- Email: [email protected]
Isolated Appendiceal Endometriosis
CASE REPORT
Isolated Appendiceal Endometriosis Amanda Yang Shen, MB BS, Aaron Stanes, MB BS (Hons) Eastern Health, Melbourne Australia
Abstract Background: Pain associated with appendiceal endometriosis can mimic other intra-abdominal pathology. The diagnosis is usually obvious during gross inspection of the appendix; however, the absence of classical macroscopic appearances may lead to missed cases in which the patient’s pain is misdiagnosed and inappropriately managed. Case: A 34-year-old woman presented with cyclical right iliac fossa pain and an elevated serum C-reactive protein of 13 mg/L (normal < 5 mg/L). Diagnostic laparoscopy showed an isolated appendiceal mass with no pelvic endometriosis, and an appendectomy was performed. Histopathology demonstrated appendiceal endometriosis without macroscopic involvement of other pelvic organs. Conclusion: This case gives insight into the pathophysiology of endometriosis. We advocate routine appendectomy in women with unexplained recurrent abdominal pain because a diagnostic laparoscopy may miss isolated endometriosis of the appendix, and we now have evidence that this may have no external features suggesting the diagnosis. Additionally, endometriosis can involve the gastrointestinal tract without involvement of the reproductive organs. This is important information in the further progression of theories underlying the pathophysiology of endometriosis.
Résumé Antécédents : Les douleurs associées à l’endométriose appendiculaire peuvent imiter d’autres pathologies intraabdominales. Le diagnostic est habituellement évident durant l’inspection macroscopique de l’appendice; toutefois, l’absence d’apparence macroscopique classique peut mener à manquer des cas dans lequel la douleur du patient est mal diagnostiquée et traitée de façon inappropriée. Cas : Une femme de 34 ans présentait une douleur cyclique de la fosse iliaque droite et un sérum de protéine C-réactive élevé de 13 mg/l (normal < 5 mg/l). La coelioscopie exploratoire a montré une masse appendiculaire isolée sans endométriose pelvienne, et une appendicectomie a été effectuée. L’histopathologie a démontré une endométriose appendiculaire sans envahissement macroscopique d’autres organes pelviens. Conclusion : Ce cas donne un aperçu de la pathophysiologie de l’endométriose. Nous recommandons l’appendicectomie de routine chez les femmes présentant une douleur abdominale récurrente
Key Words: Appendicectomy, endometriosis, gynaecology, laparoscopy Competing Interests: None declared. Received on January 29, 2016 Accepted on March 23, 2016 http://dx.doi.org/10.1016/j.jogc.2016.06.006
inexpliquée parce qu’une coelioscopie exploratoire peut manquer une endométriose isolée de l’appendice, et nous avons maintenant la preuve qu’il ne peut y avoir aucun signe externe suggérant le diagnostic. De plus, l’endométriose peut envahir le tractus gastrointestinal sans envahissement des organes reproductifs. Ce sont des informations importantes dans la progression des théories déterminant la pathophysiologie de l’endométriose. Copyright ª 2016 The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. Published by Elsevier Inc. All rights reserved.
