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CASE REPORT – OPEN ACCESSInternational Journal of Surgery Case Reports 4 (2013) 54– 57

Contents lists available at SciVerse ScienceDirect

International Journal of Surgery Case Reports

j ourna l ho me pa ge: www.elsev ier .com/ locate / i j scr

n unusual cause of acute appendicitis: Appendiceal endometriosis

rif Emrea, Sami Akbulutb,∗, Mehmet Yilmazb, Zehra Bozdagc

Department of Surgery, Malatya State Hospital, 44300 Malatya, TurkeyDepartment of Surgery, Inonu University Faculty of Medicine, 44280 Malatya, TurkeyDepartment of Pathology, Malatya State Hospital, 44300 Malatya, Turkey

r t i c l e i n f o

rticle history:eceived 8 May 2012eceived in revised form 17 July 2012ccepted 29 July 2012vailable online 19 September 2012

eywords:ppendicitisndometriosisnusual presentation

a b s t r a c t

INTRODUCTION: While endometriosis is a common disorder in women of reproductive age, appendicealendometriosis accounts for less than 1% of all pelvic endometriotic lesions. Appendiceal involvementmay present as acute appendicitis and definitive diagnosis is made by only postoperative histologicalexamination.PRESENTATION OF CASE: In this study, we present two cases of female patients who underwent an appen-dectomy presumed diagnosis as acute appendicitis, and a histopathological examination of the retrievedspecimen revealed appendiceal endometriosis.DISCUSSION: Endometriosis is defined as the presence of ectopic endometrial tissue outside the liningof the uterine cavity. Gastrointestinal endometriosis is observed in 3–37% of all endometriosis cases,whereas appendiceal endometriosis accounts for only about 3% of gastrointestinal endometriosis. Appen-

diceal endometriosis is usually asymptomatic, although it sometimes causes abdominal cramps, nausea,chronic pelvic pain, lower gastrointestinal hemorrhage, intussusception, perforation, or acute appendici-tis.CONCLUSION: Appendiceal endometriosis is an unusual histopathological finding. A preoperative diag-nosis is difficult, but this condition should be considered when women of childbearing age present withclinical symptoms of acute appendicitis.

© 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

. Introduction

Endometriosis, defined as the presence of endometrial glandsnd stroma outside the uterine cavity, is estimated to affect 4–50%f reproductively aged women and results in pelvic pain andnfertility in up to 50% of these patients.1–3 Endometriosis is mostommonly found in the gynecologic organs and pelvic peritoneumut may also involve the gastrointestinal system, greater omen-um, surgical scars, and the mesentery, but is rarely found atistant sites such as the kidney, lungs, skin, and nasal cavity.4

astrointestinal endometriosis (GE) is observed in 3–37% of allndometriosis cases, but appendiceal endometriosis (AE) accountsor only about 3% of GE and <1% of total endometriosis cases.5–7 AE

ay not only cause symptoms of acute and chronic appendicitisut also causes cyclic and chronic right lower quadrant pain,elena, lower intestinal hemorrhage, cecal intussusception, and

ntestinal perforation.3 Here, we describe two cases of AE thatresented as acute appendicitis.

∗ Corresponding author at: Department of Surgery, Division of Liver Transplanta-ion, Inonu University Faculty of Medicine, 44280 Malatya, Turkey.

E-mail address: akbulutsami@gmail.com (S. Akbulut).

