隐源性多灶性溃疡性狭窄性小肠炎

隐源性多灶性溃疡性狭窄性小肠炎(CMUSE)是一种以小肠多部位狭窄和多灶性浅溃疡为特点的小肠疾病,主要的临床表现为慢性或复发性肠梗阻。溃疡一般较浅,位于黏膜层和黏膜下层。纤维性狭窄部位较短(一般1~2 cm),相互间隔较紧凑(多为2~10 cm),无瘘管形成。外科手术治疗后复发率高,糖皮质激素可有效防止再次手术,多数患者成为激素依赖状态。     

隐源性多灶性溃疡性狭窄性小肠病一例

隐源性多灶性溃疡性狭窄性小肠病(cryptogenic multifocal ulcerous stenosing enteritis,CMUSE)是一种罕见疾病,其病因及发病机制尚不明确.国外报道其典型临床特点为:反复腹痛、肠梗阻、小肠多发表浅溃疡引起狭窄、术后复发倾向及激素治疗有效.国内尚无病例报道.本文报道北京协和医院确诊的1例CMUSE患者,并通过病例分析和文献复习总结CMUSE的临床特点,以进一步提高临床医师对小肠非特异性溃疡性病变的诊断水平.     

双气囊小肠镜临床应用进展

小肠疾病一直是消化系统疾病诊疗的瓶颈,但随着消化内镜技术的不断发展,尤其是双气囊小肠镜的问世,使我们对不明原因消化系出血、小肠肿瘤等的诊断有了新的认识.并且随着双气囊小肠镜技术的逐步成熟使得小肠疾病的内镜下治疗,如出血灶止血、息肉切除、内镜下黏膜切除、狭窄肠腔扩张成为可能.本文综述双气囊小肠镜诊断和治疗领域的最新进展,并且对其应用前景进行分析.     

双气囊电子小肠镜在小肠狭窄诊断中的作用

目的:比较双气囊电子小肠镜及小肠钡灌检查在疑有小肠狭窄患者中病变的检出率和诊断率,评价双气囊电子小肠镜在小肠狭窄诊断中的价值及安全性.方法:疑患小肠疾病患者76例,行双气囊电子小肠镜检查,分别经口或经肛进镜,对未检出病灶者建议择期改换进镜方式再行检查;其中疑小肠狭窄患者13例均行小肠钡灌检查.比较2种检查方法在疑小肠狭窄患者中病变的检出率、诊断率.结果:疑有小肠狭窄患者13例中有8例经口、3例经肛、2例分别经口和经肛行双气囊电子小肠镜检查,检查所用平均时间74(55-120)min,小肠狭窄病变检出率为84.6%(11/13),其中小肠肿瘤6例,克罗恩病3例,炎性狭窄2例.检出病变中双气囊电子小肠镜诊断率为69.2%(9/13),所有患者均未发生严重不良反应和并发症.疑小肠狭窄患者小肠钡灌检查的病变检出率为53.8%(7/13),其中小肠肿瘤3例,克罗恩病2例,炎性狭窄2例.在小肠钡灌检查未发现异常的6例病变中有4例在双气囊电子小肠镜检查中发现病变;3例未能正确诊断的病例在双气囊电子小肠镜检查中均得到正确诊断.双气囊电子小肠镜在小肠狭窄中的病变检出率和诊断率均明显高于小肠钡灌检查,二者均有显著差异(P<0.05).结论:双气囊电子小肠镜对小肠狭窄有较高的病变检出率和诊断率,无严重不良反应和并发症,可作为首选检查方法.     

4例疑诊隐源性多灶性溃疡性狭窄性小肠炎临床分析

目的提高临床医师对隐源性多灶性溃疡性小肠炎(cryptogenic multifocal ulcerous stenosing enteritis,CMUSE)的认识。方法回顾性分析2007年-2010年本院4例临床疑似诊断CMUSE患者的临床资料。结果 4例患者平均年龄(35.25±11.79)岁,以腹痛,排黑便症状为主。患者实验室检查有贫血症状,炎症指标均正常。内镜下见空肠或回肠多发的环行溃疡,溃疡处伴肠腔狭窄,相邻溃疡病灶之间的黏膜形态正常。病理诊断:黏膜及黏膜下层的浅溃疡。结论 CMUSE是一种小肠多发的,以黏膜及黏膜下层的浅溃疡为主的病变。     