J Obstet Gynaecol Can 2016;38(10):979e981
INTRODUCTION
E
ndometriosis and appendicitis are both commonly found in young women. Endometriosis is the pathological proliferation of functional endometrial tissue at an extrauterine site, usually around the pelvic organs and, less commonly, the bowel. Typically, there is superficial serosal spread of endometrial tissue on the bowel wall. The serosal surfaces of the uterus can also be affected, leading to adhesions that may involve the bladder or rectum.1,2
To our knowledge, there have been no previous reports of isolated appendiceal endometriosis without pelvic spread as found in this case. THE CASE
A 34-year-old woman, previously well, presented to the emergency department complaining of right iliac fossa pain radiating to the anterior right thigh. The pain had been present cyclically for several months in association with menstrual bleeding. The pain was colicky in nature with no associated signs or symptoms such as peritonism. She had begun menstruation on the day prior to presentation. The results of investigations, including full blood count, serum electrolytes, serum C-reactive protein, and abdominal ultrasound assessment, were unremarkable; ultrasound showed a right ovarian complex cyst with fluid-fluid levels (likely a hemorrhagic cyst), no free fluid, normal appearing kidneys, and the appendix was not visualized. The patient’s condition was stable, OCTOBER JOGC OCTOBRE 2016
l
979
CASE REPORT
and she was discharged with a presumed diagnosis of retrograde menstruation. The patient presented again five days later with acute worsening of the pain. It was again localized to the right iliac fossa and was constant. She had no genitourinary or gastrointestinal symptoms and was afebrile. Serum C-reactive protein was 13 mg/L (normal < 5 mg/L); all other investigations (including CBC, serum urea, serum creatinine, serum electrolytes, serum calcium, serum magnesium, serum phosphate. and liver function tests) were within normal limits, and pregnancy was excluded with a negative serum b-hCG. Because the ongoing pain was suspicious for a diagnosis of appendicitis, laparoscopic appendectomy was performed. The laparoscopy was uncomplicated, and the appendix was removed. Old blood was noted in the posterior cul-de-sac, and the right ovarian cyst seen earlier on ultrasound had persisted. The appendix was not inflamed and the serosa was intact, but macroscopic features suggestive of a carcinoid tumor in the appendix were noted. No endometriosis was seen in the abdominal or pelvic cavity intraoperatively (Figure). The specimen was sent for histopathological analysis. Histopathology demonstrated endometriosis involving the appendix and mesoappendix. A lobulated, firm mass measuring 15 15 mm was present at the tip of the 35 15 15 mm appendix. This was shown to be endometrial tissue (glands surrounded by stroma) involving the appendiceal wall and mesoappendix associated with the presence of hemosiderin-laden macrophages (seen in areas of bleeding). There was no evidence of malignancy. The patient made a full recovery with resolution of her pain.
Figure. Intraoperative appearance of appendiceal mass during laparoscopy
DISCUSSION
Intestinal or appendiceal endometriosis is a well-described pathological entity; published case reports date from 1951, when nine cases of endometriosis of the appendix were described.2 The mode of spread of endometrial tissue is controversial, and several theories have been noted. These include transtubal retrograde implantation, metastasis, and embryonic celomic metaplasia, the last of which possibly explains unusual locations of growth such as the lung, thigh, and forearm.2,3 Cellular metaplasia has also been postulated as a cause. Endometrium and peritoneum are derivatives of the same cell lineage, and this could explain the extrauterine occurrence of endometrial tissue as well as cases that follow hysterectomy. Chronic inflammation may play a role in the cellular metaplasia; in addition, endometriosis has been known to occur in men on estrogen therapy.4,5 In our patient, inflammation around the endometriotic lesion in the appendix may have led to new serosal formation around it, concealing the appendiceal pathology. If so, our case highlights the important point that endometriosis can be a cause of localized pathology even when pelvic spread is not seen. Although usually asymptomatic, appendiceal endometriosis may lead to numerous complications. A case control study found that endometriosis involving the appendix might be associated with higher rates of cyclical disturbance of bowel habits and a higher likelihood of having multiple areas of pelvic involvement, corresponding to greater disease severity.6 In our case, the patient reported cyclical right iliac fossa pain associated with menses, which was also described by Akbulut et al. in a case of perforated appendicitis due to endometriosis.7 Although not the case in our patient, appendicitis secondary to appendiceal endometriosis has also been described. 8
CONCLUSION
Spread of endometriosis to involve the appendix is a rare phenomenon that can lead to a variety of presentations and complications. To our knowledge, isolated appendiceal endometriosis without superficial pelvic spread has not been previously reported, marking this as a unique case that may provide insight into the pathophysiology of endometriosis and of chronic abdominal pain with no apparent cause. Consequently, removing appendices with an unusual appearance, even without evidence of appendicitis, during diagnostic laparoscopy may benefit women with unexplained abdominal pain because isolated
980
l
OCTOBER JOGC OCTOBRE 2016
Isolated Appendiceal Endometriosis
appendiceal endometriosis may be the cause of the patient’s symptoms.
