始したが静脈栄養が主体のため直ビ値が再上昇し、日齢97に腸瘻を閉鎖した。体重は良好に増加し、日齢160頃より直ビ値は低下し始め、日齢222に直ビ値1mg/dlとなり退院した。

超低出生体重児(以下ELBWI)における腸重積症の報告は少なくその特徴は知られていない.今回我々はその2例を経験したので報告する.

症例は5歳女児.先天性嚢胞性腺腫様奇形にて日齢6に右肺下葉切除術を施行した.3歳頃から胸郭変形と喘息様呼吸器症状を認め,3歳8か月時には肺炎で入院した.胸部CT検査では縦隔の左側への偏位とそれに伴う左気管支の圧排による閉塞性肺炎像を認め,この頃より進行する胸郭の陥凹と左右差が顕著となり,持続する喘息様呼吸器症状を認めるようになった.5歳5か月時にRavitch手術を施行し,呼吸器症状の改善を認めた.新生児期に胸部手術を施行した呼吸障害を伴う漏斗胸に対して,Ravitch手術は有用であった.

症例は15歳,女児.褐色細胞腫または傍神経節腫の疑いで当院を紹介受診した.腹部CT検査で左後腹膜腫瘤と多発性膵嚢胞,^<123>I-metaiodobenzylguanidineシンチグラフィで左後腹膜腫瘤に一致して集積像を認めた.開腹腫瘍生検術で,傍神経節腫と診断した.左腎静脈は腫瘍に巻き込まれており,左腎合併切除を伴う腫瘍完全切除を施行した.本症例はvon Hippel-Lindau(VHL)病の関与が考えられ,VHL遺伝子の変異とその発現を検討した.本症例では非腫瘍部腎臓組織でVHL遺伝子の生殖細胞変異を認め,さらに腫瘍組織で体細胞変異を2か所に認めた.また同遺伝子の発現は,腫瘍組織で消失していた.以上より本症例は,VHL病と考えられた.傍神経節腫において,VHL病の診断基準を満たさなくとも,VHL病の関与する症例が存在し,慎重な全身検索と経過観察が重要と考えられた.

We herein report the case of a 35-month-old female child presenting with mesenchymal hamartoma of the liver (MHL), with t(11;19)(q13;q13.4) originating in the caudate lobe. This case is the eighth known description of a cytogenetic abnormality in mesenchymal hamartoma of the liver. It is similar to the seven cases previously reported, in that one of the breakpoints involves the chromosome band 19q13.3 or 19q13.4, but it is the first report of an abnormality originating in the caudate lobe.