FERTILITY AND STERILITY威 VOL. 80, NO. 2, AUGUST 2003 Copyright ©2003 American Society for Reproductive Medicine Published by Elsevier Inc. Printed on acid-free paper in U.S.A.
TECHNIQUES AND INSTRUMENTATION
Laparoscopy-assisted transinguinal extracorporeal gonadectomy in six patients with androgen insensitivity syndrome Ahmet Yalinkaya, M.D., and Murat Yayla, M.D. Department of Obstetrics and Gynecology, Dicle University Medical School, Diyarbakir, Turkey
Objective: To describe a new surgical technique to treat prophylactic gonadectomy in patients with androgen insensitivity syndrome. Design: Prospective study. Setting: University hospital. Patient(s): Six patients with androgen insensitivity syndrome. Intervention(s): Laparoscopy-assisted transinguinal or suprapubic extracorporeal gonadectomy. Main Outcome Measure(s): Accessibility, operation time, and cost-effectiveness. Result(s): Laparoscopy-assisted transinguinal extracorporeal gonadectomy under general anesthesia was performed successfully, with no complications, in all patients as a one-day procedure. The operation times were 12 to 22 minutes (average, 16 minutes). Histopathologic examination revealed testicles in all cases. The cost of each operation was approximately $500 to $1,000. Conclusion(s): Laparoscopy-assisted transinguinal or suprapubic and extracorporeal gonadectomy has several advantages over laparotomy and operative laparoscopy, including short operating time, safety, simplicity, and cost-effectiveness. (Fertil Steril威 2003;80:429 –33. ©2003 by American Society for Reproductive Medicine.) Key Words: Androgen insensitivity syndrome, laparoscopy-assisted transinguinal extracorporeal gonadectomy
Received December 17, 2002; revised and accepted March 25, 2003. Reprint requests: Ahmet Yalinkaya, M.D., Department of Obstetrics and Gynecology, Dicle University Medical School, Diyarbakir, Turkey (FAX: 90-412-2488520; E-mail:
[email protected]). 0015-0282/03/$30.00 doi:10.1016/S0015-0282(03) 00666-6
The androgen insensitivity syndrome is an X-linked recessive genetic disorder with XY karyotype that is caused by androgen receptor defects (1). It has two forms: complete and partial. The prevalence of the syndrome is estimated to be at least 1 in 20,000 persons (2). The complete form is the more common variety, occurring in 1 in 20,000 to 64,000 male births. Persons with androgen insensitivity syndrome have a 20% to 30% risk for gonadal cancer. Prophylactic bilateral gonadectomy is usually recommended to avoid malignant change within the intra-abdominal testicle. The operation is usually deferred because such changes are uncommon before puberty and because post-pubertal removal allows feminization to develop spontaneously (3, 4). Bilateral gonadectomy has been traditionally performed by laparotomic techniques. Recently, operative laparoscopy has become the most common method of removal of the go-
nads (3, 5, 6), but this technique can be timeconsuming and expensive (7). We describe laparoscopy-assisted transinguinal extracorporeal gonadectomy, a new technique that is relatively easy for an experienced laparoscopist to perform.
MATERIALS AND METHODS We prospectively analyzed six patients with the androgen insensitivity syndrome who presented to the Department of Obstetrics and Gynecology at Dicle University, School of Medicine from September 2000 to March 2001. Two patients 18 and 22 years of age with complete androgen insensitivity syndrome and four patients 14, 18, 19, and 45 years of age with partial androgen insensitivity syndrome and primary amenorrhea underwent surgery prospectively. The 22-year-old patient had been married for 6 months; all other patients were single. The 18- and 19-year-old 429
FIGURE 1 Phenotype of six patients with androgen insensitivity syndrome who underwent laparoscopy-assisted transinguinal extracorporeal gonadectomy.
Yalinkaya. Extracorporeal gonadectomy in AIS patients. Fertil Steril 2003.
patients with partial androgen insensitivity syndrome were sisters.
through inguinal canals while the patients performed the Valsalva maneuver.
All patients underwent systemic and gynecologic examinations, including ultrasonography. Pelvic ultrasonography and karyotyping were done as diagnostic tests, and intravenous pyelography was performed in all patients to rule out urinary tract abnormalities. Estrogen, T, LH, and FSH were measured for differential diagnosis.
In all patients, T and LH levels were higher than normal women. The FSH levels were similar to those in normal women in the two patients with complete androgen insensitivity syndrome but were much higher in the four patients with partial androgen insensitivity syndrome. All results of intravenous pyelography were normal. The karyotype was 46,XY in all patients (Table 1).
