http://informahealthcare.com/gye ISSN: 0951-3590 (print), 1473-0766 (electronic) Gynecol Endocrinol, 2015; 31(6): 438–440 ! 2015 Informa UK Ltd. DOI: 10.3109/09513590.2015.1014786
ADNEXAL AGENESIS
Unilateral adnexal agenesis and dermoid cyst: fertility implications Franca Fruzzetti, Fiorella Bucci, Daria Perini, and Angiolo Gadducci
Gynecol Endocrinol Downloaded from informahealthcare.com by Nyu Medical Center on 07/29/15 For personal use only.
Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy
Abstract
Keywords
We report the loss of potential fertility in a 23-year-old nulliparous woman who attended the outpatient gynaecological endocrinology clinic for amenorrhea and hot flushes started after a laparoscopic surgery for dermoid cyst. During surgery an unilateral ovarian and fallopian tube agenesis were found. The hormonal evaluation was performed. The results evidenced high plasma levels of LH and FSH. Their values were 56.6 and 121.3 mUI/ml, respectively. The concentrations of plasma E2 were 5 20 pg/ml and of AMH were 50.08 ng/ml. Genetic analysis showed normal karyotype (46, XX). We think that if an embryological defect is suspected, an appropriate counselling about fertility preservation along with the assessment of hormonal levels before ovarian surgery should be recommended to all women in the reproductive age in order to offer future reproductive chances.
Adnexal agenesis, dermoid cyst, fertility preservation, genital ridge defects
Introduction Unilateral ovarian and fallopian tube agenesis is a rare event. Few cases are described in the literature [1–3]. Three possible causes have been hypothesized to explain this rare malformation: (1) an adnexal torsion with consequent ischemia and atresia of the organs; (2) an ischemia due to a vascular accident that causes a maldevelopment of the ovary and tube [4]; (3) an embryological defect in the development of the genital ridge and the caudal end of the Mullerian duct [2,5]. The adnexal torsion may occur in fetal life [6] or during childhood [4] or during pregnancy [7]. Such events, except when they occur in fetal life, are frequently associated with pelvic pain. The embryological defects, when they include all Mullerian structures, are commonly associated with uterus and kidney defect such as unicornuates uterus or renal agenesis, or both [8,9]. Such defects are probably due to an inadequate blood flow with a consequent alteration in autocrine and paracrine signals [10]. At the present time it is not possible to assume which one of the above mechanisms plays a major role. Indeed, it has been estimated that the incidence of the true congenital unilateral absence of the ovary and fallopian tube is about 1/11 240 women [6]. A single ovary, also in the case of oophorectomy, may be a cause for concern to women and medical staff about fertility potential. Some controversial data are present in the literature. Studies performed on rabbits, cats, mice and pigs have shown an ovarian hypertrophy and a compensatory ovulatory rate of the remaining ovary after unilateral oophorectomy [11,12]. In humans, Lass [13] reported that women with a single ovary responded less well to ovarian stimulation than women with both ovaries in terms of the number of follicles, concentrations of estradiol and number of oocytes retrieved but do not, in general, have reduced fertility potential to conceive. In agreement with Address for correspondence: Franca Fruzzetti, Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Via Roma 56, 56127 Pisa, Italy. E-mail: [email protected]
History Received 11 December 2014 Revised 21 January 2015 Accepted 30 January 2015 Published online 20 April 2015
this, Khan et al. [14] demonstrated that women with unilateral ovary had higher antral follicle count at baseline when compared with the ipsilateral ovary in referent women. The women with unilateral ovary had more follicles and oocytes when they underwent hormonal stimulation than the ipsilateral ovary of women with both ovaries. This appears to suggest compensatory follicular recruitment of the remaining ovary. In these women unilateral oophorectomy results in compensatory follicular recruitment in the remaining ovary at the time of ovarian stimulation for in vitro fertilization [14]. By contrast, other clinical studies suggest that women with a single ovary may have a decreased ovarian reserve, which may shorten the reproductive life span and increase the risk of an early menopause [15–17]. According to these authors, the possession of one ovary may be crucial for women who already have a diminished ovarian reserve. As consequence, an aggressive surgery in case of a single ovary may compromise the fertility potential. The aim of this manuscript is to discuss the clinical management of a nulliparous woman with ovarian and fallopian tube agenesis and a dermoid cyst on the single ovary.
