- Email: [email protected]
Unilateral pelvic endometriosis and congenital unilateral ovarian agenesis
CORRESPONDENCE
285
Unilateral pelvic endometriosis and congenital unilateral ovarian agenesis Sir, The striking unilateral pelvic endometriosis documented in two recently encountered women from a busy specialist endoscopic practice with a heavy streaming of patients with
286
CORRESPONDENCE
known or suspected endometriosis, and both with the rare congenital unilateral ovarian agenesis, has prompted this report. Such a congenital anomaly has an estimated incidence of 1 in 11 240 females1 but the number of reported cases is ridiculously small: only 15 in the last 32 years, not including the two cases reported below. Case 1 was a 17-year-old nulliparous woman, with a medical history of depression and fibromyalgia, who presented with severe dysmenorrhoea, right iliac fossa and bladder-distension pain. The patient underwent a laparoscopy in November 2004 at which peritoneal endometriosis was noted (diagnosis was confirmed histologically), affecting the left pelvic side wall, the left aspect of the cul-de-sac and left uterovesical fold. Adhesions were noted between the left ovary pelvic side wall; however, the right ovary was absent. Both fallopian tubes were normal in appearance and position; however, hydrotubation using methylene blue solution did not demonstrate tubal patency. No endometriosis was seen on the right hand aspect of the pelvis. Post-operative renal ultrasound showed normal kidneys. A peripheral blood karyotype was normal 46,XX. Case 2 was an 18-year-old nulliparous woman, with a medical history of recurrent urinary tract infections, who presented with increasing dysmenorrhoea and a recent episode of right iliac fossa pain. A mass was found in the right inguinal region, which was biopsied and histologically confirmed to be endometriosis. She underwent a laparoscopy in July 2004, which revealed endometriosis involving the right round ligament and right ovary. The right ovary was otherwise normal, as were both fallopian tubes. No tubal patency tests were performed. The left ovary was noted to be absent. The endometriosis was excised with a combined laparoscopic and open inguinal approach. A post-operative renal ultrasound revealed cystic changes in both kidneys. A post-operative normal peripheral blood karyotype (46,XX) was found. Unilateral absence of the ovary may result from a congenital malformation or torsion of the ovarian pedicle leading to avascular necrosis with separation of this tissue and subsequent resorption.2 Ovarian agenesis may occur in the presence or absence of an adjacent fallopian tube. The unilateral congenital absence may arise from a defect of the caudal end of the mu¨llerian duct and the genital ridge. Alternatively, it may be due to a developmental defect of the whole mu¨llerian and mesonephric duct system on one side.3 Absence of the fallopian tube may result from canalisation failure of the mu¨llerian duct1 which can also lead to the development of a unicornate uterus. Torsion of the ovarian pedicle at any stage leading to avascular necrosis of the ovary may also account for its absence.4 Such torsion is most likely to present with acute abdominal pain, nausea and vomiting if occurring in childhood or adulthood. It may, however, also be asymptomatic suggesting the event occurred during fetal life, as in a case reported by Dueck et al.5 This hypothesis could be supported by previous radiological imaging suggesting the presence of both ovaries or by the finding of separated structures in the abdominal cavity at surgery which were later histologically confirmed to be ovarian tissue.6,7 The true aetiology of unilateral ovarian absence remains unclear in most of the few cases reported. The absence of any other anatomical abnormalities, as in these
Pathology (2007), 39(2), April
two cases, would suggest a vascular accident rather than a developmental anomaly as the more likely cause.2,8,9 Both of our cases presented with pelvic pain, which was most likely secondary to endometriosis. It is possible but highly unlikely that this pain masked acute recent events of ovarian torsion. Renal ultrasound revealed bilateral polycystic kidneys in the second patient. The close embryological relationship between the mu¨llerian and wolffian ducts is thought responsible for the concurrence of crossed renal ectopia and ovarian absence, as reported by Zaitoon and Florentin.10 Renal tract investigation is thus very important. The observation that the pelvic peritoneal endometriosis was unilateral in both cases, despite normal fallopian tubes being present bilaterally, and on the contralateral side to the ovarian agenesis, is of uncertain significance but clearly not serendipitous. It gives rise to speculation as to local influences in pathogenesis of endometriosis in these two patients. It may add support to the metaplastic theory in the evolution of endometriosis, if the ovarian agenesis were developmental and accompanied by an abnormally restricted sequestration of