{"paper_id":"5382163e-c706-4337-ab6d-46f596cd1d71","body_text":"BioMed Central\nPage 1 of 4\n(page number not for citation purposes)\nJournal of Medical Case Reports\nOpen AccessCase report\nIntussusception of the appendix secondary to endometriosis: a case \nreport\nSamia Ijaz*, Surjit Lidder, Waria Mohamid, Martyn Carter and \nHilary Thompson\nAddress: Department of General Surgery, Lister Hospital, Coreys Mill Lane, Stevenage, Hertfordshire, SG1 4AB UK\nEmail: Samia Ijaz* - samiaijaz@hotmail.com; Surjit Lidder - surjitlidder@doctors.org.uk; Waria Mohamid - wariamohamid@hotmail.com; \nMartyn Carter - mjcarter@nhs.net; Hilary Thompson - hhthompson@nhs.net\n* Corresponding author    \nAbstract\nIntroduction: Intussusception of the appendix is an ex tremely rare condition that ranges from\npartial invagination of the appe ndix to involvement of the enti re colon. Endometriosis is an\nexceptionally rare cause of appendiceal intussusception and only very few cases have been reported\nin the literature to date.\nCase presentation: A 40 year-old woman presented to clin ic with a long history of lower\nabdominal pain, loose motions and painful, he avy periods. Subsequent  colonoscopy revealed\nsubmucosal endometriotic nodules in the sigmoid as well as a polyp thought to be arising from the\nappendix, which had inverted itself. She was refe rred to a colorectal surgeon because the polyp\ncould not be removed endoscopically despite seve ral attempts. At laparotomy, the appendix had\nintussuscepted but it was possible to reduce it and therefore a simple appendicectomy was carried\nout. On histology, there were widespread endometrial deposits within the wall of the appendix and\nthis was thought to be the basis for the intussusception.\nConclusion: Histological evidence of the lead point is of crucial importance in cases of appendiceal\nintussusception, in order to exclude an underly ing neoplastic process. Consequently, surgical\nresection is necessary either through an open  or a laparoscopic approach. Gastrointestinal\nendometriosis should be considered as a cause of appendiceal intussusception in post-menarchal\nwomen with episodic symptoms and proven disease.\nIntroduction\nIntussusception of the appendix is an extremely unusual\nclinical entity. A study by Collins [1] described an inci-\ndence of 0.01% based on 71,000 appendiceal specimens.\nThe condition ranges from partial invagination of the\nappendix to involvement of the whole colon where the\nappendix may protrude from the anus [2]. It occurs pre-\ndominantly in the first decade of life, with a 4:1 male to\nfemale ratio, and may be more common than tradition-\nally believed because transient appendiceal intussuscep-\ntion has been reported on barium enema in\nasymptomatic patients [3].\nThe coincidence of endometriosis and intussusception is\neven more rare with few cases reported in the literature.\nPublished: 22 January 2008\nJournal of Medical Case Reports 2008, 2:12 doi:10.1186/1752-1947-2-12\nReceived: 11 November 2007\nAccepted: 22 January 2008\nThis article is available from: http://www.jmedicalcasereports.com/content/2/1/12\n© 2008 Ijaz et al; licensee BioMed Central Ltd. \nThis is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), \nwhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.\n\nJournal of Medical Case Reports 2008, 2:12 http://www.jmedicalcasereports.com/content/2/1/12\nPage 2 of 4\n(page number not for citation purposes)\nCase presentation\nA 40-year-old woman presented to gastroenterology out-\npatients clinic with a several month history of right iliac\nfossa pain and loose motions. Apart from longstanding\ndysmenorrhoea and menorrhagia, she did not have any\nother symptoms. There was no past medical history to\nnote and no family history of endometriosis. A clinical\nexamination of the patient, including a full gynaecologi-\ncal examination, was within normal limits. Preliminary\ninvestigations revealed an iron deficiency anaemia with a\nhaemoglobin level of 11.1 g/dl, a mean corpuscular vol-\nume of 71 fl and a low ferritin level of 8.4 ng/ml. A colon-\noscopy was duly organised which showed a sessile 1 cm\npolyp in the caecum [see figure 1]. On biopsy, this proved\nto be a metaplastic polyp. A subsequent attempted\npolypectomy was unsuccessful so the patient was referred\nto a tertiary centre where another attempt at polypectomy\nwas carried out. At this point, the polyp looked to be aris-\ning from the appendix, which itself was inverted. In addi-\ntion, submucosal nodules in the sigmoid were noted and\nthese were thought to be endometrial in origin as the\npatient had a long history of painful and heavy periods.