Keywords
appendix - intussusception - adults - endometriosis
Introduction
The intussusception of the appendix is the invagination of the appendix into the cecum,
accompanied by changes in peristalsis.[1] This condition is considered a rare finding, with an incidence of 0.01%. In the
clinical assessment, the main symptoms presented by the patients are abdominal pain,
vomiting, and blood in the rectum. However, their presentation varies. Thus, while
some people have acute symptoms similar to those of appendicitis, other patients have
intermittent symptoms, which can persist for weeks or months. The most common cause
of appendix intussusception in adult patients is endometriosis, which accounts for
approximately 33% of the cases.[2]
Endometriosis can be defined as the presence of endometrial glands and stroma outside
the uterine cavity. This condition affects around 10% of women who menstruate, and
it is present in approximately 70% of the cases of chronic pelvic pain and in 50%
of the cases of infertility.[3] The clinical assessment of endometriosis is variable, and many individuals are asymptomatic.
Most of the symptomatic cases tend to present moderate to severe pelvic pain. Endometriosis
in the appendix can result in an asymptomatic condition, but it can also present as
acute appendicitis, intestinal obstruction due to intussusception, lower gastrointestinal
bleeding, or intestinal perforation.[3]
Given that intussusception of the appendix due to endometriosis is an extremely rare
finding, there is a scarcity of studies on the subject in the literature, and there
is a need for more publications on the subject.[2] We herein report a case of a patient with intussusception of the cecal appendix
secondary to endometriosis.
Case Report
A 37-year-old, White, single, female patient, diagnosed with deep endometriosis 3
years before, consulted with a coloproctologist complaining of recurrent abdominal
pain in the right iliac fossa associated with her menstrual period for 2 years, with
no change in her bowel habits, bleeding or constitutional symptoms. The physical examination
revealed pain on palpation of the right iliac fossa, with no signs of peritonitis.
The patient had undergone a colonoscopy two years before, when the symptoms began,
in which a tubular adenomatous polyp with low-grade dysplasia was identified in the
sigmoid, with no other endoscopic findings.
The patient returned for a review, presenting partial relief of the symptoms after
starting the treatment for endometriosis with hormone blockade, but continued to present
discomfort in her right iliac fossa, with the same characteristics as those at the
start of the investigation. A new colonoscopy was recommended to assess deep endometriosis
and as a follow-up after resection of the adenomatous polyp. Upon further examination,
the endoscope was introduced up to the terminal ileum, showing an intraluminal formation
4 cm long in the cecum, near the concentration of the tapeworms, apparently in the
region of the appendicular ostium, with a slightly enanthematous mucosa, but with
the architecture of the crypts preserved on biopsy ([Fig. 1]). A 5-mm sessile polyp was also identified in the sigmoid colon, which was removed.
The material collected was sent for an anatomopathological examination. The sessile
sigmoid polyp turned out to be a tubular adenoma with low-grade dysplasia, and the
biopsy of the cecal lesion identified tissue corresponding to the cecal appendix.
Fig. 1 Lesion identified in the cecum during colonoscopy: 4-cm long intraluminal formation,
apparently in the region of the appendicular ostium, with slightly enanthematous mucosa.
The next step in the investigation involved a magnetic resonance imaging (MRI) scan
to evaluate the colonoscopic findings, which showed hyperintense foci, compatible
with ectopic endometrium in the uterine junctional zone, with other foci involving
part of the uterus and ligaments, the anterior wall of the rectum, and the sigmoid
colon, with parietal thickening and vegetation, possibly related to colonic endometriosis
([Fig. 2]). The cecal appendix had diffusely thickened walls and increased volume (2.0 cm
in diameter), which may correspond to a case of mucocele, and its base projected into
the lumen of the cecum.
Fig. 2 Total abdomen magnetic resonance imaging scan in a T2-weighted fast-spin echo (T2
FSE) sequence. (A) Coronal section with hyposignal in the cecal appendix (blue arrow). (B) Axial section with hyposignal in the cecal appendix (green arrows).
The main diagnostic hypothesis was of a pathological appendix with suspected intussusception,
and the patient was referred for surgery. A right laparoscopic ileocolectomy was performed,
with resection of 15 cm of the terminal ileum and cecum ([Fig. 3]) and primary ileocolic anastomosis. The anatomopathological results of the surgical
specimen led to the diagnosis of intussusception of the cecal appendix with a marked
chronic inflammatory process and associated endometriosis of said appendix. The resection
margins were viable, with four mesocolic and mesenteric lymph nodes showing reactive
lymphoid hyperplasia and sinusoidal congestion. The patient progressed satisfactorily
and was discharged from the hospital on the fifth postoperative day, without any complications.
At the 60-day review, she showed a significant improvement in her right iliac fossa
pain.
Fig. 3 Surgical specimen with resection of the terminal ileum and cecum and intussusception
of a pathological appendix. The segments of the small and large intestines measure
6.5 cm and 4.5 cm in length respectively and have a grayish-violaceous serosa with
congested vessels, in which you can see an area of mesoretraction. At the opening,
close to the ileocecal valve, an area of protrusion lesion can be seen in the lumen
of the cecum, in which the cecal appendix can be identified, measuring approximately
4.5 cm in length and 1.5 cm in maximum diameter, with a shiny gray serosa and mucosa
with preserved folding.