210-2612/$ – see front matter © 2012 Surgical Associates Ltd. Published by Elsevier Ltdttp://dx.doi.org/10.1016/j.ijscr.2012.07.018

2. Case reports

2.1. Case-1

A 31-year-old female patient was admitted to our emergencyclinic with a complaint of right lower quadrant abdominal pain,which had persisted for 3 days and had deteriorated progressively.The patient stated that the pain she suffered from was not associ-ated with her menstrual cycle. The patient already had one child,and had been using an intrauterine device for contraceptive pur-poses for the last 2 years. Her medical history was unremarkableother than a laparoscopic cholecystectomy undertaken 6 yearsbefore. The vital findings were stable, with a body temperature of36.5 ◦C. A physical examination revealed severe abdominal tender-ness and guarding with a voluntary component in the right lowerquadrant. Moreover, Rovsing’s sign was positive. No prominentpathology was detected on a plain abdominal X-ray. Abdominalultrasonography depicted inflammation in the near vicinity ofthe appendix, along with a 6-mm wall thickness. Additionally, a25 mm × 18 mm hypoechoic mass lesion was detected in the neigh-borhood of the intestinal loops, superior to the right ovary, and

medial to the appendicular lodge. The white blood cell count andpercentage of neutrophils were 6850/mm3 and 79.1%, respectively.A urine analysis showed no pyuria or hematuria; moreover, a urinehuman chorionic gonadotropin assay was negative. The findings

. All rights reserved.

CASE REPORT – OPEN ACCESSA. Emre et al. / International Journal of Surgery Case Reports 4 (2013) 54– 57 55

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Fig. 2. Focus of endometriosis containing endometrial glands and stroma withinthe muscular layer in the neighborhood of the appendicular mucosa (hematoxylinand eosin, 40×).

Fig. 3. Focus of endometriosis containing endometrial glands and periglandular

Fig. 1. Appearance of resected appendectomy specimen with endometriosis.

rom the patient’s physical examination and ultrasonographyere consistent with a perforated appendix or a periappendicular

bscess. Hence, a laparotomy was performed through McBurney’sncision. During the exploration, the distal part of the appendix

as hard, irregular and edematous; thus, an appendectomy waserformed in a standard fashion. Both a thorough scan of the small

ntestines in the proximal direction and a meticulous inspection ofhe right tuboovarian structures were normal. The operation waserminated upon detection of no further pathology. The histopatho-ogical evaluation revealed a focus of endometriosis containingndometrial glands and stroma within the muscular layer in theeighborhood of the appendicular mucosa (Fig. 1). After ensuringhat no surgical complications occurred during the postoperativeeriod, the patient was referred to a gynecologist for furtherssessment.

.2. Case-2

A 45-year-old-female patient presented to our emergency clinicith complaints of nausea, vomiting and abdominal pain in the

ight lower quadrant which started 12 h ago. The patient wasarried and had two children. On the examination a marked

ebound tenderness was defined in the right lower quadrant. Nonding was found in the blood assay except leukocytosis. Ery-hrocyturia was found in the urine analysis. Tubular lesion withcute appendicitis was seen on the abdominal ultrasonographyerformed in another center. The patient was taken to the operationith presumed diagnosis of acute appendicitis. On the laparotomy,

retrocecal extenting hyperemic and edematous appendix wasbserved. Right tuboovarian structures and distal ileal segmentsere examined and no pathological gross finding was found. The

urgery was terminated after the appendectomy. The pathologyas reported as endometriosis and the patient referred to a gyne-

ologist (Figs. 2 and 3). She referred again to our hospital withhe complaint of cyclic pain in the McBurney incision site after 6

onths of the surgery. The patient reported that this complaintad repeated in each premenstrual period. For this reason, the

ormed incision was explored with presumed diagnosis of scarndometriosis. A mass sized about 3 cm × 3 cm that was adher-nt to surrounding tissues was excised with the scar tissue. Fasciaas repaired with prolene suture. Her pathology was coherent

ith scar endometriosis. The patient was referred again to the

ynecologist.

stroma contained within the muscular layer of the appendix (hematoxylin and eosin,100×).

