1 SUBMITTED 27 MAY 21 1 REVISION REQ. 25 JUL 21; REVISION RECD. 12 AUG 21 2 ACCEPTED 9 SEP 21 3 ONLINE-FIRST: SEPTEMBER 2021 4 DOI: https://doi.org/10.18295/squmj.9.2021.138 5 6 Progressive Dilatation as a Successful Treatment for Y duplication of Urethra 7 A case report 8 *Akash Pati, Subrat K. Sahoo, Bikasha B. Tripathy 9 10 Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, 11 India 12 *Corresponding Author’s e-mail: patiakash.ap@gmail.com 13 14 Abstract 15 Duplication of urethra is a rare congenital anomaly that has been reported in case reports and 16 case series. A Y-shaped urethral duplication is the rarest variant as per the classification 17 suggested and hence lacks a standardized treatment option. We report a case of Y-duplication of 18 urethra diagnosed during neonatal age and presented to us at nine years of age. The patient had 19 undegone a vesicostomy at seventh day of life for passing urine per anus and was lost to follow 20 up thereafter. An attempt at disconnection of the duplicated urethral tract to anus after 21 colostomy, at eight years of age had failed. The patient was managed successfully by progressive 22 dilatation of the orthotopic urethra, which required multiple stages, followed by separation of the 23 urethra from the rectum. At three years follow up the patient is continent and asymptomatic. 24 Keywords: Y duplication of the urethra; PADUA technique; Rectourinary fistula; Urethral 25 duplication. 26 27 Introduction 28 Duplication of the urethra is a rare congenital anomaly of the lower urinary tract, occurring more 29 commonly in males. Multiple variants of duplication anomalies have been described for lower 30 urinary tract. Duplication of the urethra can occur along with the bladder or in isolation. When it 31 2 affects the urethra alone, it usually occurs in the sagittal plane on a single phallus. Several types 32 of anatomic variations in the duplicated urethra have been identified. The accessory urethra may 33 be incomplete or complete, opening in the midline at normotopic, episapdaic, hypospadiac or 34 perineoanal region. There is no standard treatment described for the condition as it is rare. The 35 progressive augmentation by dilating the urethra anterior (PADUA) technique was used 36 successfully for urethral hypoplasia,1 but promising results have not been described for its use in 37 “Y duplication” of urethra. We describe a case, where the PADUA technique has been used to 38 successfully manage the Y duplication of urethra and have discussed the possible reason for our 39 successful outcome. 40 41 Case Report 42 A nine-year-old male child presented to us with a cutaneous vesicostomy, and right transverse 43 loop colostomy done elsewhere. The child was passing urine per anus at birth, and was unable to 44 pass urine per urethra, for which a vesicostomy was done at seventh day of life. An 45 ultrasonogram of the abdomen done during neonatal age was suggestive of left ectopic kidney in 46 midline, sub umbilical position and healthy right kidney and bladder. Following the vesicostomy 47 the patient was lost to follow up. At eight years of age, the patient again presented to a surgeon 48 and had underwent a right transverse colostomy. As per the records, an attempted surgical 49 disconnection between the urethra and anal orifice through the perineal approach had failed. 50 51 At the time of presentation to us, the patient was passing most of the urine per vesicostomy and 52 was occasionally dribbling drops of urine per anus. A small pit-like opening was present on the 53 tip of the glans. The rest of the genitalia and abdominal examination was unremarkable. On 54 digital rectal examination, anal opening of the fistula was palpable anteriorly just inside the anal 55 verge. A functional nuclear imaging revealed a small ectopic kidney in the left lower abdomen, 56 contributing to 23% of total renal function, and there was no morphological or functional 57 abnormality in the right kidney. A voiding cystourethrogram done after blocking the 58 vesicostomy demonstrated a very thin caliber anterior urethra and a normal posterior urethra. The 59 urinary bladder was smooth in outline and of small volume with bilateral grade two 60 vesicoureteric reflux. During micturition, the dye was flowing into the rectum. The distal 61 colostogram was unremarkable. 62 3 63 Cystoscopy performed through the vesicostomy revealed a small capacity bladder with a smooth 64 wall. The left ureteric orifice was ectopic, opening medially on the trigone while the right 65 ureteric orifice was orthotopic. Bladder neck was regular, and scope could be negotiated through 66 the bladder neck into the posterior urethra. There was a fistulous opening seen in the posterior 67 wall of the posterior urethra distal to the veru. A standard 0.018” straight tip guidewire inserted 68 through the fistulous opening in the posterior urethra was retrieved from the anal canal. 69 70 There was a small pit at the tip of the glans resembling a stenotic meatus. A 0.018” straight 71 flexible tip guidewire introduced through this meatal pit and was retrieved at the stoma of the 72 vesicostomy under guidance of a cystoscope inserted through the vesicostomy. A well lubricated 73 three French gauge (Fr) ureteral catheter was inserted over the guidewire smoothly and was 74 retrieved through the vesicostomy. The catheter was left indwelling, with a plan for weekly 75 dilatation. Every week, after adequate lubrication, a progressively larger catheter was passed 76 gently over a guidewire and left indwelling as a ring stent. This mode of progressive dilation 77 continued for several weeks until a dilatation of 16 Fr was achieved. [Figure 1]. A voiding 78 cystourethrogram after blocking the vesicostomy [Figure 2 A] was performed which confirmed 79 good caliber of the orthotopic urethra. 