Methods This is a case report of a nulliparous patient who attended the outpatient gynaecological endocrinology clinic of the Department of Obstetrics and Gynaecology of Pisa. The reason for the consultation was the appearance of amenorrhea and hot flushes after pelvic surgery. Her medical history was not remarkable. She referred only one abdominal pain event during childhood (age 7) that did not require medical treatment or hospitalization because of spontaneous recovery. The patient never had abdominal surgery before the age of 22. As for gynaecological history, the menarche was at age 13. Before surgery, the length of the menstrual cycles was 21 days, the menstrual flow was reported as excessive, during 5 days. At age 22 she started to have regular intercourse. Because of the request for a contraceptive method, she underwent a routine
Gynecol Endocrinol Downloaded from informahealthcare.com by Nyu Medical Center on 07/29/15 For personal use only.
DOI: 10.3109/09513590.2015.1014786
gynaecological examination. The external genitalia, uterus and vagina appeared normal. Pelvic ultrasound examination showed a right ovary mass (60 millimeters) compatible with a dermoid cyst. The left ovary was not visible. Serum tumour markers, specifically carbohydrate antigen 125 (CA-125), human chorionic gonadotropin (bHCG), a-fetoprotein and lactate dehydrogenase, were negative. Because of her age and given the benign appearance on ultrasound and negative serum tumour markers, a surgical approach with laparoscopy and ovarian cystectomy was suggested. A preoperative ultrasound examination confirmed the presence of the cyst on the right ovary and the ‘‘no visibility’’ of the left ovary. During laparoscopy many pelvic adhesions were found between the sigmoid colon and the uterus. The right ovary with the cyst was in the Douglas cul-de-sac. Confirming the ultrasound suspicion, the left ovary was not evidenced and there was only the proximal end of the left fallopian tube. Methylene blue chromopertubation did not result in spill from the left fallopian tube. The dermoid cyst was carefully removed taking care to preserve the ovarian tissue. The histological analysis confirmed mature cystic teratoma. The same analysis did not find the presence of ovarian tissue. After laparoscopy the woman did not have menstrual cycles. Because of the persistence of amenorrhea and hot flushes, after three months from the surgery she was referred by her family doctor to our outpatient clinic for the study of gynaecological endocrinological diseases. After a pregnancy exclusion, blood sample was collected for the determination of luteinizing hormone (LH), follicle-stimulating hormone (FSH), antimullerian hormone (AMH), estradiol (E2), thyroid-stimulating hormone (TSH), prolactin (PRL) plasma levels. The evaluation of the antibody anti-organs (ovary, adrenal, thyroid, gastric wall) as well as the determination of lupus anticoagulant (LAC) and anticardiolipin antibody (ACLA) was performed. The analysis of the karyotype was also planned.
Results The hormonal evaluation evidenced high plasma levels of LH and FSH. Their values were 56.6 and 121.3 mUI/ml, respectively. The concentrations of plasma E2 were 5 20 pg/ml and of AMH were 50.08 ng/ml. Genetic analysis showed normal karyotype (46, XX). The presence of X fragile was excluded. The results of other parameters tested proved negative. A 3D pelvic ultrasound showed no uterine malformations and the urinary tract was normal.
