Surgery Today Jpn. J. Surg. (1992) 22:470-473

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© Springer-Verlag 1992

Intussusception of an Appendiceal Mucocele: Report of a Case TSUKASA TSUNODA, l TOSHIFUMI ETO, l HARUNOBU MAEDA, ~ NOBUO MOCHINAGA, 1 MASAZUM! TERADA, 1 SHIGETOSHI MATSUO, 1 RYOICHI TSUCHIYA,2 and YOSHIKO TAKAHARA 3 ~The Second Department of Surgery, Nagasaki University School of Medicine, 7-1 Sakamoto Nagasaki, 852 Japan, 2 Shimane Medical University, Izumo, Japan, 3 Takahara Chuo Hospital, Nagasaki, Japan

Abstract: A 66 year old Japanese female was admitted to our department for the examination and treatment of a mass in the cecum. She had experienced no symptoms or signs other than a positive test result for fecal occult blood. The mass in the cecum was confirmed by barium enema, colonofiberscopy and CT scanning. The presumptive diagnosis was a submucosal tumor of the cecum, however, a laparotomy subsequently revealed intussusception of an appendiceal mass. An ileocaecal resection with an ileocolic anastomosis was therefore performed and the mass was histologically diagnosed as a mucocele of the appendix. This patient is only the 24th case of intussusception of a mucocele of the appendix to be reported in Japan. A review of the available literature on this condition follows the case report. Key Words: mucocele, intussusception

Introduction

Mucoceles of the appendix are found in only 0.2 to 0.3 per cent of all appendectomy materials. 1'2 Because intussusception of the appendix is also uncommon, 3"4 the combined occurrence of these two coditions is extremely rare. In this paper, we report a case of intussusception of a mucocele of the appendix, and discuss the clinical features and preoperative diagnosis of this condition with reference to the literature. Case R e p o r t

A 66 year old Japanese female was admitted to the Second D e p a r t m e n t of Surgery, Nagasaki University Hospital on September 11, 1989 for examination and Reprint requests to: T. Tsunoda (Received for publication on Jan. 31, 1991; accepted on Nov. 1, 1991)

treatment of a mass in the cecum. Although displaying no symptoms, the patient had had a positive test result for occult blood in the feces at a regular health check on August 1, 1989. A colonofiberscopy and barium enema performed at Takahara Chuo Hospital on August 21, 1989 had revealed a submucosal mass in the cecum. A liver cyst and bilateral renal cysts had also been found on ultrasonography. Physical examination revealed the patient to be in a good nutritional state and in no acute distress. The abdominal mass was not palpable and full blood count, blood chemistry and serum C E A levels were all within normal limits. Plain X-rays of the abdomen showed normal gas patterns and no evidence of a soft tissue mass or any abnormal calcifications. Upper gastrointestinal series were normal. Barium enema with air contrast revealed a spherical mass in the lower cecum with no demonstration of the appendix (Fig. 1) and multiple diverticula of the sigmoid colon. Colonofiberscopy disclosed a protuberant lesion covered with normal colonic mucosa (Fig. 2). CT scanning showed a homogeneous lowdensity mass in the right lower abdomen (Fig. 3). A presumptive diagnosis of a benign submucosat tumor in the cecum was made on the basis of these findings. Laparotomy was carried out on September 25, 1989. There was no evidence of ascites or any adhesions. The mass developed in the appendix and consisted of four enlarged cystic masses ranging in size from 2 to 5 cm. The largest mass, 5 cm in diameter, was intussuscepted into the cecum and there were no signs of any mucinous implants or pseudomyxoma peritonei in the abdominal cavity. Therefore, an ileocaecal resection with an ileocolic anastomosis was performed. The resected specimen is shown in Figure 4 to demonstrate the intussuscepted state found during surgery. The cystic masses contained white-grayish mucinous fluid and did not communicate with the cecal lumen (Fig. 5). Microscopically, the mucosa of the cystic

T. Tsunoda et al.: Intussusception of an Appendiceal Mucocele

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Fig. 3. CT scanning shows a 4 x 4.5 cm homogeneous lowdensity mass in the right lower abdomen. There are no calcifications in the mass

Fig. 1. Barium enema shows a spherical mass in the lower cecum with no demonstration of the appendix

Fig. 4. Resected specimen. The cecum is opened to show the intussuscepted state of the appendiceal mucocele. The largest mass has intussuscepted into the cecum Fig. 2. Colonofiberscopy shows a protruding lesion in the cecum covered with normal colonic mucosa

masses consisted of pseudostratified c o l u m n a r epit h e l i u m of high m u c o i d c o n t e n t (Fig. 6). T h e histological diagnosis was thus established as a m u c o c e l e of the appendix.

