Presenter: Kristen M Meier, MD
Email: kristen.meier@beaumont.org
@kris10meier
@nathannovotny
Authors: Kristen M Meier, MD; Nathan M Novotny, MD; Brian D Odom, MD; Donald P Gibson, MD; Zachary J Liss, MD; Beaumont Health
A 2-month-old male presented to the emergency department with a febrile urinary tract infection. Workup revealed multiple urologic pathologies including a complete urethral duplication, a urachal cyst and solitary kidney with vesicoureteral reflux. At 3 months of age, he was taken to the operating room for a novel and minimally invasive treatment of his extremely rare condition involving laparoscopic ligation of the accessory duplicated urethra and removal of his urachal remnant.
First cystoscopy was completed and a sensor wire advanced into the bladder through the accessory urethra. The remainder of the case was completed laparoscopically, first removing the urachal remnant, taking down the bladder and dissecting off the accessory duplicated urethra. The accessory urethra was transected and the stump ligated. Follow up showed successful removal of both entities. He is doing well with plans for future removal of the penile aspect of his duplicated urethra.
Intended audience: Healthcare professionals and clinicians.
A 2 month old male presented to the emergency department with a febrile urinary tract infection. He was admitted to the hospital and treated with antibiotics. Pediatric urology was consulted after renal ultrasound demonstrated a congenital solitary right kidney with associated hydronephrosis. The patient improved clinically and was discharged on antibiotics with plans for future prophylaxis. Unfortunately, he returned to the emergency department the following day with erythema and drainage from his umbilicus. Abdominal ultrasound showed concern for a urachal remnant. He was subsequently discharged with antibiotics and outpatient follow-up with pediatric urology and pediatric surgery for further management. Workup has demonstrated multiple urologic pathologies, including a complete urethral duplication with associated hypospadius, a large urachal cyst, as well as a solitary right kidney with vesicoureteral reflux, and an element of chronic kidney disease. At 3 months of age, he was taken to the operating room by pediatric urology and surgery for a novel and minimally invasive treatment of his extremely rare condition involving laparoscopic ligation of the accessory duplicated urethra and removal of his urachal remnant. The patient was placed supine and preoperative antibiotics were given. Examination showed two urethra openings as shown, with an accessory urethra on the dorsal aspect of the glands and a hypospatic subcoronal ventral urethra. First, an 8 French pediatric cystoscope was advanced into the hypospadic ventral urethral meatus. Cystoscopic examination demonstrated a normal appearing penile urethra, viru montanum, and sphincter complex, as well as a normal appearing bladder neck. Pancystoscopy yielded a somewhat lateral and gaping right ureteral orifice, but no left ureteral orifice. Attempts were made to insert a catheter into the accessory dorsal urethra, but this would not pass. A 0.35 sensor wire was passed into the bladder. At this point, the cystoscope was removed from the ventral urethra. The sensor wire was left in the dwelling, and a Foley catheter was placed in the ventral urethra. A 3 millimeter incision was made in the left upper quadrant, and the abdomen insufflated with a varis needle. Additional port placement is as demonstrated. The large urachal cyst was dissected off the abdominal wall and carried down to the bladder, noting an obvious transition between the urachal remnant and bladder. At this point, the remnant was excised. There was a small opening in the bladder through which the laparoscope was driven. Both the accessory urethra containing the sensor wire and a normal appearing ventral urethra containing the Foley catheter were seen. The scope was then returned to the abdomen and the bladder further mobilized down to the bladder neck. The accessory dorsal urethra containing the wire was isolated and a plane was created between the two urethras. The dissection was challenging, but successfully completed using a combination of a 3 millimeter sealer, Maryland dissector, and sharp dissection. The accessory urethra was opened on its anterior surface where it was completely divided, taking care not to enter the normal urethra. After this, the bladder dome was closed with two layers of 30 barbed suture. Next, the proximal aspect of the accessory urethra was ligated with a looped PDS suture. An additional 30 vicral suture and looped PDS suture were placed to ensure the accessory urethra was completely ligated. The bladder was irrigated without evidence of leak from either the accessory urethral stump or the dome where the urachal remnant had been removed. A Jackson Pratt drain and Foley catheter were left, and all fascia and skin incisions were closed. Follow-up VCUG one week later showed no extravasation from his urethral stump or urechal remnant site. The patient continues to do well. He has had no further infections, his renal function is improving, and he is growing appropriately. We plan to return to the OR in the future for removal of the penile portion of his accessory urethra when he is older. In summary, this is a male infant who presented with a febrile UTI and was found to have multiple complex and rare congenital urologic anomalies. He underwent successful and novel treatment with laparoscopic ligation of the urethral duplication and removal of his urachal remnant.
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