Discussion Data on the short- and long-term implications on the fertility potential of having a single ovary are scarce. In animals a compensatory effect of a single ovary has been described, but such an effect has not been clearly demonstrated in humans. Some studies suggest that women with a single ovary have reduced ovarian reserve, basically because of depletion of the total number of primordial follicles in their ovary [13,15–17]. Cramer et al. [17] found that women who lose an ovary at an early age had a strong risk of early menopause. Indeed, unilateral oophorectomy can significantly affect the fertility potential of women who already have suspected reduced ovarian reserve (e.g. those in their late thirties to early forties, with raised basal FSH concentrations). In other words, the possession of only one ovary may be crucial in women, in general, and in particular in those who already have a diminished ovarian reserve. Laparoscopic surgery has been advocated for the management of benign ovarian tumors, and ovarian cystectomy should be the preferred surgical approach for adolescents to ensure a better conservation of the ovarian tissue [18]. This surgical approach
Ovarian agenesis
439
attempts to minimize the threat to future reproductive potential by limiting resection of viable ovarian tissue. Reddy and Laufer [19] demonstrated that after cystectomy the affected ovary resumes a normal size and volume postoperatively, suggesting that the attenuated ovarian cortex naturally resorbs. The importance of a conservative surgical approach is highlighted by the recent studies that noted that women with a prior history of unilateral oophorectomy more frequently sought consultation for infertility compared to women without a similar prior surgical history [13]. In our case there was the coexistence of monolateral ovary and tube agenesis and mature teratoma in the single ovary without uterine and kidney malformations. It is generally accepted that teratoma arises from germ cells that originate in the hindgut (route of the mesentery) toward the genital ridge (primitive gonad). These totipotential cells may give rise to a variety of tissues originating from the three primitive embryonic layers. Migration along the hindgut explains how teratoma may develop in multiple locations [20]. In the literature one case including an ectopic ovary, tubes interruptions and cystic teratoma is reported [21]. These authors maintain that the mesenchyme of the genital ridges initiates ovary migration [22] and an alteration at this level can cause a maldescent of the organs, with consequently an ectopic ovary and an ovary dermoid cyst. These occur because mesenchyme loses the ability to sustain the primordial gonadal cells in meiotic arrest [21]. A torsion of the ovarian pedicle, which can occur in adult life or childhood, or even before birth has been hypothesized. Our patient remembered just a single episode of abdominal pain in childhood. It was not specific and lasted only for a short time, so it was not identified as acute abdomen. Because of the existence of multiple abnormalities in our patients, we maintain that in this case most probably the cause is an embryological defect in the development of the genital ridge supported by inadequate blood flow. This could explain the ovary and tube agenesis on the left side and the development of dermoid cyst on the right side. During laparoscopy only the dermoid cyst was removed, as confirmed by histological examination. However, immediately after surgery the patient had amenorrhea with elevated FSH plasma levels, and undetectable AMH and E2 levels. The explanation for this unexpected event is difficult because care had been taken by the surgeon to preserve ovarian tissue in consideration of the age of the young woman. Few studies in the literature discuss the dermoid cyst enucleation procedure. One of the drawbacks of cystectomy may be ovarian volume reduction due to coincidental tissue removal and large electrosurgical coagulation, thus compromising the ovarian tissue. Candiani et al. [23] and Morelli et al. [24] maintained that the main factor responsible for ovarian tissue reduction was damage to the ovarian stroma. The sharp increase in FSH observed in our woman could be related to the electrocauterization, possibly in the presence of a reduced potential fertility as suggested by Lass [13], since the histological analysis did not find the presence of ovarian tissue. In line with this hyporthesis, Li et al. [25] demonstrated a significant increase in the mean FSH value and significant decreases in the basal antral follicle count, mean ovarian diameter and peak systolic velocity caused by electrocauterization. Because of this, Morelli et al. [24] proposed a laparoscopic surgical approach with a better identification of the cleavage plane by ovarian mesial incision. The greater ovarian cortical thickness at the mesial side allows better and easier cleavage plane identification, thus providing a reduced risk of bleeding by small vessels of the ovarian wall, eventually requiring minimal electrocauterization. The author concluded that with this approach the ovarian reserve is not compromised in spite of the closeness of the site of incision to the ovarian hilum.
Gynecol Endocrinol Downloaded from informahealthcare.com by Nyu Medical Center on 07/29/15 For personal use only.
