Appendiceal mucocoeles occur when there is abnormal accumulation of mucus within the appendix. The tenacious and viscous mucus causes obstruction of the appendiceal neck and results in dilatation of the lumen.
The reported prevalence at appendectomy is 0.2-0.3%. They are thought to typically present in middle aged individuals.
The term mucocele is simply a macroscopic description of an appendix that is grossly distended by mucus 7. They result from chronic appendiceal obstruction that may be caused by either benign or malignant lesions:
- mucosal hyperplasia (most common 11)
- mucinous neoplasms
- appendiceal carcinoid
- adjacent caecal tumour
- myxoglobulosis: seen as with multiple small intraluminal globules which can calcify and produce 1-10 mm mobile calcifications
Fluoroscopy: barium enema
If a contrast examination is performed, there is usually non-filling or partial-filling of the appendix. Where there is a large mucocoele, the associated mass effect can cause indentation or lateral displacement of the caecum.
Typically cystic mass with variable internal echogenicity 8. The presence of an "onion sign" (sonographic layering within a cystic mass) is considered a highly suggestive feature 2,6.
- typically seen as a well-circumscribed, low-attenuation, spherical or tubular mass contiguous with the base of the caecum
- the finding of curvilinear mural calcification suggests the diagnosis, but is seen in less than 50% of cases
- intra-luminal bubbles of gas, or an air-fluid level within a mucocele suggest the presence of superinfection, which can occur in both benign and malignant mucocoeles
Seen as a rounded right iliac fossa mass.
Typical signal characteristics include:
- T1: depending on the mucin concentration, the signal may be variably hypointense to isointense 9
- T2: hyperintense
Treatment and prognosis
Treatment is usually surgical.
- rupture: may lead to pseudomyxoma peritonei if the underlying cause is neoplastic 11
- can act as a lead point and lead to an ileo-colic intussusception 9
Differentiating benign (non-neoplastic mucocoele and mucinous cystadenoma) and malignant (mucinous cystadenocarcinoma) appendiceal lesions can be difficult. Wang et al 10 found a statistically significant difference in wall irregularity and soft-tissue thickening between malignant and benign cases.
- 1. Bennett GL, Tanpitukpongse TP, Macari M et-al. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol. 2009;192 (3): W103-10. doi:10.2214/AJR.08.1572 - Pubmed citation
- 2. Caspi B, Cassif E, Auslender R et-al. The onion skin sign: a specific sonographic marker of appendiceal mucocele. J Ultrasound Med. 2004;23 (1): 117-21. J Ultrasound Med (full text) - Pubmed citation
- 3. Bartlett C, Manoharan M, Jackson A. Mucocele of the appendix - a diagnostic dilemma: a case report. J Med Case Rep. 2007;1 : 183. doi:10.1186/1752-1947-1-183 - Free text at pubmed - Pubmed citation
- 4. Madwed D, Mindelzun R, Jeffrey RB. Mucocele of the appendix: imaging findings. AJR Am J Roentgenol. 1992;159 (1): 69-72. AJR Am J Roentgenol (citation) - Pubmed citation
- 5. Attarde V, Patil P, Apte A et-al. Sonographic appearance of a giant appendicular mucocele. J Clin Ultrasound. 2011;39 (5): 290-2. doi:10.1002/jcu.20809 - Pubmed citation
- 6. Francica G, Lapiccirella G, Giardiello C et-al. Giant mucocele of the appendix: clinical and imaging findings in 3 cases. J Ultrasound Med. 2006;25 (5): 643-8. J Ultrasound Med (citation) - Pubmed citation
- 7. Pickhardt PJ, Levy AD, Rohrmann CA et-al. Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 2003;23 (3): 645-62. Radiographics (full text) - doi:10.1148/rg.233025134 - Pubmed citation
- 8. Kim SH, Lim HK, Lee WJ et-al. Mucocele of the appendix: ultrasonographic and CT findings. Abdom Imaging. 1998;23 (3): 292-6. Pubmed citation
- 9. Honnef I, Moschopulos M, Roeren T. Appendiceal mucinous cystadenoma. Radiographics. 2008;28 (5): 1524-7. Radiographics (full text) - doi:10.1148/rg.285075160 - Pubmed citation
- 10. Wang H, Chen YQ, Wei R et-al. Appendiceal mucocele: A diagnostic dilemma in differentiating malignant from benign lesions with CT. AJR Am J Roentgenol. 2013;201 (4): W590-5. doi:10.2214/AJR.12.9260 - Pubmed citation
- 11. Spyropoulos C, Rentis A, Alexaki E et-al. Appendiceal mucocele and pseudomyxoma peritonei; the clinical boundaries of a subtle disease. Am J Case Rep. 2014;15: 355-60. doi:10.12659/AJCR.890837 - Free text at pubmed - Pubmed citation
The appendix can be affected by numerous inflammatory, infectious and neoplastic conditions:
- appendiceal mucocoele
neoplasms of the appendix
- appendiceal adenoma
- appendiceal adenocarcinoma
- appendiceal lymphoma
- appendiceal mucinous cystadenoma
- appendiceal mucinous adenoma
- appendiceal mucinous cystadenocarcinoma
- appendiceal mucinous adenocarcinoma
- appendiceal neuroendocrine tumours