Discussion
Intussusception of the cecal appendix is a rare, difficult-to-diagnose condition with
an incidence of 0.01%.[1]
[2] It is more prevalent in the adult population (76%), with a predominance in females
(70%), and it is more commonly associated with cases of endometriosis, appendicular
mucocele, Crohn's disease, adenoma, and adenocarcinoma.[2]
[4]
The clinical picture of appendicular intussusception is variable, ranging from asymptomatic
to nonspecific acute and chronic symptoms, or those that can mimic acute appendicitis.[5] Chaar et al.[2] have reported that nonspecific chronic symptoms are more common, occurring in 63%
of the cases, with abdominal pain (78%), vomiting and melena (26%), and hematochezia
(28%) standing out.
According to Costa et al.,[5] the etiology of appendicular intussusception may be related to anatomical factors,
such as a large proximal appendicular lumen, hyperperistalsis, a fully mobile appendix
or a thin mesoappendix. Among the factors that trigger intussusception are foreign
bodies, fecaliths, polyps, adenocarcinomas, carcinoid tumors, parasites, endometriosis,
and lymphoid hyperplasia.[1]
[2]
[5] In adults, endometriosis is the most common etiology, responsible for 33% of the
cases, while approximately 7% of the cases are caused by carcinoid tumors, and 6%,
by adenocarcinomas.2
It is estimated that endometriosis affects around 10% of women of reproductive age,
causing involvement of the intestinal tract in 3.8 to 37% of the patients.[1]
[6] Most of these cases develop the disease in the rectum and rectosigmoid junction,
with involvement of the cecal appendix being rare, occurring in approximately 3% of
cases.[1]
[7] Endometrial tissue in the intestine most often affects the serous and muscular layers,
causing adhesions and hyperplasia of the smooth muscle; the mass formed is propelled
by peristalsis and, if it affects the cecal appendix, it can cause its intussusception
in the cecum, as in the case herein presented.[2]
[8]
The suspicion of endometriosis as the etiology of intestinal intussusception arises
mainly due to a previous diagnosis of the disease or a clinical history of complaints
such as dysmenorrhea, dyspareunia, chronic pelvic pain, and dyskinesia.[6] Based on this hypothesis, a targeted investigation into differential diagnoses is
essential.
The diagnosis of cecal appendix intussusception is an arduous task, and the condition
is often confused with neoplasms.[2]
[5] In addition, most cases are only identified during surgery (57%), while preoperative
diagnosis (32%) is less frequent.[2] Chaar et al.[2] have reported that preoperative diagnosis after the 2000s with more improved imaging
tests has resulted in increased sensitivity.
Initially, ultrasound (US), computed tomography (CT) of the abdomen, radiography with
barium enema and endoscopic studies can be performed.[5]
[9] During colonoscopy, the lesion is usually observed as a polypoid mass covered by
normal mucosa and a central depression in the orifice.[5] Computed tomography is more accurate than US, although both tests help in the diagnosis.[3] Magnetic resonance imaging has been used[10] for better preoperative assessment and differentiation from mucoceles, showing hyperintense
foci compatible with ectopic endometrium, suggestive of colonic endometriosis.
However, imaging tests and endoscopic biopsies play a limited role in ruling out malignancies,
and the suspicion of endometriosis is not well assessed in colonoscopy biopsies, since
most endometrial implants are located in the outer layers of the intestinal wall.[8] In these cases, endoscopic biopsy often shows normal mucosa, as in the case herein
described. Therefore, the definitive diagnosis, in most cases, is established by surgical
approach and complete analysis of the specimen.[2]
In general, laparoscopic cecectomy is chosen, with preservation of the ileocecal valve,
but it may also be necessary to perform ileocecectomy or right hemicolectomy if there
is an association with ileocecal or ileoileal intussusception. Laparoscopy also enables
the assessment of the peritoneal cavity in search of other foci of endometriosis.[9]
In addition to surgery, the treatment after the definitive diagnosis of endometriosis
etiology may involve hormone suppression therapy, which presents benefits mainly in
preventing recurrences of intestinal endometriosis. In these cases, the first line
of treatment is low dose progesterones or combined oral contraceptives, both of which
present few side effects and are inexpensive. Follow-up with a gynecologist and coloproctologist
is essential.[6]
Conclusion
Intussusception is a rare occurrence that is difficult to diagnose in the adult population,
and it involves the colon less often than the small intestine. Despite the rarity
of the clinical picture, the case herein reported, of a female patient with cecal
appendix intussusception due to endometriosis, who underwent surgical treatment with
good postoperative evolution, demonstrates the need to include this hypothesis in
the differential diagnosis of patients with similar symptoms of recurrent abdominal
pain in the right iliac fossa related to the menstrual period.
Bibliographical Record
Sofia Marasca Giongo, Gabriela Pinho Fillmann, Marcelo Garcia Toneto, Lúcio Sarubbi
Fillmann, Alessandro Batista Soares, Mariana Tanus Stefani, Marina Tonin, Ana Laura
Avila Caumo, Irina Maria Ayala Lopez. Intussusception of the Cecal Appendix Secondary
to Endometriosis: Case Report. Journal of Coloproctology 2025; 45: s00451802596.
DOI: 10.1055/s-0045-1802596