440
F. Fruzzetti et al.
By evaluating this case, some considerations can be made useful to define the potential management of dermoid cyst or other cysts in case of a pre-surgical suspicious of adnexal agenesia or in case of a previous monolateral oophorectomy. In this patient the ultrasound examination performed before surgery was unable to find the left ovary. The diagnosis of ovarian agenesis with ultrasound is obviously difficult to be definitively made. However, when unilateral ovarian agenesis is suspected, the assessment of FSH, AMH levels and the antral follicular count to evaluate the fertility of the woman before ovarian surgery should be recommended. Based on the result of these parameters, it should be possible to evaluate an initial ovarian failure and discuss with the patient the chance to perform an oocyte cryopreservation in order to offer a reproductive possibility. However also in the presence of a normal ovarian function, before an ovarian surgery the possibility of a cryopreservation should be always discussed with the patient and their parents. Moreover, a surgical approach aimed at preserving ovarian tissue with a minimal electrocauterization should be advisable.
Declaration of interest The authors report no declarations of interest.
References 1. Pabuccu E, Kahraman K, Taskın S, Atabekoglu C. Unilateral absence of fallopian tube and ovary in an infertile patient. Fertil Steril 2011;96:55–7. 2. Uckuyu A, Ozcimen EE, Sevinc Ciftci FC. Unilateral congenital ovarian and partial tubal absence: report of four cases with review of the literature. Fertil Steril 2009;91:936 e5–8. 3. Rapisarda G, Pappalardo EM, Arancio A, La Greca M. Unilateral ovarian and fallopian tube agenesis. Arch Gynecol Obstet 2009;280: 849–50. 4. Goktolga U, Ceyhan T, Ozturk H, et al. Isolated torsion of fallopian tube in a premenarcheal 12-year-old girl. J Obstet Gynaecol Res 2007;33:215–17. 5. 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. 6. Sivanesaratnam V. Unexplained unilateral absence of ovary and fallopian tube. Eur J Obstet Gynecol Reprod Biol 1986;22:103–5. 7. Yalcin OT, Hassa H, Zeytinoglu S, Isiksoy S. Isolated torsion of fallopian tube during pregnancy: report of two cases. Eur J Obstet Gynecol Reprod Biol 1997;74:179–82. 8. Muppala H, Sengupta S, Martin JE. Unilateral absence of tube and ovary with renal agenesis and associated pyloric stenosis: communication. Eur J Obstet Gynecol Reprod Biol 2008;137:123.
Gynecol Endocrinol, 2015; 31(6): 438–440
9. Haydardedeoglu B, Simsek E, Kilicdag EB, et al. A case of unicornuate uterus with ipsilateral ovarian and renal agenesis. Fertil Steril 2006;85:750.e1–7500000. 10. Mylonas I, Hansch S, Markmann S, et al. Unilateral ovarian agenesis: report of three cases and review of the literature. Arch Gynecol Obstet 2003;268:57–60. 11. Lipschuz A. Dynamics of ovarian hypertrophy under experimental conditions. Br J Exp Biol 1924–1925;2:331–46. 12. Dailey RA, Peters JB, First NL, et al. Effect of unilateral ovariectomy in the Yorkshire ND Poland China pre-puberal gilt. J Anim Sci 1969;29:775–9. 13. Lass A. The fertility potential of women with a single ovary. Hum Reprod Update 1999;5:546–50. 