European Journal of Obstetrics & Gynecology and Reproductive Biology, 42 (1991) 81-84 0 1691 Elsevier Science Publishers B.V. All rights reserved 0028-2243/91/$03.50
EUROBS 01146
of the perineum; report of two new cases and a review of literature David L. Cheng ‘, Debra S. Heller From the Departments
of ’ Surgery.
2p3and Changyul
Oh 1
’ Pathology and ’ Obstetrics and Gynecology, Mount Sinai School of Medicine of the City University of New York and The Mount Sinai Hospital, New York, U.S.A.
Accepted for pub~cation 27 November 1990
The incidence of endometriosis at the episiotomy site is quite rare. We have experienced two perineal endometriosis over a 9 year period. The typical clinical history and local findings enable us to make the correct diagnosis of both The treatment of choice is complete surgical excision of endometrial tissue and usually permanent cure. A review of the English literature showed there were 66 cases reported previously. The pathogensis of endometriosis and various modality of treatment were also discussed. Endometriomium;
cases of cases. obtains possible
Perineal mass; Tumor at episiotomy scar
Introduction
The occurrence of endometriosis at the site of an episiotomy scar is quite rare. It is often difficult to recognize, which may lead to improper management. However, careful case history and the typical clinical course with local findings usually enable one to make the correct diagnosis. We report our two cases of perineal endometriosis and present a review of the English literature.
Correspondence: Changyul Oh, M.D., Mount Sinai Medical Center, Department of Surgery, Box 1259, One Gustave L. Levy Place, New York, NY 10029, U.S.A.
Case 1
A.T., a 33-year-old Hispanic female, presented with complaint of per&ml pain and a fluctuating perianal mass for 8 months. Seven years previously, the patient had a traumatic vaginal delivery with a right episiotomy. She noticed a painful mass with an increase in size at the episiotomy site during her menstrual periods. Physical examination revealed a 2 cm, circumscribed and indurated mass located at the right anterior perianal region at the site of the episiotomy (Fig. 1). With her clinical history and local findings, perianal endometrioma was suspected. The perianal mass was completely excised under general anesthesia, and
82
Fig. 1. The artery clamp pointing to a circumscribed located at the site of episiotomy scar.
mass
was found to contain chocolate-colored material. Microscopic section revealed fibroadipos tissue and muscle involved by endometriosis consisting of both endometrial glandular and stromal elements (Fig. 2). The patient has been well since her surgery. Case 2 A.W., a 42-year-old white female, presented with the complaint of perirectal pain 7 years after a normal vaginal delivery in 1968. The pain invariably occurred seven to ten days after her menses.
Fig. 2. Microscopic view showing endometriosis in fibrous tissue, consisting of endometrial glands and stroma. H&E x 4 original maginification.
A painful mass in her perineum at the site of the previous episiotomy was noted. The mass was bluish in color and increased in size with her menses. It was diagnosed as an anal fistula, and a fistulotomy was performed in 1978 without success. It was reoperated on in 1980 by other surgeons without improvement. Because the pain persisted, and the mass became larger, she sought further treatment with us in January of 1990. Her past medical history was significant only for hypothyroidism, which was treated with cytomel. Physical examination revealed an indurated and tender mass in the right anterior perineal area. A preoperative diagnosis of perineal endometriosis was entertained based on her clinical course and the local appearance of the lesion. At the time of operation, a 3 cm bluish and relatively superficial mass containing dark blood was found. The mass was completely excised, and the diagnosis of endometrioma was confirmed histologically. Patient has been asymptomatic since the surgery. Discussion A review of the English literature revealed that there have been 66 cases of perineal endometriosis documented since 1923. According to Prince et al. [l], the first case was apparently reported by Schickele. They added one new, and additionally disclosed 25 cases of perineal endometrioma by reviewing the world literature in 1957 [l]. Since then, numerous cases have been reported by various authors, with a total of 66 cases [2-131. The patients’ ages ranged from 19 to 45 years with an average age of 33. All the patients had at least one successful pregnancy previously, and all but five patients had clearly suffered some type of vaginal trauma [8,11]. Forty-one patients had episiotomies performed during child birth. Five patients had prior curettage, and 15 patients had both episiotomies and curettages. The onset of symptoms varied from 1 month to 14 years after the initial trauma. All cases were confirmed histologically to be endometriosis. Six cases of recurrences were reported. Various theories have been advanced for the pathogenesis of endometriosis; namely, hematogenous or lymphatic spread, coelomic metaplasia,
83
