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Year : 2021  |  Volume : 4  |  Issue : 2  |  Page : 47-49

A rare case of duplicated appendix presenting as acute appendicitis

Department of General Surgery, Mallareddy Institute of Medical Sciences, Hyderabad, Telangana, India

Date of Submission07-May-2021
Date of Decision01-Jul-2021
Date of Acceptance06-Jun-2021
Date of Web Publication09-Nov-2021

Correspondence Address:
Dr. Shiva Kumar Rathlawath
MIG-2/180, 9th Phase, Kphb Colony, Hyderabad - 500 085, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijcs.ijcs_5_21

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Appendiceal duplications are one among the rare congenital anomalies of the gastrointestinal tract. This is an interesting case report of a 24-year-old male who was diagnosed to have acute appendicitis. On exploration, the patient had duplicated appendix; both were inflamed and adherent to each other with separate base. Appendectomy was performed on both, and histopathology confirmed appendicitis in both appendices. Duplicated appendix represents a challenging clinical scenario in case of right iliac fossa pain. Surgeons should be aware of this rare congenital anomaly to avoid missing the duplication that may result in serious life-threatening consequences.

Keywords: Appendiceal duplication, appendicitis, Cave-Wallbridge classification

How to cite this article:
Damera VV, Rathlawath SK. A rare case of duplicated appendix presenting as acute appendicitis. Indian J Colo-Rectal Surg 2021;4:47-9

How to cite this URL:
Damera VV, Rathlawath SK. A rare case of duplicated appendix presenting as acute appendicitis. Indian J Colo-Rectal Surg [serial online] 2021 [cited 2022 May 20];4:47-9. Available from: https://www.ijcrsonweb.org/text.asp?2021/4/2/47/330167

  Introduction Top

The vermiform appendix is a diverticulum arising from the posteromedial wall of cecum about 2 cm inferior to the ileocecal orifice. Variation in the size and position of the appendix is a common finding, but duplication is rare. The incidence of appendiceal duplication is estimated to range from 0.004% to 0.009%, which was first described in 1892 by Picoli.[1],[2] About 141 cases of double appendix have been reported, worldwide.[3] In adults, usually, it constitutes an incidental finding during laparotomy for a different cause.[4]

  Case Report Top

A 24-year-old male presented to the emergency with right lower quadrant pain over the past 48 h with low-grade fever. Initially, pain was present in the periumbilical region which migrated to the right iliac fossa. On clinical evaluation, the patient was febrile (38.2°C) and had tachycardia. Examination of the abdomen revealed localized tenderness at McBurney's point. Laboratory findings showed leukocytosis (16,300 cells/mm3).

Abdominal ultrasound was done and revealed aperistaltic noncompressible tubular blind ending structure measuring 9 mm in the right iliac fossa, suggestive of acute appendicitis.

The patient was planned for open appendectomy. McBurney's incision was given, and on exploration, there were omental adhesions; on further dissection, two tubular structures were identified which were adherent to each other with separate base to single cecum and located on tenia coli [Figure 1]. Both the appendices were inflamed and appendectomy was performed. The patient had uneventful recovery and was discharged on the 5th postoperative day.
Figure 1: Intraoperative picture showing two inflamed appendices

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Histopathology confirmed the intraoperative findings of appendiceal duplication, with features of acute appendicitis of one specimen and acute suppurative necrotizing appendicitis of the second specimen [Figure 2] and [Figure 3].
Figure 2: Low-power microscopic view of first specimen with acute appendicitis. Image on the left showing the structure of appendix and on the right side showing neutrophils infiltrating the muscle layer

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Figure 3: Low-power microscopic view of the second structure. Image on the left side showing the histologic composition similar to appendix and section on the right side showing acute inflammatory infiltrate (mainly neutrophils) infiltrating the muscle layer

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  Discussion Top

Appendiceal duplication is a rare entity with reported incidence of two cases in 50,000 appendices.[1] Appendiceal duplications were first classified by Cave in 1936[5] and later modified by Wallbridge[6] in 1963 and Biermann[7] in 1993 [Table 1]. There are reports of other rarer forms of anatomic variation of appendix such as horseshoe appendix[8] and triple appendix.[9]
Table 1: ModifiedCave-Wallbridgeclassification

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Although the normal embryogenesis of appendix is well known, the exact cause of appendiceal duplication is not clear. Several theories were proposed to explain the embryological origin of appendicular duplication. These include persistence of transient appendix-like structure in Type B2 and partial fusion of transient appendix-like structure with the normal appendix precursor in Type A. Type B1 duplication has been attributed to the failure of proper differentiation of the cloaca and Type C to the partial twinning of hindgut structures.[10]

Type B1 and Type C variations are often associated with other congenital abnormalities, in particular, duplications of reproductive organs and imperforate anus. In the most common variation, Type B2, there are no associated congenital abnormalities and only intraoperative findings might indicate a concealed duplication.[3]

Our case was identified as Type B2 which is the most common subtype. A double appendix may be either asymptomatic which is detected incidentally or may present with symptoms due to inflammation of one or both appendix. Preoperative diagnosis of appendiceal duplication is usually difficult during routine imaging examinations. In this case, the patient presented to the emergency with acute abdominal pain, and ultrasound was suggestive of inflamed appendix but did not show second appendix. Intraoperatively, we identified the two appendices and resected both. Appendiceal duplication was confirmed histologically by the presence of lumens that are lined by appendiceal mucosa, lymphoid follicles, and two layers of musculature.

Appendiceal duplication must be distinguished from solitary diverticulum of cecum, which is found on the inner aspect of the ileocecal angle and does not contain lymphoid tissue histologically.[6]

  Conclusion Top

Although appendiceal duplication occurs rarely, awareness of this congenital anomaly and thorough intraoperative inspection is essential to avoid potential life-threatening complications and medicolegal consequences.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Collins DC. A study of 50,000 specimens of the human vermiform appendix. Surg Gynecol Obstet 1955;101:437-45.  Back to cited text no. 1
Kjossev KT, Losanoff JE. Duplicated vermiform appendix. Br J Surg 1996;83:1259.  Back to cited text no. 2
Nageswaran H, Khan U, Hill F, Maw A. Appendiceal duplication: A comprehensive review of published cases and clinical recommendations. World J Surg 2018;42:574-81.  Back to cited text no. 3
Christodoulidis G, Symeonidis D, Spyridakis M, Koukoulis G, Manolakis A, Triantafylidis G, et al. Acute appendicitis in a duplicated appendix. Int J Surg Case Rep 2012;3:559-62.  Back to cited text no. 4
Cave AJ. Appendix Vermiformis Duplex. J Anat 1936;70:283-92.  Back to cited text no. 5
Wallbridge PH. Double appendix. Br J Surg 1962;50:346-7.  Back to cited text no. 6
Biermann R, Borský D, Gogora M. Die Appendicitis duplex–Eine pathologische Rarität (Double appendicitis–A rare pathologic entity). Chirurg 1993;64:1059-61.  Back to cited text no. 7
Mesko TW, Lugo R, Breitholtz T. Horseshoe anomaly of the appendix: A previously undescribed entity. Surgery 1989;106:563-6.  Back to cited text no. 8
Tinckler LF. Triple appendix vermiformis–A unique case. Br J Surg 1968;55:79-81.  Back to cited text no. 9
Coker DD, Hartong JM, Conklin HB. Duplication of the vermiform appendix: Case report. Mil Med 1975;140:331-2.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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