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T. Tsunoda et al.: Intussusception of an Appendiceal Mucocele

Fig. 6. Histology (HE. x 100). The mucosa of the cystic mass is comprised of pseudostratified columnar epithelium

Fig. 5. White-grayish mucinous fluid is contained in the cystic mass

The patient was discharged on the 23rd day after surgery following an uneventful postoperative course and has been well ever since.

Discussion Only 24 cases of intussusception of a mucocele of the appendix have been reported in Japan,5-s including the present case. The details of these cases are shown in Table 1. There were 11 males and 13 females with a mean (___SE) age of 55.2 + 3.5 years, ranging from 21 to 86 years, and 21 patients (87.5 per cent) were aged 40 years or older. The two most c o m m o n symptoms were abdominal pain, seen in 21 cases (87.5 per cent) and vomiting, seen in 9 cases (37.5 per cent). An abdominal mass was able to be palpated by the patient in 3 cases (12.5 per cent). There were two asymptomatic patients including our case. On physical examination, a right lower quadrant mass was palpated in 21 cases (87.5 per cent). Douglas et al collected 27 cases of this condition from Western countries and reported the mean age to be 48 years with a female preponderance. 9 There

were very few differences in the clinical manifestations observed in Japan and the West. The operative procedures in the collected series consisted mainly of ileocecal resection and cecectomy, selected on the basis of intraoperative judgment of the extent of the lesion. The overall prognosis was good, although there were no malignant lesions in any of the collected cases. With special reference to the preoperative diagnosis, an intussuscepted state was correctly diagnosed in only 50 per cent of the cases, the most frequent diagnosis of the lesion being an ileocecal mass or a cecal submucosal tumor. It thus appears extremely difficult to diagnose a mucocele of the appendix preoperatively. A superficial calcium deposit within the right lower quadrant mass on a plain X-ray of the abdomen, often expressed as a peripheral curvilinear calcification, was considered to be indicative of a mucocele. 1° However, this was described in only one case of the collected series and not detected in our own case. Characteristic findings of a mucocele by imaging modalities such as barium enema, ultrasonography and CT scanning have been reported. 3'~1-14 The findings of barium e n e m a were displacement of the cecum and a failure to fill the appendix with contrast medium, 3"11 also seen in our case. Ultrasonographic findings demonstrated a cystic mass with a serpiginous polypoid intramural projection 12 or a through-transmitting mass with echogenic foci, 11"13 while the findings of CT scanning demonstrated a low-attenuating mass, 11'13 which was compatible with our case, or a cystic mass with a thick calicified wall.14 Nevertheless, it still seems difficult to establish a correct preoperative diagnosis unless both a right lower quadrant mass and characteristic calcifications are detected. In conclusion, although intussusception of a mucocele of the appendix is rare, it should be considered when

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T. Tsunoda et al.: Intussusception of an Appendiceal Mucocele Table 1. Cases of intussusception of an appendiceal mucocele reported in the Japanese literature Authors (year)

Age/sex

Symptoms

Pal mass

Preop Dx

+ ND + + + + + +

Intus ND Intus Intus Intus Ca of IC ND Intus of IC Mass IC Mass Appendicitis Intus Perityphlic abscess of IC tumor Intus of IC Mass Intus of app tumor Cecal SMT Cecel SMT Cecel SMT Intus of app mucocele Intus of app abscess or cecal SMT Intus of cecal tumor Intus of intestinal cyst App mucocele Intus of SMT of IC Cecal SMT

1. 2. 3. 4. 5. 6. 7. 8.

Nomura (1913) 5 Kimura (1922) 5 Suzuki (1935) 5 Kanemori (1941) 5 Nakashima (1952) 5 Ogawa (1957) 5 Sugimoto (1958) 5 Tokunaga (1960) 5

40/M 22/M 40/M 47/F 52/F 46/F 56/M 64/M

P P, V P, M P, V P, V, F P, V P, V P, V

9. 10. 11. 12. 13.

