053 Totally extraperitoneal approach (TEP) for gonadectomy for differences in sex development (DSD): Report of 2 cases

Abstract

Introduction: 17-β-hydroxysteroid dehydrogenase type-3 deficiency (17βHSD-3) is a rare cause of 46XY DSD. Gonadectomy is often offered to prevent further virilization and to continue living in a female gender role [ [1] Chuang J. Vallerie A. Breech L. Saal H.M. Alam S. Crawford P. Rutter M.M. Complexities of gender assignment in 17β-hydroxysteroid dehydrogenase type 3 deficiency: is there a role for early orchiectomy?. Int J Pediatr Endocrinol. 2013; 12: 15 Crossref Google Scholar ]. Both laparotomic and laparoscopic approaches to gonadectomy had been described for DSDs [ [2] Dellaportas D. Chatzipapas I. Pandraklakis A. Michala L. Laparoscopic bilateral gonadectomy and inguinal hernia repair with mesh for complete androgen insensitivity syndrome: A case report. J Pediatr Adolesc Gynecol. 2019; 32 (Epub 2019 Feb 27): 443-445 Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar ]. Retrieval and removal of testes located in the inguinal canal can be technically challenging and requires collaboration with surgical colleagues. Cases: We present 2 cases of DSD with 17βHSD-3. One of them is 16 years old, while the other one is 20 years old. Both cases presented with amenorrhea and clitoral enlargement, whereas the second case also had deepening of voice. Blood tests for both revealed elevated testosterone and androstenedione. MRI findings were similar showing bilateral gonads located in the inguinal canals. Genetic tests revealed 46 XY karyotype and HSD17B3 gene mutation. Both subjects opted to continue with a female gender role and opted for gonadectomy to prevent further virilization. Gonadectomy was performed with a totally extraperitoneal laparoscopic approach. An infraumbilical incision was made and care was taken to enter the potential space above the rectus sheath. Then a balloon was placed via the port to distend the extraperitoneal space and then the laparoscope was inserted. Secondary ports at the suprapubic region and the left lower quadrant were placed. The gonads were mobilized and gonadectomy performed. Gonadectomy was successfully performed for the first case, but for the second case, there was failure to mobilize the gonad and operation was completed with inguinal incisions. Conclusion: Totally extraperitoneal laparoscopic approach for hernia repair is a well-established technique familiar to surgeons [ [3] McKernan J.B. Laws H.L. Laparoscopic repair of inguinal hernias using a totally extraperitoneal prosthetic approach. Surg Endosc. 1993; 7: 26-28 Crossref PubMed Scopus (264) Google Scholar ]. This was the first time the TEP approach has been described for gonadectomy for DSD. By cooperating with surgeons who has abundant experience in operating in this region, one can allow utilization of techniques which were already commonly used. It raises the importance as gynecologists, we should be mindful of technological advances in other specialties as with other aspects of DSD, collaboration with other specialties may lead to advances in patient management.