and implantation [13,14]. The first theory attempts to explain the occurrence of endometriosis by metastatic spread of endometrium at the time of menstruation [13]. The coelomic metaplasia doctrine postulates that abnormal differentiation of germinal epithelium in the pelvic peritoneum leads to endometriosis because the endometrium is developmentally related to coelornic germinal epithelium [13]. The implantation theory states that the transtubal regurgitation of menstrual blood carrying endometrial particles results in attachment and growth of endometrioma [14]. None of these theories have been conclusively proven. Despite these controversies, the implantation theory appears to best explain the pathogenesis of perineal endometriosis. The mucosal lining of the uterine cavity provides a plausible anatomical basis for the implantation theory. The uterine endometrium consists of two zones, the superficial zona functionalis and the inner zona basalis. The zona functionalis undergoes decidual transformation during the menstrual cycle. The zona basalis is capable of reconstituting the uterine lining. During menses or parturition, the zona functionalis degenerates and is shedded along with varying amounts of the zona basalis. When the cells from the zona basalis are transported to a favorable environment such as a fresh wound from an episiotomy, the cells become attached and develop into endometriosis [12]. As wound-healing progresses, the trapped endometrial cells continue to grow and eventually reach a critical size to cause discomfort and pain to the patients. Evidence to support this hypothesis comes from Paul1 and Tedeschi [9], who found fifteen cases of perineal endometriosis in a series of 2028 patients who had episiotomies along with endometrial curettage following delivery. They compared this group with another group of 13800 patients who had only episiotomies at the time of parturition but who failed to develop perineal endometrioma. This study suggested that with curettage, more viable cells from the zona basalis are transplanted to the perineum and result in endometriosis. The diagnosis of perineal endometriosis can be made based upon the typical clinical history and local findings. All patients have some type of perineal trauma, either an episiotomy or curettage.
The time of onset of the symptoms is varied and has been reported to be as long as 14 years, similar to our cases [l]. The variable latent period can be due to the fact that microscopic implants of endometrial tissue require a certain amount of time before they achieve a sufficient size to produce symptoms. The majority of patients complain of cyclic perianal pain with an increasingly painful mass during the menstrual period. Physical examination usually reveals a tender bluish perineal mass. Differential diagnosis include anal fistula, anal abscess, thrombosed hemorrhoid, sebaceous cyst, dermoid cyst or malignancies. The typical signs and symptoms usually help to make the diagnosis, but errors in diagnosis may lead to multiple unnecessary procedures, as seen in our second case. Many of the previously reported cases show similar situations with long time intervals between onset of symptoms and final definitive treatment, with undue suffering to the patient [4]. Once the diagnosis is made, the treatment of choice is complete excision of the perineal endometrial tissue. With the exception of one case, where the mass disappeared after pregnancy [6], the entire endometrial mass must be removed, or recurrence is likely to take place [12]. Some clinicians have advocated hormonal manipulations using testosterone or medroxyprogesterone with varying degrees of success [4,12]. Although the medical treatment may achieve symptomatic relief, the perineal mass often persists [12]. Because most of these patients are relatively young and healthy, surgical excision is essential, particularly if one suspects malignancy. Complete surgical removal is the quickest and most effective means of achieving permanent cure. Conclusion Two cases of perineal endometriosis are presented. A review of the English literature show that there are 66 cases reported previously. Although the pathogenesis of endometriosis is not fully understood, the transplantation of endometrial particles at time of vaginal trauma appears to best explain the occurrence of this condition. Because patients often present with typical history and similar local findings, correct diagnosis can be
84
made. Permanent cure can be achieved with wrgical excision. References 1 Prince LN, Abrams J. Endometriosis of the perineum: review of the literature and case report. Am J Obstet Gynewl 1957;73:890-893. 2 Murray RR. Endometriosis of an episiotomy scar. Armed Forces Med J 1959;10:1463-1468. 3 Stingi A. An unusual case of endometriosis in a perineal scar. Kiin Med (Wein) 1%0;15:325-329, 4 Trampuz V. Endometriosis of the perineum. Am J Obstet Gynewl 1962;84:1522-1525. 5 Binder SS. Endometriosis of the vulva and perineum. Pacif Med Surg 1965;73:294-296. 6 Beischer N. Endometriosis of an episiotomy scar cured by pregnancy. Obstet Gynecol 1966;28:15-21. 7 McGivney J, Mazuji MR. Endometriosis of episiotomy scar: a case report. Am Surg ‘1966;32:469-471.
