Appendiceal intussusception from endometriosis

An asymptomatic 39-year-old woman was noted to have occult bleeding by faecal immunohistochemical test. Investigations showed a 3 cm×2 cm caecal lesion by colonoscopy that was consistent with abdominal CT scan findings of a 1.6 cm×1.9 cm×2.3 cm enhancing focus in the medial caecal wall. The patient underwent laparoscopic right hemicolectomy after diagnostic laparoscopy and intraoperative colonoscopy revealed an intussuscepted appendix. The final histopathology was an appendiceal intussusception secondary to endometriosis.

surgery, gastrointestinal surgery, general surgery

Appendiceal intussusception is a rare condition that has an incidence of 0.01%. It is characterised by abnormal appendiceal peristalsis due to anatomical or pathological factors leading to its invagination into the caecum. The most common lead point in adults is endometriosis (33%), followed by mucocoele (19%).1 A review of literature by Chaar et al reported appendiceal intussusception to be more common in the adult population (76%) than in children (24%), with female predominance (72%), and often presents in the fourth decade of life.1 It usually presents with non-specific chronic symptoms; most frequently, abdominal pain (78%).1 This condition represents a diagnostic challenge for physicians, and is often diagnosed intraoperatively or postoperatively. Preoperative imaging plays an important role in the decision-making process on surgical management, such as abdominal CT scan or ultrasonography; while colonoscopy allows direct visualisation of the lesion. Management is surgical since an appendiceal intussusception increases the likelihood of developing secondary ileocaecal intussusception.2 Appendectomy was the most commonly performed procedure1; however, in the absence of a histopathological diagnosis and an intraoperative assessment suspicious for malignancy, a right hemicolectomy is mandated.3 This report described the management of a premenopausal woman who underwent laparoscopic right hemicolectomy for appendiceal intussusception. Histopathology later revealed the intussusception to have been caused by endometriosis. Case presentation An asymptomatic 39-year-old woman, with no comorbidities and a previous low-segment caesarean section, came for consult due to occasional hypogastric pain associated with occult bleeding detected by faecal immunohistochemical test (FIT) that was requested by her primary care physician. Investigations Work-up for the occult bleeding was initiated with a colonoscopy (figure 1). This showed a 3 cm×2 cm lesion in the caecum, with failure of identification of the appendiceal lumen. A diagnosis of an appendiceal intussusception was contemplated and so the endoscopist decided to forego performing a biopsy due to a high risk of perforation. A whole abdominal CT scan with triple contrast enhancement demonstrated a 1.6 cm×1.9 cm×2.3 cm enhancing focus in the medial caecal wall, with the appendix not clearly delineated (figure 2). Complete blood count and electrolytes were normal; while the Ccarcinoembryonic antigen (CEA) was 1.3 ng/mL. Treatment Provided the abovementioned findings, the impression for the caecal lesion was an appendiceal intussusception versus a caecal polyp at the appendiceal orifice. The proposed management plan was a combined endoscopic and laparoscopic surgery—endoscopic mucosal resection with possible oversewing of the caecal wall, or a partial caecal resection, should the lesion be determined to be a polyp; versus a laparoscopic right hemicolectomy, if it were an intussusception. On insertion of an optical port and occlusion of the terminal ileum with a bowel grasper to prevent small bowel dilatation, an intraoperative colonoscopy was done. Carbon dioxide (CO2) was used to insufflate the bowel lumen to avoid extensive dilatation of the colon since CO2 is more rapidly absorbed compared with other gases used for insufflation. Similar to the preoperative colonoscopy, the lesion was found in the caecum, obscuring the appendiceal lumen. Intraperitoneally, dense adhesions were noted surrounding the caecum with note of a ‘puckered’ area in the supposed area of the appendiceal base (figure 3). The appendix itself was not identified at its expected anatomical location—at the convergence of the taenia coli. The diagnosis of appendiceal intussusception was confirmed on examination of the resected colon (figure 4), consistent with the colonoscopic finding. Correlating these findings, and with malignancy not having been ruled out in the absence of a definitive histopathological diagnosis, the team decided to proceed with a laparoscopic right hemicolectomy. After mobilisation of the colon, and ligation of the ileocolic vessels and the right branch of the middle colic vessels, bowel resection was performed at the specimen extraction site. An extracorporeal side-to-side ileocolic anastomosis was then done to restore intestinal continuity. Outcome and follow-up The patient had an uneventful postoperative course, and was discharged on full diet on the second postoperative day. The final histopathology report was that of a benign appendix with intussusception and intramuscular endometriosis (figure 5A, B). No signs of dysplasia or malignancy were seen. She has since been started on hormonal suppression.

