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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 8  |  Issue : 2  |  Page : 91-94

Mucocele of the appendix: A case report and review of literature


Department of Surgery, Aminu Kano Teaching Hospital Kano, PMB 3052, Kano, Nigeria

Date of Web Publication30-May-2019

Correspondence Address:
Dr. Ibrahim Umar Garzali
Department of Surgery, Aminu Kano Teaching Hospital Kano, PMB 3052, Kano
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_34_18

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  Abstract 


Mucocele represents mucinous dilation of the lumen of vermiform appendix resulting from obstruction or increase secretion. It is a rare disease with an incidence of 0.2–0.7% of all appendectomies and 8–10% of all appendiceal tumours. The clinical presentation is atypical with most cases usually asymptomatic. Mucocele of the appendix indicates dilation of the appendix lumen irrespective of the aetiology. Four types of appendiceal mucocele are defined by the cause of obstruction: retention cysts, epithelial hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma. Mucocele of the appendix is a rare condition of the appendix that requires high index of suspicion for a definitive preoperative diagnosis. The treatment for appendiceal mucocele is typically surgical resection. We report a case of a 37-year-old woman with recurrent right iliac fossa pain of 20 years duration. Abdomen was full, moved with respiration with a positive pointing sign. There was marked right iliac fossa tenderness with rebound tenderness and guarding. She had an abdominopelvic ultrasonography that revealed features of acute appendicitis. She had surgery with intraoperative finding of marked distension of the appendix from the tip to the base with necrosis at the base, a right hemicolectomy was done.

Keywords: Hemicolectomy, mucocele, vermiform appendix


How to cite this article:
Garzali IU, Elyakub AI, Suleiman IE, Oloko NL, Muktar IA. Mucocele of the appendix: A case report and review of literature. Arch Int Surg 2018;8:91-4

How to cite this URL:
Garzali IU, Elyakub AI, Suleiman IE, Oloko NL, Muktar IA. Mucocele of the appendix: A case report and review of literature. Arch Int Surg [serial online] 2018 [cited 2019 Aug 24];8:91-4. Available from: http://www.archintsurg.org/text.asp?2018/8/2/91/259460




  Introduction Top


Mucocele represents mucinous dilation of the lumen of vermiform appendix resulting from obstruction or increase secretion.[1],[2] It was first described by Rokitansky in 1842.[3] It is a rare disease with an incidence of 0.2–0.7% of all appendectomies and 8–10% of all appendiceal tumours.[4],[5],[6] Females are predominantly affected with highest incidence seen after 40 years of age.[5],[7],[8] There are four histologic types of appendiceal mucocele: retention cyst, mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma.[5],[8],[9] The clinical presentation is atypical with most cases usually asymptomatic, those that are symptomatic may present with features of recurrent acute appendicitis with incidental finding during appendectomy, as was this case.[7],[9],[10],[11] Other mode of presentations include: peritonitis especially after rupture of the mucocele, abdominal mass, pelvic mass, gastrointestinal bleeding and a single case has been reported to present with haematuria.[6],[10],[12],[13],[14] The treatment for appendiceal mucocele is typically surgical resection. Surgical approaches for appendiceal mucocele vary from simple appendectomy to right hemicolectomy with lymph node dissection depending on their malignancy. However, due to the difficulty in differentiating cystadenoma from cystadenocarcinoma before surgery, overtreatment is often done, as was done for our patient.[15] Great care is required in handling a mucocele during surgery as it can rupture and cause pseudomyxoma peritonei which has a high mortality.[6],[9],[16]


  Case Report Top


We report a case of a 37-year-old woman with recurrent right iliac fossa (RIF) pain of 20 years duration. The pain was said to be colicky, intermittent pain that stops her from walking upright during the attack. It does not radiate to any part of the body. Associated nausea, anorexia but no fever, vomiting or alteration in the bowel habit and no genitourinary symptoms. Examination revealed a middle-aged woman in painful distress, afebrile, anicteric and not pale. Pulse was 92 per minute with a blood pressure of 120/80 mm Hg. She had a respiratory rate of 20 cycle per minute with vesicular breath sound. Abdomen was full, moved with respiration with a positive pointing sign. There was marked RIF tenderness with rebound tenderness and guarding. No intra-abdominal organ palpable. She had an abdominopelvic ultrasonography that revealed probe tenderness, but was not able to visualise the appendix. Baseline complete blood count, electrolyte, urea and creatinine were normal. She was counselled for appendectomy and informed consent obtained. Surgery was done under general anaesthesia via a Lanz incision deepened to the peritoneal cavity with intraoperative finding of marked distension of the appendix from the tip to the base with necrosis at the base, an ileocolic resection with end-to-end ileocolic anastomosis was done, wound closed in layers. She commenced oral sips at Day 4 postoperatively and discharged at Day 8. She came for follow up 7 days after discharge with histologic evaluation which revealed appendix lumen lined by mucus secreting epithelial cells with bland nuclei. The lumen showed mucin pool while the wall showed modest lymphoplasmocytic infiltrates. A diagnosis of mucocele of the appendix from retention was made.