14. Khan Z, Gada RP, Tabbaa ZM, et al. Unilateral oophorectomy results in compensatory follicular recruitment in the remaining ovary at time of ovarian stimulation for in vitro fertilization. Fertil Steril 2014;101:722–7. 15. Alper MM, Seibel MM, Oskowitz SP, et al. Comparison of follicular response in patients with one or two ovaries in a program of in vitro fertilization. Fertil Steril 1985;44:652–5. 16. Boutteville C, Muasher SJ, Acosta AA, et al. Results of in vitro fertilization attempts in patients with one or two ovaries. Fertil Steril 1987;47:821–7. 17. Cramer DW, Xu H, Harlow BL. Does ‘‘incessant’’ ovulation increase risk for early menopause? Am J Obstet Gynecol 1995;172: 568–73. 18. Karpelowsky JS, La Hei ER, Matthews K. Laparoscopic resection of benign ovarian tumours in children with gonadal preservation. Pediat Surg Int 2009;25:251–4. 19. Reddy J, Laufer MR. Advantage of conservative surgical management of large ovarian neoplasms in adolescents. Fertil Steril 2009; 91:1941–4. 20. Printz JL, Choate JW, Townes PL, Harper RC. The embryology of supernumerary ovaries. Obstet Gynecol 1973;41:246–52. 21. Nishiyama S, Hirota Y, Nishizawa H, et al. Bilateral interruption of mid-fallopian tubes and ovarian anomalies including ectopic ovary and cystic teratoma: a previously unreported combination. Minim Invasive Gynecol 2010;17:534–7. 22. Dietrich JE, Hertweck SP, Bond SJ. Undescended ovaries: a clinical review. Pediatr Adolesc Gynecol 2007;20:57–60. 23. Candiani M, Barbieri M, Bottani B, et al. Ovarian recovery after laparoscopic enucleation of ovarian cysts: insights from echographic short-term postsurgical follow-up. J Minim Invasive Gynecol 2005; 12:409–14. 24. Morelli M, Mocciaro R, Venturella R, et al. Mesial side ovarian incision for laparoscopic dermoid cystectomy: a safe and ovarian tissue-preserving technique. Fertil Steril 2012;98:1336–40. 25. Li CZ, Liu B, Wen ZQ, Sun Q. The impact of electrocoagulation on ovarian reserve after laparoscopic excision of ovarian cysts: a prospective clinical study of 191 patients. Fertil Steril 2009;92: 1428–35.
ADNEXAL AGENESIS
Unilateral adnexal agenesis and dermoid cyst: fertility implications Franca Fruzzetti, Fiorella Bucci, Daria Perini, and Angiolo Gadducci
Gynecol Endocrinol Downloaded from informahealthcare.com by Nyu Medical Center on 07/29/15 For personal use only.
Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy
Abstract
Keywords
We report the loss of potential fertility in a 23-year-old nulliparous woman who attended the outpatient gynaecological endocrinology clinic for amenorrhea and hot flushes started after a laparoscopic surgery for dermoid cyst. During surgery an unilateral ovarian and fallopian tube agenesis were found. The hormonal evaluation was performed. The results evidenced high plasma levels of LH and FSH. Their values were 56.6 and 121.3 mUI/ml, respectively. The concentrations of plasma E2 were 5 20 pg/ml and of AMH were 50.08 ng/ml. Genetic analysis showed normal karyotype (46, XX). We think that if an embryological defect is suspected, an appropriate counselling about fertility preservation along with the assessment of hormonal levels before ovarian surgery should be recommended to all women in the reproductive age in order to offer future reproductive chances.
