- Email: [email protected]
Early Polycystic Ovary Syndrome as a Possible Etiology of Unexplained Premenarcheal Ovarian Torsion
J Pediatr Adolesc Gynecol (2009) 22:265e269
Original Study Early Polycystic Ovary Syndrome as a Possible Etiology of Unexplained Premenarcheal Ovarian Torsion Anish A. Shah, MD, Creighton E. Likes, MD, and Thomas M. Price, MD Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina, USA
Abstract. Study Objective: To study evidence of polycystic ovary syndrome (PCOS) in premenarcheal adolescents with unexplained ovarian torsion. Design: Retrospective observational case series. Setting: Tertiary university clinical center Participants: Six premenarcheal adolescents and six adults with acute ovarian torsion Intervention: A chart review. Main Outcome Measures: Contralateral ovarian size, operative findings, ovarian pathology, hormone testing Results: Five of the six premenarcheal cases had no pathologic explanation for their ovarian torsion. In four of the cases, size measurements of the contralateral ovary were noted to be larger than the criterion of their respective age group. Three of the four cases had either an ovarian volume (28.5 cm3) or an area (16.0 cm2 and 57.6 cm2) that was above the size criterion for a polycystic ovary (volume O10 cm3 or area O 5.5 cm2). Pathology of a wedge biopsy of one of the contralateral ovaries suggested evidence of polycystic ovary. Finally, hormone testing available in three of the cases revealed elevated testosterone levels in two. Among the adults, half of the cases had a pathologic explanation for ovarian torsion. One out of the five cases had a contralateral ovary that was significantly enlarged and this was noted in a woman with a diagnosis of PCOS. The remaining two cases had extensive necrosis of the torsed ovary and no other diagnosis was made. Conclusion: We propose that premenarcheal girls presenting with ovarian torsion, without obvious ovarian pathology, be screened for ultrasound and biochemical evidence of PCOS. In those with evidence of PCOS, treatment with oral contraceptives should be considered taking into account the age and pubertal development, to decrease ovarian volume.
Key Words. Polycystic ovary syndrome—Ovarian torsion—Premenarcheal—Adolescence
Introduction
Address correspondence to: Thomas M. Price, MD, Duke Fertility Center, Duke University Medical Center, 5704 Fayetteville Road, Durham, NC 27713.; E-mail: [email protected]
Ovarian torsion is the fifth most common gynecologic surgical emergency in females of all ages with a prevalence of 2.7%.1,2 Twisting of the ovary compromises vascular pedicles in the suspensory ligaments, impeding lymphatic and venous drainage and decreasing arterial flow. Continued arterial perfusion often leads to an enlarged ovary that eventually develops necrosis, infarction, and local hemorrhage, resulting in peritonitis, and even systemic infection and inflammation.1,3e5 Therefore, it is essential to diagnose and treat ovarian torsion as early as possible to preserve the adnexa. Though ovarian torsion has been described in all age groups, it is most common in reproductive aged women due to the increased frequency of physiologic and pathologic ovarian neoplasms.6,7 Ovarian cysts and neoplasms account for up to 94% of cases in adult women.4e6,8,9 Other etiologies include pregnancy and ovulation induction, which have a reported incidence of 12% and 8%, respectively.7,10 In general, processes that lead to enlarged ovaries predispose to torsion. Procedures that result in adnexal hypermobility such as a previous tubal ligation or laparoscopic hysterectomy may contribute to torsion. Right ovarian torsion is more common than left with a frequency ratio of 5:2, thought to be due to the sigmoid colon reducing available space for ovarian movement.2,3,5,6,9 In the adolescent premenarcheal population, anatomical factors such as ovarian cyst or neoplasm account for approximately half of the cases of ovarian torsion. Thus, 50% of cases in this population have normal ovaries that have undergone torsion.6,8 We
Ó 2009 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc.