\nThe polyp was not removed and the patient was referred\nto the colorectal surgeons and gynaecologists for a possi-\nble right hemicolectomy, total abdominal hysterectomy\nand bilateral salpingo-oophorectomy.\nA preoperative CT scan of her abdomen and pelvis did not\nreveal any firm evidence of endometriosis and only noted\nsmall cysts on both ovaries.\nAt the time of the operation, the appendix had intussus-\ncepted and a simple appendicectomy, rather than a right\nhemicolectomy, was carried out in the absence of any\nother findings at laparotomy.\nOn histology, the wall of the appendix had widespread\nendometrial deposits [see Figures 2 and 3] and there was\nno evidence of malignancy. In addition, the cervix and fal-\nlopian tubes were within normal limits and the ovaries\nboth had multiple follicular cysts and germinal inclusion\ncysts and there were leiomyomas within the myometrium.\nDiscussion\nAppendiceal intussusception is uncommon and typically\nfound at the time of operation. An incidence rate of 0.01%\nhas been reported in the literature [1]. Usually associated\nwith males in the first decade, patients tend to present\nwith symptoms of vague colicky lower abdominal pain\nwith or without symptoms of small bowel obstruction.\nEndometriosis is defined as the proliferation and function\nof endometrial tissue outside the endometrial cavity. The\nreported incidence in pre-menopausal women is in the\norder of 8–15%. Although the disease classically involves\nthe pelvic organs and pelvic peritoneum, seeding has been\nobserved in surgical scars, around the umbilicus, in the\ninguinal canal, intestines, bladder, heart and lungs. The\nexact aetiology of endometriosis is unknown but there are\ntwo main theories on its pathogenesis. The transportation\ntheory presumes that endometrial cells are transported to\ndistant sites through surgical manipulation, menstrual\nshedding via the fallopian tubes or through lymphatic or\nvascular spread. Alternatively, the metaplastic theory sug-\ngests that embryonic coelomic mesothelium dedifferenti-\nates into endometrial tissue in response to inflammation\nor trauma [4,5]. The most common symptoms of\nendometriosis are dysmenorrhoea, pelvic pain and infer-\ntility but patients can also be asymptomatic.\nColonoscopy view of suspected caecal polypFigure 1\nColonoscopy view of suspected caecal polyp.\nLow power (5 × 10) view of caecal wall showing endometri-otic glands and stroma within the submucosaFigure 2\nLow power (5 × 10) view of caecal wall showing endometri-\notic glands and stroma within the submucosa. Haematoxylin \nand eosin stain.\n\n\nPublish with BioMed Central   and  every \nscientist can read your work free of charge\n\"BioMed Central will be the most significant development for \ndisseminating the results of biomedical research in our lifetime.\"\nSir Paul Nurse, Cancer Research UK\nYour research papers will be:\navailable free of charge to the entire biomedical community\npeer reviewed and published immediately upon acceptance\ncited in PubMed and archived on PubMed Central \nyours — you keep the copyright\nSubmit your manuscript here:\nhttp://www.biomedcentral.com/info/publishing_adv.asp\nBioMedcentral\nJournal of Medical Case Reports 2008, 2:12 http://www.jmedicalcasereports.com/content/2/1/12\nPage 3 of 4\n(page number not for citation purposes)\nThe incidence of gastrointestinal endometriosis varies\nbetween 3–37% of those women who have proven dis-\nease. The rectum and sigmoid colon are most commonly\ninvolved, followed by the rectovaginal septum, small\nintestine, caecum and appendix. It usually takes the form\nof asymptomatic, small, serosal deposits. Under cyclical\nhormonal influences these deposits may proliferate and\ninfiltrate the bowel wall. Cyclical haemorrhage from the\nendometrioma then leads to an intense, localised fibrosis\nwithin the bowel wall that can result in the formation of\nstrictures. In addition, serosal deposits can lead to the for-\nmation of adhesions between neighbouring pelvic struc-\ntures or bowel loops [6].