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.

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.

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

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  Image from "Diseases of children (1916)".

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  Image from "Diseases of infancy and childhood (1914)".

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  Ectopia vesicae post-operation.

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  Ectopia vesicae in a 23-year-old man.

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  Extrophy of the bladder.

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  Image from "Gaillard's medical journal (1882)".

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  5-year-old girl with ectopia vesicae.

• Madl-OP I.jpg

  Madl Operation Image I.

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  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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