WEST AFRICAN JOURNAL OF MEDICINE CASE REPORT
Ileal Endometriosis Presenting as Acute Small Intestinal Obstruction: A Case Report Iléales endométriose présentant comme des petites Occlusion intestinale aiguë: A Case Report O. I. Alatise*, D. Sabageh†, S. O. Ogunniyi‡, O. O. Olaofe† ABSTRACT BACKGROUND: The gastrointestinal tract is the most common site of extrapelvic endometriosis, affecting 5–15% of women with pelvic endometriosis. Among women with intestinal endometriosis, rectum and sigmoid colon are the most commonly involved areas. Terminal ileum is rarely involved in endometriosis. Similarly, bowel endometriosis is an uncommon cause of intestinal obstruction. OBJECTIVE: To present a rare occurrence of ileal endometriosis presenting with acute small intestinal obstruction. METHODS: A 34-year-old woman presented with a two-month history of intermittent, colicky abdominal pain which became more intense with associated vomiting of three days prior to presentation. Besides full clinical evaluation, she had other investigations including abdominal X-rays, ultrasonography, ECG, and echocardiography. The results of these informed the need for myomectomy. RESULTS: Besides the abdominal pain, the patient also complained of a supra-pubic swelling and menorrhagia. Physical examination showed an incisional hernia, and a suprapubic mass. The results of evaluation were consistent with incisional hernia complicated by imminent adhesive intestinal obstruction. She had had secondary infertility and has had myomectomy due to copious menstrual flow which was complicated with incisional hernia. She was managed initially conservatively for adhesive small bowel obstruction which failed. She had exploratory laparotomy with small intestinal resection and end to end anastomosis. Histopathology of the resected mass revealed ileal endometriosis. CONCLUSION: This report highlights the importance of histopathological assessment of resected specimens in the diagnosis of intestinal obstruction due to intestinal endometriosis. This disease should, therefore, be considered during the evaluation of women of child bearing age. WAJM 2010; 29(5): 352–345.
RÉSUMÉ
Keywords: Endometriosis, Ileum, Intestinal Obstruction, Diagnosis
Mots-clés: l’endométriose, l’iléon, une occlusion intestinale, Diagnostic.
CONTEXTE: Le tube digestif est la localisation la plus fréquente de l’endométriose extrapelvienne, affectant 5-15% des femmes atteintes d’endométriose pelvienne. Chez les femmes atteintes d’endométriose intestinale, du rectum et du côlon sigmoïde sont les zones les plus communément impliquées. iléon terminal est rarement impliqué dans l’endométriose. De même, l’endométriose intestinale est une cause rare d’occlusion intestinale. OBJECTIF: présenter une rareté de l’endométriose iléale présentant une occlusion aiguë de l’intestin grêle. MÉTHODES: Une femme de 34 ans s’est présenté avec une histoire de deux mois des intermittents, des douleurs abdominales de type colique qui est devenue plus intense avec vomissements associés de trois jours avant la présentation. Outre l’évaluation clinique complète, elle avait d’autres enquêtes, y compris les radiographies abdominales, échographie, électrocardiogramme, une échocardiographie. Les résultats de ces informés de la nécessité pour la myomectomie. RÉSULTATS: Outre les douleurs abdominales, la patiente se plaint également d’une sus-pubienne gonflement et la ménorragie. L’examen physique a montré une éventration, et une masse sus-pubienne. Les résultats de l’évaluation étaient compatibles avec éventration compliquée par un adhésif imminente occlusion intestinale. Elle avait eu l’infertilité secondaire et a eu une myomectomie en raison de flux menstruel abondant qui a été compliquée avec éventration. Elle a été gérée d’abord prudente, pour une occlusion intestinale adhésif petite qui a échoué. Elle avait une laparotomie exploratrice avec résection de l’intestin grêle et une anastomose de bout en bout. Histopathologie de la masse réséquée a révélé l’endométriose iléale. CONCLUSION: Ce rapport souligne l’importance de l’évaluation histopathologique des pièces de résection chirurgicale dans le diagnostic d’occlusion intestinale due à l’endométriose intestinale. Cette maladie doit donc être considéré lors de l’évaluation des femmes en âge de procréer. WAJM 2010; 29 (5): 352–345.
Departments of *Surgery, College of Health Sciences, Obafemi Awolowo University/Surgery, † Morbid Anatomy, Obafemi Awolowo University Teaching Hospitals Complex. ‡ Obstetrics & Gynaecology, College of Health Sciences, Obafemi Awolowo University/ Department of Obstetrics & Gynaecology, Obafemi Awolowo University Teaching Hospital Complex, Ile-Ife. Correspondence: Dr Olusegun Isaac Alatise, Department of Surgery, Obafemi Awolowo University Teaching Hospital Complex, PMB 5538, Ile-Ife, Osun State. Nigeria. Email address:
[email protected] Mobile : +234-803-385-9387 Abbreviation: ECG, Electrocardiography; GI, Gastroinstestinal.