1083-3188/09/$36.00 doi:10.1016/j.jpag.2008.11.005
266
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion
believe that one possible etiology of unexplained ovarian torsion in this population is early polycystic ovary syndrome (PCOS). Since PCOS likely begins long before its physical manifestations during late adolescence and early adulthood, we believe the increased ovarian volume associated with PCOS predisposes these adolescents to ovarian torsion.11e14 Methods With IRB approval, we reviewed the medical records of all children aged 8e12 years old and adults with the diagnosis of ovarian torsion resulting in oophorectomy in a ten year period (1997e2007) at Duke University Medical Center, Durham, NC. Using the International Classifications of Diseases, Ninth Revision code to identify potential cases of ovarian torsion (code 620.5, torsion of ovary, ovarian pedicle, or fallopian tube, torsion: accessory tube hydatid of Morgagni), we were able to identify twelve cases. Six cases were from premenarcheal girls and six were adults. The adult population had no evidence of infertility. We then abstracted the medical records for data that included the patient’s age, race, menarcheal status, imaging studies, surgical findings, surgical outcomes, pathology reports, and laboratory tests. Medical records were abstracted by a single investigator (C.E.L.) and then abstracted by a second investigator (A.A.S.) with no significant disagreement. Results In the six cases of acute ovarian torsion among premenarcheal girls, the mean age was 10 years (Table 1). Torsion of the right ovary was found in 50% of the cases. One case had a pathologic diagnosis of a mature teratoma. The other five cases were noted as benign with no evidence of any ovarian cyst or neoplasm. The contralateral ovary was imaged preoperatively or inspected during the operation with size measurements in four of the six cases. All ovaries with measurements were larger than would be expected when compared to standard measurements with their respective ages. Two patients had a calculable ovarian volume of 28.5 cm3 and 7.2 cm3 on preoperative ultrasound, which was significantly greater than the expected volumes of 1.8 cm3 0.8 and 2.6 cm3 0.9 for a 10- and 11-year-old, respectively.15,16 Two other cases had measurements performed intraoperatively by the surgeon and were noted to be 4 5 cm (or a maximal area of 16.0 cm2) and 8 9 cm (or maximal area of 57.6 cm2). Interestingly, in all four cases the ultrasonographer or the surgeon commented on the contralateral ovary appearing polycystic and significantly enlarged. In one case, the surgeon performed a wedge biopsy on the contralateral ovary
and on final pathology it was noted to have serous cystadenofibroma and extensive hilar fibrosis. Hormone testing was performed in three of the premenarcheal cases (Table 1). One girl had an elevated free testosterone of 12.2 ng/dL (normal ! 8.5 ng/dL). In two other cases total testosterone levels were measured at 22 and 51 ng/dL (normal 10e35 ng/dL). Luteinizing hormone and follicle-stimulating hormone were evaluated in two of the cases and noted to have a normal ratio in both. Finally, dehydroepiandrosterone sulfate (DHEAS) levels were noted within the normal range for their respective ages in all three of the cases. Among the adults, the mean age was 29 years. Five of the six cases showed torsion of the right ovary. Three of the cases had abnormal pathologic features: two mucinous cystadenomas and one large ovarian cyst. Two other cases were noted to have extensive necrosis on final pathologic examination and thus no other specific diagnosis could be ascertained. Their respective contralateral ovaries were noted to be normal. All six cases had preoperative ultrasound measurements; however, in only one case was the contralateral ovary noted to be very enlarged with numerous tiny follicles. This patient had a diagnosis of PCOS and the torsed ovary in this case had no additional pathologic diagnosis. Fig. 1 shows a laparoscopic view of the non-torsed ovary of the 10-year-old patient described in Table 1. Three months prior, she had undergone right salpingooophorectomy for torsion. Acute onset of left pelvic pain with questionable ovarian blood flow led to a repeat laparoscopy showing an enlarged, but non-torsed ovary. Oophoropexy was performed by shortening the ovarian ligament. Discussion The diagnostic criteria for PCOS include polycystic appearing ovaries, oligo- anovulation, clinical or biochemical signs of unexplained hyperandrogenism and no other disease process with similar presentation such as congenital adrenal hyperplasia.11e13 The etiology, although unclear, is likely the result of a number of intrinsic ovarian genetic traits interacting with congenital or environmental factors that begin long before the physical manifestations during late adolescence.11e14 In the adolescent population, ovarian enlargement is one of the most specific findings of excessive ovarian stroma. Ovarian volume of O10 cm3 or maximal area of O5.5 cm2 are characteristic of a polycystic ovary based on international consensus definition.12,17 In our study, the contralateral ovary was used as a surrogate of the torsed ovary since the polycystic features of PCOS are bilateral. Again, it would be expected that the torsed ovary would be enlarged due to the continued arterial flow in the setting of obstructed venous and
Age (yrs)
Race
Contralateral Ovary (cm)
Operative Findings
Pathology
11
White
2.6 1.4 3.8 (vol: 7.2 cm3)
8
Black
4 5 (area: 16.0 cm2)
11
White
No description
10
White
5.1 4.1 2.6 (vol: 28.5 cm3)
Left ovary with multiple follicles, it measures 3x2 cm. Right ovary completely torsed. Hemorrhagic right ovarian complex measuring 7x5 cm. Left ovary slightly enlarged, 4x5 cm but normal. Right adnexa very enlarged. Left ovary normal. Right ovary 8 cm necrotic. Left ovary enlarged, dilated left tube.