3. Discussion

Acute appendicitis is one of most common acute surgical condi-tions of the abdomen, and an appendectomy is one of the mostfrequently performed operations worldwide. Obstruction of theappendiceal lumen seems to be essential for developing an appen-diceal infection. Although fecaliths and lymphoid hyperplasia arethe usual causes of an obstruction, some unusual factors can alsobe involved.8 While enterobius vermicularis and carcinoid tumorsoccupy the first two ranks among the unusual factors revealedthrough a histopathological examination of appendix specimensfrom a patient undergoing surgery with a diagnosis of acute appen-dicitis, appendiceal endometriosis ranks eighth, with an incidenceof 3%.8

Endometriosis is a common benign gynecologic disorder and hasbeen divided into internal and external forms according to local-ization. It is called internal endometriosis when the endometrialtissue is found within the uteral muscles. External endometriosis

is commonly found in genital organs and the pelvic peritoneum,although it may be seen in the gastrointestinal system, omen-tum majus, mesenterium, liver, and operation scars but rarely in

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CASE REPORT6 A. Emre et al. / International Journ

ther organs.9 The true prevalence of endometriosis is difficulto quantify, as women with the disease are often asymptomatic,nd imaging modalities have low sensitivities for diagnosis. Var-ous authors have estimated that up to 15% of all reproductivege women and one-third of infertile women have endometri-sis. The precise etiology that completely explains the cause andathogenesis of endometriosis is unknown. Two main hypothe-es have been proposed to explain its cause: one suggests thatultipotential mesenchymal cells may undergo metaplasia into

ndometriosis under proper circumstances; the other hypothesistates that viable endometrial cells are implanted from retrogradeenstruation through the fallopian tubes.10

The most common sites where endometriosis occurs arehe ovaries (60–75%), uterosacral ligaments (30–65%), cul-de-sac20–30%), gastrointestinal tract (3–37%), ureters (1–2%), bladder<1%), and scar tissue (<1%).9 Endometriosis can affect various partsf the gastrointestinal system from the small intestine to the anus.9

he rectosigmoid region (72%) is the site most commonly involved,ollowed by the recto-vaginal septum (13%), small bowel (7%),ecum (4%), and appendix (3%).7,11

Appendiceal endometriosis was first described in 1860 byon Rokitansky. Numerous studies since then have reported

hat the prevalence of appendiceal endometriosis is 0.8–22%,epending on the population evaluated. Appendiceal endometri-sis is divided into primary and secondary forms. The primaryorm includes histopathological evidence of endometriosis withinhe appendix with no clinicopathological evidence of extra-ppendicular endometriosis. The secondary form is associatedith internal and/or external endometriosis. The majority of

tudies address the similarities between appendiceal endome-riosis and tubo-ovarian endometriosis. Moreover, most patientsiagnosed with appendiceal endometriosis suffer from men-trual irregularities and uterine leiomyomas. Similarly, primaryppendiceal endometriosis cases have been reported withoutnvolvement of any other gynecological organ.2,9,12 Of 125 patientsn whom various surgical procedures were undertaken due tonfertility, 65 underwent an appendectomy, and in 20% appen-iceal endometriosis was evident in the pathological evaluation.he above-mentioned results have revived the debate as tohether an elective appendectomy should be undertaken inatients destined to undergo surgery for endometriosis. We believehat the decision for an elective appendectomy should be dic-ated by a meticulous inspection including the length, diameter,olor, and relationship of the appendix with the surroundingissues.

Patients with appendiceal endometriosis can be categorizednto four groups in terms of symptomatology: patients presenting

ith acute appendicitis, patients with an appendix invagination,atients manifesting atypical symptoms such as abdominal colic,ausea, and melena, and asymptomatic patients. The most com-only seen group are patients who present with appendicitis, and

he condition mostly occurs during menstruation.4,9,13

Appendiceal intussusception, first described by Mc Kidd in 1958,s defined as invagination of part of appendix vermiformis into itswn or cecal lumen. Etiologic factors are divided into anatomicnd pathologic. Some irritants such as mucoceles, parasites, foreignody, carcinoid tumor, granulomas, polyps, lipomas are consid-red to lead intussusceptions by causing abnormal peristaltism.ne of the etiologic agents causing appendiceal intussusceptions isppendiceal endometriosis which is always associated with chronicbrosis, inflammation, and changes in intestinal wall. The resultinguscular thickening serves as a starting point for hyperperistalsis

y causing contraction of the appendiceal segment at which it is

ocated, making it prone to intussusception. Appendiceal intussus-eption secondary to endometriosis was first described by Ingersolt al. in 1945. Various symptoms of classical appendicitis, recurrent

