Herlyn Werner Wunderlich Syndrome with an incidental finding of Concomitant Ipsilateral Adrenal Agenesis

 

Herlyn Werner Wunderlich Syndrome (HWWS) is arare form of mullerian duct anomaly characterized by a combination of uterine didelphys,obstructed hemivagina, and ipsilateral renal agenesis. Internationally, its incidence is 0.1-3.8% and among the reported cases, none presented with a concomitant adrenal glandagenesis. This is the first case report of a diagnosed Herlyn Werner Wunderlichsyndrome with a concomitant unilateral adrenal gland agenesis.

 

This is a case study of a10-year old Filipino who presented withsevere cyclic abdominal pain. MRI resultshowed two separate uteri and cervices,obstructed left hemivagina, absent leftkidney, and adrenal gland. This patient hasno symptoms of adrenal insufficiency, andserum cortisol and ACTH levels were withinnormal limits. She underwent vaginoscopyand resection of the longitudinal vaginalseptum. Postoperatively, the regularmenstrual cycle continued and was nolonger associated with dysmenorrhea.Follow-up after six months revealed norecurrence of hematacolpos on 3Dtransrectal sonography.

CONCLUSION

The rarity of HWWS is a major contributory factor in delayed diagnosis; however, if detected early, timely decompressionof vaginal obstruction could avoid complications and preserve reproductive capacity. A high index of suspicion is needed for patients who presentwith cyclic pelvic pain during puberty and patients who have findings of renal anomaly. The preferred surgical approach for these patients isresection of the vaginal septum since the main reason for the symptoms is the physical obstruction of the menstrual flow, which predisposes toendometriosis due to the retrograde flow of menstruation. This procedure is associated with a successful reproductive performance in the future.

The significance of the incidental finding of concomitant adrenal agenesis in a diagnosed HWWS suggests that other abnormalities or defects maybe associated with this syndrome and not just what is presently known. The congenital anomaly involving the reproductive organ poses a challengein the patient’s capacity to bring about a successful pregnancy. Adding to that is the possibility of developing adrenal insufficiency during stressfulsituations or pregnancy. It is crucial to consider these things in managing this patient.

The body of knowledge is ever-changing and fast-growing. What we know now may only be a fraction of what will be discovered in the future. Thiscase report hopes to be a beacon which will pave a way for future studies that is more focused on unilateral gland agenesis.