Birth defects, reconstructive surgery: childhood surgical history illuminates pelvic problems.(Gynecology)

BALTIMORE -- An increasing number of girls with birth defects who had corrective renal or bladder surgeries in infancy and childhood are surviving to adulthood, and they are requiring extra care during pregnancy and gynecologic procedures. Dr. John Gearhart said at a urogynecology meeting sponsored by Johns Hopkins University.

Patients who have birth defects or who underwent reconstructive surgery for birth defects during infancy or childhood may have intrinsic sphincter weakness and bladder dysfunction, said Dr. Gearhart, professor and director of pediatric urology at Johns Hopkins University, Baltimore.

Improved methods of prenatal evaluation now allow physicians to diagnose many defects before birth, which allows surgeons to operate on newborns whose conditions are discovered prenatally.

The most common congenital anomalies in the upper urinary tract that can cause urinary incontinence in females are ectopic ureters and ectopic ureteroceles, Dr. Gearhart said.

Ectopic ureters are always associated with a duplicated system--two ureteral buds exit from the mesonephric duct, and one ends up positioned ectopically. These usually present prenatally, but they can present in early childhood. The buds can be close together or separated. Ideally, a surgeon will operate on an ectopic ureter during infancy. An ectopic ureter can take root in the bladder neck or vagina. When a young girl has a foul-smelling vaginal discharge, use an ultrasound of the kidneys and pelvis to look for an ectopic ureter, Dr. Gearhart advised.

Treatment for an ectopic ureter involves removing the upper pole of the kidney, which is usually done laparoscopically. "However, if there is reflux into the distal ureter we will remove it down into the "pelvis and remove the distal upper pole ureter," Dr. Gearhart said at the meeting.

Sometimes the lower pole must be reimplanted, but reimplantation may not happen if there is no reflux, he said. Thus, in the absence of reflux, a remnant of the ectopic ureter may be left behind.