3. Liu DT, Hitchcock A. Endometriosis: its association with retrograde menstruation, dysmenorrhoea and tubal pathology. Br J Obstet Gynaecol 1986;93:859e62.
ACKNOWLEDGEMENT
4. Suginami H. A reappraisal of the coelomic metaplasia theory by reviewing endometriosis occurring in unusual sites and instances. Am J Obstet Gynecol 1991;165:214e8.
The woman whose story is told in this case report has provided signed permission for its publication.
5. Schrodt GR, Alcorn MO, Ibanez J. Endometriosis of the male urinary system: a case report. J Urol 1980;124:722e3.
REFERENCES
6. Abrão M, Dias J, Rodini G, Podgaec S, Bassi M, Averbach M. Endometriosis at several sites, cyclic bowel symptoms, and the likelihood of the appendix being affected. Fertil Steril 2010;94:1099e101.
1. Cameron I, Rogers S, Collins M, Reed M. Intestinal endometriosis: presentation, investigation, and surgical management. Int J Colorect Dis 1995;10:83e6. 2. Collins D. Endometriosis of the vermiform appendix. AMA Arch Surg 1951;63:617e22.
7. Akbulut S, Dursun P, Kocbiyik A, Harman A, Sevmis S. Appendiceal endometriosis presenting as perforated appendicitis: report of a case and review of the literature. Arch Gynecol Obstet 2009;280:495e7. 8. Curbelo-Peña Y, Guedes-De la Puente X, Saladich-Cubero M, MolinasBruguera J, Molineros J, De Caralt-Mestres E. Endometriosis causing acute appendicitis complicated with hemoperitoneum. J Surg Case Rep 2015;2015(8). pii: rjv097.
OCTOBER JOGC OCTOBRE 2016
l
981
Isolated Appendiceal Endometriosis Amanda Yang Shen, MB BS, Aaron Stanes, MB BS (Hons) Eastern Health, Melbourne Australia
Abstract Background: Pain associated with appendiceal endometriosis can mimic other intra-abdominal pathology. The diagnosis is usually obvious during gross inspection of the appendix; however, the absence of classical macroscopic appearances may lead to missed cases in which the patient’s pain is misdiagnosed and inappropriately managed. Case: A 34-year-old woman presented with cyclical right iliac fossa pain and an elevated serum C-reactive protein of 13 mg/L (normal < 5 mg/L). Diagnostic laparoscopy showed an isolated appendiceal mass with no pelvic endometriosis, and an appendectomy was performed. Histopathology demonstrated appendiceal endometriosis without macroscopic involvement of other pelvic organs. Conclusion: This case gives insight into the pathophysiology of endometriosis. We advocate routine appendectomy in women with unexplained recurrent abdominal pain because a diagnostic laparoscopy may miss isolated endometriosis of the appendix, and we now have evidence that this may have no external features suggesting the diagnosis. Additionally, endometriosis can involve the gastrointestinal tract without involvement of the reproductive organs. This is important information in the further progression of theories underlying the pathophysiology of endometriosis.
