Bladder exstrophy (also known as ectopia vesicae) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. The severity of these defects is widely variable.
The estimated incidence of bladder exstrophy is 1:10,000-50,000 live births 3,5. There is a recognized male predilection with a male to female ratio of ~3:1 6. Most cases are sporadic.
Bladder exstrophy is thought to be caused by a developmental defect of the cloacal membrane which results in a subsequent eversion of the bladder mucosa. This then protrudes out as a mass-like lesion.
- extension of the bladder defect into the urethra
- bilateral inguinal herniation
- OEIS complex
- vaginal duplication
- clitoral cleft
Imaging findings include a soft-tissue mass extending from a large infra-umbilical anterior wall defect which may be close to the umbilical arterial exits. The absence of a normal urinary bladder and a low-lying umbilical cord insertion 4 may also indicate the diagnosis.
Failure of the pubic bones to meet in the midline (widened pubic symphysis). This appearance on AP plain radiograph of the pelvis has been likened to a manta ray swimming towards you (manta ray sign) 7. Hurley stick appearance of distal ureters has been described in excretory urogram 8.
Amniotic fluid volumes are often normal.
Treatment and prognosis
Treatment is with surgical intervention (primary closure/excision with urinary diversion) and the prognosis is generally good.
- urinary incontinence
- acute pyelonephritis
- increased incidence of malignancy in the extruded bladder
- 1. Daltro P, Fricke BL, Kline-fath BM et-al. Prenatal MRI of congenital abdominal and chest wall defects. AJR Am J Roentgenol. 2005;184 (3): 1010-6. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Emanuel PG, Garcia GI, Angtuaco TL. Prenatal detection of anterior abdominal wall defects with US. Radiographics. 1995;15 (3): 517-30. Radiographics (abstract) - Pubmed citation
- 3. Ben-chaim J, Docimo SG, Jeffs RD et-al. Bladder exstrophy from childhood into adult life. J R Soc Med. 1996;89 (1): 39P-46P. - Free text at pubmed - Pubmed citation
- 4. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
- 5. Fotter R, Baert AL. Pediatric Uroradiology. Springer Verlag. (2008) ISBN:3540330046. Read it at Google Books - Find it at Amazon
- 6. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
- 7. Roller BL, Dyer RB. The "manta ray" pelvis. Abdominal radiology (New York). 41 (8): 1666-7. doi:10.1007/s00261-016-0706-y - Pubmed
- 8. Syed Z.H. Jafri, Marco A. Amendola, Ananias C. Diokno. Lower Genitourinary Radiology. (2012) ISBN: 9781461216483