双气囊小肠镜对小肠出血的诊断价值

目的探讨双气囊小肠镜对小肠出血的诊断价值及安全性。方法对2007年1月-2008年12月我院经常规检查怀疑为小肠出血的56例患者行双气囊小肠镜检查,分别接受经口或经肛双气囊小肠镜检查。主要分析检查时间、内镜插入深度、确诊情况和并发症发生率。结果所有患者无严重并发症,操作时间为35-150 min,平均（76±22）min;平均插入小肠长度经口和经肛分别为（254±126）cm和（182±103）cm。小肠病变中阳性发现46例（82.1%）,主要包括血管畸形、小肠溃疡、克罗恩病、慢性非特异性炎症、肿瘤、息肉及憩室等。结论双气囊小肠镜检查安全有效,为小肠出血疾病的诊断提供了新的手段。     

双气囊电子小肠镜的操作规范

小肠是消化吸收的主要部位,分为十二指肠、空肠和回肠,长约5 m,游离于腹膜内,并被肠系膜束缚形成多个复合肠襻,是整个胃肠道中最难检查的部分.因此,小肠疾病的诊断远落后于胃肠道其他部位,X线小肠钡餐检查、核素扫描、动脉造影及传统的推进型小肠镜等检查方法均存在着诊断阳性率低、定性与定位不准确等缺点. 双气囊电子小肠镜是日本富士公司研发生产的新型小肠镜,有希望获得整个小肠的影像学资料,并可取得活检标本.以下介绍双气囊电子小肠镜的操作规范.     

双气囊小肠镜对不完全小肠梗阻的诊断价值及安全性分析

目的 探讨双气囊小肠镜(DBE)对不完全性小肠梗阻的诊断价值与安全性.方法 对21例确诊的不完全性小肠梗阻患者行DBE检查,收集并分析相关临床数据.结果 21例患者共进行22次DBE检查,20例发现异常病变,阳性检出率95.2％.小肠不全性梗阻增生性病变6例,病变部位以空肠最多;溃疡瘢痕狭窄10例,病变部位以回肠最多.检查前、中、后的心率、血氧饱和度、收缩压、舒张压变化均无统计学差异(P均＞0.05).采用视觉模拟评分量表对小肠镜检查前及麻醉清醒后1、6和24h的腹痛程度进行评分,各时间点比较无统计学差异(P均＞0.05).结论 依靠恰当的麻醉和操作方法,DBE对不完全性小肠梗阻有较高的检出率,安全可行.     

双气囊小肠镜诊断小肠克罗恩病的作用

目的 探讨双气囊小肠镜在小肠克罗恩病诊断中的作用.方法 对28例疑有小肠疾病者行双气囊小肠镜检查,分析双气囊小肠镜检查对小肠疾病,尤其是小肠克罗恩病的检出率、诊断准确性、患者依从性和不良反应发生率等.结果 28例中,小肠病变总检出率为71.43%(20/28),小肠克罗恩病检出率为21.43%(6/28),占所有检出病变的30%(6/20).6例双气囊小肠镜检查判断为克罗恩病者中,结合病理、治疗效果和临床随访,6例均确诊为克罗恩病,诊断准确率为100%(6/6).结论 双气囊小肠镜检查是一项针对小肠疾病安全、有效的检查方法 ,是诊断小肠克罗恩病较为理想的方法 .     

双气囊小肠镜在68例小肠疾病诊断中的价值

目的 评价双气囊小肠镜对小肠疾病的诊断价值及安全性和耐受性.方法 2003年5月至2005年7月,对68例经常规检查无异常发现、疑患小肠疾病患者进行双气囊小肠镜检查,其中不明原因反复消化道出血39例、不完全性小肠梗阻7例、慢性腹痛14例、慢性腹泻8例.结果 68例患者中,36例经口进镜,25例经肛进镜,7例患者分别经口及经肛进镜检查.除3例因肠腔狭窄中止进镜外,其余病例均能检查1/2-3/4的小肠,7例患者结合经口及经肛途径完成全小肠检查.68例患者中41例检出阳性病灶,总阳性率为60.3%;其中不明原因消化道出血阳性率为62.6%（26/39）,不完全性小肠梗阻阳性5例,慢性腹痛阳性率为43%（6/14）,慢性腹泻阳性4例.除11例经口进镜者行异丙芬静脉全身麻醉外,其他经口及经肛进镜患者均能耐受整个检查,未出现出血、穿孔等严重并发症.结论 双气囊小肠镜是一种对小肠疾病诊断价值较高、安全可靠的检查手段.     

