Vol. 57, No.2, February 1992
FERTILITY AND STERILITY
Printed on acid-free paper in U.S.A.
Copyright © 1992 The American Fertility Society
Laparoscopic gonadectomy in 46XY female patient
Eliezer Shalev, M.D.* Avinoam Zabari, M.D. Shabtai Romano, M.D. Raphael Luboshitzky, M.D.
Department of Obstetrics and Gynecology and the Endocrinology Unit, Central Emek Hospital, Afula, Israel
A normal XY karyotype can be found in a phenotypic female with sexual infantilism, normal or tall stature, and streak gonads. The term pure gonadal dysgenesis has been applied to these XY females (1). The risk of gonadal neoplasia in XY gonadal streaks is high, dictating early prophylactic removal of the streaks (2). Traditionally, this procedure has been performed by laparotomy (1,2). In the following case we will present a novel approach of laparoscopic removal of the streaks.
noted beneath the tubes, and the left pelvic kidney was clearly identified. Through two additional puncture sites, the streaks were grasped and pulled, allowing coagulation and cutting of the parietal peritoneum to which the gonades are attached (Fig. 1). Extreme care must be exercised in identifying and avoiding damage to uterus. The excisional procedure took about 10 minutes. The patient was released on the 1st postoperative day in good condition. Pathological examination of the streaks revealed fibrotic tissue with no malignant cells.
CASE REPORT
The patient, a 15-year-old phenotypic female, presented with primary amenorrhea and very puor breast development. On examination she was a tall eunuchoid female. Pubic hair was scant, external genitalia hypoplastic, and the vagina 2 cm in length. Vital signs were normal as were kidney and liver function tests. Endocrinologic investigation revealed an elevated follicle-stimulating hormone level of 42 mUjmL and low testosterone level of 0.2 ngjmL. Her karyotyping was 46XY. Intravenous pyelography revealed a left pelvic kidney. Operative laparoscopy was performed under general anesthesia. A rudimentory uterus and thin short tubes were observed. Streak gonades were
Received July 29, 1991; revised and accepted October 25, 1991.
* Reprint requests: Eliezer Shalev, M.D., Department of Obstetrics and Gynecology, Central Emek Hospital, Afula 18 101, Israel.
Vol. 57, No.2, February 1992
Figure 1 Line drawing of the procedure. The broken line represents the line for coagulation and cutting. T, tube; G, gonades; U, uterus.
Shalev et al.
Communications-in-brief
459
DISCUSSION
Patients with 46XY gonadal dysgenesis typically present in adolescence or early adult life with primary amenorrhea and failure to develop secondary sex characteristics. These patients are at increased risk (25% to 30%) of gonadal malignancy, especially gonadoblastoma and dysgerminoma. Laparotomy and prophylactic removal ofthe streaks is thus considered an indication in these cases (1, 2). Recently, laparoscopic removal of the ovaries was described (3, 4). The advantages of this technique are short procedure and recovery time and minor additional psychological stress. Furthermore, where diagnosis is unclear, laparoscopy can serve for both diagnosis and treatment. SUMMARY
The risk of gonadal neoplasia in XY gonadal streaks is high, dictating early prophylactic removal
460
Shalev et al.
Communications· in-brief
of the streaks. Laparoscopic removal of the streaks is recommended. Key Words: Laparoscopic gonadectomy, operative laparoscopy, streak gonades, 46XY female.
REFERENCES 1. DeWhurst J. Genetic and congenital sexual disorders. In:
Gynecological therapeutics. Hawkins DF, editor. London: Bailliere Tindall, 1981:1-36. 2. MacMahon RA, Cussen J, Walters W AM. Importance of early diagnosis and gonadectomy in 46XY female. J Pediatr Surg 1980;15:642-5. 3. Parker WH, Jonathan SB. Management of selected cystic adnexal masses in postmenopausal women by operative laparoscopy: a pilot study. Am J Obstet Gynecol 1990;163: 1574-7. 4. Johns A. Laparoscopic oophorectomy/oophorecystectomy. In: Pitkin RM, Scott JR, editors. Clinical obstetrics and gynecology. Philadelphia: J. B. Lippincott, 1991;34:460-6.
