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- One hundred and sixty-two cases of suspected congenital abnormalities of the urinary tract notified to the Northern Region Fetal Abnormality Survey Register between the beginning of 1984 and the end of 1986 were studied. These cases represented 14.3% of total notifications, which in turn were 0.93% of total births. There were a further 25 cases notified but not due for delivery until early 1987; 10 of these were urological. Thirty-five cases (22%) notified proved postnatally to have normal urinary tracts and 21 (13.2%) had unsuspected urinary tract abnormalities. Of the 106 cases in which a suspected urinary tract abnormality was confirmed postnatally, confirmation was obtained at perinatal death in 32 and by ultrasound, radiographic and radioisotope investigation in the remainder; 34.5% of all urological cases registered and 75% of the confirmed survivors were treated surgically. The urological abnormality most frequently missed was posterior urethral valves. Attempts to diagnose the fetal sex were seldom made but when they were the prediction was usually male and usually correct. The perinatal mortality in this group was 0.43/1000 births, a contribution of nearly 4% to overall perinatal mortality in the Northern Region. [ABSTRACT FROM AUTHOR]

- A series of 46 children treated by the author since January 1972 for congenital posterior urethral valves is presented: 22% were diagnosed at birth, 28% as neonates and 52% in the first 3 months of life. Ninety-three per cent had unilateral or bilateral dilatation of the upper urinary tract at the time the valves were diagnosed and 72% had ureteric reflux. Unilateral reflux occurred into the left ureter twice as often as the right. Renal failure was present at the time of diagnosis in 72% of all of the children but in 83% of those aged less than 3 months. Surface urinary diversion was used minimally during post-operative management and contributed little to the recovery of renal function. Reflux disappeared spontaneously in one-third of the refluxing ureters. Ureteric dilatation subsided spontaneously in 57% of dilated ureters. Surgery was performed mostly for reflux. Non-refluxing ureteric dilatation was made worse by surgery in a few instances and in others the dilatation improved with time rather than as a result of surgery. Renal function returned to normal in over 60% of the children who were in renal failure at diagnosis. Measurement of glomerular filtration rate was the most accurate method of predicting recovery of renal function: a value of less than 50% of normal for age at the time of diagnosis forecast persistent chronic renal failure with all its attendant complications. [ABSTRACT FROM AUTHOR]

- Eight children, 3 girls and 5 boys, with single ectopic ureters arising from non-concentrating kidneys are described. Two children, both girls, complained of persistent wetting with a pattern suggestive of ectopic ureter: 2 of the boys presented in the neonatal period with massive abdominal distension: the remaining children developed a urinary infection or chronic urinary retention. Diagnosis depends on an appreciation of the possibility that a non-concentrating kidney on intravenous urography may be giving rise to an ectopic ureter and, in the boys, on a correct interpretation of cystourethrographic findings. Careful endoscopic examination of the urethra and in the girls of the vagina also will lead to the identification of the ectopic ureteric orifice in most cases. When the ectopic ureter joins the male genital tract there may be anextravesical ureterocele which can be recognised cystoscopically. The condition is treated by nephroureterectomy. [ABSTRACT FROM AUTHOR]

Eighty six children with neuropathic urinary incontinence who had been treated by clean intermittent catheterisation for more than five years were reviewed. Eighty five had congenital lesions, and one traumatic paraplegia. During the day, 72 (84%) patients were dry or had minor stress incontinence only, 11 were damp but controlled with pads, and only three were continuously wet. Eleven abandoned clean intermittent catheterisation, five because of poor control, four by choice despite good control, and only two because of deterioration of upper urinary tract disease. Most of them usually had bacteriuria, but only 32 developed occasional symptomatic infections. Urinary calculi occurred in six, epididymitis in three, and urethral problems in four. Upper urinary tract dilatation did not arise in a previously normal renal system in any patient during clean intermittent catheterisation. Clean intermittent catheterisation was successful in controlling wetting with few serious complications, and was well tolerated. It remains the method of choice for the management of neuropathic urinary incontinence in such children. [ABSTRACT FROM AUTHOR]

- The hypothesis that testicular descent may be governed by Müllerian inhibiting substance (MIS) was investigated by observing the position of the testes in 13 children with XY karyotype and persistent Müllerian duct syndrome (PMDS). It was found that there was a direct relationship between failure of testicular descent and the degree of development of the Müllerian system. Where the Müllerian system was complete, the testes were in an ovarian position but where only the vagina was present, the testes were sometimes found in the inguinal region. To discover whether excessive androgen activity in females might produce ovarian descent, the position of the ovaries in 15 children with severe adrenogenital syndrome was observed. Despite complete genital masculinisation in three children and almost complete in six, all but one ovary was in the normal position: that ovary had descended in an inguinal hernia sac. These findings suggest that MIS rather than androgens may be responsible for the first or abdominal phase of testicular descent. Even excessive androgen activity failed to cause ovarian descent. [ABSTRACT FROM AUTHOR]