28 March 2018: Articles 
Torsion of the Vermiform Appendix: A Case Report and Review of Literature
Rare disease
Wan Amir Wan Hassan ABCDEFG , Yeng Kwang Tay ABCDEF , Marjan Ghadiri DEDOI: 10.12659/AJCR.908725
Am J Case Rep 2018; 19:365-368
Abstract
BACKGROUND: Torsion of the vermiform appendix is a rare condition that presents with symptoms analogous to those of common acute appendicitis; therefore, it is often diagnosed during surgery. It was first described by Payne et al. in 1918. Since then, there has been wide recognition of a primary and a secondary form of the condition, affecting both the pediatric and adult populations. We present a case of an adult patient and conducted a literature review in the adult demographic.
CASE REPORT: We report the case of a 30-year-old man who presented with clinically acute appendicitis. Laparoscopically, we diagnosed a torsion of the vermiform appendix secondary to a mucocele process. Histology confirmed a low-grade mucinous cystoadenoma, with a hemorrhagic necrosis of the wall, in keeping with torsion.
CONCLUSIONS: Torsion of the vermiform appendix is a rare condition that presents similar to acute appendicitis, and therefore is often diagnosed intraoperatively. Since first described, 33 cases in adults were identified in the English literature, and recognition of a primary or secondary form has emerged. Preoperative radiological imaging is rarely useful in diagnosis. To the best of our knowledge, this is the eighth reported case in the English literature of a torsion of the vermiform appendix secondary to a mucinous cystoadenoma.
Keywords: Appendix, Cystadenoma, Mucinous, Torsion Abnormality
Background
Torsion of the vermiform appendix is a rare condition that presents with symptoms analogous to those of common acute appendicitis; thus, it is often diagnosed during surgery. It was first described by Payne et al. in 1918 [1]. Since then, there has been wide recognition of a primary and a secondary form of the condition, affecting both the pediatric and adult populations. We present a case of torsion of the vermiform appendix secondary to mucinous cystoadenoma, with a review of the literature in the adult demographic.
Case Report
A 30-year-old man presented with a 24-h history of progressive onset of abdominal pain associated with nausea and anorexia. The pain was localized centrally and migrated to the right iliac fossa. He reports there was no radiation of pain or any febrile symptoms. His bedside observations were within normal limits. Abdominal examination revealed significant tenderness with localized peritonism on the right iliac fossa. Rovsing’s sign was also positive. Laboratory findings were un-remarkable except for leukocytosis of 15.1×109/L (normal range 4.0–11.0). Radiological imaging was not performed, as he was clinically diagnosed with acute appendicitis.
Upon laparoscopy, the appendix was located in the right iliac fossa but was grossly distended and gangrenous in appearance (Figure 1). It was rotated 720 degrees anticlockwise at its base. A standard laparoscopic appendicectomy was performed with two 5-mm ports inserted at the left iliac fossa and suprapubic area. The appendix was assessed carefully and deemed appropriate to proceed laparoscopically, as it did not appear to be necrotic or at high risk of rupture with manipulation. Its position was paracecal, which did not require significant laparoscopic manipulation to define the anatomy. The appendix was untwisted completely, and the mesoappendix was dissected with the appendiceal artery clipped and transected. The base of appendix was ligated using 2 loops of polydioxanone suture (PDS) and the appendix was retrieved using a specimen pouch plastic bag. The umbilical port site wound had to be extended inferiorly to deliver the specimen. The patient recovered post-operatively without any complications and was discharged home the next day. The specimen measured 120 mm in length, with a maximum diameter of 30 mm (Figure 2). Histology finding was a low-grade mucinous cystoadenoma, with a hemorrhagic necrosis of the wall, in keeping with torsion.
Discussion
Torsion of the vermiform appendix is a rare disorder that presents with a clinical picture similar to acute appendicitis; therefore, it is often diagnosed intraoperatively, to the surgeon’s surprise. It was first reported in the English literature by Payne et al. in 1918 [1]. Since then, there has been widespread recognition of a primary and secondary form of appendiceal torsion.