Cryptogenic multifocal ulcerous stenosing enteritis: Radiologic features and clinical behavior

AIM To investigate the characteristic radiologic findings of cryptogenic multifocal ulcerous stenosing enteritis(CMUSE) which can be differentiated from other similar bowel disease and to assess their clinical behavior.METHODS Twenty pathologically and clinically confirmed CMUSE patients(males:females = 8:12; mean age: 40.4 years) between March 2002 and August 2015 from seven academic centers in South Korea were retrospectively reviewed. We evaluated small bowel series(SBS; n = 25), computed tomography(CT) enterography(n = 21), magnetic resonance(MR) enterography(n = 2), and abdominopelvic CT(n = 18) images, focusing on enteric and perienteric manifestations. Any change in radiologic features during followup period was recorded. We evaluated clinical data including presenting symptoms, laboratory finding and presence of relapse from electronic medical records. Histopathologic findings were also evaluated. RESULTS The main symptoms were abdominal pain(n = 12) and anemia(n = 10). All patients showed small bowel strictures(n = 52, mean: 2.6 per patient) on initial CT/MR, located in the ileum(n = 47) or jejunum(n = 5). Strictures showed short-length(mean: 10.44 mm) and circumferential bowel wall thickening(mean: 5.56 mm) with layered enhancement(n = 48) that were also noted on initial SBS(n = 36) with shallow ulcers(n = 10). Some ulcerative lesions or wall thickening progressed into strictures on follow-up SBS/CT, and some strictures revealed recurrent ulceration on followup SBS. There were no penetrating disease features like fistula or abscess and no gastrointestinal tract involvement except the small bowel. Nine patients experienced disease recurrence(median relapse-free period: 32 mo) even post-operatively. Histopathologic features of surgically resected specimens were characterized as multiple superficial ulcerations confined to mucosa or submucosa and multiple strictures.CONCLUSION Under characteristic radiologic findings with multiple short-segmental strictures and/or shallow ulcers of the small intestine, CMUSE should be considered when assessing patients with recurrent abdominal pain and anemia.     

Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis

BACKGROUND/AIMS: Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathophysiology of the disease, and to propose a treatment strategy. METHODS: A total of 220 French gastroenterology departments were contacted to review patients with unexplained small bowel strictures. Of 17 responses, 12 corresponded to a diagnosis of CMUSE. These patients were hospitalised between 1965 and 1993 and their medical records were reviewed. RESULTS: All patients (mean age 42.1 (4.4) years) had intestinal and five had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient had heterozygous type I C2 deficiency (28 base pair gene deletion). Two to 25 (mean 8.3 (1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils. CONCLUSIONS: CMUSE is an independent entity characterised by steroid sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.     

Genetic analysis is helpful for the diagnosis of small bowel ulceration

The widespread use of capsule endoscopy and balloonassisted endoscopy has provided easy access for detailed mucosal assessment of the small intestine. However, the diagnosis of rare small bowel diseases, such as cryptogenic multifocal ulcerous stenosing enteritis(CMUSE), remains difficult because clinical and morphological features of these diseases are obscure even for gastroenterologists. In an issue of this journal in 2017, Hwang et al reviewed and summarized clinical and radiographic features of 20 patients with an established diagnosis of CMUSE. Recently, recessive mutations in the PLA2G4A and SLCO2A1 genes have been shown to cause small intestinal diseases. The small bowel ulcers in each disease mimic those in the other and furthermore those found in nonsteroidal anti-inflammatory drug-induced enteropathy. These recent and novel findings suggest that a clinical diagnosis exclusively based on the characteristics of small bowel lesions is possibly imprecise. Genetic analyses seem to be inevitable for the diagnosis of rare small bowel disorders such as CMUSE.     

The chronological sequence in the pathology of Crohn's disease

Sixty-three consecutive reexcisions of bowel for recurrent Crohn's disease where the former excision had removed all apparent disease were studied. Nine pathologic parameters (small ulcers, granulomas, regeneration, metaplasia, submucosal lymphoid follicles, transmural inflammation, large ulcers, sinuses, and strictures) were correlated to the interval of time since the former excision to document the disease progression. By regression analysis, only the presence of strictures or large ulcers correlated with time. First quartile times suggest that small ulcers and granulomas were the earliest lesions of those studied. Regeneration, metaplasia, submucosal lymphoid follicles, and transmural inflammation appear to be sequelae of ulceration. Sinuses and strictures are late complications of ulceration. The results confirm clinical, radiological, and subjective pathological impressions of the progress of the disease and suggest a central role for ulceration in this disease.     

