Bladder exstrophy

Bladder exstrophy is a complex congenital anomaly that involves the protrusion of the bladder through the abdominal wall. This condition lies within the spectrum of the exstrophy-epispadias complex. It is associated with several other anomalies and requires a multi-faceted approach to management. This article aims to provide an in-depth understanding of its embryology, clinical presentation, diagnosis, management, and implications for affected individuals.

File:Case of ectopia vesicae Wellcome L0062458.jpg

Case of ectopia vesicae

Embryology

During early embryonic development, the cloacal membrane's failure to rupture and inadequate mesenchyme causes bladder exstrophy. This leads to:

• Exposed bladder mucosa on the lower abdomen.
• Disruption in the development of the anterior abdominal wall and underlying structures.

File:Classical bladder exstrophy.jpg

Classical bladder exstrophy

Clinical Presentation

• Bladder: The posterior wall of the bladder is exposed on the lower abdominal wall.
• Urethra: It may be split (epispadias) or may appear shortened.
• Pelvic Bones: Separation of the pubic symphysis and external rotation of the pelvic bones.
• Anorectal Anomalies: Disruption of the puborectal sling and sphincters.
• External Genitalia: It may be malformed, with males having a short, broad penis and females having a bifid clitoris and separated labia.

Diagnosis

• Antenatal Ultrasound: May show a lower abdominal mass with absence of a normally filled bladder.
• Physical Examination: Exposed bladder mucosa and associated anomalies are usually evident at birth.
• Radiological Examinations: Pelvic X-ray can confirm pubic diastasis. Additional imaging like MRI may be done to evaluate associated anomalies.

Management

Managing bladder exstrophy is challenging and involves a multi-disciplinary approach:

• Initial Management: Sterile coverings to protect the exposed bladder and surgical consultation.
• Primary Closure: Surgical closure of the bladder and abdominal wall is usually attempted in the neonatal period.
• Reconstructive Surgeries: Additional procedures might be needed for urinary continence and to address genital and orthopedic anomalies.
• Long-Term Follow-Up: Regular monitoring for urinary function, renal function, and potential complications is essential.

Complications and Prognosis

• Urinary Incontinence: Continence can be a challenge and may require additional surgeries.
• Renal Dysfunction: Due to associated urological anomalies.
• Fertility Issues: Especially in males, due to associated anomalies and surgeries.
• Psychosocial Impact: Issues related to body image and self-esteem can arise and may require psychological support.

With appropriate surgical care and follow-up, many individuals with bladder exstrophy lead normal lives, albeit with potential challenges related to the condition.

See Also

• Congenital anomalies of the kidney and urinary tract (CAKUT)
• Urogenital embryology
• Epispadias

Gallery

• Image from "Diseases of children (1916)".
  Image from "Diseases of children (1916)".
• Image from "Diseases of infancy and childhood (1914)".
  Image from "Diseases of infancy and childhood (1914)".
• Ectopia vesicae post-operation.
  Ectopia vesicae post-operation.
• Ectopia vesicae in a 23-year-old man.
  Ectopia vesicae in a 23-year-old man.
• Extrophy of the bladder.
  Extrophy of the bladder.
• Image from "Gaillard's medical journal (1882)".
  Image from "Gaillard's medical journal (1882)".
• 5-year-old girl with ectopia vesicae.
  5-year-old girl with ectopia vesicae.
• Madl Operation Image I.
  Madl Operation Image I.
• Image from "The practice of surgery (1853)".
  Image from "The practice of surgery (1853)".

References

• Ebert, A. K., Reutter, H., Ludwig, M., & Rösch, W. H. (2009). The exstrophy-epispadias complex. Orphanet Journal of Rare Diseases.
• Gearhart, J. P., & Mathews, R. (1996). Exstrophy-epispadias complex and bladder anomalies. Urologic Clinics of North America.

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