In our review of the English literature, 33 cases of torsion of the vermiform appendix in adults were identified, including the present case [1–32] (Table 1). The mean age is 42 years old, with a range of 18 to 79 years old, and a 19: 14 female-to-male sex ratio. The rotation of torsion varies from 180 to 1800 degrees, and although anticlockwise rotation is often reported as the most common rotation, our review of the literature in the adult population shows that clockwise rotation is most common (12 clockwise
Primary torsion has been associated with anatomical variation in which the mesoappendix is fan-shaped, with a narrow base, and the absence of the azygotic fold that normally attaches the appendix laterally, or a long appendix [33,34]. It has also been described as a secondary ischemic or necrotic change with luminal distension distally to the torsion site in the absence of any primary lesion.
Secondary torsion is associated with an identifiable pathology such as a fecalith, mucocele, carcinoid tumor, or cystoadenoma. Theoretically this would cause the appendix to first be engorged and distended, before rendering it unstable and more likely to twist.
Preoperative imaging is often unhelpful in diagnosis. Out of the 33 reported cases in adults, 13 had preoperative imaging that is not a plain film, and of those, only 3 reported cases had a radiologically diagnosed twisted appendix [22,23,30]. Hamada et al. described a target-like appearance at the base of the appendix on ultrasound akin to that seen in cases of ovarian and testicular torsion, whereas in the case reported by Herbert et al., a whorl of mesenteric fat and vessels around the appendiceal axis was seen on CT. On MRI, Stark et al. considered mesenteric edema and abrupt tapering of the base of appendix to be signs of torsion.
Conclusions
Torsion of the vermiform appendix is a rare condition that presents similar to acute appendicitis and is therefore often diagnosed intraoperatively. Since first described, 33 cases in adults were identified in the English literature, and recognition of a primary or secondary form has emerged. Preoperative radiological imaging is rarely useful in diagnosis. To the best of our knowledge, the present case is the eighth report in the English literature of a torsion of the vermiform appendix, secondary to a mucinous cystoadenoma. Whether surgery is carried out in open or laparoscopic approach, great care should be taken to avoid iatrogenic rupture of the appendiceal mucocele. We would not hesitate to convert to open to prevent iatrogenic rupture and spillage of content/cystadenoma in a more difficult situation.
References:
1.. Payne JE, A case of torsion of the appendix: Br J Surg, 1918; 6; 327
2.. Bevers EC, Torsion of the appendix: Lancet, 1920; 1; 597
3.. Flatley G, Torsion of the vermiform appendix associated with pregnancy: Lancet, 1936; 1; 1357
4.. Chan KP, Volvulus complicating mucocele of the appendix: Br J Surg, 1965; 52; 713-14, pmid: 14338327
5.. Ghent WR, Carnovale BV, Primary volvulus of the appendix: Can Med Assoc J, 1966; 95; 926-27, pmid: 5921752
6.. De Bruin AJ, Torsion of appendix: Med J Aust, 1969; 1; 581, pmid: 5782098
7.. Killiam AR, An unusual case of appendicitis: Torsion produced by a mesoappendiceal lipoma: Ann Surg, 1969; 35; 648-49
8.. Legg NGM, Torsion complicating mucocele of appendix: J R Coll Surg Edinb, 1973; 18(4); 236, pmid: 4719798
9.. Finch DRA, Torsion of appendix: Br J Clin Pract, 1974; 28(11); 391-92, pmid: 4461040
10.. Cassie GF, Torsion of mucocele of the appendix caused by a carcinoid tumor: Br J Surg, 1953; 41(165); 105-6, pmid: 13082023
