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Towards a better understanding of pediatric urachal anomalies
Joseph Gleason, MD, Darius Bagli, MD, Armando Lorenzo, MD, Joao Luiz Pippi Salle, MD, Martin Koyle, MD, Walid Farhat, MD.
The Hospital for Sick Children, Toronto, ON, Canada.
Urachal anomalies are thought to be associated with an increased risk of bladder adenocarcinoma in adults, which has an incidence of approximately 1 in 5 million annually. Despite the rarity of this feared outcome, the literature is split on how to manage these lesions when diagnosed in children. Recent literature suggests excision of even incidentally discovered urachal anomalies is recommended. We sought to examine the presentation, diagnosis and management of radiologically diagnosed pediatric urachal anomalies in a tertiary care center with a catchment of approximately 10 million people.
Methods: Our radiology database (2000-2012) was queried for all children under the age of 18 who were diagnosed with a urachal anomaly radiographically. The charts of those patients were individually reviewed to confirm the diagnosis, and this list was cross-referenced to the operative procedures performed in the same time range to identify the patients who underwent surgical intervention. Patient demographics, presentation and histopathologic data were collected, in addition to indication for excision and the imaging modality used for diagnosis.
Results: Over a 13 year time period, 731 patients were radiographically diagnosed with a urachal anomaly, either due to symptoms or incidentally. Of those, only 61 (8.3%) underwent surgical excision. Imaging diagnoses were persistent urachal remnants (50%), sinus tract/patent urachus (18%), and urachal cysts (32%). Ultrasonography was the most common imaging modality used in the group who underwent excision (89%), followed by fluoroscopy (7%) and VCUG (4%). Indications for imaging and treatment in the surgical group were umbilical drainage (43%), palpable mass (25%), pain (14%), UTIs (11%) and hematuria (3%). However, 25% of those excised were incidentally diagnosed, and prophylactic surgery was undertaken because of recent recommendations made in the literature. The mean age at excision was 5.6 years (range 3 days to 17.1 years), and 64% were male. The majority of the specimens contained epithelial elements (74%), with urothelium present in 52% of all specimens (bladder cuff tissue excluded), followed by intestinal or cuboidal epithelium in 15% each. No complications were reported in those undergoing simple excision, and all symptomatic patients were cured of their presenting symptoms.
Conclusions: This large retrospective case series demonstrates that although a large number of urachal anomalies were diagnosed in this single medical center, the percentage undergoing excision was rather low. This group of 670 patients who were not operated upon now form a large cohort whose longitudinal follow up will be presented to help objectively ascertain the natural history of asymptomatic pediatric urachal anomalies. Though removal of urachal anomalies carries a relatively low degree of morbidity, prophylactic excision may nevertheless not be necessary in asymptomatic children.
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