11, 12
Black
8 9 (area: 57.6 cm2)
First surgery: Enlarged left ovary w/ clot and hemorrhage 10x9 cm. Right ovary w/ multiple small cysts, 8x9 cm. Second Surgery: (10 mo later) Multiple small cysts in right ovary
First surgery: Left ovary: 12.5 18 6 cm, benign normal. Wedge Bx of Right ovary: Benign serous cystadenofibroma. Second Surgery: Right ovary: 5.0 2.5 1.5 cm, multiple follicles, extensive hilar/fibrous tissue.
9
White
No description
Left ovary torsed, 7x5 cm. Right ovary normal
10.5 8.5 4 cm, Mature teratoma
Hormone Testing
9.5 5.5 5 cm, Benign, normal
N/A
6.5 6 2.5 cm, Benign, normal
Test (total): 22 ng/dL, DHEAS: 111 mcg/dL, E2: 14 pg/mL
15 13.5 8.2 cm, Benign, normal
N/A
7.9 7.2 2.3 cm, Benign, normal
LH: !0.1 mIU/mL, FSH: !0.1 mIU/ mL, 17OHP: 174 ng/dL, DHEAS: 56 mcg/dL Test (total): 51 ng/dL, TSH: 2.53 uIU/ mL LH: 1.6 mIU/mL, FSH: 1.7 mIU/mL, DHEAS: 104 mcg/dL, Test (free): 12 pg/mL E2: 43 pg/mL
N/A
DHEAS 5 Dehydroepiandrosterone Sulfate (normal range: 35 e 130 mcg/dL), FSH 5 Follicle-Stimulating Hormone (normal range: 0.4 e 6.9 mIU/mL), LH 5 Luteinizing Hormone (normal range: !4.0 mIU/mL), Test 5 Testosterone (normal range, total: 10 e 35 ng/dL, free: undetectable e 8.5 pg/mL), E2 5 Estradiol (normal range: undetectable e 24 pg/mL), 17OHP 5 17-hydroxyprogesterone (normal range: !100 ng/dL), TSH 5 Thyroid Stimulating Hormone (normal: range 0.34 e 5.66 uIU/mL)
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion
Table 1. Findings of all Premenarcheal Cases
267
268
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion
Fig 1. Enlarged contralateral ovary in the 10 year old patient with previous ovarian torsion.
lymphatic outflow. Interestingly, in three of the four adolescent girls with measurements of their contralateral ovary available, their ovarian volume or area was much larger than the minimal criteria of 10 cm3 or 5.5 cm2.12,17 Even in the adolescent girl who did not meet volume criteria for PCOS, her volume of 7.2 cm3 was still significantly enlarged when compared to her age group, which has an average of 2.6 cm3 0.9.16 Furthermore, in the one case where a wedge biopsy was performed, the ovary was noted to have extensive hilar fibrosis possibly due to an increase in ovarian stroma as seen in PCOS.17 Also, when comparing the adult population, the only significantly enlarged contralateral ovary was noted in a woman with PCOS. Hormone testing available in the premenarcheal adolescents revealed elevated testosterone levels in two of the three cases. With features of polycystic ovaries and biochemical signs of hyperandrogenism, these two cases meet the diagnostic criteria for PCOS. None of the girls showed elevated DHEAS levels or revealed a history of premature adrenarche. Ibanez et al18 was the first to describe the correlation between premature adrenarche and PCOS when they noted that half of their study population later developed PCOS. Also, of the remaining girls who did not develop PCOS, one sixth had polycystic ovaries.18 The most recognized risk factor for torsion in the adult population is ovarian enlargement, often due to functional cyst or neoplasm. This has not been seen as frequently in premenarcheal girls, thus leading to other hypotheses including congenitally long supportive ligaments, mesovarium, and mesosalpynx resulting in hypermobility.2,3,9 Our theory of enlarged polycystic ovaries as a risk factor for torsion in this age group has not been previously proposed. Another supporting observation for the role of PCOS in torsion in premenarcheal girls is the occurrence of
