Endometriosis of the Terminal Ileum Simulating the Clinical, Roentgenographic, and Surgical Findings in Crohn''s Disease

A 38-yr-old nulliparous female presented with endometriosis of the terminal ileum which mimicked the clinical, roentgenographic, and surgical findings of Crohn's disease. The patient presented with diarrhea, right lower quadrant pain, fever, and significant weight loss. At surgery, the terminal ileum was inflamed, indurated, thickened, and angulated. Ileal endometriosis may be expected to simulate Crohn's disease, because intestinal endometriosis frequently produces local bowel inflammation, adhesions, stricture, and angulation. Ileal endometriosis should be carefully considered in the differential diagnosis of Crohn's disease in menstruating females who are nulliparous and have dysmenorrhea, dyspareunia, dyschezia, menometrorrhagia, or other perimenstrual symptoms. It is important not to mistake endometriosis for Crohn's disease because endometriosis has a different therapy.     

CCR2 expressing CD4+ T lymphocytes are preferentially recruited to the ileum in Crohn's disease

BACKGROUND AND AIMS: Chemokine receptors are key determinants of leucocyte trafficking. While the chemokine receptor CCR9 and its chemokine ligand CCL25 (TECK) mediate lymphocyte homing to the healthy small intestine, the chemokine receptors important for recruitment during intestinal inflammation are undefined. Animal studies have suggested potential roles for CCR2 and CCR5 in inflammatory bowel disease (IBD). The aim of this study was to understand the role of CCR2 in human IBD. METHODS: Resections of ileum or colon were obtained from patients undergoing surgery for small bowel Crohn's disease (SBCD; n = 10), Crohn's colitis (n = 5), ulcerative colitis (n = 6), and non-IBD related conditions (control ileum n = 11; control colon n = 11). Expression of CCR2 by lamina propria lymphocytes (LPLs) was determined by both flow cytometry and immunohistochemistry. As a functional correlate, chemotaxis assays using the CCR2 ligand, CCL2 (MCP-1), were performed. Expression of CCR2 by peripheral blood lymphocytes was determined by flow cytometry. RESULTS: There were greater than 30-fold more CCR2(+) LPLs in SBCD than in control ileum (29.3% (19.9-55.1) v 0.9% (0.4-11.5); p = 0.0007). Specifically, CCR2(+)CD4(+) LPLs were increased (p = 0.002) whereas CCR2(+)CD8(+) LPLs were not. Increased expression included both memory (CD45RO(+); p = 0.005) and na?ve (CD45RO(-); p = 0.01) CCR2(+) populations. The increase in CCR2(+) LPLs in SBCD was confirmed by both immunohistochemistry (p = 0.0002) and enhanced chemotactic responses to CCL2. CCR2 expression was not increased in the peripheral blood of patients with SBCD, suggesting ongoing recruitment of the CCR2(+) population to the ileum. In contrast with SBCD, there was no significant increase in CCR2(+) LPLs in Crohn's colitis or ulcerative colitis samples. CONCLUSIONS: The chemokine receptor CCR2 appears to be an important contributor to accumulation of CD4(+) T lymphocytes in the ileum in small bowel Crohn's disease. Blockade of CCR2 may provide a novel therapeutic alternative.     

Role of EUS and EUS-guided FNA in the diagnosis of symptomatic rectosigmoid endometriosis

BACKGROUND: Rectosigmoid endometriosis is an underrecognized cause of GI symptoms in women. Pelvic magnetic resonance imaging and CT have a low sensitivity in making this diagnosis. The role of EUS and EUS-guided FNA (EUS-FNA) in the diagnosis of rectosigmoid endometriosis in symptomatic patients is not well studied. METHODS: A review of medical records identified 5 women who were diagnosed with rectosigmoid endometriosis by EUS and EUS-FNA over a period of 1 year. OBSERVATIONS: Five women with nonspecific GI complaints underwent EUS examination of a rectosigmoid subepithelial mass found on colonoscopy. EUS revealed a hypoechoic lesion infiltrating the muscularis propria and the serosa of the rectal wall, and extending outside the rectal wall, findings consistent with rectosigmoid endometriosis. This diagnosis was confirmed by EUS-FNA, surgical exploration, and/or the patient's clinical course. CONCLUSIONS: EUS and EUS-FNA are noninvasive, sensitive techniques for the diagnosis of rectosigmoid endometriosis in symptomatic patients.     

