Herlyn–Werner–Wunderlich Syndrome: Presentation and Surgical Management Options for Five Cases

Background: Herlyn?Werner?Wunderlich syndrome is a rare Müllerian-duct anomaly consisting of uterine didelphys, a unilateral obstructed hemivagina, and ipsilateral renal agenesis. This article presents clinical and laparoscopic findings as well as the surgical management of 5 cases with various clinical scenarios. Cases: Case 1: An 11-year-old premenarchal female presented with chronic vaginal discharge and negative cultures. She had a uterus didelphys, a unilateral partial obstructed hemivagina, and ipsilateral renal agenesis. She underwent excision of the hemivagina. Case 2: A 14-year-old female presented with severe dysmenorrhea. As part of the work-up for a two-vessel cord at birth, an ultrasound revealed an absent left kidney. Magnetic resonance imaging suggested uterine didelphys and a left hematometrocolpos. At surgery, the MRI findings were confirmed. She underwent excision of the left hemivagina. Case 3: A 23-year-old asymptomatic female was referred following an incidental finding of a duplicated uterine horn and hematocolpos on ultrasound. She had a uterus didelphys, a unilateral obstructed hemivagina, and ipsilateral renal agenesis. She underwent excision of the hemivagina. Case 4: A 26-year-old female presented with severe dysmenorrhea and chronic pelvic pain. She had a uterus didelphys and a high right-sided obstructed hemivagina, with no hematocolpos, hematometra, hematosalpinx, endometrioma, and ipsilateral renal agenesis. She underwent a hemihysterectomy, unilateral salpingectomy, and endometrioma excision. Case 5: A 15-year-old female had a history of severe dysmenorrhea. During laparoscopy for an acute abdomen, she was noted to have uterus didelphys. Further evaluation revealed a hypoplastic cervix and a high right-sided obstructed hemivagina, with minimal hematocolpos, hematometra and ipsilateral renal agenesis. She underwent a hemihysterectomy. Results: The patients recovered well and, in most cases, had complete resolution of their symptoms. In Case 4, the patient's symptoms were greatly reduced. Conclusions: In patients with obstructed hemivaginas that are diagnosed early, resection of the vaginal septum is a convenient and effective treatment modality. Hemihysterectomy should be considered in patients with cervical hypoplasia/aplasia and high position of obstructed hemivaginas, and in patients with severe endometriosis, tubal damage, and extensive pelvic adhesions. (J GYNECOL SURG 31:46)Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/140097/1/gyn.2014.0081.pd

Robotic surgery in the management of benign complex adnexal masses with a frozen pelvis in women desiring to preserve fertility

Background: A “frozen pelvis” is a term often used to describe extensive pelvic adhesions. It is considered as one of the most challenging situations that a gynecologic surgeon can face. It commonly is caused by extensive endometriosis and pelvic inflammatory disease. We present two cases with extensive pelvic adhesive disease with the aim to illustrate the value of robotic assisted laparoscopy in the management of benign complex adnexal masses with severe pelvic adhesive disease in women desiring to preserve the fertility. Case 1: A 27 year old female referred to our clinic for evaluation of a complex left adnexal mass. Trans-vaginal ultrasound scan and an MRI suggested bilateral endometriomas. She was noted to have stage IV endometriosis. Da Vinci robot assisted left salpingo-oophorectomy was performed. Case 2: A 43 year old female presented with recurrent episodes of pelvic inflammatory disease following an unsuccessful in vitro fertilization procedure. A trans-vaginal ultrasound scan showed a complex left adnexal mass. She was noted to have extensive pelvic adhesions secondary to chronic pelvic inflammatory disease. Da Vinci robotic assisted left salpingectomy was performed, while preserving the left ovary. Conclusion: Our experience and review of literature suggest that in hands of an experienced surgeon, the inherent advantages of robotic assisted operative laparoscopy makes it a safe and attractive alternative to conventional operative laparoscopy and laparotomy for managing benign complex adnexal masses with concomitant severe pelvic adhesive disease in women desiring to preserve their fertility