asynchronous bilateral ovarian torsion (ABOT) at a rate of approximately 11.4%. ABOT is the subsequent torsion of the remaining ovary which may occur months to years later. In a report of four cases, the mean time was 15.7 months with a range of 7 to 30 months. No underlying pathology was found.2 Due to the catastrophic nature of ABOT, oophoropexy of the non-affected ovary should be considered in cases of premenarcheal torsion. Techniques include shortening the ovarian ligament,19 fixation of the ovary to the posterior abdominal wall,20 or fixation of the ovary to the pelvic side wall21 with permanent suture. We propose that premenarcheal girls presenting with ovarian torsion, without obvious ovarian pathology, be screened for ultrasound and biochemical evidence of PCOS. In those with evidence of PCOS, treatment with oral contraceptives should be considered taking into account the age and pubertal development, to decrease ovarian volume. Although it was not studied in premenarcheal girls, a 6-month treatment with oral contraceptives in adults with PCOS resulted in a 37% decrease in ovarian volume22 from 14.0 to 9.4 cm3. A decrease in ovarian volume may further decrease the risk of ovarian torsion. References 1. Pena JE, Ufberg D, Cooney N, et al: Usefulness of doppler sonography in diagnosis of ovarian torsion. Fertil Steril 2001; 75:1041 2. Beaunoyer M, Chapdelaine J, Boucharch S, et al: Asynchronous bilateral ovarian torsion. J Pediatr Surg 2004; 39:746 3. Ozcan C, Celik A, Ozok G, et al: Adnexal torsion in children may have a catastrophic sequel: asynchronous bilateral torsion. J Pediatr Surg 2002; 37:1617 4. Anders JF, Powell EC: Urgency of evaluation and outcome of acute ovarian torsion in pediatric patients. Arch Pediatr Adolesc Med 2005; 159:532 5. Kokoska ER, Keller MS, Weber TR: Acute ovarian torsion in children. Am J Surg 2000; 180:462 6. Cass D: Ovarian Torsion. Semin Pediatr Surg 2005; 14:86 7. Rackow BW, Patrizio P: Successful pregnancy complicated by early and late adnexal torsion after in vitro fertilization. Fertil Steril 2007; 87:697 8. Varras M, Tsikini A, Polyzos D, et al: Uterine adnexal torsion: Pathologic and gray scale ultrasound findings. Clin Exp Obstet Gynecol 2004; 31:34 9. Rousseau V, Massicot R, Darwish AA, et al: Emergency management and conservative surgery of ovarian torsion in children: A report of 40 cases. J Pediatr Adolesc Gynecol 2008; 21:201 10. Gorkemli H, Camus M, Clasen K: Adnexal torsion after gonadotrophin ovulation induction for IVF or ICSI and its conservative treatment. Arch Gynecol Obstet 2002; 267:4 11. Azziz R: Diagnostic criteria for polycystic ovary syndrome: A reappraisal. Fertil Steril 2005; 85:1343 12. Mortensen M, Rosenfield RL, Littlejohn E: Functional significance of polycystic-size ovaries in healthy adolescents. J Clin Endocrinol Metab 2006; 91:3786
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion 13. Frank S: Polycystic ovary syndrome in adolescents. Int J Obes (Lond) 2008; 32:1035 14. Rosenfield RL: Clinical Review: Identifying children at risk for polycystic ovary syndrome. J Clin Endocrinol Metab 2007; 92:787 15. Herter LD, Golendziner E, Flores JA, et al: Ovarian and uterine sonography in health girls between 1 and 13 years old: Correlation of findings with age and pubertal status. AJR Am J Roentgenol 2002; 178:1531 16. Badouraki M, Christoforidis A, Economou I, et al: Sonographic assessment of uterine and ovarian development in normal girls aged 1 to 12 years. J Clin Ultrasound 2008; 36:539 17. Balen AH, Laven JS, Tan SL, et al: Ultrasound assessment of polycystic ovary: international consensus definitions. Hum Reprod Update 2003; 9:505
269
18. Iban˜ez L, Potau N, Virdis R, et al: Postpubertal outcome in girls diagnosed of premature pubarche during childhood: increased frequency of functional ovarian hyperandrogenism. J Clin Endocrinol Metab 1993; 76:1599 19. Djavadian D, Braendle W, Jaenicke F: Laparoscopic oophoropexy for the treatment of recurrent torsion of the adnexa in pregnancy: Case report and review. Fertil Steril 2004; 82:933 20. Abes M, Sarihan H: Oophoropexy in children with ovarian torsion. Eur J Pediatr Surg 2004; 14:168 21. Righi RV, McComb PF, Fluker MR: Laparoscopic oophoropexy for recurrent adnexal torsion. Hum Reprod 1995; 10:3136 22. Somunkiran A, Yavuz T, Yucel O, et al: Anti-Mu¨llerian hormone levels during hormonal contraception in women with polycystic ovary syndrome. Eur J Obstet Gynecol Reprod Biol 2007; 134:196
