Appendiceal intussusception is not a common disease and is rarely diagnosed preoperatively. In our case, a 25-year-old male patient living in Ho Chi Minh City came to Medic Medical Center complaining about his epigastric abdominal pain, which lasting for 3 days. His body temperature was not high and he did not have any other symptoms. He recalled similar pain which had gone away without any treatment three months ago. Abdominal ultrasound showed abnormalities in appendix and cecum. During performing colonoscopy, we suspected appendiceal intussusception, and following computed tomography showed the images of enlarged appendix with fluid-filled lumen and signs of intussusception at the appendix base. The patient underwent an operation to remove the appendix and appendiceal intussusception was confirmed. Microscopic result was consistent with chronic appendicitis.
Intussusception of the appendix vermiformis in adults is a rare condition caused by anatomical and pathological factors such as tumors and is rarely diagnosed before surgery.
Although most appendiceal tumors are benign, tubular adenoma is an unusual lesion. Furthermore, carcinoma of the appendix is a distinctly rare phenomenon. The combination of both a carcinoma and intussusception has been regarded as extremely rare. Here, we report a case with intussusception of the appendix induced by primary appendiceal adenocarcinoma and discuss the clinical features, classification, preoperative diagnosis and therapy of this condition together with a review of the literature.
Intussusception of the appendix is an uncommon pathological condition. The incidence of invagination of the appendix is 0.01% in a large autopsy series. The etiology of appendiceal intussusception has been proposed by Fink et al. to be divided into anatomical and pathological causes. Anatomical variations include a fetal-type cecum, a wide appendicular lumen and a thin, mobile appendix. Reported pathological conditions include worms, endometrial implants and tumors. Various classifications of appendiceal intussusception have been attempted. Forshall divided cases into a primary type and a secondary or compound type. Langsam et al. classified the disorder into four types according to the relationship of the intussusceptum and intussuscipiens. In our case, the appendiceal tumor as a leading point seemed to have induced the complete appendiceal intussusception.
Preoperative diagnosis of intussusceptions of the appendix is often difficult because it is a rare clinical entity; only a few cases can be diagnosed by barium enema and colonoscopy. Many cases have been diagnosed as filling defects or polypoid tumors of the cecum. Careful endoscopic examination, identifying the appendiceal orifice, should be required in the case of cecal polyp. When differential diagnosis is difficult, computed tomography or abdominal ultrasound is more useful. A definite finding of intussusception of the appendix in CT is the invaginated appendix in the cecal cavity.
Tumors of the appendix are uncommon; also, most tumors are benign. Adenoma of the appendix is also a rare condition. Only 50 cases of appendiceal adenoma were reported among 30 000 appendectomies. Moreover, primary adenocarcinoma is a very rare entity, which in most cases arises from a pre-existing adenoma. Ohno et al. first reported appendiceal intussusception induced by tubulovillous adenoma with carcinoma in situ similar to our case; the combination of both a carcinoma in adenoma and intussusception has been regarded as extremely rare.
Treatment of appendiceal intussusception is mainly surgical. The procedure varies from reduction of intussusception with appendectomy to right hemicolectomy. A right hemicolectomy or ileocecal resection with lymph node dissection should be performed when carcinoma is diagnosed preoperatively or during surgery. In the last decade, laparoscopic procedures have been applied to the treatment of almost all colonic diseases, including both benign and malignant lesions. As Schmidt et al. reported laparoscopic management of appendiceal intussusception, laparoscopic procedures will be more useful as a minimally invasive treatment.
The clinical manifestation of appendiceal intussusception with primary appendiceal tumor resembles a large cecal polyp with a wide stalk, but its treatment is completely different. Endoscopic removal should be performed carefully in cases of polypoid lesions in the cecum, taking into consideration the possibility of an invaginated appendix. Failure to recognize this condition may result in unexpected complications such as consequent peritonitis.