8 Cheleden J. Endometriosis of the perineum: report of two cases. Southern Med J 1968;61:1313-1314. 9 Paul1 T, Tedeschi LG. Perineal endometriosis at the site of episiotomy scar. Obstet Gynecol 1972;40:28-34. 10 Ramsey WH. Endometrioma involving the perianal tissue: report of a case. Dis Co1 Rect 1971;14:366-367. 11 Gordon PH, Schottler JL, Balcos EC, Goldberg SM. Perianal endometrioma; report of five cases. Dis Co1 Rect 1976;19:260-265. 12 Hambrick E, Abcaiian H, Smith D. Perineal endometrioma in episiotomy incisions: clinical features and management. Dis Co1 Rect 1979;22:550-552. 13 Wittich AC. Endomet~~is in an episiotomy scar: review of the literature and report of case. J Am Osteopath Assoc 1982;82:22-23. 14 Sampson J. The development of the implantation theory for the origin of peritoneal endometriosis. Am J Obstet Gynecol 1940;40:549-557.
EUROBS 01146
of the perineum; report of two new cases and a review of literature David L. Cheng ‘, Debra S. Heller From the Departments
of ’ Surgery.
2p3and Changyul
Oh 1
’ Pathology and ’ Obstetrics and Gynecology, Mount Sinai School of Medicine of the City University of New York and The Mount Sinai Hospital, New York, U.S.A.
Accepted for pub~cation 27 November 1990
The incidence of endometriosis at the episiotomy site is quite rare. We have experienced two perineal endometriosis over a 9 year period. The typical clinical history and local findings enable us to make the correct diagnosis of both The treatment of choice is complete surgical excision of endometrial tissue and usually permanent cure. A review of the English literature showed there were 66 cases reported previously. The pathogensis of endometriosis and various modality of treatment were also discussed. Endometriomium;
cases of cases. obtains possible
Perineal mass; Tumor at episiotomy scar
Introduction
The occurrence of endometriosis at the site of an episiotomy scar is quite rare. It is often difficult to recognize, which may lead to improper management. However, careful case history and the typical clinical course with local findings usually enable one to make the correct diagnosis. We report our two cases of perineal endometriosis and present a review of the English literature.
Correspondence: Changyul Oh, M.D., Mount Sinai Medical Center, Department of Surgery, Box 1259, One Gustave L. Levy Place, New York, NY 10029, U.S.A.
