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Chiari III
Joseph Junewick, MD FACR
over 6 years ago
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Bladder Extrophy

Case Detail

Anatomy: Genitourinary
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: CR
Activities:
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History

Newborn female with anterior abdominal wall mass.


Case Images


Diagnosis

Bladder Extrophy

Findings

CR – Midline rounded soft tissue mass in the lower abdomen, thoracic butterfly vertebra and pubic diastasis.

Discussion

Potential etiologies for bladder extrophy include 1) Failure of the cloacal membrane to be reinforced by ingrowth of mesoderm, 2) Abnormal development of the cloacal membrane, preventing lower abdominal wall development, 3) Abnormal caudal development with midline fusion below rather than above the cloacal membrane, 4) Abnormal caudal insertion of the body stalk-failure of interposition of mesenchymal tissue in the midline. Timing of the insult determines the variant of the exstrophy-epispadias complex that will result.

Bladder extrophy occurs 1 in 10,000 to 50,000 births with a slight male predominance. The risk of recurrence in a family is one in 100; children of parents with exstrophy have a one in 70 chance of having the defect. Diagnosis is usually made on prenatal sonography with the inability to localize the fluid-filled bladder on multiple exams.

Postnatally, the diagnosis is usually by an atypical appearance of the lower abdomen. Defects include 1) distorted pelvic floor musculature, 2) external rotation of the pelvic bones, 3) abnormal umbilicus, 4) high incidence of inguinal hernias, 5) anorectal defects, 6) male and female epispadius, 7) undescended testicles, 8) stenotic introitus.



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