Table 1 Summary of the clinical presentation, investigations, management, and follow-up of the three cases with abnormal communication between the lower urinary tract and the anus (the so-called Y-type urethral duplication)

Case 1

- Urine flow through the anus; few drops through penile urethra (Fig. 1a)

- Recurrent epididymo-orchitis

- Slight anterior anal misplacement

- Congenital deafness

- Bilateral mild hydroureteronephrosis (more on left)

- Increased wall thickness of urinary bladder

- Bilateral cortical scaring in DMSA renal isotopic scan

- Non uniform bladder contour (pear-shaped)

- Abnormal accessory channel communicating between urinary bladder and anus (Fig. 1d)

- Hypoplastic anterior urethra; patent posterior urethra

- Bilateral vesico-ureteric reflux (grade III)

Penile corporeal dysgenesis: penis is formed of a single corporeal body

- Trial of dilatation of hypoplastic penile urethra (PADUA)→failed

- Permanent supra-vesical port for CIC (Mitrofanoff)

- Separation and transfer of distal end of posterior accessory channel from the anus to the perineum anteriorly

- Recurrent stricture of perineal meatus that required reoperation twice

- Follow-up duration: 10 years

- The patient passes urine through perineal meatus, yet we opt for CIC (supra-vesical port) to ensure adequate bladder drainage

- The patient is kept on prophylactic antibiotic and anticholinergic drugs to control recurrent UTIs

Case 2

- Urine flow through the anus; few drops through penile urethra (Fig. 1b)

- Marked bilateral hydroureteronephrosis

- Left kidney is small in size with poor corticomedullary differentiation and thinned parenchyma

- Increased wall thickness of urinary bladder

- Bilateral cortical scaring in DMSA renal isotopic scan

- Non uniform bladder contour with posterior para-ureteric diverticulae (hutch diverticulae)

- Abnormal posterior channel communicating between posterior urethra and anus (Fig. 1e)

- Hypoplastic irregular anterior urethra; patent posterior urethra

- Bilateral vesico-ureteric reflux (grade VI)

Although the penis was formed of three corporeal bodies, yet it showed some degree of disorganization (irregularities and interruption of corporeal bodies)

- Initially, suprapubic cystostomy at presentation

- Ligation and excision of accessory posterior channel + perineal urethrotomy using the distal patent end of orthotopic posterior urethra (1st stage)

- Ureteric reimplantation + Permanent supra-vesical port for CIC

- Reconstruction of anterior urethra using inner preputial island tube (2nd stage)

- Anastomosing the reconstructed anterior urethra to the perineal urethrotomy (3rd stage)

- Follow-up duration: 6 years

- Weak urinary stream through reconstructed neo-urethra (meatus is distal penile)

- Patient is kept on regular CIC (supra-vesical port) to ensure adequate bladder drainage and protect upper tract

Case 3

- Urine flow through abnormal orifice on the anterior margin of the anus; few drops through penile urethra (Fig. 1c)

- Slight anterior anal misplacement + constipation

- Normal renal ultrasound

- Normal renal scan

- Abnormal posterior channel communicating between posterior urethra and anus

- Hypoplastic penile urethra; patent posterior urethra

Penile corporeal dysgenesis: penis is formed of a single corporeal body (Fig. 2)

- After counseling with parents, we decided not to perform reconstruction of the lower urinary tract as far as the urinary system is in equilibrium

- Instead of circumcision, a dorsal vertical split of the prepuce (preputeoplasty) was performed to spare preputial tissues for possible reconstruction of anterior urethra (if the patient or parents would change their mind)

- Cut-back anoplasty for constipation

- Follow-up duration: 12 months

- He is urinating through ectopic perineal orifice on anterior anal margin

- Close follow-up to ensure adequate bladder evacuation

- The option of shifting to a permanent supra-vesical port for CIC is explained to parents if turns to be necessary