Appendiceal intussusception is a rare disease with an incidence of 0.01% as reported by Collins.4 This condition is characterised by abnormal appendiceal peristalsis due to anatomical or pathological factors leading to its invagination into the caecum. Anatomical considerations include a mobile mesoappendix, a thin appendix and a wider proximal appendiceal lumen.2 5 6 Pathological intrinsic conditions that may act as lead point for its intussusception include adenocarcinoma, carcinoid tumour, mucocoele, polyps, parasites, endometriosis and lymphoid hyperplasia.1 6–8 Endometriosis is the most common lead point (33%) in adults, followed by mucocoele (19%).1 A review of literature by Chaar et al reported appendiceal intussusception to be more common in the adult population (76%) than in children (24%), with female predominance (72%), and often presents in the fourth decade of life.1 Clinical manifestations include a wide variety of symptoms from purely asymptomatic, acute or non-specific chronic (63%) symptoms; most commonly abdominal pain (78%), vomiting (26%) and melaena or haematochezia (23%).1 The reported incidence of endometriosis involving the gastrointestinal tract ranges from 15% to 37%, and most commonly affects the rectum and the rectosigmoid junction (72%). Appendiceal involvement accounts for only 3% of cases.9 10 Endometrial tissue is histologically seen affecting the serosa and muscular layer of the intestinal tract, including presence of stroma, fibrosis and haemosiderin-laden macrophages. Serosal involvement can result to fibrosis and adhesions, and implants can cause hyperplasia of the muscular layer, constricting the appendiceal lumen mimicking a mass. This can become a lead point for hyperperistalsis, resulting to appendicular invagination into the caecum.9 11 Appendiceal intussusception represents a diagnostic challenge for physicians, and is often diagnosed intraoperatively or postoperatively. Preoperative imaging plays an important role in the decision-making process on surgical management. Pathognomonic abdominal CT scan findings include a target lesion or a concentric central mass, while ultrasonography will show an onion-skin like lesion, or multiple concentric hyperechoic and hypoechoic rings. Colonoscopy allows direct visualisation of the lesion, with appearance of a polypoid mass covered in normal mucosa and a central dimple at the appendiceal orifice.11 However, in some cases this can only be diagnosed with certainty during surgery itself, in which case good surgical judgement plays a crucial role. The request to have a FIT done was made by the patient’s primary care physician as part of her ‘annual exam’. This digression from established guidelines on screening for colorectal cancer is commonly practiced in our country, particularly in urban centres. Unfortunately, we have no existing national guidelines on colorectal cancer screening. Management is surgical since an appendiceal intussusception increases the likelihood of developing secondary ileocaecal intussusception.5 Appendectomy was the most common procedure performed (42%), followed by ileocecectomy (27%) and right hemicolectomy (21%).1 In the setting of a benign looking appendix with no suspicion for malignancy, an appendectomy is the treatment of choice.3 12 Suspicion for an appendiceal mass or cecal neoplasm may warrant exploration of the abdomen. In the absence of a histopathological diagnosis and an intraoperative assessment suspicious for malignancy, a right hemicolectomy is mandated.3 The patient was also started on hormonal suppression with the intent of inhibiting further ectopic endometrial growth, prevent disease progression and reduce risk of recurrence. Similar to appendiceal intussusception, an ileocolic intussusception due to endometriosis shows the same clinical presentation and approach to management, which makes it a strong differential diagnosis, as documented in a few published reports.13–16 It is important to have a high index of suspicion when presented with lower abdominal/hypogastric pain, palpable mass and heme-positive stools, as these signs and symptoms imply an otherwise surgical entity. Two of these patients with ileocaecal intussusception due to endometriosis underwent ileocaecal resection, while the other two had right hemicolectomy. Despite the rarity of these disease entities, both appendiceal and ileocolic intussusceptions secondary to endometriosis warrant the same approach to diagnosis and surgical management. Learning points. Appendiceal intussusception is a rare and difficult condition to diagnose. When symptomatic, this may manifest with non-specific signs and symptoms. In the absence of a histopathological diagnosis, a right hemicolectomy—the recommended surgery for appendiceal malignancy—may be the most prudent surgical intervention to be done in cases of appendiceal intussusception. Particularly for uncommon cases, communication among various specialties is vital in ensuring the most ideal outcome for the patient. Footnotes Contributors: MPJL is the lead author and primary surgeon. VC reviewed all pertinent literature for the case. MFM is one of the surgical assists and did manuscript editing. RL is one of the surgical assists and did manuscript editing. 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. Competing interests: None declared. Provenance and peer review: Not commissioned; externally peer reviewed. Ethics statements Patient consent for publication Obtained.

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