80 81 The fistulous connection between the posterior urethra and anal canal was closed via a perineal 82 approach. The perineal body muscles were interposed between the anterior wall of the rectum 83 and the urethra to prevent a recurrence. The colostomy and vesicostomy were reversed after four 84 weeks. Postoperatively, the indwelling bladder catheter was kept for ten days and on removal, 85 the patient could pass urine per urethra. The patient was on clean intermittent self-catheterization 86 and a weekly calibration schedule until six months following vesicostomy closure. 87 88 A good urethral caliber was seen on voiding cystourethrogram, performed after closing the 89 vesicostomy and at six months follow up. [Figure 2B&C]. Uroflowmetry done immediately after 90 the vesicostomy closure had a flow rate of 5ml/sec which increased to 12 ml/sec at six months 91 follow-up and there was no post void residue. At three years follow up, the patient is continent 92 4 with a normal voiding stream. The parents of the patient provided informed consent for 93 publication of this case. 94 95 Discussion 96 Y duplication of the urethra occurs when the prostatic urethra splits into two channels with one 97 coursing to the glans, and the other more functional one extends ventrally to the perineal area 98 near the anus. Less than 50 cases of Y duplication of urethra have been described in English 99 literature.2 The orthotopic urethra is characteristically stenotic. This form of aberration is usually 100 associated with other congenital anomalies in the genitourinary and gastrointestinal tract. In the 101 present case described, there was an associated left ectopic kidney with bilateral vesicoureteric 102 reflux. 103 104 Being a rare condition there is no standardized method of treatment for the anomaly. Several 105 surgeons have mobilised the perineal urethra to the scrotal skin level and laid open the 106 orthotopic stenoptic urethra 3,4. At a second stage they have performed a urethroplasty. Singh and 107 Rawat have also performed a similar method, but they have completely excised the orthotopic 108 urethra and have tried to reconstruct the neourethra from locally available healthy tissue.5 Sinha 109 et al have tried to reinforce the urethra by strips of mucosa from the anterior anorectum.6 110 Passerini – Glazel et al have successfully managed the orthotopic urethral hypopalasia by 111 PADUA technique.1 The authors have reported a slow progressive dilatation of the hypoplastic 112 urethra to be successful in 6 out of 8 cases. We have employed the same method of gradual 113 progressive dilatation of anterior urethra in the case of Y duplication of the urethra. Some 114 authors do not agree with the PADUA technique of dilatation, as repeated trauma leads to 115 stricture formation and the hypoplastic urethra is not usable.5 Lima et al have reported, PADUA 116 technique, to be ineffective in two cases of Y duplication of urethra as both the cases have 117 developed urethral stenosis.7 118 119 The urethra is a distensible structure and probably remains hypoplastic because of the presence 120 of a low-pressure channel to the rectum. As a result, the anterior urethra is not challenged by the 121 voiding pressure and urinary stream and remains hypoplastic in cases with Y duplication. The 122 cause of failure of the PADUA technique, as mentioned by different authors can be summarized 123 5 as multiple injuries to the hypoplastic urethra resulting in stricture. A gentle progressive 124 dilatation is likely to have less trauma on the orthotopic urethra. Hence, a smooth progressive 125 dilatation will give a successful result without a stricture of the urethra. The procedure of 126 dilatation is such that the catheter is large enough to fill the lumen of the urethra but not forced.1 127 128 Conclusion 129 We could successfully manage the patient using the PADUA technique described for urethral 130 hypoplasia. The patient had a good urinary stream from the orthotopic meatus and had no post 131 void residue. Gentle progression of the dilatation was the key to success. However, more cases 132 of Y duplication of urethra should be managed by slow progressive dilatation of the hypoplastic 133 orthotopic urethra before any definite conclusion is formulated. 134 135 Authors’ Contribution 136 AP conceptualized the report. AP, SKS and BBT were involved in data collection and 137 acquisition, preparation of the original manuscript and revision of the manuscript. All authors 138 approved the final version of the manuscript. 139 140 References 141 1. Passerini-Glazel G, Araguna F, Chiozza L, Artibani W, Rabinowitz R, Firlit CF. (1998)The 142 P.A.D.U.A. (Progressive Augmentation by Dilating the Urethra Anterior) Procedure for the 143 Treatment of Severe Urethral Hypoplasia. Journal of Urology. 140(5 Part 2):1247–9. 144 2. Wagner JR, Carr MC, Bauer SB, et al. Congenital posterior urethral perineal fistulae: a unique 145 form of urethral duplication. Urology 1996; 48:277 - 80. 146 3. Podesta Miguel L., Medel Ricardo, Castera Roberto, Duarte Adolfo C. (1998)Urethral 147 duplication in children: surgical treatment and results. Journal of Urology. 160(5):1830–3. 148 4. Wakhlu A, Wakhlu AK. (1998)Management of H-type urethral duplication. Journal of 149 Pediatric Surgery. 33(8):1238–42. 150 5. Singh S, Rawat J. Y-type urethral duplication in children: Management strategy at our 151 center.(2013) J Indian Assoc Pediatr Surg. 18(3):100–4. 152 6 6. Sinha S, Sen S, Chacko J, Thomas G, Karl S, Mathai J. (2006)Y-duplication of the male 153 urethra: use of anterior anorectal wall for posterior urethral lengthening. Ped Surgery Int. 154 22(6):529–31. 155 7. M. Lima, F. Destro, M. Maffi, D. Persichetti Proietti, G. Ruggeri. (2017)Practical and 156 functional classification of the double urethra: A variable, complex and fascinating 157 malformation observed in 20 patients. Journal of Pediatric Urology, 13(1): 42.e1-42.e7 158 159 160 Figure1: Ring stent (Black Arrow) of 16 Fr kept indwelling passing through orthotopic 161 urethra and exiting through vesicostomy. 162 7 163 Figure 2: Voiding cystourthrogram done after blocking the vesicostomy (A), immediately 164 after closing the vesicostomy(B) and at 6 months follow up (C). 165