INTRODUCTIONThe term congenital 'bladder outlet obstruction (BOO)' describes the collection of conditions in which the normal, urethral egress of urine from the fetal bladder is impaired. The term is interchangeable with fetal 'lower urinary tract obstruction', as used by other authors. 1,2 After considering normal urinary tract embryology, we describe the epidemiology of congenital BOO and the primary anatomical disorders associated with it. We then proceed to describe its fetal and postnatal clinical manifestations and then consider therapies and interventions which have been used to manage the condition. We not only focus on urethral and bladder disease with constitutes BOO itself, but also describe associated kidney disorders which, via chronic renal excretory failure, are important causes of morbidity. Rather than provide an exhaustive review, we emphasise studies published in the last decade, and therefore readers are referred to other reviews 3-7 citing numerous earlier references.
EMBRYOLOGYCongenital BOO is but one entity in a spectrum of renal tract anomalies which include kidney malformations (e.g. absent, small and abnormally-differentiated kidneys) and ureter malformations (e.g. duplicated, refluxing and obstructed uterers). 8 In fact, the lower and upper renal tract develop harmoniously, 9,10 so that the former is ready to receive urine first produced by metanephric kidneys between 8-10 weeks of human gestation, when the first layers of vascularised, and thus filtering, glomeruli are forming. In the seventh week after fertilisation, the cloaca divides into the urogenital sinus and the rectum. By the end of the first trimester the upper part of the sinus has differentiated into a muscularised urinary bladder, with the lower part forming the opening, or 'proximal ', urethra. 11 Ureteric smooth muscle is also forming at this time 12-13 and its peristalsis propels urine from the renal pelvis towards the bladder. 14