Case 1
A.T., a 33-year-old Hispanic female, presented with complaint of per&ml pain and a fluctuating perianal mass for 8 months. Seven years previously, the patient had a traumatic vaginal delivery with a right episiotomy. She noticed a painful mass with an increase in size at the episiotomy site during her menstrual periods. Physical examination revealed a 2 cm, circumscribed and indurated mass located at the right anterior perianal region at the site of the episiotomy (Fig. 1). With her clinical history and local findings, perianal endometrioma was suspected. The perianal mass was completely excised under general anesthesia, and
82
Fig. 1. The artery clamp pointing to a circumscribed located at the site of episiotomy scar.
mass
was found to contain chocolate-colored material. Microscopic section revealed fibroadipos tissue and muscle involved by endometriosis consisting of both endometrial glandular and stromal elements (Fig. 2). The patient has been well since her surgery. Case 2 A.W., a 42-year-old white female, presented with the complaint of perirectal pain 7 years after a normal vaginal delivery in 1968. The pain invariably occurred seven to ten days after her menses.
Fig. 2. Microscopic view showing endometriosis in fibrous tissue, consisting of endometrial glands and stroma. H&E x 4 original maginification.
A painful mass in her perineum at the site of the previous episiotomy was noted. The mass was bluish in color and increased in size with her menses. It was diagnosed as an anal fistula, and a fistulotomy was performed in 1978 without success. It was reoperated on in 1980 by other surgeons without improvement. Because the pain persisted, and the mass became larger, she sought further treatment with us in January of 1990. Her past medical history was significant only for hypothyroidism, which was treated with cytomel. Physical examination revealed an indurated and tender mass in the right anterior perineal area. A preoperative diagnosis of perineal endometriosis was entertained based on her clinical course and the local appearance of the lesion. At the time of operation, a 3 cm bluish and relatively superficial mass containing dark blood was found. The mass was completely excised, and the diagnosis of endometrioma was confirmed histologically. Patient has been asymptomatic since the surgery. Discussion A review of the English literature revealed that there have been 66 cases of perineal endometriosis documented since 1923. According to Prince et al. [l], the first case was apparently reported by Schickele. They added one new, and additionally disclosed 25 cases of perineal endometrioma by reviewing the world literature in 1957 [l]. Since then, numerous cases have been reported by various authors, with a total of 66 cases [2-131. The patients’ ages ranged from 19 to 45 years with an average age of 33. All the patients had at least one successful pregnancy previously, and all but five patients had clearly suffered some type of vaginal trauma [8,11]. Forty-one patients had episiotomies performed during child birth. Five patients had prior curettage, and 15 patients had both episiotomies and curettages. The onset of symptoms varied from 1 month to 14 years after the initial trauma. All cases were confirmed histologically to be endometriosis. Six cases of recurrences were reported. Various theories have been advanced for the pathogenesis of endometriosis; namely, hematogenous or lymphatic spread, coelomic metaplasia,
83
and implantation [13,14]. The first theory attempts to explain the occurrence of endometriosis by metastatic spread of endometrium at the time of menstruation [13]. The coelomic metaplasia doctrine postulates that abnormal differentiation of germinal epithelium in the pelvic peritoneum leads to endometriosis because the endometrium is developmentally related to coelornic germinal epithelium [13]. The implantation theory states that the transtubal regurgitation of menstrual blood carrying endometrial particles results in attachment and growth of endometrioma [14]. None of these theories have been conclusively proven. Despite these controversies, the implantation theory appears to best explain the pathogenesis of perineal endometriosis. The mucosal lining of the uterine cavity provides a plausible anatomical basis for the implantation theory. The uterine endometrium consists of two zones, the superficial zona functionalis and the inner zona basalis. The zona functionalis undergoes decidual transformation during the menstrual cycle. The zona basalis is capable of reconstituting the uterine lining. During menses or parturition, the zona functionalis degenerates and is shedded along with varying amounts of the zona basalis. When the cells from the zona basalis are transported to a favorable environment such as a fresh wound from an episiotomy, the cells become attached and develop into endometriosis [12]. As wound-healing progresses, the trapped endometrial cells continue to grow and eventually reach a critical size to cause discomfort and pain to the patients. Evidence to support this hypothesis comes from Paul1 and Tedeschi [9], who found fifteen cases of perineal endometriosis in a series of 2028 patients who had episiotomies along with endometrial curettage following delivery. They compared this group with another group of 13800 patients who had only episiotomies at the time of parturition but who failed to develop perineal endometrioma. This study suggested that with curettage, more viable cells from the zona basalis are transplanted to the perineum and result in endometriosis. The diagnosis of perineal endometriosis can be made based upon the typical clinical history and local findings. All patients have some type of perineal trauma, either an episiotomy or curettage.
