Urinary Retention in an Adolescent Male with Partial Bladder Duplication, Complete Urethral Duplication, and Glans Duplication in the Coronal Plane
Charlotte Q. Wu MD* and Edwin A. Smith, MD
Division of Pediatric Urology, Children's Healthcare of Atlanta, Emory University School of Medicine; Atlanta, GA, USA
Introduction: Diphallia is exceedingly rare, with an incidence of 1 to 5.5 million births. Glans duplication (GD) is the rarest form of diphallia and occurs almost exclusively in the sagittal plane1. We describe the first case of GD in the coronal plane with isolated genitourinary anomalies. Our patient had associated features of urethral duplication, partial bladder duplication, and presented with urinary retention as an adolescent.
Cases: The patient is a healthy 14-year-old male seen initially as a prenatal consult for enlarged bladder and hydronephrosis. Post-natal exam was notable only for GD, with a right dominant glanular hypospadiac meatus, a left orthotopic meatus, and a single penile shaft. Workup by ultrasound initially demonstrated normal kidneys and bladder, though revealed right hydronephrosis and a dilated bladder with over 400mL capacity by 9 months of age. Voiding cystourethrogram (VCUG) and MR urogram showed bilateral grade 3-4 vesicoureteral reflux (VUR) and confirmed partial bladder duplication with a right and left urethra and three apparent corporal bodies. He had mild bladder residuals and was managed for several years with timed voids and prophylactic antibiotics, with improvement of VUR, resolved hydronephrosis, and no infections. More recently, he presented with elevated bladder residuals and febrile UTIs. An appendicovesicostomy was created. Correction of the penile anomaly has been offered separately, though family has opted to manage conservatively.
Discussion: Lower urinary tract duplication anomalies can occur in a variety of configurations and are more commonly associated with bowel or spine anomalies or with syndromes2. The etiology of lower tract duplication remains poorly understood, though it appears multiple embryopathies may account for the spectrum of anatomic variations1,2.
1 Jesus LE, et al. Glans duplication: In-depth review and proposal of a new classification. J Pediatr Urol. 2017 Apr;13(2):172-176. doi: 10.1016/j.jpurol.2016.12.010. Epub 2017 Jan 18. Review. PubMed PMID: 28161406
2 Matta H, Nawaz A, Jacobsz AW, Al-Salem A. A rare association of urogenital duplication and anorectal malformation. J Pediatr Surg. 2002 Nov;37(11):1623-5. PubMed PMID: 12407552
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