subperitoneal ‘pluripotential’ cells from which endometriosis could later arise. Alternatively, the lack of the intense local hormonal influence from a missing ovary may have been sufficient to explain the successful seeding of shed endometrial cells only on the side of the normal ovary. Relevant to both of these propositions is the previously noted observation of pelvic endometriosis occurring despite the presence of bilateral ovarian dysgenesis.11,12 It seems fatuous to speculate that the absent ovaries accompanied normallooking but obstructed fallopian tubes and that the endometrial cells shed from the contralateral fallopian tubes were mysteriously unable to cross the midline and implant. Keith Johnston* Peter Russell{ Anshu Shukla* Geoffery Reid* Michael Cooper* *Sydney Women’s Endosurgery Centre, St George Private Hospital, Kogarah, {Symbion Laverty Pathology, Macquarie Park, and Department of Pathology, University of Sydney, New South Wales, Australia Contact Dr K. Johnston. E-mail: [email protected]
1. Sivanesaratnam V. Unexplained unilateral absence of ovary and fallopian tube. Eur J Obstet Gynecol Reprod Biol 1986; 22: 103–5. 2. Eustace DL. Congenital absence of fallopian tube and ovary. Eur J Obstet Gynecol Reprod Biol 1992; 46: 157–9. 3. Dare FO, Makinde OO, Makinde ON, Odutayo R. Congenital absence of an ovary in a Nigerian woman. Int J Gynaecol Obstet 1989; 29: 377–8. 4. James DF, Barber HR, Graber EA. Torsion of normal uterine adnexa in children. Report of three cases. Obstet Gynecol 1970; 35: 226–30. 5. Dueck A, Poenaru D, Jamieson MA, Kamal IK. Unilateral ovarian agenesis and fallopian tube maldescent. Pediatr Surg Int 2001; 17: 228–9. 6. Sebastian JA, Baker RL, Cordray D. Asymptomatic infarction and separation of ovary and distal uterine tube. Obstet Gynecol 1973; 41: 531–5.
CORRESPONDENCE
7. Silva PD, Glasser KE, Virata RL. Spontaneously acquired, unilateral absence of the adnexa. A case report. J Reprod Med 1995; 40: 63–4. 8. Georgy FM, Viechnicki MB. Absence of an ovary and uterine tube. Obstet Gynecol 1974; 44: 441–2. 9. Sirisena LA. Unexplained absence of an ovary and uterine tube. Postgrad Med J 1978; 54: 423–4. 10. Zaitoon MM, Florentin H. Crossed renal ectopia with unilateral agenesis of fallopian tube and ovary. J Urol 1982; 128: 111. 11. Bosze P, Gaal M, Toth A, Laszlo J. Endometriosis and streak gonad syndrome. Arch Gynecol 1987; 240: 253–4. 12. Kourounis G, Fotopoulos A, Decavalas G, Michail G. Endometriosis and ovarian dysgenesis: a case report. Clin Exp Obstet Gynecol 2004; 31: 311–2.
DOI: 10.1080/00313020701230690
287
285
Unilateral pelvic endometriosis and congenital unilateral ovarian agenesis Sir, The striking unilateral pelvic endometriosis documented in two recently encountered women from a busy specialist endoscopic practice with a heavy streaming of patients with
286
CORRESPONDENCE
known or suspected endometriosis, and both with the rare congenital unilateral ovarian agenesis, has prompted this report. Such a congenital anomaly has an estimated incidence of 1 in 11 240 females1 but the number of reported cases is ridiculously small: only 15 in the last 32 years, not including the two cases reported below. Case 1 was a 17-year-old nulliparous woman, with a medical history of depression and fibromyalgia, who presented with severe dysmenorrhoea, right iliac fossa and bladder-distension pain. The patient underwent a laparoscopy in November 2004 at which peritoneal endometriosis was noted (diagnosis was confirmed histologically), affecting the left pelvic side wall, the left aspect of the cul-de-sac and left uterovesical fold. Adhesions were noted between the left ovary pelvic side wall; however, the right ovary was absent. Both fallopian tubes were normal in appearance and position; however, hydrotubation using methylene blue solution did not demonstrate tubal patency. No endometriosis was seen on the right hand aspect of the pelvis. Post-operative renal ultrasound showed normal kidneys. A peripheral blood karyotype was normal 46,XX. Case 2 was an 18-year-old nulliparous woman, with a medical history of recurrent urinary tract infections, who presented with increasing dysmenorrhoea and a recent episode of right iliac fossa pain. A mass was found in the right inguinal region, which was biopsied and histologically confirmed to be endometriosis. She underwent a laparoscopy in July 2004, which revealed endometriosis involving the right round ligament and right ovary. The right ovary was otherwise normal, as were both fallopian tubes. No tubal patency tests were performed. The left ovary was noted to be absent. The endometriosis was excised with a combined laparoscopic and open inguinal approach. A post-operative renal ultrasound revealed cystic changes in both kidneys. A post-operative normal peripheral blood karyotype (46,XX) was found. Unilateral absence of the ovary may result from a congenital malformation or torsion of the ovarian pedicle leading to avascular necrosis with separation of this tissue and subsequent resorption.2 Ovarian agenesis