11.. Petersen KR, Brooks L, Pedersen H, Torsio appendicis vermiformis. Report of an unusual case: Acta Chir Scand, 1982; 148(4); 383-84, pmid: 7136443
12.. Dickson DR, Jennings WK, Mucocele of the appendix complicated by torsion and gangrene: Calif Med, 1953; 79(4); 317-18, pmid: 13094547
13.. Won OH, Waxman M, Torsion of vermiform appendix: JAMA, 1977; 237(13); 1312-13
14.. Abu Zidan FM, al-Hilaly MA, al-Atrabi N, Torsion of a mucocele of the appendix in a pregnant woman: Acta Obstet Gynecol Scand, 1992; 71(2); 140-42, pmid: 1316043
15.. Moten AL, Williams RS, Torsion of the appendix: Med J Aust, 2002; 177(11–12); 632
16.. Tzilinis A, Vahedi MH, Wittenborn WS, Appendiceal torsion in an adult: Case report and review of literature: Curr Surg, 2002; 59(4); 410-11, pmid: 16093177
17.. Bowling CB, Lipscomb GH, Torsion of the appendix mimicking ovarian torsion: Obstet Gynecol, 2006; 107(2 Pt 2); 466-67, pmid: 16449151
18.. Bestman TJR, van Cleemput M, Detournay G, Torsion of the vermiform appendix: A case report: Acta Chir Belg, 2006; 106(2); 228-29, pmid: 16761485
19.. Rajendran N, Ameen S, Rohatgi A, Laparoscopic management of a torted appendix: Ann R Coll Surg Engl, 2006; 88(6); W6-7
20.. Rudloff U, Malhotra S, Volvulus of an appendiceal mucocele: Report of a case: Surg Today, 2007; 37(6); 514-17, pmid: 17522774
21.. Kitagawa M, Kotani T, Yamano T, Secondary torsion of vermiform appendix with mucinous cystadenoma: Case Rep Gastroenterol, 2007; 1(1); 32-37, pmid: 21487469
22.. Hebert JJ, Pickhardt PJ, MDCT diagnosis of an appendiceal mucocele with acute torsion: Am J Roentgenol, 2007; 189(1); W4-6, pmid: 17579135
23.. Hamada T, Kosaka K, Shigeoka N, Torsion of the appendix secondary to appendiceal mucocele: Gray scale and contrast-enhanced sonographic findings: J Ultrasound Med, 2007; 26(1); 111-15, pmid: 17182716
24.. Wani I, Kitagawa M, Rather M, Torsion of vermiform appendix with fecalith: A case report: Cases J, 2008; 1(1); 20, pmid: 18577201
25.. Wani I, Maqbool M, Sheikh T, Secondary torsion of vermiform appendix: J Emerg Trauma Shock, 2010; 3(2); 206-7
26.. Lee CH, Lee MR, Kim JC, Torsion of a mucocele of the vermiform appendix: A case report and review of the literature: J Korean Surg Soc, 2011; 81(Suppl.1); S47-50, pmid: 22319738
27.. Marsdin EL, Griffiths C, Primary torsion of vermiform appendix mimicking acute appendicitis: BMJ Case Rep, 2011; 2011 pii: bcr0320113979
28.. Dimitriadis PA, Makar RR, Randall JK, Ramus J, Appendiceal torsion associated with undescended caecum: A case report and review of the literature: BMJ Case Rep, 2012; 2012 pii: bcr2012006932
29.. Mishin I, Ghidirim G, Zastavnitsky G, Popa C, Torsion of an appendiceal cystadenoma. Report of a case and review of literature: Ann Ital Chir, 2012; 83(1); 75-78, pmid: 22352223
30.. Stark C, Jousi M, Enholm B, Preoperative assessment and treatment of appendiceal mucocele complicated by acute torsion: A case report: BMC Res Notes, 2014; 7; 1, pmid: 24382056
31.. Grebic D, Lovasic F, Benjak I, Lovasic I, Secondary torsion of vermiform appendix with mesoappendiceal lipoma: Ann Saudi Med, 2015; 35(2); 173-75, pmid: 26336028
32.. Dubhashi SP, Khadav B, Appendicular torsion: Niger J Surg, 2016; 22(1); 41-42, pmid: 27013858
33.. Dewan PA, Woodward A, Torsion of the vermiform appendix: J Pediatr Surg, 1986; 21(4); 379, pmid: 3701558
34.. Sarin YK, Pathak D, Torsion of vermiform appendix: Indian Pediatr, 2006; 43(3); 266-67, pmid: 16585826
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