A rare Acute Abdomen: Appendix Duplication

Abstract

Turkish Abstract

Introduction

Acute appendicitis (AA) is the most common non-traumatic emergency surgical pathology. The lifetime risk for the development of acute appendicitis in the general population has been reported to be 7% ¹. Clinically, AA is characterized by nausea and periumbilical pain initially beginning in the visceral phase, followed by appendix locus pain in the parietal phase. The appendix is localized posteromedially and is on the inferior ileocecal valve of the caecum in 65% of the population ². However, the position of the appendix may be para-ileal, post-ileal, promontory, pelvic, sub-caecal, paracolic and retro-caecal in 35% of the population ². Additionally, clinical differences can be noted depending on the congenital location and number of anomalies of the appendix. Here, we a patient with appendix vermiformis duplex diagnosed during emergency laparotomy. The two appendices had two different origins.

Case Report

A 24-year-old male was admitted to the emergency services with a complaint of abdominal pain 12 h previously. He was found to have defensive tenderness and rebound positivity at McBurney?s point. On abdominal computed tomography (CT)scans, an 8-mm-diameter inflated appendix was observed. His white blood cell count was 15400/mm³. On intraoperative exploration, an inflamed appendix measuring 1 cm in diameter and 8 cm in length was observed on the caecum 2 cm away from a non-inflamed appendix measuring 4mm in diameter and 3 cm in length. Appendectomy was performed for both appendices. On the third postoperative day, he was discharged without any problems. The patient was examined for congenital malformation of other intraabdominal organs, and no other malformation was detected. Intraoperative image of duplication of appendix and microscopic images of both appendices are shown in the figures.

Figure 1 Intraoperative image of appendix duplication

Figure 2 Microscopic images of both appendices

Discussion

Appendix duplication was first described by Picoli in 1892 in a female patient with a double uterus, double colon and double vagina ^3,4. In 1968, Tinckler?s description of the double penis, double bladder and triple appendix revealed that appendiceal congenital anomalies can coexist with other abdominal organ congenital malformations ³. Till now, fewer than 100 cases have been reported ⁵.

The aetiology of double appendix or horseshoe appendix is still unclear ⁶. Collins reported an appendix duplication rate of 1 / 12,500 ageneses and 1 / 25,000 duplications in 50,000 appendices specimen, and Kjossev reported an appendix duplication rate of 1/10.956 ^7,8. A classification system for duplication was first developed by Cave in 1936 ^9,10, and it was modified by Wallbridge in 1963 ¹¹ and then by Biermann in 1993 ¹². A complete classification system was introduced by Calota et al ¹³. This system classified anomalies according to the number and shape of anomalies.

Number Anomalies

1. Congenital agenesis
2. Multiforious appendices:

A: appendiceal duplication (partially) in ?Y-shaped?

B: duplex appendix on a single cecum:

• B₁ - ?avian type/bird-like ? with intestinal and/or genitourinary anomalies
• B₂- ?taenia coli cecum type? without other anomalies
• B₃ - ?taenia coli hepatic flexura type?
• B₄- ?taenia coli splenic flexura type?

C: duplex appendix on two caeca with hindgut, genitourinary tract, lower vertebral column maldevelopment

D: triplex appendix:

• ?new-born type? with/without other congenital anomalies
• ?adult type? without other congenital anomalies

Shape Anomalies

            Horseshoe-shaped appendix:

                        -with frontal disposal

                        -with sagittal disposal

According to the Calota classification system, the present case could be classified as type B2. In addition to a physical examination, ultrasonography (USG) and abdominal CT are used as gold standard diagnostic methods for AA. However, in appendix duplication, USG and CT are not very useful. Preoperative diagnosis of a duplicated appendix is not easy ¹⁴.The diagnosis is usually made intraoperatively or in the post-mortem autopsy. Two separate lumens can be very rarely seen in the barium graphite. Appendix duplication should be distinguished from a solitary diverticulum of the caecum and from appendiceal diverticulosis ¹⁵. Treatment involves the excision of both appendices, in particular, to avoid medicolegal responsibilities. In our case, a single lumen was observed on performing abdominal CT and duplication was seen intraoperatively, as in general clinical practice. Two appendectomies were performed to avoid medicolegal problems.

In conclusion, we presented a rare case of appendix vermiformis duplex diagnosed during emergency laparotomy.

References

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Information Presentation

11^th National Trauma and Emergency Surgery Congress in 2017 in Turkey