Small bowel ulcers

Opinion statement Treatment of small intestinal ulcers depends on the cause of the ulcers. Ulcerations of the small intestine (apart from duodenal ulcers) are uncommon. Two diverse syndromes of idiopathic small bowel ulcerations include the isolated nonspecific ulcer and idiopathic chronic ulcerative enteritis (ICUE). Treatment of isolated nonspecific ulcers include discontinuation of medications known to cause nonspecific ulcerations, balloon dilation of strictures, and segmental resection of involved segments. Treatment of ICUE includes a diet trial of gluten restriction and consideration of early surgery because of the potential for associated malignancies. Steroid therapy has not been shown to be effective in the treatment of ICUE.     

Ischemic stricture of the small intestine associated with acute pancreatitis

Summary A 37-yr-old man underwent an open drainage operation for severe acute pancreatitis and received respiratory ventilation support for 4 mo because of respiratory failure based on disseminated intravascular coagulation (DIC) and septic shock. Under intensive care, he sometimes had bloody diarrhea for about 6 wk. Colonoscopic findings suggested that the bleeding had derived from the small intestine. The patient then gradually recovered from acute pancreatitis and was discharged from the hospital. Thereafter, he suffered relapses of ileus and his symptoms progressively worsened. The patient underwent a second operation about 2 yr after the onset of acute pancreatitis. At celiotomy, multiple stenoses of the distal ileum measuring about 60 cm in length were found and the segment was resected. The resected specimen demonstrated six separate circumferential strictures and shallow ulcerations. Histologically, multiple ulcerations were restricted to the mucosa and were accompanied by marked submucosal edema and fibrosis. The mucosa between the ulcers revealed chronic regenerative changes: intimal thickening of small mesenteric arteries causing luminal narrowing and organized thrombosis in small mesenteric veins. Therefore, these were considered to be a series of segmental ischemic lesions. Note that delayed ischemic stricture of the small intestine may occur as a chronic complication of acute pancreatitis.     

Wireless capsule endoscopy detects small bowel ulcers in patients with normal results from state of the art enteroclysis

BACKGROUND: Wireless capsule endoscopy (WCE) is a new technology for small bowel imaging. AIM: To report our initial experience with sensitivity of high quality enteroclysis in patients with small bowel ulcers detected by WCE. METHODS: Medical records of all patients referred for WCE from December, 2001 to April, 2002 at our institution were reviewed. All patients had negative upper and lower endoscopies and small bowel barium studies before WCE. RESULTS: There were 40 patients (19 female, mean age 57.3 yr) during this study period. Three patients had multiple small bowel ulcers detected by WCE. One with ileal ulcers and abdominal pain had an enteroclysis at another hospital before WCE. Review of the study at our institution showed that it was of excellent quality and was normal. Two patients with chronic iron deficiency anemia had multiple small bowel ulcers and were referred after WCE for a repeat small bowel barium study by biphasic enteroclysis performed by experienced GI radiologists. The radiologists were told in advance of the WCE findings. Both studies were considered technically to be of perfect quality. Despite this, both studies were negative. All 3 patients improved after therapy for Crohn's disease. CONCLUSIONS: Our data indicates that WCE may be more sensitive for small bowel ulcers than the best enteroclysis available.     

Double-balloon enteroscopy-assisted dilatation avoids surgery for small bowel strictures: A systematic review

AIM To evaluate the therapeutic role of double-balloon enteroscopy(DBE) in small bowel strictures and to propose a standard approach to small bowel strictures.METHODS Systematic review of studies involving DBE in patients with small bowel strictures. Only studies limited to small bowel strictures were included and those with ileo-colonic strictures were excluded. RESULTS In total 13 studies were included,in which 310 patients were dilated. The average follow-up time was 31.8 mo per patient. The complication rate was 4.8% per patient and 2.6% per dilatation. Surgery was avoided in 80% of patients. After the first dilatation,46% were treated with re-dilatation and only 17% required surgery.CONCLUSION DBE-assisted dilatation avoids surgery in 80% of patients with small bowel strictures and is safe and effective. We propose a standardized approach to small bowel strictures.     

Multiple strictures in Crohn's disease of the small bowel: a benign variant

Involvement of both jejunum and ileum is uncommon in Crohn's disease of the small bowel. We report five patients with multiple strictures of the small bowel associated with one or more intervening segments of dilated bowel. A diagnosis of Crohn's disease was delayed because none of the patients experienced diarrhea. Despite the early radiologic appearance of extensive small bowel disease, only three patients have required surgery, a limited surgical resection of 65-75 cm was possible, and long-term prognosis has been favorable.