Superior vesical fissure–VACTERL association in a Ugandan child: case report and literature review

Abstract Background Classic bladder exstrophy is defined as the presence of several abnormalities of the genitourinary and musculoskeletal systems. The patient with exstrophy is born with an open bladder that lies on the anterior abdominal wall contiguous with an epispadiac penis or bifid clitoris and divergent rectus muscles. The pelvis is characterized by a wide diastasis of the symphysis pubis caused by a 30% bony deficit of the anterior pubic rami in combination with a 12-degree and 18-degree external rotation of the posterior and anterior aspects of the pelvis, respectively. Superior vesical fissure is a variant of classical bladder exstrophy where only the upper portion of the bladder is open near the umbilicus. Superior vesical fissure represents an exstrophy-epispadias variant marked by superior bladder dehiscence, absence of lower tract exposure, musculoskeletal changes typical of exstrophy, and preservation of normal external genitalia. Case report We present a case of superior vesical fissure in a neonate born at 38 weeks with VACTERL association—(vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb anomalies). The female infant displayed a 2 cm long, vertical, exposed moist, red and shiny mucosa along the midline on the lower part of the anterior abdominal wall with urine being witnessed draining out of it. Radiologically, the symphysis pubis was normal. In addition, the patient had a low anorectal malformation in form of a vestibular fistula, left clubfoot, sacral dysplasia, multiple vertebral segmentation anomalies of the lower thoracic and lumbar spine (fused vertebra, hemivertebra), unilateral left lumbar rib, developmental dysplasia of the left hip, cardiac anomalies (small VSD), fibromatosis colli, and left renal agenesis, comprising the VACTERL association. Surgical management included cysto-vaginoscopy, repair of the superior vesical fissure, double-barrel sigmoidostomy for the vestibular fistula. The patient had a transurethral catheter for 5 days postoperatively. Subsequently, anterior sagittal anorectoplasty (ASARP) was performed eight months later, and 8 months later, a sigmoidostomy reversal was done. At two years follow up - May 2026- she has shown favorable follow-up outcome. The child passes Bristol type 5 stool 3–4 times a day without soiling. She is continent for urine. Conclusion Superior vesical fissure is a scarcely reported variant of classical bladder exstrophy. Literature documents 30 SVF cases globally, none linked to VACTERL. The anomaly is surgically manageable, but requires multidisciplinary coordination for associated abnormalities. Successful surgical repair and multidisciplinary care were achieved. Awareness of this variant and its potential associations help improve diagnosis and management in similar complex congenital malformations.