may occur in the presence or absence of an adjacent fallopian tube. The unilateral congenital absence may arise from a defect of the caudal end of the mu¨llerian duct and the genital ridge. Alternatively, it may be due to a developmental defect of the whole mu¨llerian and mesonephric duct system on one side.3 Absence of the fallopian tube may result from canalisation failure of the mu¨llerian duct1 which can also lead to the development of a unicornate uterus. Torsion of the ovarian pedicle at any stage leading to avascular necrosis of the ovary may also account for its absence.4 Such torsion is most likely to present with acute abdominal pain, nausea and vomiting if occurring in childhood or adulthood. It may, however, also be asymptomatic suggesting the event occurred during fetal life, as in a case reported by Dueck et al.5 This hypothesis could be supported by previous radiological imaging suggesting the presence of both ovaries or by the finding of separated structures in the abdominal cavity at surgery which were later histologically confirmed to be ovarian tissue.6,7 The true aetiology of unilateral ovarian absence remains unclear in most of the few cases reported. The absence of any other anatomical abnormalities, as in these
Pathology (2007), 39(2), April
two cases, would suggest a vascular accident rather than a developmental anomaly as the more likely cause.2,8,9 Both of our cases presented with pelvic pain, which was most likely secondary to endometriosis. It is possible but highly unlikely that this pain masked acute recent events of ovarian torsion. Renal ultrasound revealed bilateral polycystic kidneys in the second patient. The close embryological relationship between the mu¨llerian and wolffian ducts is thought responsible for the concurrence of crossed renal ectopia and ovarian absence, as reported by Zaitoon and Florentin.10 Renal tract investigation is thus very important. The observation that the pelvic peritoneal endometriosis was unilateral in both cases, despite normal fallopian tubes being present bilaterally, and on the contralateral side to the ovarian agenesis, is of uncertain significance but clearly not serendipitous. It gives rise to speculation as to local influences in pathogenesis of endometriosis in these two patients. It may add support to the metaplastic theory in the evolution of endometriosis, if the ovarian agenesis were developmental and accompanied by an abnormally restricted sequestration of subperitoneal ‘pluripotential’ cells from which endometriosis could later arise. Alternatively, the lack of the intense local hormonal influence from a missing ovary may have been sufficient to explain the successful seeding of shed endometrial cells only on the side of the normal ovary. Relevant to both of these propositions is the previously noted observation of pelvic endometriosis occurring despite the presence of bilateral ovarian dysgenesis.11,12 It seems fatuous to speculate that the absent ovaries accompanied normallooking but obstructed fallopian tubes and that the endometrial cells shed from the contralateral fallopian tubes were mysteriously unable to cross the midline and implant. Keith Johnston* Peter Russell{ Anshu Shukla* Geoffery Reid* Michael Cooper* *Sydney Women’s Endosurgery Centre, St George Private Hospital, Kogarah, {Symbion Laverty Pathology, Macquarie Park, and Department of Pathology, University of Sydney, New South Wales, Australia Contact Dr K. Johnston. E-mail: [email protected]
1. Sivanesaratnam V. Unexplained unilateral absence of ovary and fallopian tube. Eur J Obstet Gynecol Reprod Biol 1986; 22: 103–5. 2. Eustace DL. Congenital absence of fallopian tube and ovary. Eur J Obstet Gynecol Reprod Biol 1992; 46: 157–9. 3. Dare FO, Makinde OO, Makinde ON, Odutayo R. Congenital absence of an ovary in a Nigerian woman. Int J Gynaecol Obstet 1989; 29: 377–8. 4. James DF, Barber HR, Graber EA. Torsion of normal uterine adnexa in children. Report of three cases. Obstet Gynecol 1970; 35: 226–30. 5. Dueck A, Poenaru D, Jamieson MA, Kamal IK. Unilateral ovarian agenesis and fallopian tube maldescent. Pediatr Surg Int 2001; 17: 228–9. 6. Sebastian JA, Baker RL, Cordray D. Asymptomatic infarction and separation of ovary and distal uterine tube. Obstet Gynecol 1973; 41: 531–5.
CORRESPONDENCE
7. Silva PD, Glasser KE, Virata RL. Spontaneously acquired, unilateral absence of the adnexa. A case report. J Reprod Med 1995; 40: 63–4. 8. Georgy FM, Viechnicki MB. Absence of an ovary and uterine tube. Obstet Gynecol 1974; 44: 441–2. 9. Sirisena LA. Unexplained absence of an ovary and uterine tube. Postgrad Med J 1978; 54: 423–4. 10. Zaitoon MM, Florentin H. Crossed renal ectopia with unilateral agenesis of fallopian tube and ovary. J Urol 1982; 128: 111. 11. Bosze P, Gaal M, Toth A, Laszlo J. Endometriosis and streak gonad syndrome. Arch Gynecol 1987; 240: 253–4. 12. Kourounis G, Fotopoulos A, Decavalas G, Michail G. Endometriosis and ovarian dysgenesis: a case report. Clin Exp Obstet Gynecol 2004; 31: 311–2.
DOI: 10.1080/00313020701230690
287