Ileal polyposis as manifestation of neurofibromatosis syndrome.

A 13-year-old girl presented with features of intestinal obstruction. At surgery, the terminal 25 cm of ileum, which was resected along with the right colon, showed plexiform neurofibromatosis of the serosa and mesentery, hyperplastic submucosal and myenteric nerve plexuses and proliferation of neural tissue in the lamina propria, which manifested as diffuse polyposis of the ileal mucosa. The patient had a single inconspicuous external neurofibroma and a few café-au-lait spots on the back.     

Acute small bowel obstruction caused by endometriosis：A case report and review of the literature

Gastrointestinal involvement of endometriosis has been found in 3%-37% of menstruating women and exclusive localization on the ileum is very rare（1%-7%）.Endometriosis of the distal ileum is an infrequent cause of intestinal obstruction,ranging from 7% to 23% of all cases with intestinal involvement.We report a case in which endometrial infiltration of the small bowel caused acute obstruction requiring emergency surgery,in a woman whose symptoms were not related to menses.Histology of the resected specimen showed that endometriosis was mainly prevalent in the muscularis propria and submucosa and that the mucosa was not ulcerated but had inflammation and glandular alteration.Endometrial lymph node involvement,with a cystic glandular pattern was also detected.     

Epithelial permeability to proteins in the noninflamed ileum of Crohn's disease?

BACKGROUND & AIMS: Crohn's disease (CD) is associated with a disturbed intestinal barrier. Permeability studies have focused on inert molecules, but little is known about transepithelial transport of macromolecules with antigenic potential in humans. The aim of this study was to quantify permeation and to characterize passage routes for macromolecules in ileal mucosa in CD. METHODS: Noninflamed and inflamed ileal mucosa specimens from patients with CD (n = 12) and ileal specimens from patients with colon cancer (n = 7) were studied regarding transmucosal permeation of ovalbumin, dextran (mol wt, 40,000), and 51Cr-EDTA for 90 minutes in vitro in Ussing chambers. Transepithelial passage routes for fluorescent ovalbumin and dextran 40,000 were investigated by confocal microscopy. RESULTS: Noninflamed ileum from CD patients showed increased permeation of ovalbumin compared with ileum from colon cancer patients (P < 0.05). Dextran permeation was equal in the three groups, whereas 51Cr-EDTA permeability was increased in inflamed ileum. Ovalbumin passed both transcellularly and paracellularly, but dextran followed a strictly paracellular route. Both markers were subsequently endocytosed by cells of the lamina propria. CONCLUSIONS: Noninflamed ileal mucosa from patients with CD shows increased epithelial permeability to ovalbumin, probably by augmented transcytosis. This increase in antigen load to the lamina propria could be an initiating pathogenic event in CD.     

Role of colonoscopy in the diagnostic work-up of bowel endometriosis

AIM:To evaluate the accuracy of colonoscopy for the prediction of intestinal involvement in deep pelvic endometriosis.METHODS:This prospective observational study was performed between September 2011 and July 2014.Only women with both a clinical and imaging diagnosis of deep pelvic endometriosis were included.The study was approved by the local ethics committee and written informed consent was obtained in all cases.Both colonoscopy and laparoscopy were performed by expert surgeons with a high level of expertise with these techniques.Laparoscopy was performed within4 wk of colonoscopic examination.All hypothetical colonoscopy findings(eccentric wall thickening with or without surface nodularities and polypoid lesions with or without surface nodularities of endometriosis)were compared with laparoscopic and histological findings.We calculated the sensitivity,specificity,positive predictive value and negative predictive value for the presence of colonoscopic findings of intestinal endometriosis.RESULTS:A total of 174 consecutive women aged between 21-42 years with a diagnosis of deep pelvic endometriosis who underwent colonoscopy andsurgical intervention were included in our analysis.In 76 of the women(43.6%),intestinal endometrial implants were found at surgery and histopathological examination.Specifically,38 of the 76 lesions(50%)were characterized by the presence of serosal bowel nodules;28 of the 76 lesions(36.8%)reached the muscularis layer;8 of the 76 lesions(10.5%)reached the submucosa;and 2 of the 76 lesions(2.6%)reached the mucosa.Colonoscopic findings suggestive of intestinal endometriosis were detected in 7 of the174(4%)examinations.Colonoscopy failed to diagnose intestinal endometriosis in 70 of the 76 women(92.1%).A colonoscopic diagnosis of endometriosis was obtained in all cases of mucosal involvement,in 3of 8 cases(37.5%)of submucosal involvement,in no cases of muscularis layer involvement and in 1 of 38cases(2.6%)of serosa involvement.The sensitivity,specificity,positive predictive and negative predictive values of colonoscopy for the diagnosis of intestinal endometriosis were 7%,98%,85%and 58%,respectively.CONCLUSION:Being an invasive procedure,colonoscopy should not be routinely performed in the diagnostic work-up of bowel endometriosis.     