Adnexal agenesis, dermoid cyst, fertility preservation, genital ridge defects
Introduction Unilateral ovarian and fallopian tube agenesis is a rare event. Few cases are described in the literature [1–3]. Three possible causes have been hypothesized to explain this rare malformation: (1) an adnexal torsion with consequent ischemia and atresia of the organs; (2) an ischemia due to a vascular accident that causes a maldevelopment of the ovary and tube [4]; (3) an embryological defect in the development of the genital ridge and the caudal end of the Mullerian duct [2,5]. The adnexal torsion may occur in fetal life [6] or during childhood [4] or during pregnancy [7]. Such events, except when they occur in fetal life, are frequently associated with pelvic pain. The embryological defects, when they include all Mullerian structures, are commonly associated with uterus and kidney defect such as unicornuates uterus or renal agenesis, or both [8,9]. Such defects are probably due to an inadequate blood flow with a consequent alteration in autocrine and paracrine signals [10]. At the present time it is not possible to assume which one of the above mechanisms plays a major role. Indeed, it has been estimated that the incidence of the true congenital unilateral absence of the ovary and fallopian tube is about 1/11 240 women [6]. A single ovary, also in the case of oophorectomy, may be a cause for concern to women and medical staff about fertility potential. Some controversial data are present in the literature. Studies performed on rabbits, cats, mice and pigs have shown an ovarian hypertrophy and a compensatory ovulatory rate of the remaining ovary after unilateral oophorectomy [11,12]. In humans, Lass [13] reported that women with a single ovary responded less well to ovarian stimulation than women with both ovaries in terms of the number of follicles, concentrations of estradiol and number of oocytes retrieved but do not, in general, have reduced fertility potential to conceive. In agreement with Address for correspondence: Franca Fruzzetti, Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Via Roma 56, 56127 Pisa, Italy. E-mail: [email protected]
History Received 11 December 2014 Revised 21 January 2015 Accepted 30 January 2015 Published online 20 April 2015
this, Khan et al. [14] demonstrated that women with unilateral ovary had higher antral follicle count at baseline when compared with the ipsilateral ovary in referent women. The women with unilateral ovary had more follicles and oocytes when they underwent hormonal stimulation than the ipsilateral ovary of women with both ovaries. This appears to suggest compensatory follicular recruitment of the remaining ovary. In these women unilateral oophorectomy results in compensatory follicular recruitment in the remaining ovary at the time of ovarian stimulation for in vitro fertilization [14]. By contrast, other clinical studies suggest that women with a single ovary may have a decreased ovarian reserve, which may shorten the reproductive life span and increase the risk of an early menopause [15–17]. According to these authors, the possession of one ovary may be crucial for women who already have a diminished ovarian reserve. As consequence, an aggressive surgery in case of a single ovary may compromise the fertility potential. The aim of this manuscript is to discuss the clinical management of a nulliparous woman with ovarian and fallopian tube agenesis and a dermoid cyst on the single ovary.
Methods This is a case report of a nulliparous patient who attended the outpatient gynaecological endocrinology clinic of the Department of Obstetrics and Gynaecology of Pisa. The reason for the consultation was the appearance of amenorrhea and hot flushes after pelvic surgery. Her medical history was not remarkable. She referred only one abdominal pain event during childhood (age 7) that did not require medical treatment or hospitalization because of spontaneous recovery. The patient never had abdominal surgery before the age of 22. As for gynaecological history, the menarche was at age 13. Before surgery, the length of the menstrual cycles was 21 days, the menstrual flow was reported as excessive, during 5 days. At age 22 she started to have regular intercourse. Because of the request for a contraceptive method, she underwent a routine
Gynecol Endocrinol Downloaded from informahealthcare.com by Nyu Medical Center on 07/29/15 For personal use only.
DOI: 10.3109/09513590.2015.1014786
gynaecological examination. The external genitalia, uterus and vagina appeared normal. Pelvic ultrasound examination showed a right ovary mass (60 millimeters) compatible with a dermoid cyst. The left ovary was not visible. Serum tumour markers, specifically carbohydrate antigen 125 (CA-125), human chorionic gonadotropin (bHCG), a-fetoprotein and lactate dehydrogenase, were negative. Because of her age and given the benign appearance on ultrasound and negative serum tumour markers, a surgical approach with laparoscopy and ovarian cystectomy was suggested. A preoperative ultrasound examination confirmed the presence of the cyst on the right ovary and the ‘‘no visibility’’ of the left ovary. During laparoscopy many pelvic adhesions were found between the sigmoid colon and the uterus. The right ovary with the cyst was in the Douglas cul-de-sac. Confirming the ultrasound suspicion, the left ovary was not evidenced and there was only the proximal end of the left fallopian tube. Methylene blue chromopertubation did not result in spill from the left fallopian tube. The dermoid cyst was carefully removed taking care to preserve the ovarian tissue. The histological analysis confirmed mature cystic teratoma. The same analysis did not find the presence of ovarian tissue. After laparoscopy the woman did not have menstrual cycles. Because of the persistence of amenorrhea and hot flushes, after three months from the surgery she was referred by her family doctor to our outpatient clinic for the study of gynaecological endocrinological diseases. After a pregnancy exclusion, blood sample was collected for the determination of luteinizing hormone (LH), follicle-stimulating hormone (FSH), antimullerian hormone (AMH), estradiol (E2), thyroid-stimulating hormone (TSH), prolactin (PRL) plasma levels. The evaluation of the antibody anti-organs (ovary, adrenal, thyroid, gastric wall) as well as the determination of lupus anticoagulant (LAC) and anticardiolipin antibody (ACLA) was performed. The analysis of the karyotype was also planned.
