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Updated: Sep 3 2017

Congenital Bladder Disorders

  • Snapshot
    • A 2-year-old boy is brought to his pediatrician’s office for evaluation of a “wet umbilicus.” His parents report noticing a persistently wet belly button since birth, and attributed that to healing after birth. However, now that he is 2-years-old and starting to interact with other children, they are concerned that he may be bullied for having a wet umbilicus. His past medical history is only significant for a large umbilicus at birth. On physical exam, clear liquid is leaking from his umbilicus without erythema or purulence. He is scheduled for an ultrasound later that week.(Patent urachus)
  • Exstrophy of the Bladder
    • Clinical definition
      • failure of caudal fold closure of ventral abdominal wall to close around the bladder
    • Epidemiology
      • incidence
        • 3-5 per 100,000
      • demographics
        • males > females
        • more frequent in first-born children
        • Caucasian > other ethnicities
    • Associated conditions
      • often associated with epispadias (congenital defect of penis)
        • often part of exstrophy-epispadias complex (EEC)
      • inguinal hernia
    • Presentation
      • phyical exam
        • low set umbilicus
        • open bladder plate and exposed urethra
        • inguinal hernia
        • may have genital abnormalities
    • Imaging
      • prenatal ultrasound
        • indications
          • routine prenatal care
          • findings
            • absence of bladder filling
            • diminutive genitalia
      • MRI
        • indications
          • to confirm findings of ultrasound
    • Treatment
      • reconstructive surgery
    • Complications
      • ↑ risk of adenocarinoma
  • Urachal Disorders
    • Clinical definition
      • congenital bladder disorder that results from a failure to obliterate the urachus
        • urachus is the duct between fetal bladder and the umbilicus
    • Epidemiology
      • incidence
        • 1% or less of boys
    • Classification
      • patent urachus
        • complete failure of urachus to obliterate
        • presentation
          • physical exam
            • giant umbilical cord at birth
            • persistent urine discharge from umbilicus
        • complications
          • urinary tract infections
      • urachal cyst
        • failure of urachus to completely obliterate
        • results in persistence of fluid-filled cavity between umbilicus and bladder
        • presentation
          • physical exam
            • umbilical mass
            • infection with tenderness and erythema below the umbilicus
        • complications
          • adenocarcinoma
      • vesicourachal diverticulum
        • failure of urachus to completely obliterate
          • to a lesser extent than that in urachal cyst
        • presentation
          • physical exam
            • outpouching of the bladder
            • ureteral obstruction
    • Imaging
      • ultrasound
        • initial imaging
    • Treatment
      • surgical resection
        • indication
          • for correction of urachal cyst and patent urachus
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