Influence of inflammatory bowel disease on intestinal microflora.

The microflora of the jejunum, ileum, and colon has been studied from operative samples in Crohn's disease (n = 30), ulcerative colitis (n = 15), and controls (n = 40). There was no significant difference in the flora of patients with ulcerative colitis compared with controls. In Crohn's disease there was a significant increase in E. coli (P less than 0.001) and B. fragilis (P less than 0.001) in the ileum and of E. coli (P less than 0.001) and lactobacilli (P less than 0.01) in the colon. The abnormal ileal flora in Crohn's disease was unrelated to serological evidence of disease activity (indices: ESR, serum albumin, serum seromucoids), diameter of the ileum, or excision of the ileocaecal valve. The abnormal colonic flora in Crohn's disease was not related to presence of macroscopic colitis.     

Augmented increase in tight junction permeability by luminal stimuli in the non-inflamed ileum of Crohn's disease

BACKGROUND: Crohn's disease is associated with deranged intestinal permeability in vivo, suggesting dysfunction of tight junctions. The luminal contents are important for development of neoinflammation following resection. Regulation of tight junctions by luminal factors has not previously been studied in Crohn's disease. AIMS: The aim of the study was to investigate the effects of a luminal stimulus, known to affect tight junctions, on the distal ileum in patients with Crohn's disease. PATIENTS: Surgical specimens from the distal ileum of patients with Crohn's disease (n=12) were studied, and ileal specimens from colon cancer patients (n=13) served as controls. METHODS: Mucosal permeability to 51Cr-EDTA and electrical resistance were studied in Ussing chambers during luminal exposure to sodium caprate (a constituent of milk fat, affecting tight junctions) or to buffer only. The mechanisms involved were studied by mucosal ATP levels, and by electron and confocal microscopy. RESULTS: Baseline permeability was the same in non-inflamed ileum of Crohn's disease and controls. Sodium caprate induced a rapid increase in paracellular permeability--that is, increased permeation of 51Cr-EDTA and decreased electrical resistance--which was more pronounced in non-inflamed ileum of Crohn's disease, and electron microscopy showed dilatations within the tight junctions. Moreover, sodium caprate induced disassembly of perijunctional filamentous actin was more pronounced in Crohn's disease mucosa. Mucosal permeability changes were accompanied by mitochondrial swelling and a fall in epithelial ATP content, suggesting uncoupling of oxidative phosphorylation. CONCLUSIONS: The tight junctions in the non-inflamed distal ileum of Crohn's disease were more reactive to luminal stimuli, possibly mediated via disturbed cytoskeletal contractility. This could contribute to the development of mucosal neoinflammation in Crohn's disease.     

Small bowel endometriosis masquerading as regional enteritis

Summary A 35-year-old female presented with recurrent right lower quadrant pain, nausea, and vomiting. She was afebrile with diffuse abdominal tenderness. Plain x-ray of abdomen revealed small bowel obstruction. A barium x-ray of the small bowel showed stricture of the terminal ileum. A CT scan of the abdomen showed a 6-cm mass in right lower quadrant. She was empirically managed as having Crohn's disease. She underwent laparotomy after failure of medical management with high-dose steroids. There was ulceration and narrowing of terminal ileum. Frozen sections revealed endometriosis. Ileocecectomy was performed. Histopathology of resected specimen confirmed the diagnosis of endometriosis, and there was no evidence of chronic inflammatory bowel disease or neoplasia. Ileal endometriosis should be considered in the differential diagnosis of Crohn's disease in menstruating females presenting with perimenstrual symptoms.     