Original Study Early Polycystic Ovary Syndrome as a Possible Etiology of Unexplained Premenarcheal Ovarian Torsion Anish A. Shah, MD, Creighton E. Likes, MD, and Thomas M. Price, MD Department of Obstetrics and Gynecology, Duke University Medical Center, Durham, North Carolina, USA
Abstract. Study Objective: To study evidence of polycystic ovary syndrome (PCOS) in premenarcheal adolescents with unexplained ovarian torsion. Design: Retrospective observational case series. Setting: Tertiary university clinical center Participants: Six premenarcheal adolescents and six adults with acute ovarian torsion Intervention: A chart review. Main Outcome Measures: Contralateral ovarian size, operative findings, ovarian pathology, hormone testing Results: Five of the six premenarcheal cases had no pathologic explanation for their ovarian torsion. In four of the cases, size measurements of the contralateral ovary were noted to be larger than the criterion of their respective age group. Three of the four cases had either an ovarian volume (28.5 cm3) or an area (16.0 cm2 and 57.6 cm2) that was above the size criterion for a polycystic ovary (volume O10 cm3 or area O 5.5 cm2). Pathology of a wedge biopsy of one of the contralateral ovaries suggested evidence of polycystic ovary. Finally, hormone testing available in three of the cases revealed elevated testosterone levels in two. Among the adults, half of the cases had a pathologic explanation for ovarian torsion. One out of the five cases had a contralateral ovary that was significantly enlarged and this was noted in a woman with a diagnosis of PCOS. The remaining two cases had extensive necrosis of the torsed ovary and no other diagnosis was made. Conclusion: We propose that premenarcheal girls presenting with ovarian torsion, without obvious ovarian pathology, be screened for ultrasound and biochemical evidence of PCOS. In those with evidence of PCOS, treatment with oral contraceptives should be considered taking into account the age and pubertal development, to decrease ovarian volume.
Key Words. Polycystic ovary syndrome—Ovarian torsion—Premenarcheal—Adolescence
Introduction
Address correspondence to: Thomas M. Price, MD, Duke Fertility Center, Duke University Medical Center, 5704 Fayetteville Road, Durham, NC 27713.; E-mail: [email protected]
Ovarian torsion is the fifth most common gynecologic surgical emergency in females of all ages with a prevalence of 2.7%.1,2 Twisting of the ovary compromises vascular pedicles in the suspensory ligaments, impeding lymphatic and venous drainage and decreasing arterial flow. Continued arterial perfusion often leads to an enlarged ovary that eventually develops necrosis, infarction, and local hemorrhage, resulting in peritonitis, and even systemic infection and inflammation.1,3e5 Therefore, it is essential to diagnose and treat ovarian torsion as early as possible to preserve the adnexa. Though ovarian torsion has been described in all age groups, it is most common in reproductive aged women due to the increased frequency of physiologic and pathologic ovarian neoplasms.6,7 Ovarian cysts and neoplasms account for up to 94% of cases in adult women.4e6,8,9 Other etiologies include pregnancy and ovulation induction, which have a reported incidence of 12% and 8%, respectively.7,10 In general, processes that lead to enlarged ovaries predispose to torsion. Procedures that result in adnexal hypermobility such as a previous tubal ligation or laparoscopic hysterectomy may contribute to torsion. Right ovarian torsion is more common than left with a frequency ratio of 5:2, thought to be due to the sigmoid colon reducing available space for ovarian movement.2,3,5,6,9 In the adolescent premenarcheal population, anatomical factors such as ovarian cyst or neoplasm account for approximately half of the cases of ovarian torsion. Thus, 50% of cases in this population have normal ovaries that have undergone torsion.6,8 We
Ó 2009 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc.
