British Association of Paediatric Urologists consensus statement on the management of the primary obstructive megaureter

Abstract

A peer-reviewed consensus guideline for the management of the primary megaureter has been established. The guideline is based on current evidence and peer practice and the BAPU recognized that new techniques requiring further studies may have a role in future management.

Dilatação Endoscópica de Balão para Tratamento de Megaureter Obstrutivo Primário: Experiência de um Centro

“…5 However, ureteric reimplantation in infants less than one-year of age may be challenging due to the discrepancy between the grossly dilated ureter and the small infantile bladder and the concern regarding possible iatrogenic bladder dysfunction, such as VUR. 2 Therefore, some authors consider alternative temporizing or permanent interventions avoiding reimplantation in infancy.…”

“…These were later subdivided by King into primary and secondary. 2 Primary obstructive megaureter (POM) is most commonly caused by a distal adynamic ureteral segment. A few histopathologic characteristics of the adynamic segment have been described such as abnormal neuromodulation, abnormal collagen deposition and abnormalities in the interstitial cells of Cajal.…”

“…3 The timing of smooth muscle differentiation in the distal ureter is unknown but may be the key to explain pathogenesis of primary (or congenital) megaureter. The process whereby the circular muscle pattern, which is typical of the fetal ureter, changes progressively into double muscle layers on the full term infant, may last up to two years, 2 and may explain why this condition resolves spontaneously in approximately 80% of those patients diagnosed prenatally and that is why conservative management is safe initially. 4 Surgical management is indicated when initial differential renal function (DRF) is less than 40% and when conservative management fails.…”

“…4 Surgical management is indicated when initial differential renal function (DRF) is less than 40% and when conservative management fails. 2 Traditionally, the surgical management of POM has been via ureteral reimplantation with or without ureteral remodeling. However, in newborns and children under one year of age this technique is very difficult and has a high complication rate.…”

Single Nucleotide Variants in A Family of Monozygotic Twins Discordant for the Phenotype Congenital Megaureter: A Genomic Analysis

“…Ultrasound [US] scan was performed in all cases after 28 weeks of gestation. Megaureter was considered to be present in case of an ureter diameter superior to 7 mm in fetal US [3,5]. Patients were followed up at the Pediatric Nephrology Unit of the Hospital das Clínicas -Universidade Federal de Minas Gerais [UFMG], Brazil and all of them underwent postnatal image and laboratory investigation according to a systematic protocol as detailed described elsewhere [29].…”