Endometriosis of the appendix: Report of three cases

1. Introduction Endometriosis is a common condition that can affect up to 15% of women of childbearing age and 2–5% of post-menopausal women. This condition can lead to significant morbidity and is a frequent cause of surgical intervention for chronic pelvic pain. Endometriosis commonly involves the adnexa but can be present anywhere in the peritoneal cavity. Endometriosis involving the appendix is rare and preoperative diagnosis is difficult. In this paper we illustrate three different clinical presentations of endometriosis of the appendix and their subsequent management. 2. Case report 1 A 53-year-old postmenopausal female with known history of endometriosis presented to our clinic for screening colonoscopy. She also complained of mild right lower quadrant tenderness at Mc Burney’s point. Endoscopic findings were consistent with an appendiceal submucosal mass and invagination at the base of the appendix (Fig. 1). Biopsies of the mass were consistent with colonic mucosa with lymphoid aggregates. A computed tomograph (CT) of the abdomen and pelvis did not demonstrate any pelvic or abdominal abnormalities. The patient underwent a diagnostic laparoscopy. The appendix was tethered to the terminal ileum. There were several white plaques involving the terminal ileum. Because of the operative findings a laparoscopic ileocecectomy was performed. The specimen histopathology was consistent with endometriosis involving the appendix and the serosal layer of the ileum. The patient went on to make an uneventful recovery with resolution of her abdominal discomfort. 3. Case report 2 A 49-year-old female presented with a history of intermittent right lower quadrant pain for 4 years. She was evaluated with an abdominal CT, which demonstrated a dilated fluid filled appendix (Fig. 2). Optical colonoscopy was performed and demonstrated a 2 cm extrinsic mass adjacent to the appendiceal ostia. The mucosal surface was normal. She underwent a diagnostic laparoscopy. Intra-operatively, dense adhesions were encountered in the right lower quadrant. An appendectomy was performed. Her postoperative course was uneventful. Histopathologic examination demonstrated chronic appendicitis and endometriosis involving the serosa of the appendix. 4. Case report 3 A 55-year-old healthy female underwent a screening colonoscopy, significant for an inverted appendix (Fig. 3). Her past medical history was significant for laparoscopic hysterectomy for dysfunctional uterine bleeding. An endoscopic biopsy of the inverted appendix revealed normal mucosa. The patient underwent diagnostic laparoscopy. Intraoperatively, the appendix was absent at the confluence of the tenia. An ileocecectomy was performed. Her postoperative course was uneventful. When the specimen was opened, the entire appendix appeared inverted. Histologically, there was muscular hyperplasia with endometrial gland and stroma which consistent with endometriosis (Fig. 4). 5. Discussion Endometriosis is a condition characterized by the growth of the endometrial tissue outside the uterine cavity. It was initially described by von Rokitansky in 1860. The majority of endometrial implants are located in the dependent portions of the female pelvis. The most common sites of endometriosis involvement are the ovaries (54.9%), posterior broad ligament (35.2%), anterior cul-de-sac (34.6%), the posterior cul-de-sac (34.0%), and the uterosacral ligament (28.0%). Endometriosis is occasionally discovered in the umbilicus and in surgical scars, especially following Caesarean section. Rarely, it is seen in the kidney, lung, skin and diaphragm. Involvement of the gastrointestinal tract is reported to affect between 3% and 37% of patients with pelvic endometriosis. When endometriosis does involve the GI tract it commonly involves the recto-sigmoid (72%), the recto-vaginal septum (13%), small intestine (7%), cecum (3.6%) and the appendix (3%). The histological diagnosis of endometriosis is readily made when both endometrial glands and stroma are present outside of the uterus. The diagnosis can sometimes be difficult in long standing cases in which the tissue is obscured by secondary fibrosis. In the intestine, endometriosis usually involves the serosa and the subserosa. Sometimes the muscularis propria, the submucosa and the mucosa may be involved especially in symptomatic patients. Endometriosis has a wide variety of clinical presentations. Common symptoms include pelvic pain, secondary dysmenorrhea, dyspareunia, irregular or heavy menstrual period and infertility. Approximately one third of patients with endometriosis are asymptomatic. Endometriosis of the gastrointestinal tract may mimic gastrointestinal pathology causing abdominal cramps, change in bowel habits, nausea, rectal bleeding and bowel obstruction. Endometriosis of the appendix can present with chronic pelvic pain, melena, lower gastrointestinal bleeding, or intussusception of the appendix. Appendiceal endometriosis is not an uncommon incidental finding during laparoscopy or laparotomy. It is a common diagnosis when an inverted or bulbous appendiceal orifice is noted at the time of colonoscopy. Endometriosis of the appendix during pregnancy is very rare and it is associated with higher risk of bowel perforation, especially in the 3rd trimester. Laboratory tests are of limited value. CT of the abdomen and pelvis may show evidence of acute appendicitis, or appendiceal abnormality. Laparoscopy is considered the gold standard for the diagnosis of endometriosis. Grossly, endometriosis appears as raised flame-like patches, whitish opacifications, yellow-brown discoloration, or reddish-blue irregularly shaped islands. If there is an involvement of the mucosa, the differential diagnosis should include crohn’s disease, cancer and ischemia. Pain is the most common indication for surgical management. The published rate of endometriosis of the appendix varies widely depending on the type of clinical practice. Reports from tertiary centers reported a higher incidence of disease likely because they are referral centers for complicated disease. A study of 50,000 specimen of the human appendix by Collins reported the incidence of endometriosis of the appendix as 0.054. Marudanayagam et al. examined 2660 appendix specimens and found that the incidence of appendiceal endometriosis was 0.3%. Uohara and Kovara reported the incidence of endometriosis of the appendix as 0.80% in 1496 appendectomies. Smith reported an incidence of 0.62%, and Haydon reported the incidence as 0.17% in 569 who presented with endometriosis. Comparative analysis of the diagnosis of 866 cases of endometriosis including 53 cases of intestinal endometriosis revelled two cases of endometriosis of the appendix, reported by Thiersten et al. with an incidence of 0.22%, while Scott and Te reported an incidence of 1.3% in 516 patients who underwent an operation for gynecological reasons. Sutton and Hardy reported an incidence of endometriosis of the appendix 0.0217% in 6911 appendectomies. The goal of surgical treatment of bowel endometriosis is to remove the disease and restore the bowel continuity and function. The treatment of endometriosis of the appendix is appendectomy, ileocecectomy, and right hemicolectomy (Table 1). At laparotomy or laparoscopy, a careful examination of the abdominal cavity is carried out in order to fully evaluate the extent of disease. Often, involvement of the uterus and adnexa requires a multidisciplinary team approach. When there is appendiceal intussusception, gentle traction is applied to reduce the intussuscepted appendix. This should be performed with some caution. Malignancies or benign tumor of the appendiceal region can present with invagination. If the diagnosis is not clear, ileocecectomy or right hemicolectomy should be performed. Because endometriosis of the appendix maybe missed by visual inspection alone, incidental appendectomy is recommended in patients with severe endometriosis. Small bowel involvement is rare, and the ileocecal region is the most common affected area. Superficial spots of endometriosis can be vaporized with CO2 laser. More extensive lesions are treated by small bowel resection and anastomosis. Superficial lesions involving the rectal wall can be dissected off the wall using diathermy. 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