Fertility and Sterility
FERTILITY AND STERILITY
Printed on acid-free paper in U.S.A.
Copyright © 1992 The American Fertility Society
Laparoscopic gonadectomy in 46XY female patient
Eliezer Shalev, M.D.* Avinoam Zabari, M.D. Shabtai Romano, M.D. Raphael Luboshitzky, M.D.
Department of Obstetrics and Gynecology and the Endocrinology Unit, Central Emek Hospital, Afula, Israel
A normal XY karyotype can be found in a phenotypic female with sexual infantilism, normal or tall stature, and streak gonads. The term pure gonadal dysgenesis has been applied to these XY females (1). The risk of gonadal neoplasia in XY gonadal streaks is high, dictating early prophylactic removal of the streaks (2). Traditionally, this procedure has been performed by laparotomy (1,2). In the following case we will present a novel approach of laparoscopic removal of the streaks.
noted beneath the tubes, and the left pelvic kidney was clearly identified. Through two additional puncture sites, the streaks were grasped and pulled, allowing coagulation and cutting of the parietal peritoneum to which the gonades are attached (Fig. 1). Extreme care must be exercised in identifying and avoiding damage to uterus. The excisional procedure took about 10 minutes. The patient was released on the 1st postoperative day in good condition. Pathological examination of the streaks revealed fibrotic tissue with no malignant cells.
CASE REPORT
The patient, a 15-year-old phenotypic female, presented with primary amenorrhea and very puor breast development. On examination she was a tall eunuchoid female. Pubic hair was scant, external genitalia hypoplastic, and the vagina 2 cm in length. Vital signs were normal as were kidney and liver function tests. Endocrinologic investigation revealed an elevated follicle-stimulating hormone level of 42 mUjmL and low testosterone level of 0.2 ngjmL. Her karyotyping was 46XY. Intravenous pyelography revealed a left pelvic kidney. Operative laparoscopy was performed under general anesthesia. A rudimentory uterus and thin short tubes were observed. Streak gonades were
Received July 29, 1991; revised and accepted October 25, 1991.
* Reprint requests: Eliezer Shalev, M.D., Department of Obstetrics and Gynecology, Central Emek Hospital, Afula 18 101, Israel.
Vol. 57, No.2, February 1992
Figure 1 Line drawing of the procedure. The broken line represents the line for coagulation and cutting. T, tube; G, gonades; U, uterus.
Shalev et al.
Communications-in-brief
459
DISCUSSION
Patients with 46XY gonadal dysgenesis typically present in adolescence or early adult life with primary amenorrhea and failure to develop secondary sex characteristics. These patients are at increased risk (25% to 30%) of gonadal malignancy, especially gonadoblastoma and dysgerminoma. Laparotomy and prophylactic removal ofthe streaks is thus considered an indication in these cases (1, 2). Recently, laparoscopic removal of the ovaries was described (3, 4). The advantages of this technique are short procedure and recovery time and minor additional psychological stress. Furthermore, where diagnosis is unclear, laparoscopy can serve for both diagnosis and treatment. SUMMARY
The risk of gonadal neoplasia in XY gonadal streaks is high, dictating early prophylactic removal
460
Shalev et al.
Communications· in-brief
of the streaks. Laparoscopic removal of the streaks is recommended. Key Words: Laparoscopic gonadectomy, operative laparoscopy, streak gonades, 46XY female.
REFERENCES 1. DeWhurst J. Genetic and congenital sexual disorders. In:
Gynecological therapeutics. Hawkins DF, editor. London: Bailliere Tindall, 1981:1-36. 2. MacMahon RA, Cussen J, Walters W AM. Importance of early diagnosis and gonadectomy in 46XY female. J Pediatr Surg 1980;15:642-5. 3. Parker WH, Jonathan SB. Management of selected cystic adnexal masses in postmenopausal women by operative laparoscopy: a pilot study. Am J Obstet Gynecol 1990;163: 1574-7. 4. Johns A. Laparoscopic oophorectomy/oophorecystectomy. In: Pitkin RM, Scott JR, editors. Clinical obstetrics and gynecology. Philadelphia: J. B. Lippincott, 1991;34:460-6.
Fertility and Sterility
