Abstract
The optimal management of patients with appendiceal endometriosis has not been determined because of the difficulty of establishing a preoperative diagnosis. There are no reports of preoperative hormone therapy for a patient with appendiceal endometriosis. We report a patient who underwent resection of appendiceal endometriosis after hormone therapy. A 40-year-old woman with history of recurrent pelvic abscesses presented to the emergency department with lower abdominal pain. The recurrent pelvic abscesses were synchronised with her menstrual cycle. CT scan demonstrated a 25 mm contrast-enhanced luminal structure adjacent to the cecum, which was thought to be a mucocele of the appendix. Considering the recurrent symptoms during menstruation, endometriosis was suspected. Treatment with a gonadotropin-releasing hormone agonist was started for appendiceal endometriosis, which alleviated the symptoms. After 3 months, elective laparoscopic appendectomy was performed. Preoperative hormonal therapy is an option for patients with appendiceal endometriosis, especially when there is concern for dense adhesions.
Keywords: reproductive medicine, gastrointestinal surgery, general surgery
Background
Appendiceal endometriosis is an uncommon condition usually diagnosed histologically after appendectomy for acute appendicitis. According to previous reports, 0.054%–0.03% of all excised appendix specimens revealed endometriosis.1 2 However, due to difficulty diagnosing this condition, the true prevalence is unknown. Appendiceal endometriosis is usually managed operatively if it is diagnosed during surgery for endometriosis. If it is diagnosed preoperatively, there are three options for treatment including surgery, hormonal therapy and combined surgery and hormonal therapy. However, since it is rarely diagnosed preoperatively, optimal management has not been defined. To the best of our knowledge, there are no reports of preoperative hormonal therapy for appendiceal endometriosis. For this patient, surgery alone was not considered the best option due to the risk of injury to other organs with suspected severe inflammation and adhesions. She also wished to become pregnant, which made permanent hormonal therapy unfavourable. For these reasons, we chose combined therapy.We report a patient who underwent successful laparoscopic resection of appendiceal endometriosis after hormone therapy.
Case presentation
A 40-year-old gravida 1 para 0 woman with a history of infertility treatment and recurrent pelvic abscesses presented to the emergency department with lower abdominal pain. The pain was not associated with nausea or vomiting. Her last menstrual period started the day before presentation. On physical examination, the patient was afebrile and in moderate distress with abdominal pain. The abdomen was not distended, but there was tenderness in the right lower quadrant. Her usual menstrual cycle was 28 days, and she had dysmenorrhoea relieved by ibuprofen. She underwent laparoscopic right salpingectomy for a fallopian tube abscess and laparoscopic drainage of a pelvic abscess 9 moths previously. The right fallopian tube did not contain endometrial tissue.
Investigations
The patient’s white blood cell count was elevated at 20 300 /µL, but C reactive protein, as well as other chemistry and coagulation studies, were within normal limits. CT scan demonstrated a 25 mm contrast-enhanced luminal structure adjacent to the cecum, which was thought to be a mucocele of the appendix (figure 1).
Figure 1.
CT scan demonstrating a 25 mm contrast-enhancing luminal structure adjacent to the cecum, thought to be mucocele of the appendix.
Treatment
Considering the presentation with recurrent fevers and abdominal pain during menstruation, endometriosis was suspected. Severe inflammation and dense adhesions were predicted based on the history of recurrent abscesses and the operative findings of two previous operations, which revealed dense adhesions. Immediate surgery was thought to carry an elevated risk of injuriy to other organs, especially the ureters. After discussion with gynaecology specialists, we decided to wait a few months for surgery to maximise the chance of preserving the left fallopian tube and ovaries and to reduce the risk of injury to other organs. Dienogest, a gonadotropin-releasing hormone (GnRH) agonist, was started at a dose of 1 mg two times per day. After starting this therapy, the pain decreased to the level where she felt only mild pain and discomfort with bowel movements. Three months later, elective laparoscopic appendectomy was performed. Laparoscopic examination showed the terminal ileum adherent to the right adnexa and pelvis and no abscess cavity. Adhesiolysis revealed the appendix with a swollen tip, with a small area of adhesions to the ileum and right adnexa. There were no nodules in the appendix. The base of the appendix was resected using a linear stapler, the appendix was removed in a retrograde fashion and the base cauterised. The operative time was 91 min with minimal blood loss.
Outcome and follow-up
The postoperative course was uneventful, and the patient was discharged on postoperative day 2. The histopathology was consistent with endometriosis involving the appendix (figure 2). After 1 year of follow-up, she remains asymptomatic with no evidence of recurrence.
Figure 2.
Appendix specimen.
Discussion
Appendiceal endometriosis
Endometriosis is a common condition defined by the presence of endometrial glands and stroma outside the uterus. When those glands and stroma invade the bowel wall, it is called bowel endometriosis. While 8%–12% of patients with endometriosis have intestinal involvement—the rectum and sigmoid colon account for 90% of all cases.3 Compared with the rectum and sigmoid colon, appendiceal endometriosis is rare, and the actual prevalence is unknown. Mabrouk et al4 reported that 2.6% of patients who underwent surgery for symptomatic endometriosis had appendiceal endometriosis. For patients with deep infiltrating endometriosis, the prevalence is reported to be approximately 8%–13%.5 However, some cases are diagnosed incidentally when appendectomy is performed for appendicitis. In 1963, Collins1 reported a study of 71 000 appendix specimens with appendiceal endometriosis in 0.054%. In the study by Marudanayagam et al2 in 2006, 0.3% of 2660 appendix specimens revealed endometriosis. Although appendiceal endometriosis can present as acute appendicitis, perforation or intussusception, most patients have no symptoms specific to this condition.6 7 In the present patient, menstrual cycle-associated pain and fever with CT scan findings of an enlarged appendix lead to the diagnosis.