Résumé Antécédents : Les douleurs associées à l’endométriose appendiculaire peuvent imiter d’autres pathologies intraabdominales. Le diagnostic est habituellement évident durant l’inspection macroscopique de l’appendice; toutefois, l’absence d’apparence macroscopique classique peut mener à manquer des cas dans lequel la douleur du patient est mal diagnostiquée et traitée de façon inappropriée. Cas : Une femme de 34 ans présentait une douleur cyclique de la fosse iliaque droite et un sérum de protéine C-réactive élevé de 13 mg/l (normal < 5 mg/l). La coelioscopie exploratoire a montré une masse appendiculaire isolée sans endométriose pelvienne, et une appendicectomie a été effectuée. L’histopathologie a démontré une endométriose appendiculaire sans envahissement macroscopique d’autres organes pelviens. Conclusion : Ce cas donne un aperçu de la pathophysiologie de l’endométriose. Nous recommandons l’appendicectomie de routine chez les femmes présentant une douleur abdominale récurrente
Key Words: Appendicectomy, endometriosis, gynaecology, laparoscopy Competing Interests: None declared. Received on January 29, 2016 Accepted on March 23, 2016 http://dx.doi.org/10.1016/j.jogc.2016.06.006
inexpliquée parce qu’une coelioscopie exploratoire peut manquer une endométriose isolée de l’appendice, et nous avons maintenant la preuve qu’il ne peut y avoir aucun signe externe suggérant le diagnostic. De plus, l’endométriose peut envahir le tractus gastrointestinal sans envahissement des organes reproductifs. Ce sont des informations importantes dans la progression des théories déterminant la pathophysiologie de l’endométriose. Copyright ª 2016 The Society of Obstetricians and Gynaecologists of Canada/La Société des obstétriciens et gynécologues du Canada. Published by Elsevier Inc. All rights reserved.
J Obstet Gynaecol Can 2016;38(10):979e981
INTRODUCTION
E
ndometriosis and appendicitis are both commonly found in young women. Endometriosis is the pathological proliferation of functional endometrial tissue at an extrauterine site, usually around the pelvic organs and, less commonly, the bowel. Typically, there is superficial serosal spread of endometrial tissue on the bowel wall. The serosal surfaces of the uterus can also be affected, leading to adhesions that may involve the bladder or rectum.1,2
To our knowledge, there have been no previous reports of isolated appendiceal endometriosis without pelvic spread as found in this case. THE CASE
A 34-year-old woman, previously well, presented to the emergency department complaining of right iliac fossa pain radiating to the anterior right thigh. The pain had been present cyclically for several months in association with menstrual bleeding. The pain was colicky in nature with no associated signs or symptoms such as peritonism. She had begun menstruation on the day prior to presentation. The results of investigations, including full blood count, serum electrolytes, serum C-reactive protein, and abdominal ultrasound assessment, were unremarkable; ultrasound showed a right ovarian complex cyst with fluid-fluid levels (likely a hemorrhagic cyst), no free fluid, normal appearing kidneys, and the appendix was not visualized. The patient’s condition was stable, OCTOBER JOGC OCTOBRE 2016
l
979
CASE REPORT
and she was discharged with a presumed diagnosis of retrograde menstruation. The patient presented again five days later with acute worsening of the pain. It was again localized to the right iliac fossa and was constant. She had no genitourinary or gastrointestinal symptoms and was afebrile. Serum C-reactive protein was 13 mg/L (normal < 5 mg/L); all other investigations (including CBC, serum urea, serum creatinine, serum electrolytes, serum calcium, serum magnesium, serum phosphate. and liver function tests) were within normal limits, and pregnancy was excluded with a negative serum b-hCG. Because the ongoing pain was suspicious for a diagnosis of appendicitis, laparoscopic appendectomy was performed. The laparoscopy was uncomplicated, and the appendix was removed. Old blood was noted in the posterior cul-de-sac, and the right ovarian cyst seen earlier on ultrasound had persisted. The appendix was not inflamed and the serosa was intact, but macroscopic features suggestive of a carcinoid tumor in the appendix were noted. No endometriosis was seen in the abdominal or pelvic cavity intraoperatively (Figure). The specimen was sent for histopathological analysis. Histopathology demonstrated endometriosis involving the appendix and mesoappendix. A lobulated, firm mass measuring 15 15 mm was present at the tip of the 35 15 15 mm appendix. This was shown to be endometrial tissue (glands surrounded by stroma) involving the appendiceal wall and mesoappendix associated with the presence of hemosiderin-laden macrophages (seen in areas of bleeding). There was no evidence of malignancy. The patient made a full recovery with resolution of her pain.
