Normal Functioning Pelvic Lump Kidney: Isolated Anomaly - Radiological Evaluation.

There is wide spectrum of Mullerian dysgenesis presenting in different ways. Routine ultrasound done of a child for pain abdomen discovered a pelvic mass supero-posterior to the urinary bladder. This was diagnosed as fused pelvic kidneys by various cross sectional imaging modalities like Computerized tomography (CT) and Magnetic resonance imaging (MRI).We present a 4-years male child who was diagnosed as a case of “lump kidneys” by plain sonography coupled with color flow imaging (CFI). The entity usually falls in the common group of VATER (vertebral, anorectal malformation, esophageal and renal) anomalies. But our present case was having isolated anomaly and this entity is of a great rarity as seen in literature.

Mayer-Rokitansky-Küster-Hauser Syndrome with Imperforate Anus: A Rare Association and an Innovative Surgical Management.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is one of the rare disorder of Mullerian agenesis leading to non-development of uterus and vagina. Its association with anorectal malformation is rare. We report a case of MRKH syndrome with recto-vestibular in a female child. The child had undergone a sigmoid loop colostomy in the neonatal period. On clinical examination of the perineum, a fistula was present in the vestibule just below the urethral opening, but no vaginal canal was found. It was managed with an innovative surgical technique, preserving the fistula and lower rectum to function as vagina.