PEN ACCESSurgery Case Reports 4 (2013) 54– 57

right lower abdominal pain, or symptoms of intestinal obstructionmay develop in such patients.14,15

The differential diagnosis of intestinal endometriosis includesinflammatory disorders such as inflammatory bowel disease withstricture, diverticulitis, infectious diseases such as ileocolonictuberculosis and schistosomiasis, benign and malignant neoplas-tic disorders, and colon ischemia. It is important to emphasize thatno radiological or imaging finding is pathognomonic for endome-triosis; mucosal abnormalities that permit positive biopsies arerare, and tissue for a definitive diagnosis is usually obtained only atlaparotomy or laparoscopy.

Such diagnostic options as a patient’s medical history, phys-ical examination findings, blood tests (CA-125), colonoscopy,transvaginal or transrectal ultrasonography, barium enema, com-puted tomography, and magnetic resonance imaging can be utilizedto make an endometriosis diagnosis. However, none of theseoptions can establish a definitive diagnosis of gastrointestinal endo-metriosis. A definitive diagnosis of endometriosis is often madeby laparoscopy or laparotomy with a biopsy and is particularlyuseful in patients with intestinal implants. The classic peritonealimplant appears as a bluish-black “powder-burn” lesion with vari-able degrees of pigmentation and surrounding fibrosis, the darkcoloration resulting from deposition of hemosiderin However, mostperitoneal implants appear as subtle, nonpigmented lesions. Theappreciation that endometrial tissue may be nonpigmented hasincreased the yield of these procedures considerably.

As noted above, histopathological evaluations, in terms of boththe definitive and differential diagnoses, are of paramount impor-tance in diagnosing appendiceal endometriosis.13 About half ofappendiceal endometriosis involves the body and half involves thetip of the appendix. The mucosa of the appendix is mostly notaffected, whereas glandular tissue, the endometrial stroma, andhemorrhagia are seen in the muscular and seromuscular layers intwo-thirds of patients, and solely in the serosa layer in one-third ofpatients.2,9

Treatment options for women with endometriosis are cur-rently based on the severity and type of symptoms. Preventingendometriosis is not yet possible; thus, treatment commences toameliorate symptoms. Some women with endometriosis are com-pletely asymptomatic, and the implants are found incidentally atthe time of surgery for other reasons. As in our case, medical treat-ment is not required in asymptomatic cases in whom gynecologicaltests fail to document any other focus of endometriosis. How-ever, additional medical therapy should be considered following anappendectomy in cases with symptomatic secondary appendicealendometriosis.

Appendiceal endometriosis is an unusual histopathological find-ing. A preoperative diagnosis is difficult but should be included inthe differential diagnosis when women of childbearing age presentwith clinical symptoms of acute appendicitis, even prior to imag-ing. Similarly, AE should be considered in patients with a history ofmenstrual disorders.

Conflict of interest

The authors declare that there is no conflict of interest.

Funding

None.

Written informed consent was obtained from the patient forpublication of this case report and accompanying images. A copy

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CASE REPORTA. Emre et al. / International Journ

f the written consent is available for review by the Editor-in-Chieff this journal on request.

ontributors

Emre A performed surgical procedure; Akbulut S and Yilmaz Montributed writing the article and review of the literature as well asndertaking a comprehensive literature search; Bozdag Z providedistopathologic information; Akbulut S and Yilmaz M contributedesign and manuscript preparation.

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pen Accesshis article is published Open Access at sciencedirect.com. It is distribermits unrestricted non commercial use, distribution, and reproductredited.

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