1083-3188/09/$36.00 doi:10.1016/j.jpag.2008.11.005
266
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion
believe that one possible etiology of unexplained ovarian torsion in this population is early polycystic ovary syndrome (PCOS). Since PCOS likely begins long before its physical manifestations during late adolescence and early adulthood, we believe the increased ovarian volume associated with PCOS predisposes these adolescents to ovarian torsion.11e14 Methods With IRB approval, we reviewed the medical records of all children aged 8e12 years old and adults with the diagnosis of ovarian torsion resulting in oophorectomy in a ten year period (1997e2007) at Duke University Medical Center, Durham, NC. Using the International Classifications of Diseases, Ninth Revision code to identify potential cases of ovarian torsion (code 620.5, torsion of ovary, ovarian pedicle, or fallopian tube, torsion: accessory tube hydatid of Morgagni), we were able to identify twelve cases. Six cases were from premenarcheal girls and six were adults. The adult population had no evidence of infertility. We then abstracted the medical records for data that included the patient’s age, race, menarcheal status, imaging studies, surgical findings, surgical outcomes, pathology reports, and laboratory tests. Medical records were abstracted by a single investigator (C.E.L.) and then abstracted by a second investigator (A.A.S.) with no significant disagreement. Results In the six cases of acute ovarian torsion among premenarcheal girls, the mean age was 10 years (Table 1). Torsion of the right ovary was found in 50% of the cases. One case had a pathologic diagnosis of a mature teratoma. The other five cases were noted as benign with no evidence of any ovarian cyst or neoplasm. The contralateral ovary was imaged preoperatively or inspected during the operation with size measurements in four of the six cases. All ovaries with measurements were larger than would be expected when compared to standard measurements with their respective ages. Two patients had a calculable ovarian volume of 28.5 cm3 and 7.2 cm3 on preoperative ultrasound, which was significantly greater than the expected volumes of 1.8 cm3 0.8 and 2.6 cm3 0.9 for a 10- and 11-year-old, respectively.15,16 Two other cases had measurements performed intraoperatively by the surgeon and were noted to be 4 5 cm (or a maximal area of 16.0 cm2) and 8 9 cm (or maximal area of 57.6 cm2). Interestingly, in all four cases the ultrasonographer or the surgeon commented on the contralateral ovary appearing polycystic and significantly enlarged. In one case, the surgeon performed a wedge biopsy on the contralateral ovary
and on final pathology it was noted to have serous cystadenofibroma and extensive hilar fibrosis. Hormone testing was performed in three of the premenarcheal cases (Table 1). One girl had an elevated free testosterone of 12.2 ng/dL (normal ! 8.5 ng/dL). In two other cases total testosterone levels were measured at 22 and 51 ng/dL (normal 10e35 ng/dL). Luteinizing hormone and follicle-stimulating hormone were evaluated in two of the cases and noted to have a normal ratio in both. Finally, dehydroepiandrosterone sulfate (DHEAS) levels were noted within the normal range for their respective ages in all three of the cases. Among the adults, the mean age was 29 years. Five of the six cases showed torsion of the right ovary. Three of the cases had abnormal pathologic features: two mucinous cystadenomas and one large ovarian cyst. Two other cases were noted to have extensive necrosis on final pathologic examination and thus no other specific diagnosis could be ascertained. Their respective contralateral ovaries were noted to be normal. All six cases had preoperative ultrasound measurements; however, in only one case was the contralateral ovary noted to be very enlarged with numerous tiny follicles. This patient had a diagnosis of PCOS and the torsed ovary in this case had no additional pathologic diagnosis. Fig. 1 shows a laparoscopic view of the non-torsed ovary of the 10-year-old patient described in Table 1. Three months prior, she had undergone right salpingooophorectomy for torsion. Acute onset of left pelvic pain with questionable ovarian blood flow led to a repeat laparoscopy showing an enlarged, but non-torsed ovary. Oophoropexy was performed by shortening the ovarian ligament. Discussion The diagnostic criteria for PCOS include polycystic appearing ovaries, oligo- anovulation, clinical or biochemical signs of unexplained hyperandrogenism and no other disease process with similar presentation such as congenital adrenal hyperplasia.11e13 The etiology, although unclear, is likely the result of a number of intrinsic ovarian genetic traits interacting with congenital or environmental factors that begin long before the physical manifestations during late adolescence.11e14 In the adolescent population, ovarian enlargement is one of the most specific findings of excessive ovarian stroma. Ovarian volume of O10 cm3 or maximal area of O5.5 cm2 are characteristic of a polycystic ovary based on international consensus definition.12,17 In our study, the contralateral ovary was used as a surrogate of the torsed ovary since the polycystic features of PCOS are bilateral. Again, it would be expected that the torsed ovary would be enlarged due to the continued arterial flow in the setting of obstructed venous and
Age (yrs)
Race
Contralateral Ovary (cm)
Operative Findings
Pathology
11
White
2.6 1.4 3.8 (vol: 7.2 cm3)
8
Black
4 5 (area: 16.0 cm2)
11
White
No description
10
White
5.1 4.1 2.6 (vol: 28.5 cm3)
Left ovary with multiple follicles, it measures 3x2 cm. Right ovary completely torsed. Hemorrhagic right ovarian complex measuring 7x5 cm. Left ovary slightly enlarged, 4x5 cm but normal. Right adnexa very enlarged. Left ovary normal. Right ovary 8 cm necrotic. Left ovary enlarged, dilated left tube.