Results The hormonal evaluation evidenced high plasma levels of LH and FSH. Their values were 56.6 and 121.3 mUI/ml, respectively. The concentrations of plasma E2 were 5 20 pg/ml and of AMH were 50.08 ng/ml. Genetic analysis showed normal karyotype (46, XX). The presence of X fragile was excluded. The results of other parameters tested proved negative. A 3D pelvic ultrasound showed no uterine malformations and the urinary tract was normal.
Discussion Data on the short- and long-term implications on the fertility potential of having a single ovary are scarce. In animals a compensatory effect of a single ovary has been described, but such an effect has not been clearly demonstrated in humans. Some studies suggest that women with a single ovary have reduced ovarian reserve, basically because of depletion of the total number of primordial follicles in their ovary [13,15–17]. Cramer et al. [17] found that women who lose an ovary at an early age had a strong risk of early menopause. Indeed, unilateral oophorectomy can significantly affect the fertility potential of women who already have suspected reduced ovarian reserve (e.g. those in their late thirties to early forties, with raised basal FSH concentrations). In other words, the possession of only one ovary may be crucial in women, in general, and in particular in those who already have a diminished ovarian reserve. Laparoscopic surgery has been advocated for the management of benign ovarian tumors, and ovarian cystectomy should be the preferred surgical approach for adolescents to ensure a better conservation of the ovarian tissue [18]. This surgical approach
Ovarian agenesis
439
attempts to minimize the threat to future reproductive potential by limiting resection of viable ovarian tissue. Reddy and Laufer [19] demonstrated that after cystectomy the affected ovary resumes a normal size and volume postoperatively, suggesting that the attenuated ovarian cortex naturally resorbs. The importance of a conservative surgical approach is highlighted by the recent studies that noted that women with a prior history of unilateral oophorectomy more frequently sought consultation for infertility compared to women without a similar prior surgical history [13]. In our case there was the coexistence of monolateral ovary and tube agenesis and mature teratoma in the single ovary without uterine and kidney malformations. It is generally accepted that teratoma arises from germ cells that originate in the hindgut (route of the mesentery) toward the genital ridge (primitive gonad). These totipotential cells may give rise to a variety of tissues originating from the three primitive embryonic layers. Migration along the hindgut explains how teratoma may develop in multiple locations [20]. In the literature one case including an ectopic ovary, tubes interruptions and cystic teratoma is reported [21]. These authors maintain that the mesenchyme of the genital ridges initiates ovary migration [22] and an alteration at this level can cause a maldescent of the organs, with consequently an ectopic ovary and an ovary dermoid cyst. These occur because mesenchyme loses the ability to sustain the primordial gonadal cells in meiotic arrest [21]. A torsion of the ovarian pedicle, which can occur in adult life or childhood, or even before birth has been hypothesized. Our patient remembered just a single episode of abdominal pain in childhood. It was not specific and lasted only for a short time, so it was not identified as acute abdomen. Because of the existence of multiple abnormalities in our patients, we maintain that in this case most probably the cause is an embryological defect in the development of the genital ridge supported by inadequate blood flow. This could explain the ovary and tube agenesis on the left side and the development of dermoid cyst on the right side. During laparoscopy only the dermoid cyst was removed, as confirmed by histological examination. However, immediately after surgery the patient had amenorrhea with elevated FSH plasma levels, and undetectable AMH and E2 levels. The explanation for this unexpected event is difficult because care had been taken by the