PERSISTENT UROGENITAL SINUS – A RARE CASE REPORT

Introduction: Urogenital disorders are one of the most common congenital anomalies presenting in infants and children. Persistent urogenital sinus is a condition where, there is failure of separation between urinary and genital system. This condition is associated with many other congenital anomalies and syndromes like spinal defects, ano-rectal and uterine malformations. There are multiple presentations known for this condition, depending on the age of presentation. Case History: We report a case of a 25 year old woman referred to our hospital with pelvic inflammatory disease. A clinical examination of the perineum revealed a single Genito-urinary meatus between the labia. The Patient had associated skin disease all over the body which was proved histopathologically as kindlers syndrome (epidermolysis bullosa) with associated oesophageal webs. There are no other associated renal, bladder, rectal and anal anomalies. Radiological evaluation through Micturating cystourethrogram (MCUG), Genitogram, Ultrasound, pelvic CT and MRI revealed low confluence persistent urogenital sinus which was confirmed through cystoscopy. There is a rare presentation of persistent urogenital sinus in a young female of reproductive age. Conclusion: This case emphasizes the need for radiologists, gynaecologists, urologists and other physicians to be aware of congenital urogenital anomalies when a patient presents with abnormal external genito-urinary openings, primary amenorrhea, pelvic cystic mass and ambiguous genitalia.