\nAppendiceal endometriosis is usually asymptomatic.\nWhen symptomatic it frequently presents as appendicitis.\nAcute appendiceal inflammation arises due to partial or\ncomplete luminal occlusion by the endometrioma [6].\nAppendiceal intussusception secondary to endometriosis\nis extremely rare with fewer than 30 cases reported in the\nliterature during the last fifty years. Endometrial involve-\nment of the appendix is usually accompanied by chronic\nfibrosis, inflammation and hyperplasia or hypertrophy of\nthe muscularis propria. This hypertrophic segment serves\nas a lead point for hyperperistalsis hence making it prone\nto intussusception particularly when combined with a\nfully mobile appendix that has a wide proximal lumen\nand a fat free mesoappendix. CT abdominal scans may\ndemonstrate a soft tissue mass in the region of the cae-\ncum, although in this particular case the CT scan did not\npoint towards the diagnosis.\nConclusion\nAs in all cases of intussusception, the index of suspicion\nmust be high as 90% of all intussusceptions in adults are\ndue to an underlying neoplastic process. Intestinal\nendometriosis should be considered as a differential diag-\nnosis in post-menarchal women who present with epi-\nsodic gastrointestinal symptoms particularly in\nconjunction with gynaecological symptoms. The gold\nstandard in the investigation of similar cases would\nappear to be laparoscopy or laparotomy followed by sur-\ngical resection in order to obtain histological evidence of\nthe lead point.\nCompeting interests\nThe author(s) declare that they have no competing interests.\nAuthors' contributions\nAll of the named authors were involved in the preparation\nof this manuscript.\nConsent\nWritten informed consent was obtained from the patient\nfor publication of this case report and any accompanying\nimages. A copy of the written consent is available for\nreview by the Editor-in-Chief of this journal.\nAcknowledgements\nThe authors would like to express their thanks to both the gynaecology and \nradiology departments for their help in this case. No funding was required \nfor this study.\nReferences\n1. Collins D: Seventy one thousand human appendix specimens.\nA final report summarising forty years' study.   Am J Proctol\n1963, 14:356-381.\n2. Burghard F: Intussusception of the vermiform appendix, the\nintussusceptum protruding from the anus.  Br J Surj 1914, 1:721.\n3. Bachman AL, Clemett AR: Roentgen aspects of primary appen-\ndiceal intussusception.  Radiology 1971, 101:531-538.\n4. Igawa HH, Ohura T, Sugihara T, Hosokawa M, Kawamura K, Kaneko\nY: Umbilical endometriosis.  Ann Plast Surg 1992, 29:266.\n5. Hasegawa T, Yoshida K, Matsui K: Endometriosis of the appendix\nresulting in perfor ated appendicitis.   Case Rep Gastroenterol\n2007, 1:27-31.\n6. Cameron IC, Rogers S, Collins MC, Reed MWR: Intestinal\nendometriosis.  Int J Colorect Dis 1995, 10:83-86.\nLow power (5 × 10) view of appendix wall showing foci of endometriosis within the muscle layerFigure 3\nLow power (5 × 10) view of appendix wall showing foci of \nendometriosis within the muscle layer. Haematoxylin and \neosin stain.\nPublish with BioMed Central   and  every \nscientist can read your work free of charge\n\"BioMed Central will be the most significant development for \ndisseminating the results of biomedical research in our lifetime.\"\nSir Paul Nurse, Cancer Research UK\nYour research papers will be:\navailable free of charge to the entire biomedical community\npeer reviewed and published immediately upon acceptance\ncited in PubMed and archived on PubMed Central \nyours — you keep the copyright\nSubmit your manuscript here:\nhttp://www.biomedcentral.com/info/publishing_adv.asp\nBioMedcentral","source_license":"CC0","license_restricted":false}