Three Cases of Disorders of Sex Development (DSD) With Inguinal Hernia Diagnosed by Laparoscopic Percutaneous Extraperitoneal Closure (LPEC)

Abstract

We have to be aware of disorders of sex development (DSD), which are found in about 1% of female infants with inguinal hernia. We treated three cases of DSD diagnosed by laparoscopic percutaneous extraperitoneal closure (LPEC) without preoperative ovarian sliding. Cases 1 and 2 were sisters, who were 7 and 4 years old, respectively. The older sister underwent Potts repair for her right inguinal hernia when she was 1.5 years old. She was scheduled to have LPEC on the left and her sister on both sides without ovarian sliding. During the operations, testis-like gonads and vas deferens were found, and no oviduct and uterus were found. Therefore, only observations were performed for suspected DSD. Both patients were diagnosed as having 46 XY DSD and complete androgen insensitivity syndrome (CAIS), and bilateral gonadectomy and LPEC were performed later. Case 3 was a 6-year-old girl, in whom no ovarian sliding was found. During the operation, no uterus was found and only observation was made for suspected DSD. She was diagnosed as having 46 XX DSD and Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, and LPEC was performed later. It is necessary to consider DSD even when no ovarian sliding is detected by preoperative ultrasonography. LPEC allows easy observation of the internal genitalia during surgery and is useful for the early detection of DSD.