Trans-umbilical management of OHVIRA syndrome by a thoracic trocar technique: A case report

Introduction

Cervical atresia in cases of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is rare. To prevent complications that may occur due to the association of cervical atresia with OHVIRA syndrome, a left hemihysterectomy and salpingectomy is occasionally needed.

Case presentation

A 14-year-old female was referred to us due to abdominal pain. She had never experienced menstrual pain until three periods preceding her presentation. Abdominal ultrasound (US) showed that the left horn of the uterus was hypoplastic and compatible with hematosalpinx. Computerized tomography (CT) of the abdomen showed a 3-cm left endometrial cavity, and an 8-cm septated lesion in the left adnexa. The left ovary could not be visualized. Magnetic resonance (MR) revealed uterus didelphys and a 6–8 cm septated lesion in the left adnexa. The left kidney was not present. The left cervix was absent. Based on these findings, OHVIRA syndrome was diagnosed. She was taken to the operating room for a hysteroscopy, which showed a single cervix, normal uterine mucosa, and a single tubal ostium. No septum was seen in the vagina. She then underwent a laparoscopy, which revealed uterus didelphys, widespread endometriosis, a large left hematosalpinx, left tubal torsion, no left ovary, and a normal right ovary. We proceeded with a left hemihysterectomy and salpingectomy using a transumbilical single-incision approach. The foci of endometriosis were suctioned and the specimen retrieved through the umbilical incision. She recovered well from the operation and was discharged home 5 days later.

Conclusion

OHVIRA syndrome should be suspected in patients with renal agenesis and an ipsilateral adnexal mass. Patients with OHVIRA and cervical atresia may need a left hemihysterectomy and salpingectomy, which can be done through a transumbilical single-incision approach.