After a detailed physical examination and laboratory results, all patients consulted a psychiatrist. Gonadectomy was then proposed to their families. Patients were informed about the operation with their parents. Physical and gynecologic examination showed that all patients had a female phenotype. The secondary sex characters of the two patients with complete androgen insensitivity syndrome cases were complete; breast, vagina, and body shape were normal. The patients with partial androgen insensitivity syndrome lacked secondary sex characteristics (Fig. 1). No ovaries or uterus were found in any patient on pelvic ultrasonography. The gonads were found bilaterally at the entrance of inguinal canals in the abdomen in four patients; in the fifth patient, one was located in the abdomen and other in the inguinal canal; and in the sixth patient, only the right labial gonad was present, but no gonad was found on the contralateral side (vanishing testicle), and the clitoris was 2 cm (clitoromegaly). The gonads could be pushed down 430
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Patients underwent laparoscopy-assisted transinguinal extracorporeal gonadectomy under general anesthesia at the surgery unit. After carbon dioxide insufflation, a 10-mm trocar and sleeve were inserted infraumbilically. The whole abdominal cavity was visualized with a 10-mm endoscope. A 5 mm suprapubic trocar and sleeve were then inserted. A grasper was introduced through the suprapubic trocar and pushed the gonads through the inguinal canal. In the meantime, the cutaneous end of the inguinal canals were incised to 10 mm, and a curved Kocher clamp was introduced through the canal and used to grasp the testicle. Once the testicle was exteriorized, the pedicle was clamped and cut with scissors under direct vision. The pedicles were sutured with 1/0 polyglactin 910 material. The stump was pushed back into the abdominal cavity. The procedure was repeated for the remaining testicle. The fascia of the inguinal canal was repaired by using 1/0
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TABLE 1 Clinical and laboratory characteristics of the patients. Characteristic Age (y) Karyotype Amenorrhea Breast Pubic hair Vaginal depth (cm) Clitoromegaly Open inguinal canal: Right Left Ultrasonographic findings: Uterus Location of gonads Entrance inguinal canal (right/left) Inguinal canal (right/left) Movement of testicles by Valsalva maneuver (right/left) FSH level (IU/L) LH level (IU/L) Estrogen level (ng/L) Progesterone level (mmol/L) Testosterone level (mmol/L)
PAIS patient
CAIS patient
PAIS patient
PAIS patient
CAIS patient
PAIS patient
14 46,XY Yes Infantile Normal 0.5 Yes
18 46,XY Yes Normal Sparse 7 No
18 46,XY Yes Infantile Sparse 1 No
19 46,XY Yes Infantile Sparse 2.5 No
22 46,XY Yes Normal Sparse 8 No
45 46,XY Yes Infantile Sparse 1.5 No
Yes No
Yes No
Yes Yes
Yes Yes
Yes Yes
Yes Yes
⫺
⫺
⫺
⫺
⫺
⫺
⫺/⫺ ⫹/⫺
⫹/⫹ ⫺/⫺
⫹/⫹ ⫺/⫺
⫹/⫹ ⫺/⫺
⫹/⫹ ⫺/⫺
⫹/⫹ ⫺/⫺
⫹/⫺ 23.7 29 119 0.72 4.46
⫺/⫹ 8.56 47 73 1.18 8.3
⫹/⫹ 39.7 47 19 0.87 4.7
⫹/⫹ 16.9 26.9 20.4 0.76 5.2
⫹/⫹ 4.6 32 68 1.44 3.4
⫹/⫹ 48.3 54.9 23.4 0.48 4.7
Yalinkaya. Extracorporeal gonadectomy in AIS patients. Fertil Steril 2003.
polyglactin 910 suture material. The abdominal fascia of the trocar sites was closed with 2/0 chromic catgut (Fig. 2).
RESULTS Table 1 shows clinical and laboratory findings. Patients had amenorrhea, sparse pubic hair, an open inguinal canal, no uterus, gonads generally at the entrance of inguinal canal, and positive findings on the Valsalva maneuver. In all patients, the T and LH levels were higher and the P level was lower than those in normal women. Levels of FSH and estrogen were similar to those in normal women in the two patients with complete androgen insensitivity syndrome, whereas the FSH level was remarkably higher. The estrogen was lower than normal in the four patients with partial androgen insensitivity syndrome. The operation time ranged from 12 to 22 minutes (average, 16 minutes). All of the gonads except two were extracted by this new technique. In one case, vanishing testicle occurred. In the other case, in which the patient’s inguinal canal was closed, we made an incision on the suprapubic area and, with laparoscopic assistance, grasped the gonad with the Kocher clamp and extracted it manually after deinsufflation. This was done to obtain optimal distance and to not cause harm to the pedicle of the testicle, which can elongate spontaneously. After the pedicle was exteriorized, it was ligated, cut, and pushed back with no difficulty. FERTILITY & STERILITY威
All patients were ambulatory 6 hours postoperatively and discharged on the same day. No intraoperative or postoperative complications occurred. Blood loss was minimal. All extirpated gonads were examined by the pathology department, which confirmed that all were testicles. No cancer was reported. Hormonal replacement with estrogen was prescribed postoperatively and recommended for each patient’s lifetime.