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INTRODUCTION Endometriosis is a common, benign, estrogen-dependent, chronic gyneacological disorder associated with pelvic pain and infertility.1-3 It is caused by the growth of endometrium outside the uterine cavity or myometrium (endometriotic implants). Endometriosis may be intraperitoneal or extraperitoneal. In decreasing order of frequency, the intra-peritoneal locations are ovaries (30%), uterosacral and large ligaments (18%–24%), fallopian tubes (20%), pelvic peritoneum, pouch of Douglas, and gastrointestinal (GI) tract. Extraperitoneal locations include cervical portion, vagina and rectovaginal septum, round ligament and inguinal hernia sac, navel, abdominal scars after gynaecological surgery (1.5%) and caesarian section. Very rarely it can also be found in the lungs, pericardium, urinary system, skin, and the central nervous system. 1–4 Distal ileum endometriosis is an uncommon cause of intestinal obstruction. Diagnosis of intestinal obstruction becomes much more difficult in a patient who has had previous surgery. The purpose of this communication is to present a 34-yearold woman with ileal endometriosis presenting with intestinal obstruction diagnosed on histopathology. Case Report A 34-year-old para1+1 woman living in one of the villages in south-western Nigeria was referred to out general surgery clinic after being diagnosed of secondary infertility at the Gyneacology Clinic. She was referred on account of a two-month history of intermittent and colicky abdominal pain, which became more intense and was associated with vomiting three days before presenting at our clinic. She also complained of a supra-pubic swelling as well as copious menstrual flow associated with a moderately severe dysmenorrhoea. Three years before these present symptoms, she had had myomectomy at a private hospital which was complicated by an incisional hernia. Physical examination revealed a young woman in painful distress. She was neither febrile nor jaundiced. She was not pale, neither was she cyanosed. There was no pitting pedal edema.
Intestinal Obstruction from Ileal Endometriosis
However, her blood pressure was recorded as 210/140 mmHg. Abdominal examination revealed positive cough impulse and a defect on the anterior abdominal wall which measured about 8cm in the widest diameter. There was tenderness on the hernia sac. There was also a firm a non tender mass lying over the pubis symphysis which measured 6cmX4cm. Vaginal and rectal examinations did not reveal any significant finding. Laboratory investigations showed a normal FBC, urinalysis, as well as, urea and electrolytes. Abdominal X-ray showed dilation of the small bowel. No intra-peritoneal mass or fluid collection was seen on abdominal USS. ECG revealed prolonged QT interval and left ventricular hypertrophy. Echocardiography showed normal left ventricular systolic function and left ventricular diastolic dysfunction with impaired relaxation. An assessment of incisional hernia with imminent adhesive intestinal obstruction was, therefore, made. She was managed conservatively for 48 hours with nil per oral, nasogastric intubation, monitoring of vital signs. She later had explorative laparatomy during which interloop adhesions were seen and an intermural mass measuring 6 x 4 x 4cm, in the ileum about 20cm from the ileocecal valve were discovered. The mass was obstructing the lumen. The proximal ileum was dilated. The left ovary was enlarged (6cm x 5cm x 4cm) and hard to touch. Other intra-abdominal organs were normal. She had excision of the pubic mass, adhesiolysis, left oophorectomy, ileal resection and anastomosis while an incisional herniorrhaphy also done. The post-operative course was uneventful and the patient left the hospital after 11 days in the hospital. Histology of the resected specimen showed endometriosis involving the ileum and causing a stricture. The bowel wall was infiltrated with hemosiderin laden macrophages, lymphocyte, neutrophils and plasma cell. Endometriosis was mainly prevalent in the muscularis propria and submucosa. The mucosal involvement showed inflammation and glandular alteration with discontinuity of the epithelium (Figure 1 and 2). Sections of suprapubic mass
showed fibrocollagenous tissue within which were seen a number of endometrial gland (Figures 3 and 4). Histology evidence of corpus lutein cyst was seen in the ovary.