Renal amyloidosis as initial clinical manifestation of Crohn's disease

We present a 20-year-old man with oligosymptomatic Crohn's disease of the terminal ileum complicated by chronic renal insufficiency due to secondary amyloidosis. Definitive diagnosis of Crohn's disease was established 7 years after the onset of the renal disease as a result of symptoms of intestinal obstruction requiring surgery. The affected colonic segment was removed and histopathological examination of the surgical specimen confirmed the diagnosis of Crohn's disease without identifying intestinal amyloidosis. Despite the surgical intervention and the absence of recurrence of inflammatory bowel disease, renal insufficiency steadily progressed, and the patient required kidney transplantation. The patient's subsequent course was excellent and no recurrence of intestinal or renal disease was observed after 8 years of follow-up.     

Neurochemical coding in the small intestine of patients with Crohn''s disease.

BACKGROUND: There have been conflicting results regarding the effect of Crohn's disease on the neurochemical composition of the enteric nervous system. AIMS: To examine the effect of Crohn's disease on the neurochemical composition of enteric nerve fibres and cell bodies using whole mount preparations of human ileum. METHODS: Whole wall ileum from seven normal subjects and nine patients with Crohn's disease was used to investigate the neurochemical composition of neurones and nerve fibres in the myenteric plexus, circular muscle, and serosa layer of ileum using immunohistochemical techniques. RESULTS: Increased tyrosine hydroxylase, 5-hydroxytryptamine, and neuropeptide Y immunoreactivity was exclusively seen in the myenteric plexus. There was increased neurofilament immunoreactivity in the myenteric plexus and nerve fibres of the circular muscle layer, and thick bundles of immunoreactive nerve fibres in the serosa layer. Increased vasoactive intestinal polypeptide, nitric oxide synthase, and pituitary adenylate cyclase activating peptide immunoreactivity was seen in the myenteric plexus and nerve fibres of the circular muscle layer, and aggregates of inflammatory cells in the serosa layer of the afflicted segment of Crohn's ileum. In addition, there was a chaotic display of nerve fibres containing some of the neuroactive substances with a high frequency of enlarged varicosities in the myenteric ganglia and/or nerve fibres of the circular muscle layer of Crohn's ileum. CONCLUSION: Results show quantitative as well as qualitative changes in the neurochemical composition of enteric nerve fibres and nerve cell bodies of Crohn's ileum. These changes and the presence of nitric oxide synthase and peptides immunoreactive inflammatory cells in the serosa layer suggest that nerve-immune interactions may have a significant role in the process of the inflammatory changes seen in Crohn's ileitis.     

Endoscopy and magnetic resonance imaging in patients with Crohn's disease: a retrospective single-centre comparative study

OBJECTIVE: There is ongoing debate about which imaging modality is best for patients with inflammatory bowel diseases. Magnetic resonance imaging (MRI) has been successfully used to evaluate the jejunum and the ileum. Because virtual colonoscopy by MRI requires bowel cleansing and/or rectal filling, endoscopy is preferred for assessment of the colon. However, hydro-MRI without special bowel preparation may be sufficient as a diagnostic tool if specifically targeted in the course of a known disease. The aim of this study was retrospectively to assess the correlation of endoscopy, histology and MRI findings for the terminal ileum and the colon in a cohort of patients with Crohn's disease. MATERIAL AND METHODS: In all, 60 patients with a confirmed diagnosis of Crohn's disease were included in the study. Here, 412 anatomical segments of the colon were analysed by MRI, 401 by endoscopy and 374 by histology. RESULTS: Presence or absence of inflammation was concordantly diagnosed in 310 segments (77.3%). The highest concordance was found for the terminal ileum and, in patients with previous surgery, the anastomosis. Sensitivity and specificity for MRI versus endoscopy, MRI versus histology and endoscopy versus histology were 64.4%/81.1%, 62.1%/86.2% and 78.2%/80.3%, respectively. CONCLUSIONS: In a retrospective analysis of patients with Crohn's disease, hydro-MRI assessment of inflammation in anatomical segments of the colon reaches acceptable concordance rates with endoscopy and histology without prior preparation of the bowel. The data justify a prospective controlled trial.     

