Bladder exstrophy

Bladder exstrophy

Cover image: Female baby with classical bladder exstrophy | By Anne-Karoline Ebert, Heiko Reutter, Michael Ludwig and Wolfgang H Rösch - Ebert, Anne-Karoline (2009-01-01). "The Exstrophy-epispadias complex". Orphanet Journal of Rare Diseases 4: 23. DOI:10.1186/1750-1172-4-23. PMID 19878548. PMC: 2777855. ISSN 1750-1172., CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=55276656

Introduction

https://www.youtube.com/watch?v=gM6mmi8sYSo

Bladder exstrophy (also known as ectopia vesicae) is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall.


Clinical features

Variable presentation

  • Abdominal wall defect
    • Occupied by exstrophied bladder + portion of urethra
  • Flattened puborectal sling
  • Separation/widening of pubic symphysis
  • Shortening of pubic rami
  • External rotation of pelvis
  • Urinary incontinence
  • UTI

Other changes:

  • Anus located more anteriorly
  • Males: Lack of anterior corporeal tissue, Epispadias (Urethra exits top of penis)
  • Girls: Wider, shorter, more vertical vagina, bifid clitoris, divergent labia

Complications

Primary Pathophysiology of Bladder Exstrophy and Complications that occur in Relation to its Management

Primary Pathophysiology (If Untreated) Complications (Associated with Management of Exstrophy)
Malignancy (related to chronic exposure of the bladder plate) Malignancy (related to the use of intestine in bladder reconstruction)
Pyelonephritis Pyelonephritis
Kidney stones Kidney and bladder stones
Total urinary incontinence Stress or urge urinary incontinence
Chronic bladder irritation Hydronephrosis
Pelvic floor insufficiency Cystocele, uterine prolapse
Abnormal hip dynamics, back pain Abnormal hip dynamics, back pain
Symphyseal diastasis, pelvic flattening Urinary outlet obstruction
Abdominal wall defect Absent umbilicus
Ventral and inguinal hernias Incisional hernias
Severe penile shortening with dorsal chordee Inadequate phallus in males with subsequent social and psychological sequelae

Diagnosis


Management

Immediate management:

  • Placement of non-adherent film over protruding bladder

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