Contents
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