  Discussion Top


Mucocele of the appendix indicates dilation of the appendix lumen irrespective of the aetiology.[1],[2],[6],[7],[8],[9] It was recognised as a pathological entity in 1842 by Rokitansky while the definition was standardised by Ferren in 1876.[3],[6],[8],[9] Mucocele of the appendix is divided into four pathological subgroups based on the epithelial characteristics.[6],[7],[9]

The first group consists of a simple retention cyst secondary to occlusion of the appendix by fecalith, scar tissue from previous inflammation or in rare cases due to endometriosis.[9],[11],[17] It has a normal or flattened epithelium, moderate luminal dilatation of up to 2 cm and it constitutes about 20% of all appendiceal mucoceles.[9] The case presented falls under this group. However, it was much larger than expected for this pathologic type.

The second group with hyperplastic epithelium and moderate luminal dilatation: this constitutes about 20% of all mucocele of the appendix.[9],[11]

Benign mucinous cystadenoma forms the third group: this is characterised by tubular adenomatous epithelium with varying degree of epithelial atypia. It produces large amounts of mucin with prominent luminal dilatation of up to 6 cm. It is the most common form, constituting about 50% of cases and with associated 20% risk of perforation.[8],[9],[11]

Finally, the fourth group encompasses the malignant mucinous cystadenocarcinomas, characterised by glandular stromal invasion and/or tumour cells in peritoneal implants, i.e. pseudomyxoma peritonei. It sometimes resembles mucinous carcinoma of the colon. It constitutes about 11–20% of all cases with 6% risk of spontaneous rupture.[8],[9],[11],[17] Cystadenoma and cystadenocarcinoma are neoplastic appendiceal mucocoeles, they constituting about 35% of all primary neoplasms of the appendix.[6],[9],[17] These lesions may occur de novo or from pre-existing simple mucocoeles.[9]

Approximately 23–50% of patients are asymptomatic, with the lesions being discovered incidentally during surgery, radiological evaluations or endoscopic procedures.[8],[11],[17],[18] These presentations include symptoms and signs of acute appendicitis, history of painless or painful RIF mass, abdominal distension with discomfort especially in cases of spontaneous rupture, features of intestinal obstruction from mucocele associated intussusceptions, incidental findings of calcified mass on plain abdominal radiograph and a well-circumscribed mass displacing the caecum medially on barium enema.[7],[9],[10],[11] Our patient presented with recurrent RIF pain for 20 years, this is similar to three of the five cases reported by Karakaya et al. in their case series and also cases reported by Dogo et al.[13],[16]

Preoperative definitive diagnosis of mucocele of the appendix is important for effective planning of adequate surgical intervention. This is, however, very difficult as most cases are diagnosed as acute appendicitis both clinically and by the relevant radiologic investigations.

Abdominopelvic ultrasound scan play a role in the diagnosis of mucocele if high index of suspicion is employed especially in patients with long-standing recurrent acute appendicitis. The findings include: atypical image, called 'onion skin', is an important sonographic marker of appendiceal mucocele. Visualisation of the appendix and its diameter exceeding 15 mm is considered as specific appendiceal mucocele index with sensitivity of 83% and specificity of 92%. Discontinuity of the appendiceal wall with leakage of the internal contents into surrounding tissues indicates rupture of the mucocele. In cases of pseudomyxoma peritonei, typical ultrasound findings are non-mobile, sonogenic ascites with multiple semi-solid masses and scalloping of the hepatic and splenic margins.[5],[9],[11],[15] Our patient had abdominopelvic USS which was normal.

