Citation: BJOG: An International Journal of Obstetrics and Gynaecology. 2012, 119, 205
Author: Myagerimath R.; Gul N.; Rowlands D.J.
Abstract: Introduction: Developmental anomalies of the mullerian duct system represent some of the most fascinating disorders that obstetricians and gynecologists encounter. Incidence rates vary between 0.1% and 3.5%. The Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Here we are presenting a case of HWW syndrome with ovarian endometrioma, an uncommon cause of abdominal pain and menstrual discomfort in an adolescent girl. Case report: A 13 year-old girl presented with history of abdominal discomfort and painful periods since menarche. Initial ultrasound pelvis suggested a Dermoid cyst, MRI scan showed a uterus didelphys with normal right horn and grossly distended left vagina. Congenital absence of left kidney and left ureter were reconfirmed on MRI scan. Examination under anaesthesia and laparoscopy confirmed normal right cervix, right hemiuterus, right tube, right ovary with a noncommunicating left hemi uterus, which had caused hematocolpos, hematometra, hematosalphinx, left ovarian endometrioma with adhesions and massive distention of left parametrium. The large parametrial swelling drained one litre of old blood. Following laparoscopic drainage of endometrioma she was treated with GnRH analogues and add back HRT for 3 months. Then she successfully underwent laparoscopic hemi hysterectomy with left salphingectomy and preservation of both ovaries. The upper part of left vagina was left open to reduce the risk of pseudocyst formation. The procedure and post operative recovery were uneventful and she is painfree. Discussion: HWW syndrome usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos. Awareness is necessary in order to diagnose and treat this disorder
properly before complications occur. Because of the wide variation in clinical presentations, mullerian duct anomalies may be difficult to diagnose. Accurate diagnosis and proper surgical management can be challenging. MRI is the preferred modality for the delineation of uterine malformation. Ipsilateral renal agenesis with a pelvic mass should raise the level of suspicion for HWW syndrome. When renal anomalies are encountered, a screening should also be made for congenital abnormalities of the reproductive tract and vice versa. Conclusion: HWW syndrome is a rare cause of abdominal pain and dysmenorrhoea in an adolescent girl. Accurate diagnosis and proper surgical management can be challenging but laparoscopic hemi hysterectomy with preservation of fertility and ovarian reserve is a possible best treatment option.
