Duplicate bladder exstrophy in a female infant: A case report

Abstract

Duplicate bladder exstrophy, a rare variant of the bladder exstrophy-epispadias complex, was diagnosed in a 5-month-old female infant presenting with a 4 × 4 cm lower abdominal mass. Physical examination revealed absent umbilical scar, wide symphysis pubis, and anteriorly displaced genitalia and anus. Imaging ruled out classic bladder exstrophy and vesicoureteral reflux. Surgical exploration confirmed a suprapubic exstrophic mucosal plaque without communication to a functional bladder. Management involved excision of the exstrophic plaque and primary abdominal wall closure. Post-operative course was normal intravenous pyelogram findings. This case emphasizes the importance of accurate diagnosis and tailored surgical approach for rare bladder exstrophy variants.

1 Introduction

Bladder exstrophy is a rare and complex congenital malformation affecting approximately 1 in 50,000 live births. It is characterized by the failure of the anterior abdominal wall and bladder to close properly during embryonic development, resulting in an exposed bladder and associated anomalies of the genitourinary system and pelvic structures. While classic bladder exstrophy presents with an open bladder visible on the abdominal surface, variants of this condition can manifest with varying degrees of severity and anatomical involvement.

This case report describes an unusual presentation of a variant form of bladder exstrophy in a 5-month-old female infant. The case is particularly noteworthy due to its atypical presentation, with a closed but extrophied bladder segment, and the successful surgical management that preserved normal bladder function.

The manuscript was prepared following the CARE guidelines ( https://www.carestatement.org ).

2 Case report

A 5-month-old female infant, weighing 5 kg, presented with a lower abdominal mass present since birth. The mass, located in the suprapubic area, measured approximately 4 × 4 cm, with a red, moist appearance and normal surrounding skin.Upon applying pressure, it partially reduced, resembling a small cup before protruding again. Physical examination revealed an absent umbilical scar, wide symphysis pubis, and anterior displacement of the genital area and anal opening( Fig. 1 ). To rule out bladder exstrophy, a Foley catheter was inserted, and the bladder was filled with serum, showing no extravasation from the mass. An abdominal ultrasound was reported as normal. The patient was admitted and underwent surgery. The surgical procedure involved a lower midline incision from the expected umbilical area, extended around the mass ( Fig. 2 A). Dissection revealed the mass’s closest attachment to the urinary bladder in the coronal plane, anterior to the bladder, with adhesions present up to the bladder neck ( Fig. 2 B and C). Careful dissection was performed without opening the bladder, which appeared normal in size with thin muscle at the attachment site. After removing the extrophied bladder tissue ( Fig. 3 ), the abdomen was closed layer by layer, supported by fascia under tension due to symphysis pubis dehiscence.Post-operatively, the patient remained in the hospital for 3 days. A post-operative intravenous pyelogram (IVP) was performed, with results reported as normal. An orthopedic consultation was advised for management of the pelvic abnormality.