Congenital anterior urethral diverticula with posterior urethral valve: rare combina- tion in a neonate: a case reportc

Congenital Anterior Urethral Diverticulum (CAUD) is defined as a cystic dilatation of the anterior urethra and is one of the rare causes of bladder outlet obstruction. The association of this urethral anomaly with the posterior urethral valve is extremely rare. In this case, we found the association of congenital urethral diverticulum and posterior urethral valve and we aimed to present it.


Introduction
Congenital Anterior Urethral Diverticulum (CAUD) is defined as a cystic dilatation of the anterior urethra and is one of the rare causes of bladder outlet obstruction.
Its embryological basis is discussed [1]. It is seen in the penile urethra in one third of cases. Two types have been defined radiologically: saccular and globular diverticula. Although there are cases with congenital anterior urethral diverticulum defined antenatally, the diagnosis is usually made postnatally. Diagnosis; It is determined by the history, clinical evaluation and radiological examinations. In this case, we aimed to report a rare combination of two anomalies associated with congenital anterior urethral diverticulum and posterior urethral valve [2].

Presentation of case
One-day-old male patient had bilateral hydronephrosis, predominantly in the left kidney, during prenatal follow-up. At the first postnatal examination after term and cesarean delivery, a swelling in the ventral penile skin was detected (Fig 1). In the urinary system ultrasonography performed at one day of age, right kidney dimensions were measured as 54 × 26 mm, grade 3-4 ectasia, and pelvis AP diameter 15 mm. Parenchyma thickness decreased and parenchyma ecogenicity grade 2 increased. Left kidney size was 59 × 28 mm, grade 4 ectasia, pelvis AP diameter was 15 mm. Bilateral ureters were dilated and tortuous. The bladder wall thickness was 9 mm, and the proximal urethra was dilated at the 2 cm segment and its diameter was 4.6 mm (Fig 2).
Diagnostic cystoscopy was performed at the age of 8 days and cystofix was applied to the patient who could not be probed via urethral route. Posterior urethral valve and anterior urethral diverticulum were visualized in cystoscopy. Due to the narrow and small urethra due to the patient's age and therefore not technically suitable for valve ablation, ablation was not performed and cystofix was used for bladder drainage. A grade 5 vesicoureteral reflux was detected in the left kidney in voiding cystourethrography performed by giving opaque cystofix. Bladder contours were irregular and diverticular filling excess was observed. Filling of the diverticulum with opaque was observed in the urethra (Fig 3). Static renal scintigraphy at the age of 2 months revealed normal size, localization, normal DMSA uptake and right kidney (100%) showing homogeneous material distribution and nonfunctioning left kidney (0%). Cystoscopy and valve resection were performed at the age of 3 weeks. At the age of four months, control cystoscopy was performed and ablation was performed for residual valve. Control cystoscopy performed before diverticulum excision showed no residual valve and then diverticulum excision and urethroplasty were performed (Fig 4).
After the spontaneous urination from the urethra after urethroplasty, cystofix was removed. In the control cystoscopy performed at the age of 19 months, it was observed that the urethra was in good. In the postoperative first year urinary system ultrasonography; A soft tissue area of 37 mm, which may belong to the kidney tissue, was observed in the left kidney. The parenchyma was greatly thinned. Right kidney dimensions were measured 60 × 27 mm.
The pelvicalyceal system in the right kidney is grade 2 ectasic. Pelvis Ap diameter measured 9 mm. The left ureter appears to be dilated with a diameter of approximately 9 mm before bladder entry. Simultaneously in static renal scintigraphy, it was observed that the left kidney was nonfunctioning (11%) and the right kidney showed normal size, normal DMSA uptake in localization and homogeneous material distribution (89%).
Postoperative first year control voiding cystourethrography showed left G5 vesicoureteral reflux. There is no reflux right side. Postvoiding urine was present. The urethra image was observed normally during the voiding phase ( Fig 5).
The expected bladder capacity was 70 ml and the bladder cystometric capacity was 70 ml in the urodynamics performed for the evaluation of bladder functions.

Results and discussion
In the development of the urethra, the formation of a continuous layer by joining and advancing urethral folds in the midline, from proximal to distal, is a multifactorial process. Any malfunction that may occur in this process may result in genital system development anomaly [3][4][5].
The Wolffian system appears as two elongated ducts. While it is associated with mesonephrons in the cranial, it opens to the urogenital sinus at the caudal.
At the end of the first month of gestation, the cloacal membrane, which will later form the urogenital system, It is reported that the treatment of congenital anterior urethral diverticulum is determined according to the size of the diverticulum and the degree of obstruction.
Small and asymptomatic diverticula can be followed conservatively.