Figure. Intraoperative appearance of appendiceal mass during laparoscopy
DISCUSSION
Intestinal or appendiceal endometriosis is a well-described pathological entity; published case reports date from 1951, when nine cases of endometriosis of the appendix were described.2 The mode of spread of endometrial tissue is controversial, and several theories have been noted. These include transtubal retrograde implantation, metastasis, and embryonic celomic metaplasia, the last of which possibly explains unusual locations of growth such as the lung, thigh, and forearm.2,3 Cellular metaplasia has also been postulated as a cause. Endometrium and peritoneum are derivatives of the same cell lineage, and this could explain the extrauterine occurrence of endometrial tissue as well as cases that follow hysterectomy. Chronic inflammation may play a role in the cellular metaplasia; in addition, endometriosis has been known to occur in men on estrogen therapy.4,5 In our patient, inflammation around the endometriotic lesion in the appendix may have led to new serosal formation around it, concealing the appendiceal pathology. If so, our case highlights the important point that endometriosis can be a cause of localized pathology even when pelvic spread is not seen. Although usually asymptomatic, appendiceal endometriosis may lead to numerous complications. A case control study found that endometriosis involving the appendix might be associated with higher rates of cyclical disturbance of bowel habits and a higher likelihood of having multiple areas of pelvic involvement, corresponding to greater disease severity.6 In our case, the patient reported cyclical right iliac fossa pain associated with menses, which was also described by Akbulut et al. in a case of perforated appendicitis due to endometriosis.7 Although not the case in our patient, appendicitis secondary to appendiceal endometriosis has also been described. 8
CONCLUSION
Spread of endometriosis to involve the appendix is a rare phenomenon that can lead to a variety of presentations and complications. To our knowledge, isolated appendiceal endometriosis without superficial pelvic spread has not been previously reported, marking this as a unique case that may provide insight into the pathophysiology of endometriosis and of chronic abdominal pain with no apparent cause. Consequently, removing appendices with an unusual appearance, even without evidence of appendicitis, during diagnostic laparoscopy may benefit women with unexplained abdominal pain because isolated
980
l
OCTOBER JOGC OCTOBRE 2016
Isolated Appendiceal Endometriosis
appendiceal endometriosis may be the cause of the patient’s symptoms.
3. Liu DT, Hitchcock A. Endometriosis: its association with retrograde menstruation, dysmenorrhoea and tubal pathology. Br J Obstet Gynaecol 1986;93:859e62.
ACKNOWLEDGEMENT
4. Suginami H. A reappraisal of the coelomic metaplasia theory by reviewing endometriosis occurring in unusual sites and instances. Am J Obstet Gynecol 1991;165:214e8.
The woman whose story is told in this case report has provided signed permission for its publication.
5. Schrodt GR, Alcorn MO, Ibanez J. Endometriosis of the male urinary system: a case report. J Urol 1980;124:722e3.
REFERENCES
6. Abrão M, Dias J, Rodini G, Podgaec S, Bassi M, Averbach M. Endometriosis at several sites, cyclic bowel symptoms, and the likelihood of the appendix being affected. Fertil Steril 2010;94:1099e101.
1. Cameron I, Rogers S, Collins M, Reed M. Intestinal endometriosis: presentation, investigation, and surgical management. Int J Colorect Dis 1995;10:83e6. 2. Collins D. Endometriosis of the vermiform appendix. AMA Arch Surg 1951;63:617e22.
7. Akbulut S, Dursun P, Kocbiyik A, Harman A, Sevmis S. Appendiceal endometriosis presenting as perforated appendicitis: report of a case and review of the literature. Arch Gynecol Obstet 2009;280:495e7. 8. Curbelo-Peña Y, Guedes-De la Puente X, Saladich-Cubero M, MolinasBruguera J, Molineros J, De Caralt-Mestres E. Endometriosis causing acute appendicitis complicated with hemoperitoneum. J Surg Case Rep 2015;2015(8). pii: rjv097.
OCTOBER JOGC OCTOBRE 2016
l
981