11, 12
Black
8 9 (area: 57.6 cm2)
First surgery: Enlarged left ovary w/ clot and hemorrhage 10x9 cm. Right ovary w/ multiple small cysts, 8x9 cm. Second Surgery: (10 mo later) Multiple small cysts in right ovary
First surgery: Left ovary: 12.5 18 6 cm, benign normal. Wedge Bx of Right ovary: Benign serous cystadenofibroma. Second Surgery: Right ovary: 5.0 2.5 1.5 cm, multiple follicles, extensive hilar/fibrous tissue.
9
White
No description
Left ovary torsed, 7x5 cm. Right ovary normal
10.5 8.5 4 cm, Mature teratoma
Hormone Testing
9.5 5.5 5 cm, Benign, normal
N/A
6.5 6 2.5 cm, Benign, normal
Test (total): 22 ng/dL, DHEAS: 111 mcg/dL, E2: 14 pg/mL
15 13.5 8.2 cm, Benign, normal
N/A
7.9 7.2 2.3 cm, Benign, normal
LH: !0.1 mIU/mL, FSH: !0.1 mIU/ mL, 17OHP: 174 ng/dL, DHEAS: 56 mcg/dL Test (total): 51 ng/dL, TSH: 2.53 uIU/ mL LH: 1.6 mIU/mL, FSH: 1.7 mIU/mL, DHEAS: 104 mcg/dL, Test (free): 12 pg/mL E2: 43 pg/mL
N/A
DHEAS 5 Dehydroepiandrosterone Sulfate (normal range: 35 e 130 mcg/dL), FSH 5 Follicle-Stimulating Hormone (normal range: 0.4 e 6.9 mIU/mL), LH 5 Luteinizing Hormone (normal range: !4.0 mIU/mL), Test 5 Testosterone (normal range, total: 10 e 35 ng/dL, free: undetectable e 8.5 pg/mL), E2 5 Estradiol (normal range: undetectable e 24 pg/mL), 17OHP 5 17-hydroxyprogesterone (normal range: !100 ng/dL), TSH 5 Thyroid Stimulating Hormone (normal: range 0.34 e 5.66 uIU/mL)
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion
Table 1. Findings of all Premenarcheal Cases
267
268
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion
Fig 1. Enlarged contralateral ovary in the 10 year old patient with previous ovarian torsion.
lymphatic outflow. Interestingly, in three of the four adolescent girls with measurements of their contralateral ovary available, their ovarian volume or area was much larger than the minimal criteria of 10 cm3 or 5.5 cm2.12,17 Even in the adolescent girl who did not meet volume criteria for PCOS, her volume of 7.2 cm3 was still significantly enlarged when compared to her age group, which has an average of 2.6 cm3 0.9.16 Furthermore, in the one case where a wedge biopsy was performed, the ovary was noted to have extensive hilar fibrosis possibly due to an increase in ovarian stroma as seen in PCOS.17 Also, when comparing the adult population, the only significantly enlarged contralateral ovary was noted in a woman with PCOS. Hormone testing available in the premenarcheal adolescents revealed elevated testosterone levels in two of the three cases. With features of polycystic ovaries and biochemical signs of hyperandrogenism, these two cases meet the diagnostic criteria for PCOS. None of the girls showed elevated DHEAS levels or revealed a history of premature adrenarche. Ibanez et al18 was the first to describe the correlation between premature adrenarche and PCOS when they noted that half of their study population later developed PCOS. Also, of the remaining girls who did not develop PCOS, one sixth had polycystic ovaries.18 The most recognized risk factor for torsion in the adult population is ovarian enlargement, often due to functional cyst or neoplasm. This has not been seen as frequently in premenarcheal girls, thus leading to other hypotheses including congenitally long supportive ligaments, mesovarium, and mesosalpynx resulting in hypermobility.2,3,9 Our theory of enlarged polycystic ovaries as a risk factor for torsion in this age group has not been previously proposed. Another supporting observation for the role of PCOS in torsion in premenarcheal girls is the occurrence of