Fig. 1: Endometriosis in Ileal Tissue
Fig. 2: Endometriosis in Ileal Tissue extending to the Mucosa DISCUSSION Gastrointestinal involvement by endometriosis has been found in 3%–37% of women, most commonly in the sigmoid colon, rectum and small bowel.1 The majority of small bowel involvement occurs in the distal ileum, especially the last l0cm of the terminal ileum. 5 Commonly, involvement occurs in the sub-serosal part of the bowel, and extension may then occur towards the luminal surface.1,4,6 However, as seen in our case, the mucosa is rarely involved. The involvement may be due to necrosis of the mucosa from the pressure exerted by the submucosa mass. While there have been a number of theories postulated to explain the origin and development of endometriosis, Sampson’s theory of retrograde spread is the most commonly accepted.1–2 This theory maintains that endometrial cells flow backward through the fallopian tubes into the peritoneal cavity during
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menstruation, subsequently seeding various surfaces. Coelomic metaplasia, vascular dissemination, stem cell differentiation and autoimmune disease are other proposed major theories.2,6,7 None of these theories seem plausible enough to explain all the clinical scenarios of endometriosis seen. Intestinal endometriosis often presents as obstruction, but rarely can present with bleeding.5–6 Obstruction can be caused by the obstruction of the lumen by the mass or adhesion. Invasion of the muscle layer of the ileum by the endometrial tissue is accompanied by fibrosis within the submucosal layer and adhesions around the serosal layer5-8. Sometimes the adhesion is caused by the ectopic endometrial tissue adhering to the peritoneum and bowel wall. As a result of cyclic bleeding in the ectopic endometrial tissue, sloughing and proliferation, inflammation and fibrosis are seen around the lesion. The fibrosis and adhesion are due to release of fibrogenic ferrous material from degraded blood, occurring with each menstrual flow from the ectopic endometrial tissue.4 The multiple adhesions seen in our case could be due to endometriosis and/or the previous surgery. Depending on the degree of scarring, ileal endometriosis can present with acute or chronic intestinal obstruction. Most reported cases of ileal endometriosis are associated with acute episodes of obstruction. 1–7 Chronic intestinal obstruction from ileal endometriosis is usually periodic and cyclical with each episode occurring very close to or during menstruation. With time the symptoms become more permanent. It is difficult to establish a preoperative diagnosis of ileal endometriosis, because symptoms can mimic a wide spectrum of diseases, including irritable bowel syndrome, infections, bowel infestation especially amoebiasis and neoplasm. 6,8 Just like previous reports have shown, it was not possible to establish a timely and accurate preoperative diagnosis, in this case, especially with reference to the vague presenting symptoms which also occur with other causes of bowel obstruction.3 A diagnosis of adhesive intestinal obstruction may readily be plausible 354
Intestinal Obstruction from Ileal Endometriosis
since adhesive bands have been documented to be the most common cause of intestinal obstruction in our centre.10 However, endometriosis of the small bowel should always be suspected in young, nulliparous patients or patients with secondary infertility with abdominal pain, in conjunction with signs of obstruction especially when there are associated disorders of the female genital tract.11 In some centers, a variety of radiological diagnostic procedures have been found useful in making a diagnosis of intestinal endometriosis, although with widely variable sensitivity and specificity. These diagnostic procedures include endoluminal ultrasound scan, magnetic resonance imaging, and multislide computerized tomographic scan.12–14 The yield with these modalities increases when they are used in combination (especially for colorectal endometriosis). Serum interleukin-6 and CA-125 may also be useful in patient1 monitoring. Most diagnoses are made during histopathology of the specimen. Microscopic examination of the intestine involving endometriosis usually discloses an endometrial stroma and gland islands located between muscular fibres, subserosa and serosa. Normally, the mucosa is found to be intact, but in some cases, the endometriotic tissue reaches it in the form of small islands, which cause intestinal bleeding coincidental with the menstrual period. In our case, although intestinal obstruction was present and mucosal involvement demonstrated histologically, the patient did not present with any gastrointestinal bleeding. Endometrial glands formed of multifocally placed cuboidal epithelium and stroma spreading under the intestinal mucosa to the serosa was observed. The diagnosis is easily confirmed using immunohistochemical stains. Ideally, a panel of markers should be performed for optimal yield. These include CK7, CA125 and ER to which endometriotic glands show positivity.15–17 They, however, show negativity to CK20 and CEA.16 The surrounding intestinal glands, on the other hand, exhibit the opposite immunophenotype. Immunohistochemistry plays a useful role in the differentiation of endometriosis from intestinal adeno-
Fig. 3: Endometriotic Tissue within the Suprapubic Mass
Fig. 4: Endometriotic Tissue within the Suprapubic Mass carcinomas with endometroid differentiation especially in mucosal biopsies where the endometriotic glands are bare and are not surrounded by endometriotic stroma or show atypical nuclear changes.16 In our case, the diagnosis was relatively easy to arrive at because the specimen was a resection specimen while the lesion consisted of classical benign endometriotic glands and stroma. In the vast majority of cases, surgery remains the treatment of choice for intestinal endometriosis.5 However, asymptomatic incidental cases may require hormonal therapy with danazol or gonadotrophin-releasing hormone (GnRH) analogues.4 Surgical treatment is indicated in women who present with pain, hemorrhage, changes in bowel habits and intestinal obstruction.1–6 The treatment for small bowel endometriosis is surgical resection of the involved bowel (medical therapy is only a temporary measure). In conclusion, we report an unusual presentation of endometriosis characterised by abrupt onset of small bowel occlusion. The diagnosis of intestinal
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endometriosis can be a challenging clinical exercise especially when it is associated with symptoms not related to the female genital tract. This report, however, highlights the importance of the histopathological assessment of resected specimens in the diagnosis of intestinal endometriosis. We, therefore, advocate that this diagnosis should be considered during the evaluation of women of child bearing age. REFERENCES 1.
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