surgeon to preserve ovarian tissue in consideration of the age of the young woman. Few studies in the literature discuss the dermoid cyst enucleation procedure. One of the drawbacks of cystectomy may be ovarian volume reduction due to coincidental tissue removal and large electrosurgical coagulation, thus compromising the ovarian tissue. Candiani et al. [23] and Morelli et al. [24] maintained that the main factor responsible for ovarian tissue reduction was damage to the ovarian stroma. The sharp increase in FSH observed in our woman could be related to the electrocauterization, possibly in the presence of a reduced potential fertility as suggested by Lass [13], since the histological analysis did not find the presence of ovarian tissue. In line with this hyporthesis, Li et al. [25] demonstrated a significant increase in the mean FSH value and significant decreases in the basal antral follicle count, mean ovarian diameter and peak systolic velocity caused by electrocauterization. Because of this, Morelli et al. [24] proposed a laparoscopic surgical approach with a better identification of the cleavage plane by ovarian mesial incision. The greater ovarian cortical thickness at the mesial side allows better and easier cleavage plane identification, thus providing a reduced risk of bleeding by small vessels of the ovarian wall, eventually requiring minimal electrocauterization. The author concluded that with this approach the ovarian reserve is not compromised in spite of the closeness of the site of incision to the ovarian hilum.
Gynecol Endocrinol Downloaded from informahealthcare.com by Nyu Medical Center on 07/29/15 For personal use only.
440
F. Fruzzetti et al.
By evaluating this case, some considerations can be made useful to define the potential management of dermoid cyst or other cysts in case of a pre-surgical suspicious of adnexal agenesia or in case of a previous monolateral oophorectomy. In this patient the ultrasound examination performed before surgery was unable to find the left ovary. The diagnosis of ovarian agenesis with ultrasound is obviously difficult to be definitively made. However, when unilateral ovarian agenesis is suspected, the assessment of FSH, AMH levels and the antral follicular count to evaluate the fertility of the woman before ovarian surgery should be recommended. Based on the result of these parameters, it should be possible to evaluate an initial ovarian failure and discuss with the patient the chance to perform an oocyte cryopreservation in order to offer a reproductive possibility. However also in the presence of a normal ovarian function, before an ovarian surgery the possibility of a cryopreservation should be always discussed with the patient and their parents. Moreover, a surgical approach aimed at preserving ovarian tissue with a minimal electrocauterization should be advisable.
Declaration of interest The authors report no declarations of interest.
References 1. Pabuccu E, Kahraman K, Taskın S, Atabekoglu C. Unilateral absence of fallopian tube and ovary in an infertile patient. Fertil Steril 2011;96:55–7. 2. Uckuyu A, Ozcimen EE, Sevinc Ciftci FC. Unilateral congenital ovarian and partial tubal absence: report of four cases with review of the literature. Fertil Steril 2009;91:936 e5–8. 3. Rapisarda G, Pappalardo EM, Arancio A, La Greca M. Unilateral ovarian and fallopian tube agenesis. Arch Gynecol Obstet 2009;280: 849–50. 4. Goktolga U, Ceyhan T, Ozturk H, et al. Isolated torsion of fallopian tube in a premenarcheal 12-year-old girl. J Obstet Gynaecol Res 2007;33:215–17. 5. 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. 6. Sivanesaratnam V. Unexplained unilateral absence of ovary and fallopian tube. Eur J Obstet Gynecol Reprod Biol 1986;22:103–5. 7. Yalcin OT, Hassa H, Zeytinoglu S, Isiksoy S. Isolated torsion of fallopian tube during pregnancy: report of two cases. Eur J Obstet Gynecol Reprod Biol 1997;74:179–82. 8. Muppala H, Sengupta S, Martin JE. Unilateral absence of tube and ovary with renal agenesis and associated pyloric stenosis: communication. Eur J Obstet Gynecol Reprod Biol 2008;137:123.
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