Hernia Uterus Inguinale A Proposed Algorithm Using the Laparoscopic Approach Kiran K. Turaga, MD, MPH, Shawn D. St. Peter, MD, Casey M. Calkins, MD, George W. Holcomb, III, MD, MBA, Daniel J. Ostlie, MD, and Charles L. Snyder, MD Abstract: The authors present a case of hernia uteri inguinale, or the presence of Mullerian duct structures in an inguinal hernia. This entity presents in phenotypic males presumably secondary to defects in the action of the Mullerian inhibiting substance and presents a pediatric surgeon with management dilemmas. The long-term complications associated with the gonads if left intact include the risk of malignancy and infertility. We describe the use of the laparoscopic technique in the management of this condition and propose an algorithm for the management of this condition. Key Words: hernia, uterus inguinal, mullerian duct syndrome (Surg Laparosc Endosc Percutan Tech 2006;16:366–367) H ernia uterus inguinale is a rare condition, classically described as herniation of a corner of the uterus and ipsilateral fallopian tube in the inguinal canal in a phenotypic male. This condition is also known as persistent Mullerian duct syndrome (PMDS), a more physiologically precise term encompassing and other associated conditions as well. 1,2 Hernia uterus inguinale is ultimately defined by the presence of female reproduc- tive structures within an inguinal hernia. These patients are typically genotypically normal males with a 46 XY karyotype. It is a variant of male pseudohermaphroditism that is generally clinically silent until identified intra- operatively by the perplexing discovery of fallopian or uterine structures during an inguinal herniorrhaphy, or operation for an undescended testis in a phenotypically normal male. Providing effective operative management and avoiding intraoperative uncertainty in this circum- stance requires awareness of the condition and a definitive plan. Therefore we describe a case and propose a simple algorithm for the management of this rare condition using the current tools of minimally invasive surgery. CASE REPORT A healthy, 5-month-old male infant was initially eval- uated for bilateral cryptorchidism. On examination, he had a well-developed phallus with a normal foreskin and hypoplastic scrotum. A gonad was palpable in the proximal left inguinal canal, and no gonad was palpable on the right. Laboratory and endocrinologic evaluation revealed normal male serum levels of leutenizing hormone, follicular stimulating hormone, testoster- one, dihydrotestosterone, free thyroxine, thyroid stimulating hormone, and 17-hydroxyprogesterone. He received four 900 U intramuscular injections of human chorionic gonadotropin without testicular descent. A left orchiopexy was successfully performed at 16 months of age. Six months later the right testes remained nonpalpable and he was scheduled for laparoscopy and orchiopexy. A 5 mm umbilical port was introduced into the abdominal cavity under direct vision through a vertical incision in the umbilicus. The abdomen was insufflated with 12 mm Hg at flow rate of 4 L/min. The right gonad was easily visualized within the extraperitoneal space cephalad to the internal ring behind a thin transparent peritoneal covering. A normal appearing vas deferens and gonadal blood supply were visualized in their normal locations and a patent processus vaginalis. This diagnostic maneuver required no additional ports and lasted less than 5 minutes total. A standard open inguinal exploration was performed that revealed a diminutive, dys- morphic gonad that was removed given its size and appearance. The hernia was repaired by high ligation of the sac. His recovery was uneventful. Pathologic examination revealed evidence of a vas deferens, epididymis, and a rudimentary uterus consistent with hernia uteri inguinale. DISCUSSION Female genital organs in an XY male with normal external phenotype can be explained by a fault in the pathway initiated by Mullerian inhibitory substance (MIS). MIS was originally described as the substance causing regression of female anlagen in males. 3 This case is 1 type of PMDS. Defects in the genes transcribing MIS or the MIS type II receptor are presumed to cause PMDS in males, in an autosomal recessive inheritance pattern. 4 These patients often present in childhood or adulthood with cryptorchidism or an inguinal hernia. Hernia uterus inguinale is one of the more common male variants of the PMDS, which has been classically described as a descended testis and herniation of an ipsilateral corner of the uterus and ipsilateral fallopian tube. The other male variant, called crossed testicular ectopia, is characterized by herniation of testes, fallopian tubes, and the uterus. A female variant of PMDS has also been described in 10% to Copyright r 2006 by Lippincott Williams & Wilkins Received for publication October 17, 2005; accepted June 20, 2006. From the Department of Pediatric Surgery, the Children’s Mercy Hospital, Kansas City, MO. Reprints: Charles L. Snyder, MD, Department of Pediatric Surgery, Children’s Mercy Hospital, 2401 Gilham Road, Kansas City, MO 64108 (e-mail: csnyder@cmh.edu). TECHNICAL REPORT 366 Surg Laparosc Endosc Percutan Tech  Volume 16, Number 5, October 2006