Updated: 9/3/2017

Congenital Bladder Disorders

Review Topic
  • 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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