asynchronous bilateral ovarian torsion (ABOT) at a rate of approximately 11.4%. ABOT is the subsequent torsion of the remaining ovary which may occur months to years later. In a report of four cases, the mean time was 15.7 months with a range of 7 to 30 months. No underlying pathology was found.2 Due to the catastrophic nature of ABOT, oophoropexy of the non-affected ovary should be considered in cases of premenarcheal torsion. Techniques include shortening the ovarian ligament,19 fixation of the ovary to the posterior abdominal wall,20 or fixation of the ovary to the pelvic side wall21 with permanent suture. We propose that premenarcheal girls presenting with ovarian torsion, without obvious ovarian pathology, be screened for ultrasound and biochemical evidence of PCOS. In those with evidence of PCOS, treatment with oral contraceptives should be considered taking into account the age and pubertal development, to decrease ovarian volume. Although it was not studied in premenarcheal girls, a 6-month treatment with oral contraceptives in adults with PCOS resulted in a 37% decrease in ovarian volume22 from 14.0 to 9.4 cm3. A decrease in ovarian volume may further decrease the risk of ovarian torsion. References 1. Pena JE, Ufberg D, Cooney N, et al: Usefulness of doppler sonography in diagnosis of ovarian torsion. Fertil Steril 2001; 75:1041 2. Beaunoyer M, Chapdelaine J, Boucharch S, et al: Asynchronous bilateral ovarian torsion. J Pediatr Surg 2004; 39:746 3. Ozcan C, Celik A, Ozok G, et al: Adnexal torsion in children may have a catastrophic sequel: asynchronous bilateral torsion. J Pediatr Surg 2002; 37:1617 4. Anders JF, Powell EC: Urgency of evaluation and outcome of acute ovarian torsion in pediatric patients. Arch Pediatr Adolesc Med 2005; 159:532 5. Kokoska ER, Keller MS, Weber TR: Acute ovarian torsion in children. Am J Surg 2000; 180:462 6. Cass D: Ovarian Torsion. Semin Pediatr Surg 2005; 14:86 7. Rackow BW, Patrizio P: Successful pregnancy complicated by early and late adnexal torsion after in vitro fertilization. Fertil Steril 2007; 87:697 8. Varras M, Tsikini A, Polyzos D, et al: Uterine adnexal torsion: Pathologic and gray scale ultrasound findings. Clin Exp Obstet Gynecol 2004; 31:34 9. Rousseau V, Massicot R, Darwish AA, et al: Emergency management and conservative surgery of ovarian torsion in children: A report of 40 cases. J Pediatr Adolesc Gynecol 2008; 21:201 10. Gorkemli H, Camus M, Clasen K: Adnexal torsion after gonadotrophin ovulation induction for IVF or ICSI and its conservative treatment. Arch Gynecol Obstet 2002; 267:4 11. Azziz R: Diagnostic criteria for polycystic ovary syndrome: A reappraisal. Fertil Steril 2005; 85:1343 12. Mortensen M, Rosenfield RL, Littlejohn E: Functional significance of polycystic-size ovaries in healthy adolescents. J Clin Endocrinol Metab 2006; 91:3786
Shah et al: Early PCOS as a Possible Etiology of Premenarcheal Ovarian Torsion 13. Frank S: Polycystic ovary syndrome in adolescents. Int J Obes (Lond) 2008; 32:1035 14. Rosenfield RL: Clinical Review: Identifying children at risk for polycystic ovary syndrome. J Clin Endocrinol Metab 2007; 92:787 15. Herter LD, Golendziner E, Flores JA, et al: Ovarian and uterine sonography in health girls between 1 and 13 years old: Correlation of findings with age and pubertal status. AJR Am J Roentgenol 2002; 178:1531 16. Badouraki M, Christoforidis A, Economou I, et al: Sonographic assessment of uterine and ovarian development in normal girls aged 1 to 12 years. J Clin Ultrasound 2008; 36:539 17. Balen AH, Laven JS, Tan SL, et al: Ultrasound assessment of polycystic ovary: international consensus definitions. Hum Reprod Update 2003; 9:505
269
18. Iban˜ez L, Potau N, Virdis R, et al: Postpubertal outcome in girls diagnosed of premature pubarche during childhood: increased frequency of functional ovarian hyperandrogenism. J Clin Endocrinol Metab 1993; 76:1599 19. Djavadian D, Braendle W, Jaenicke F: Laparoscopic oophoropexy for the treatment of recurrent torsion of the adnexa in pregnancy: Case report and review. Fertil Steril 2004; 82:933 20. Abes M, Sarihan H: Oophoropexy in children with ovarian torsion. Eur J Pediatr Surg 2004; 14:168 21. Righi RV, McComb PF, Fluker MR: Laparoscopic oophoropexy for recurrent adnexal torsion. Hum Reprod 1995; 10:3136 22. Somunkiran A, Yavuz T, Yucel O, et al: Anti-Mu¨llerian hormone levels during hormonal contraception in women with polycystic ovary syndrome. Eur J Obstet Gynecol Reprod Biol 2007; 134:196