The time of onset of the symptoms is varied and has been reported to be as long as 14 years, similar to our cases [l]. The variable latent period can be due to the fact that microscopic implants of endometrial tissue require a certain amount of time before they achieve a sufficient size to produce symptoms. The majority of patients complain of cyclic perianal pain with an increasingly painful mass during the menstrual period. Physical examination usually reveals a tender bluish perineal mass. Differential diagnosis include anal fistula, anal abscess, thrombosed hemorrhoid, sebaceous cyst, dermoid cyst or malignancies. The typical signs and symptoms usually help to make the diagnosis, but errors in diagnosis may lead to multiple unnecessary procedures, as seen in our second case. Many of the previously reported cases show similar situations with long time intervals between onset of symptoms and final definitive treatment, with undue suffering to the patient [4]. Once the diagnosis is made, the treatment of choice is complete excision of the perineal endometrial tissue. With the exception of one case, where the mass disappeared after pregnancy [6], the entire endometrial mass must be removed, or recurrence is likely to take place [12]. Some clinicians have advocated hormonal manipulations using testosterone or medroxyprogesterone with varying degrees of success [4,12]. Although the medical treatment may achieve symptomatic relief, the perineal mass often persists [12]. Because most of these patients are relatively young and healthy, surgical excision is essential, particularly if one suspects malignancy. Complete surgical removal is the quickest and most effective means of achieving permanent cure. Conclusion Two cases of perineal endometriosis are presented. A review of the English literature show that there are 66 cases reported previously. Although the pathogenesis of endometriosis is not fully understood, the transplantation of endometrial particles at time of vaginal trauma appears to best explain the occurrence of this condition. Because patients often present with typical history and similar local findings, correct diagnosis can be
84
made. Permanent cure can be achieved with wrgical excision. References 1 Prince LN, Abrams J. Endometriosis of the perineum: review of the literature and case report. Am J Obstet Gynewl 1957;73:890-893. 2 Murray RR. Endometriosis of an episiotomy scar. Armed Forces Med J 1959;10:1463-1468. 3 Stingi A. An unusual case of endometriosis in a perineal scar. Kiin Med (Wein) 1%0;15:325-329, 4 Trampuz V. Endometriosis of the perineum. Am J Obstet Gynewl 1962;84:1522-1525. 5 Binder SS. Endometriosis of the vulva and perineum. Pacif Med Surg 1965;73:294-296. 6 Beischer N. Endometriosis of an episiotomy scar cured by pregnancy. Obstet Gynecol 1966;28:15-21. 7 McGivney J, Mazuji MR. Endometriosis of episiotomy scar: a case report. Am Surg ‘1966;32:469-471.
8 Cheleden J. Endometriosis of the perineum: report of two cases. Southern Med J 1968;61:1313-1314. 9 Paul1 T, Tedeschi LG. Perineal endometriosis at the site of episiotomy scar. Obstet Gynecol 1972;40:28-34. 10 Ramsey WH. Endometrioma involving the perianal tissue: report of a case. Dis Co1 Rect 1971;14:366-367. 11 Gordon PH, Schottler JL, Balcos EC, Goldberg SM. Perianal endometrioma; report of five cases. Dis Co1 Rect 1976;19:260-265. 12 Hambrick E, Abcaiian H, Smith D. Perineal endometrioma in episiotomy incisions: clinical features and management. Dis Co1 Rect 1979;22:550-552. 13 Wittich AC. Endomet~~is in an episiotomy scar: review of the literature and report of case. J Am Osteopath Assoc 1982;82:22-23. 14 Sampson J. The development of the implantation theory for the origin of peritoneal endometriosis. Am J Obstet Gynecol 1940;40:549-557.