Isogaki (1961) 5 Harada (1962) 5 Nishimoto (1965) 5 Iwasa (1973) 5 Setsu (1978) 5

80/M 61/F 66/M 73/F 35/F

P, N P P, V P P, V

+ + + +

14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24.

Munakata (1979) 5 Takahashi (1981) 5 Nitta (1983) 5 Ueda (1983) 5 Onoda (1986) 5 Monzen (1985) 6 Monzen (1985) 6 Fujji (1986) 7 Fujii (1986) 7 Kawase (1988) 8 T S U N O D A (1990)

62/F 21/F 86/M 67/F 78/M 41/F 47/F 54/M 51/F 70/M 66/F

P, V, M M None P, BS P P P P P P None

+ + + + + + + + + + +

Operative methods IC Resection IC Resection Cecectomy IC Resection IC Resection IC Resection IC Resection IC Resection Cecectomy Appendectomy IC Resection Cecectomy IC Resection Cecectomy Appendectomy IC Resection Cecectomy IC Resection IC Resection ND Cecectomy Cecectomy IC Resection IC Resection

P, Pain; v, vomiting; M, mass; F, fever; N, nausea; BS, bloody stool; Pal, palpable; Preop DX, preoperative diagnosis; intus, intussusception; ca, carcinoma; IC, ileocecal; SMT, submucosal tumor; app, appendiceal; ND, not described

dealing with a n ileocecal mass lesion or cecal subm u c o s a l t u m o r , especially w h e n the mass lesion is a c c o m p a n i e d by calcifications.

References 1. Collins DS (1955) A study of 50,000 specimens of the human vermiform appendix. Surg Gynecol Obstet 101:437-445 2. Woodruff R, McDonald JR (1940) Benign and malignant cystic tumors of the appendix. Surg Gynecol Obstet 71:750-756 3. Fink VH, Santos AL, Goldberg SL (1967) Intugsusception of the appendix. Case reports and review of the literature. Am J Gastroenterol 42:431-441 4. Paul GJ, Ferraro LR (1967) Intussusception of appendix. NY State J Med 67:2625-2629 5. Onoda B, Hirosawa K, Nakajima K, Nakagawa T (1986) A case of intussusception of mucocele of the appendix (in Japanese with English abstract). Nippon Rinsho Geka Igakkai Zasshi (J Jpn Soc Clin Surg) 47:372-378 6. Monzen Y, Kinoshita Y, Tominaga K, Miyake H, Ishimaru H, Hayashi K (1985) Experience of treatment for two cases of intussusception by mucocele of the appendix (in Japanese). Gazo Shindan (J Medical Imagings) 15:462-466

7. Fujii M, Konn M, Ono K, Chiba M, Watabe S, Nishida S (1986) Two cases with intussusception of appendiceal mucocele: Its diagnostic value of preoperative ultrasonography (in Japanese with English abstract). Nippon Geka Gakkai Zasshi (J Jpn Surg Soc) 87:808-812 8. Kawase H, Ishida K, Satomi A, Aoki N, Takaoka A, Tomimatsu S, Kayano H, Katayama I (1988) A case of intussusception of mucocele of the appendix (in Japanese). Nippon Shokaki Geka Gakkai Zasshi (Jpn J Gastroenterol Surg) 21:147-150 9. Douglas NJ, Cameron DC, Nixon SJ, Rensberg MV, Samuel E (1978) Intussusception of a mucocele of the appendix. Gastrointest Radiol 3:97-100 10. Ogilvie HH (1915) Pseudomyxomatous cyst of the appendix with calcifications of walls. Report of a case. JAMA 64:657658 11. Beck SL, Anderson DJ, Sciorrotta AJ (1987) Mucinous appendiceal cystadenoma complicated by torsion presenting as a retroperitoneal mass. Journal of AOA 87:240-244 12. Li YP, Morin ME, Tan A (1981) Ultrasound findingsin mucocele of the appendix. JCU 9:406-408 13. Horgan JG, Chow PP, Richter JO, Rosenfield AT, Taylor KJW (1984) CT and sonography in the recognition of mucocele of the appendix. AJR 143:959-962 14. Fish B, Smulewicz JJ, Barek L (1981) Role of computed tomography in diagnosis of appendiceal disorders. NY State J Med 81:900-904

Intussusception of an appendiceal mucocele: report of a case.

A 66 year old Japanese female was admitted to our department for the examination and treatment of a mass in the cecum. She had experienced no symptoms...
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