Since it is challenging to diagnose appendiceal endometriosis preoperatively, there are no guidelines or large studies to guide management. If appendiceal endometriosis is suspected, it is common to choose surgery because it is considered easier to remove the appendix compared with the colon or rectum. In addition, it is difficult to rule out a malignancy such as a neuroendocrine tumour based on the operative findings. However, extensive procedures such as ileocecectomy are sometimes needed due to the presence of extensive adhesions. Mabrouk reported a cohort study of patients who underwent appendectomy for endometriosis. He stated that appendiceal involvement is associated with a higher bladder, deeper pelvis and ileocecal endometriosis.2
Treatment of appendiceal endometriosis
Three clinical scenarios are common in patients with appendiceal endometriosis. In the first scenario, emergency appendectomy is performed for presumed acute appendecitis, and the pathology report shows appendiceal endometriosis. There is no need to discuss treatment options in this situation because treatment is complete when the diagnosis of endometriosis is made. The second situation is discovering appendiceal involvement during surgery for endometriosis. In this case, an appendectomy is recommended because it is difficult to rule out malignancy during the operation. In the third scenario, appendiceal endometriosis was suspected preoperatively based on the patient’s history, as in the present patient.
Preoperative hormone treatment for endometriosis
Preoperative hormone treatment for patients with endometriosis is widely used, although there is no strong evidence to support its efficacy. The rationale is that hormonal therapy decreases the size of endometriosis implants and potentially decreases the extent of surgery needed.
There are several guidelines for hormonal treatment of endometriosis. The European Society of Human Reproduction and Embryology (ESHRE) issued a guideline in 2013.8 Preoperative treatment is not recommended to improve the outcome in patients with endometriosis based on a Cochrane review of preoperative and postoperative medical therapy for endometriosis.9 The primary outcomes of two randomised controlled trials included in the review were American Fertility Society Score, size of the endometrioma, the proportion who had complete excision of cysts and recurrence of cysts at 6 months. However, the extent of surgery or operative complications was not discussed here.
Several studies support the efficacy of preoperative hormonal therapy. A recent randomised controlled trial of patients with genital endometriosis showed the efficacy of combined surgical and hormonal therapy.10 Four hundred fifty patients were randomly assigned to hormone therapy, surgery or combined treatment. In this study, the preoperative hormone treatment group showed the lowest recurrence and highest cure rate. Bailey11 also supports the efficacy of preoperative hormone therapy for patients with intestinal endometriosis with the rationale that 3–6 months of hormone therapy decreases the inflammation and vascularity, which reduces the density of adhesions.
Given the preceding discussion, preoperative hormonal therapy is a reasonable option when endometriosis involves extragenital organs, such as ureters, the colorectum or a large portion of the small intestine.
Combined surgery and hormone therapy for appendiceal endometriosis
Due to its low prevalence, no studies are available on the effect of hormone therapy on appendiceal endometriosis. In the present patient, GnRH agonist therapy successfully alleviated her symptoms. However, the hormone treatment had to be stopped due to her strong desire to become pregnant. Dense adhesions were predicted preoperatively based on a long history of menstrual-associated pain and two previous operations for pelvic abscess, at which time there were dense adhesions present. To maximise preservation of the left fallopian tube and ovaries and to minimise the risk of injuries to other organs such as the ureters, surgery was delayed for 3 months after the last episode of abdominal pain and initiating GnRH agonist therapy preoperatively. The 3-month interval was based on previous reports evaluating preoperative hormone treatment for patients with ovarian endometriosis.12 13 We also considered the standard interval of more than 2 months to surgery in patients with non-operatively managed acute appendicitis. At operation, possibly due to the use of hormone therapy, adhesions surrounding the appendix were limited to a small area involving the ileum. We successfully performed laparoscopic appendectomy without complications. In patients with appendiceal endometriosis, when dense adhesions are suspected, preoperative hormone therapy is a reasonable option.
Patient’s perspective.
I was very anxious about it because no one was sure whether the pain came from menstrual periods or something else was happening in my body. I also felt emotional stress while I was repeatedly admitted to the hospital, which caused trouble for my coworkers and family.
However, I was relieved having been seen by both surgeons and gynaecologists in the early stage of treatment. I would recommend women with similar symptoms to go to the hospital, where several specialists treat the patients if necessary.
Learning points.
Appendiceal endometriosis can present with menstrual cycle-associated pain and fever.
Appendectomy in a patient with recurrent endometriosis can be challenging due to dense adhesions.
Preoperative hormonal therapy is an option for patients with appendiceal endometriosis, especially when there is a concern for the presence of dense adhesiona.
Footnotes
Contributors: All persons who meet authorship criteria are listed as authors, and all authors certify that they have participated sufficiently in the work to take public responsibility for the content, including participation in the concept, design, writing, or revision of the manuscript. KS: concept and design of study, drafting the manuscript, approval of final manuscript. KN: participation in the treatment and perioperative management of the patient, approval of final manuscript. AKL: concept and design of study, approval of final manuscript.TK: approval of final manuscript.
Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests: None declared.
Provenance and peer review: Not commissioned; externally peer reviewed.
Ethics statements
Patient consent for publication
Consent obtained directly from patient(s)
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