DISCUSSION The risk of gonadal cancer is increased in persons with the androgen insensitivity syndrome. Prophylactic gonadectomy is therefore recommended (3). The most appropriate age for prophylactic gonadectomy is 16 to 18 years. Even if the diagnosis has been made at an early age, the operation can be postponed until the development of secondary female sex characters. In older patients, however, prophylactic gonadectomy should be performed as soon as possible (6). When gonadectomy is required because of hirsutism or clitoromegaly in young patients, administration of high-dose estrogen after the gonadectomy is a common practice to aid development of secondary sex characters. Because it has protective effects, estrogen therapy should be continued for a lifetime in all patients who have had gonadectomy (1). In the past, gonadectomy was performed by laparotomy, but laparoscopy has recently become popular (5, 7). 431
FIGURE 2 Laparoscopy-assisted transinguinal extracorporeal gonadectomy. The left panel shows extracoporeal gonads; the right panel shows the end of the operation.
Yalinkaya. Extracorporeal gonadectomy in AIS patients. Fertil Steril 2003.
We describe a new technique for gonadectomy in patients with the androgen insensitivity syndrome. Ten of eleven gonads (90.9%) were successfully removed transinguinally in six patients. One gonad was vanishing, and one was removed by suprapubic skin incision; thus, we can claim a success rate of 100%. This new technique has several advantages over laparotomy and operative laparoscopy, including short operation time, simplicity, and cost-effectiveness. Extraction of the gonads through the abdominal cavity may prolong the operation, causing more intake of carbon dioxide and anesthetic drug and thus increasing the risk of related complications. In addition, laparoscopic gonadectomy requires one additional trocar, one endoscopic scissor, two endobags, and occasionally two endoloops, which can add approximately $500 to $1000 to the cost of each operation. No patient in our series experienced complications, and all were discharged on the day of surgery. The duration of gonadectomy is rarely mentioned in the literature. In a study by Arici et al. (7), the average operative time was reported to be 72 minutes (range, 35 to 95 minutes). In contrast, the average time to perform laparascopy-assisted transinguinal extracorporeal gonadectomy was 16 minutes (range, 12 to 22 minutes). Operation time was shorter in nonobese patients and may take longer in obese patients. 432
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If the testicles are in the distal part of the inguinal canal, gonadectomy can be performed without laparoscopy. In these cases, the procedure can be done under spinal or epidural anesthesia instead of general anesthesia, thus avoiding the complications of general anesthesia. Local anesthesia allows the Valsalva maneuver to be performed and the gonads to be pushed through the inguinal canal. In conclusion, we performed laparoscopy-assisted transinguinal extracorporeal gonadectomy in patients with androgen insensitivity syndrome. No patient required operative laparoscopy. The procedure seems to be cost-effective. The advantages and disadvantages of this new technique should be considered in choosing the appropriate method for gonadectomy in patients with the androgen insensitivity syndrome.
Acknowledgments: The authors thank Basar Tekin, M.D., for revising the manuscript and Ibrahim Tunik and Sue Kalpak for English-language assistance.
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implications for the role of androgens in testicular descent. J Urol 2000;164:497–501. 3. Campo S, Garcea N. Laparoscopic gonadectomy in two patients with gonadal dysgenesis. J Am Assoc Gynecol Laparosc 1998;5:305–8. 4. Hawkyard S, Poon P, Morgan DR. Sertoli tumour presenting with stress incontinence in a patient with testicular feminisation. BJU Int 1999;84: 382–3.
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5. Droesch K, Droesch J, Chumas J, Bronson R. Laparoscopic gonadectomy for gonadal dysgenesis. Fertil Steril 1990;53:360 –1. 6. Major T, Borsos A, Csiszar P. Laparoscopic removal of gonads in gonadal dysgenesis. Int J Gynaecol Obstet 1995;49:53–4. 7. Arici A, Kutteh WH, Chantilis SJ, Johns DA, Carr BR. Laparoscopic removal of gonads in women with abnormal karyotypes. J Reprod Med 1993;38:521–5.
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