Bladder exstrophy: Difference between revisions

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File:Ectopia vesicae in a man aged 23 years Wellcome L0062460.jpg

Bladder Exstrophy

Bladder exstrophy is a rare congenital anomaly that involves the protrusion of the urinary bladder through a defect in the lower abdominal wall. This condition is part of the exstrophy-epispadias complex, which includes a spectrum of anomalies affecting the urinary tract, genitalia, and pelvic structures.

Pathophysiology

Bladder exstrophy occurs due to a failure in the closure of the infraumbilical midline structures during embryonic development. This results in the bladder being exposed outside the body, with the inner surface of the bladder visible externally. The condition is often associated with other anomalies such as epispadias, where the urethra does not develop properly, and a separation of the pubic symphysis.

Epidemiology

Bladder exstrophy is a rare condition, with an estimated incidence of 1 in 30,000 to 50,000 live births. It is more common in males than females, with a male-to-female ratio of approximately 2:1.

Clinical Presentation

Infants with bladder exstrophy present with a visible defect in the lower abdominal wall, where the bladder mucosa is exposed. The umbilicus is typically low-set, and the genitalia may be malformed. In males, the penis is often short and broad with epispadias, while females may have a bifid clitoris and a short, wide vagina.

Diagnosis

Diagnosis of bladder exstrophy is usually made at birth based on the characteristic physical findings. Prenatal ultrasound may suggest the diagnosis if there is an absence of a visible bladder and other associated findings such as a low-set umbilicus and abnormal genitalia.

Management

The management of bladder exstrophy involves surgical correction, which is typically performed in stages. The initial surgery aims to close the bladder and abdominal wall, and subsequent procedures may address the reconstruction of the genitalia and urinary continence. Multidisciplinary care involving pediatric urologists, surgeons, and other specialists is essential for optimal outcomes.

Prognosis

With advances in surgical techniques, the prognosis for individuals with bladder exstrophy has improved significantly. Many patients achieve urinary continence and have normal renal function. However, long-term follow-up is necessary to monitor for complications such as urinary tract infections and renal impairment.

Also see

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-[[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:Classical bladder exstrophy.jpg|thumb]] [[File:Ectopia vesicae in a man aged 23 years Wellcome L0062460.jpg|thumb]] Bladder Exstrophy
-[[File:Case of ectopia vesicae Wellcome L0062458.jpg|Case of ectopia vesicae|500px|thumb]]
-== 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|thumb|500px]]
-== 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 ==
+Bladder exstrophy is a rare congenital anomaly that involves the protrusion of the urinary bladder through a defect in the lower abdominal wall. This condition is part of the exstrophy-epispadias complex, which includes a spectrum of anomalies affecting the urinary tract, genitalia, and pelvic structures.
-* '''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 ==
+==Pathophysiology==
-Managing bladder exstrophy is challenging and involves a multi-disciplinary approach:
+Bladder exstrophy occurs due to a failure in the closure of the infraumbilical midline structures during embryonic development. This results in the bladder being exposed outside the body, with the inner surface of the bladder visible externally. The condition is often associated with other anomalies such as epispadias, where the urethra does not develop properly, and a separation of the pubic symphysis.
-* '''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 ==
+==Epidemiology==
-* '''Urinary Incontinence:''' Continence can be a challenge and may require additional surgeries.
+Bladder exstrophy is a rare condition, with an estimated incidence of 1 in 30,000 to 50,000 live births. It is more common in males than females, with a male-to-female ratio of approximately 2:1.
-* '''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.
+==Clinical Presentation==
+Infants with bladder exstrophy present with a visible defect in the lower abdominal wall, where the bladder mucosa is exposed. The umbilicus is typically low-set, and the genitalia may be malformed. In males, the penis is often short and broad with epispadias, while females may have a bifid clitoris and a short, wide vagina.
-== See Also ==
+==Diagnosis==
-* [[Congenital anomalies of the kidney and urinary tract (CAKUT)]]
+Diagnosis of bladder exstrophy is usually made at birth based on the characteristic physical findings. Prenatal ultrasound may suggest the diagnosis if there is an absence of a visible bladder and other associated findings such as a low-set umbilicus and abnormal genitalia.
-* [[Urogenital embryology]]
+==Management==
+The management of bladder exstrophy involves surgical correction, which is typically performed in stages. The initial surgery aims to close the bladder and abdominal wall, and subsequent procedures may address the reconstruction of the genitalia and urinary continence. Multidisciplinary care involving pediatric urologists, surgeons, and other specialists is essential for optimal outcomes.
+==Prognosis==
+With advances in surgical techniques, the prognosis for individuals with bladder exstrophy has improved significantly. Many patients achieve urinary continence and have normal renal function. However, long-term follow-up is necessary to monitor for complications such as urinary tract infections and renal impairment.
+==Also see==
 * [[Epispadias]]
+* [[Congenital anomalies]]
+* [[Pediatric urology]]
+* [[Urinary tract reconstruction]]
-== Gallery ==
+{{Congenital disorders}}
-<gallery>
+{{Urology}}
-Diseases of children (1916) (14579546649).jpg|Image from "Diseases of children (1916)".
-Diseases of infancy and childhood (1914) (14769641464).jpg|Image from "Diseases of infancy and childhood (1914)".
-Ectopia vesicae after operation Wellcome L0061353.jpg|Ectopia vesicae post-operation.
-Ectopia vesicae in a man aged 23 years Wellcome L0062460.jpg|Ectopia vesicae in a 23-year-old man.
-Extrophy of the bladder Wellcome L0004960.jpg|Extrophy of the bladder.
-Gaillard's medical journal (1882) (14585661490).jpg|Image from "Gaillard's medical journal (1882)".
-Girl, aged five years, with ectopia vesicae Wellcome L0062461.jpg|5-year-old girl with ectopia vesicae.
-Madl-OP I.jpg|Madl Operation Image I.
-The practice of surgery (1853) (14797952153).jpg|Image from "The practice of surgery (1853)".
-</gallery>
-== 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''.
 [[Category:Congenital disorders]]
 [[Category:Urology]]
-[[Category:Pediatrics]]
-[[Category:Surgery]]
-{{stub}}