Abdominopelvic computed tomography scan diagnosed mucocele of the appendix with high accuracy by finding the following features: low density, thin-walled, encapsulated mass communicating with the caecum and calcifications which may be present in about 50% of cases and an appendix lumen >1.3 cm.[8],[9],[17]

Colonoscopy may reveal a characteristic mass with a central crater from which mucinous material exudes. This is known as 'volcano sign', it also rules out synchronous or metachronous colonic tumour.[8],[9],[17]

Treatment of mucocele of the appendix is surgery which can be open or laparoscopic; the laparoscopic approach has been discouraged because of the risk of rupture of the mucocele with resulting pseudomyxoma peritonei. Open surgery also has advantage of direct inspection of the appendix, mesoappendix and lymphnodes.[8],[9],[13],[15]

Appendectomy is considered to be sufficient in case of mucous hyperplasia or cystadenoma with an intact appendiceal base, as no long-term follow-up is required in this case.[8],[9],[18] The prognosis for retention cysts and adenomas is excellent: 5-year survival is 91–100%. No long-term follow up is needed for these patients.[9]

In case of cystadenoma with positive boundaries, i.e. base of the appendix is affected, caecum resection is necessary.[5],[9],[15] However, if there are features suggestive of cystadenocarcinoma like palpable lymph nodes, right hemicolectomy with thorough lymph dissection must be performed.[5],[9],[16] Long-term follow up is obligatory for these patients.

There is some difficulty in differentiating mucocele from neoplastic or non-neoplastic pathology during surgery, so there is tendency to overtreat all patients with right hemicolectomy.[6],[11] This was the case in our patient.


  Conclusion Top


Mucocele of the appendix is a rare condition of the appendix that requires high index of suspicion for a definitive preoperative diagnosis which will influence the operative approach and prevent complications like pseudomyxoma peritonei. It should be considered in patients with long standing features of recurrent acute appendicitis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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Rokitansky C. A manual of pathological anatomy, vol ii. In: Charles H, William E, editors. Philadelpia: Sherman and Son, Printers; 2016. p. 1855.  Back to cited text no. 3
    
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Asenov Y, Korukov B, Penkov N, Sedloev T, Tihtchev V, Hadzhiysca V, et al. Appendiceal mucocele-case report and review of the literature. Chirurgia (Bucur) 2015;110:565-9.  Back to cited text no. 5
    
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Akbulut S, Tas M, Sogutcu N, Arikanoglu Z, Basbug M, Ulku A, et al. Unusual histopathological findings in appendectomy specimens : A retrospective analysis and literature review. World J Gastroenterol 2011;17:1961-70.  Back to cited text no. 7
    
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Idris LO, Olaofe OO, Adejumobi OM, Kolawole AO, Jimoh AK. Case report – Open access international journal of surgery case reports giant mucocele of the appendix in pregnancy : A case report and review of literature case report – Open access. Int J Surg Case Rep [Internet] 2015;9:95-7.  Back to cited text no. 9
    
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Alese O, Irabor D. Mucinous cystadenoma of the appendix: A case report. Afr Health Sci 2010;10:99-100.  Back to cited text no. 10
    
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Rabie ME, Al Shraim M, Al Skaini MS, Alqahtani S, El Hakeem I, Al Qahtani AS, et al. Mucus containing cystic lesions “Mucocele” of the appendix: The unresolved issues. Int J Surg Oncol 2015;2015. doi: 10.1155/2015/139461.  Back to cited text no. 12
    
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Karakaya K, Barut F, Emre AU, Ucan HB, Cakmak GK, Irkorucu O. Appendiceal mucocele : Case reports and review of current literature appendiceal mucocele ucoceleeal rts and review of current literature erature Gastroenterol 2008;14:2280-3.  Back to cited text no. 13
    
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Oliphant U, Rosenthal A. Hematuria: An unusual presentation for mucocele of the appendix. Case report and review of literature. JSLS 1999;3:71-4.  Back to cited text no. 14
    
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Shiihara M, Ohki T, Yamamoto M. Preoperative diagnosis and surgical approach of appendiceal mucinous cystadenoma : Usefulness of volcano sign. Case Rep Gastroenterol 2017;11:539-44.  Back to cited text no. 15
    
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Faure M, Antwerpen UZ, Salgado RA, Antwerpen UZ, Antwerpen UZ, Parizel PM. Mucocele of the appendix : Case report and review of literature mucocele of the appendix : Case report and review of literature. JBR-BTR 2014;97:217-21.  Back to cited text no. 17
    
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