Natural history of recurrent Crohn''s disease at the ileocolonic anastomosis after curative surgery

An endoscopical and histological study was carried out in 114 patients, treated by 'curative' resection of the terminal ileum and part of the colon for Crohn's disease, in order to study the natural history of recurrent Crohn's disease. The recurrence rate of Crohn's disease in patients examined within one year of the operation was 72%. This figure did not differ significantly from that in patients examined one to three years or three to 10 years after surgery (79% and 77% respectively). Recurrence was located in the neoterminal ileum and at the anastomosis in 88% of the patients. Early endoscopic signs of recurrence were small aphthous ulcers in the neoterminal ileum. Ileal biopsies at this stage showed an important inflammatory cell infiltrate of the lamina propria with numerous eosinophils and fusion and blunting of the villi. More advanced lesions observed in patients examined one to three years after surgery, consisted of larger, often serpiginous ulcerations and nodular thickening of folds. In patients examined three to 10 years after the operation, the anastomosis was frequently stenosed and rigid, with large ulcers extending from the stenosis into the colon. Mucosal granulomas may be found in normal appearing mucosa as well as in the obviously inflamed mucosa surrounding the ulcers. These studies suggest that recurrence of Crohn's disease almost always develops in the first year after the operation. Significant endoscopic lesions may be present without clinical symptoms, particularly in the earlier stages of the disease.     

Ileal and colonic fatty acid profiles in patients with active Crohn's disease.

In patients with active Crohn's disease and in a control group the fatty acid profiles in the whole lipid fraction of ileal and colonic mucosal biopsy specimens were determined by capillary gas chromatography. The biopsy specimens in Crohn's disease patients were taken from the inflamed terminal ileum as well as from the inflamed and macroscopically normal colon. Compared with controls the fatty acid distribution in the inflamed ileal mucosa was significantly characterised by (a) a decrease of 18:2 n6 and 18:3 n3 accompanied by a substantial increase of the highly polyunsaturated fatty acids 20:4 n6, 22:4 n6, and 22:6 n3 and (b) a higher unsaturation index of total fatty acids compared with controls. These changes were similar in the inflamed colon. Additionally, both the inflamed and the macroscopically normal colonic mucosa showed an increase of saturated (18:0) and a decrease of monounsaturated fatty acids (18:1 n9). Fatty acid profiles of ileum and colon showed side variations in controls, but not in the Crohn's disease group. These data suggest that in Crohn's disease changes in the distribution of polyunsaturated fatty acids seem to be the general feature of inflamed mucosa in small and large intestine. Results further suggest that colonic fatty acid metabolism in Crohn's disease is altered by degrees, showing changes in saturated and monounsaturated fatty acids as an additional, primary event.     

The clinical significance of focal enhanced gastritis in adults with isolated ileitis of the terminal ileum

BACKGROUND: Isolated chronic ileitis in the terminal ileum, without accompanying chronic colitis, is not an uncommon finding present in biopsy specimens from patients being evaluated for chronic diarrhea. Among the many entities that should be included in the differential diagnosis are Crohn's disease and nonsteroidal antiinflammatory drugs (NSAIDs)-induced enterocolitis. In high-prevalence Crohn's disease populations, focal enhanced or active gastritis (FEG) may be a good predictor of Crohn's disease; however, this criterion may not apply in a general clinical setting. Our goal was to determine if FEG is a pathological marker of Crohn's disease in patients with isolated chronic ileitis in the terminal ileum. METHODS: We examined 46 consecutive cases of isolated chronic ileitis with concurrent stomach biopsies. These patients did not have evidence or previous history of inflammatory bowel disease. The diagnostic criteria of chronic ileitis included crypt distortion and inflammation, plasmacytosis in the lamina propria, ulceration, and/or pyloric gland metaplasia. RESULTS: Of the 46 cases reviewed, 25 (54%) cases were diagnosed with Crohn's disease later, confirmed by clinical manifestations and/or biopsies with a follow-up of up to 4 years. The stomach biopsies of these patients were either normal or demonstrated a spectrum of histological findings, including FEG, chronic gastritis with or without Helicobacter pylori organisms, chemical gastropathy, and normal tissues. FEG was more commonly present in Crohn's disease patients (36%) than in non-Crohn's disease patients (5%) (P < 0.01). CONCLUSIONS: The presence of FEG is a good indicator for the diagnosis of Crohn's disease in adult patients with isolated chronic ileitis.     

