This 9-year-old girl was referred in February 1972. At age 5 weeks, an evaluation for failure to thrive showed a solitary right kidney with severe hydronephrosis literally filling the abdomen. Pyeloplasty was attempted, but failed. An inlying nephrostomy tube was placed and was being changed monthly.
Bladder volume was only 50 ml. Dilating it hydrostatically, although done gently, ruptured the dome. The dome was closed and pyeloplasty was performed, reducing the size of the renal pelvis. A nephrostogram 10 days later showed an easy passage of contrast to the bladder. Cystogram 4 months later showed increased bladder size, but massive reflux. The ureter was reimplanted curing the reflux. In Dec.1977, because she was small (4 feet, 3 inches tall), she was referred to an Endocrinologist who made the diagnosis of Turner’s Syndrome.
Scoliosis surgery was performed in 1980 at age 18 years. The patient has remained under close metabolic supervision; she has a stable creatinine level of 1.3.
X-rays (Select Image for High-quality Version). (Top Left) Delayed IVP as neonate showing huge right hydronephrosis. (Top Right) Nephrostogram and simultaneous retrograde pyelogram at age 9 years to assess anatomy. (Bottom Left) Nephrostogram 10 days after pyeloplasty. (Bottom Right) IVP age 15 years, note normal bladder size.
Anatomy (Select Image for High-quality Version). (Before) Dismembered pyeloplasty with reduction in size of renal pelvis. (After) Reimplantation of ureter for massive reflux 6 months later.
Review of her renal studies from childhood makes it seem miraculous that she has now reached age 52 years and is not yet in need of a renal transplant! She is a law school graduate and has passed the New York and Connecticut Bar examinations. It is gratifying to have followed this young woman’s development and successes during the past 40 years!