小肠腔内超声在小肠疾病诊断中的初步临床研究

目的评估小肠腔内超声（ISIU）检查对小肠疾病诊断的价值及可行性和安全性.方法对2004年6月至2005年2月间11例因不明原因慢性腹泻、隐性出血及腹痛怀疑小肠疾病的患者行双气囊小肠镜（DBE）检查,发现局部病灶或病变肠段后,经DBE活检孔道插入特制超声小探头进行ISIU探查,取得正常肠壁以及局部病灶或病变肠段ISIU影像图并对其进行分析.结果11例患者中,DBE发现阳性病灶并行ISIU检查9例,其中成功完成ISIU探查者8例,平均用时20min.提示侵犯浆膜层的恶性肿瘤2例（术后病理证实）,表现为小肠绒毛层消失,其余小肠壁层次破坏,代之以不均低回声区域;小肠间质瘤1例（起源于肌层,术后病理证实）,表现为边界清晰的均匀低回声区域内伴有部分高回声;肠壁黏膜层来源息肉1例（活检证实）,影像特征为均匀高回声区域;早期克罗恩病2例,表现为小肠绒毛层消失,其余肠壁层次模糊伴全层小肠壁增厚;淋巴管瘤1例,表现为内伴管样结构的均匀中低回声区域;对于DBE发现的小肠毛细血管扩张症患者,ISIU影像不能明确诊断.另有1例DBE发现增殖性病灶的患者,由于病灶位置较深,位于空回肠交界部,经口DBE中超声小探头不能经由内镜活检孔道送出导致探查失败.所有ISIU探查均未发现相关的并发症.结论ISIU是一种安全有效的辅助检查手段,尤其对小肠黏膜下肿瘤的性质鉴别、早期克罗恩病的诊断及肿瘤侵犯深度的判断存在明显优势.     

Chediak-Higashi syndrome with intestinal complication. Report of a case

A 21-year-old woman with Chediak-Higashi syndrome developed intestinal lesions similar to those of Crohn's disease. Radiographic findings showed a stenotic lesion with multiple nodular changes in the ascending colon and cecum, and a longitudinal ulceration in the terminal ileum. In addition, multiple oval-shaped ulcerations were seen between the terminal ileum and descending colon. However, serial histological examinations revealed only an infiltration of lymphoid cells, which was a nonspecific finding. Though there are few reports of gastrointestinal involvement in Chediak-Higashi syndrome, such an intestinal complication may develop in patients long surviving with this disease.     

Application of the Vienna Classification for Crohn's disease to a single clinician database of 877 patients.

An international working party at the World Congress of Gastroenterology held in Vienna, Austria from September 6 to 11, 1998 defined a classification for Crohn's disease based on patient age at diagnosis (eg, less than 40 years of age, 40 years of age or older), disease location (eg, terminal ileum, colon, ileocolon or upper gastrointestinal tract) and behaviour (eg, stricturing, penetrating). Disease location in the upper gastrointestinal tract was defined by disease being present proximal to the terminal ileum, regardless of terminal ileal or colon involvement. A 20-year, single clinician database of 877 patients from a university campus hospital was used, and comprised 492 women (56.1%) and 385 men (43.9%). Of these patients, 740 (84.4%) were diagnosed before age 40 years and 137 (15.6%) were diagnosed by 40 years of age or older. Disease was located in the terminal ileum alone in 222 patients (25.3%), colon alone in 238 patients (27.2%) and ileocolon in 304 patients (34.6%). Another 113 patients (13.1%) had disease in the upper gastrointestinal tract, usually with disease also in the terminal ileum (23 patients), colon (12 patients) or ileocolon (71 patients). Only seven of 877 patients had disease located in the upper gastrointestinal tract alone with no distal disease. Disease behaviour could be classified as nonstricturing and nonpenetrating in 256 patients (29.2%), stricturing in 294 patients (33.6%) and penetrating in 327 patients (37.2%). Of the 877 patients with Crohn's disease, 837 were white, 38 were Asian and two were black. In this tertiary care setting of a single clinician practice in a Canadian teaching hospital at the University of British Columbia, Crohn's disease predominantly affects women, and young adults with a high rate of stricturing and penetrating complications.