Our experience with management of congenital urological pathologies in adulthood: What pediatric urologists should know and adult urologists adopt in pediatric practice experience

Abstract

Purpose: To summarize our experience in the management of congenital anomalies in the kidney and urinary tract (CAKUT) in adults.

Materials and methods: We conducted a retrospective chart review of all adult patients who underwent primary surgical intervention for CAKUT between 1998 and 2021.

Results: The study included 102 patients with a median age of 25 (interquartile range, 23-36.5). Of these, 85 (83.3%) patients reported normal prenatal ultrasound, and the remaining 17 (16.7%) patients were diagnosed with antenatal hydronephrosis. These patients were followed-up conservatively postnatally and were discharged from follow-up because of the absence of indications for surgical intervention or because they decided to leave medical care. All studied adult patients presented with the following pathologies: 67 ureteropelvic junction obstructions, 14 ectopic ureters, 9 ureteroceles, and 6 primary obstructive megaureters, and the remaining 6 patients were diagnosed with vesicoureteral reflux. Forty-three percent of the patients had poorly functioning moieties associated with ectopic ureters or ureteroceles. Notably, 67% of patients underwent pyeloplasty, 9% underwent endoscopic puncture of ureterocele, 3% underwent ureteral reimplantation, 6% underwent endoscopic correction of reflux, 7% underwent partial nephrectomy of non-functioning moiety, and the remaining 9% underwent robotic-assisted laparoscopic ureteroureterostomy. The median follow-up period after surgery was 33 months (interquartile range, 12-54). Post-operative complications occurred in 5 patients (Clavien-Dindo 1-2).

Conclusions: Patients with CAKUT present clinical symptoms later in life. Parents of patients diagnosed during fetal screening and treated conservatively should be aware of this possibility, and children should be appropriately counseled when they enter adolescence. Similar surgical skills and operative techniques used in the pediatric population may be applied to adults.

Keywords

Antenatal diagnosis / Congenital anomalies / Adults / Minimal invasive surgery

Acknowledgments

None.

Statement of ethics

The protocol for this research project was approved by a suitably constituted Ethics Committee of the institution (Shamir Medical Center) and conformed to the provisions of the Declaration of Helsinki (approval no. 0317-19-AS). The authors are accountable for all aspects of the work to ensure that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All people gave their informed consent prior to their inclusion in the study.

Conflict of interest statement

All authors have submitted the ICMJE uniform disclosure form. The authors declare no conflicts of interest.

Funding source

No funding was received for this study. All authors certify that they have no affiliations with or involvement in any organization or entity with any financial or non-financial interests in the subject matter or materials discussed in this manuscript

Author contributions

All authors have significantly contributed to the submitted manuscript. Specific contributions are as follows:

LC, AN, BC, SK: Conception and design, manuscript writing;

BBN, JJ: Administrative support, manuscript writing;

DK, OZS: Provision of study materials or patients, manuscript writing;

AZ, AM: Collection and assembly of data, manuscript writing;

GV, IK, AEN: Data analysis and interpretation, manuscript writing.

Data availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

[1] Halachmi S, Pillar G. Congenital urological anomalies diagnosed in adulthood—Management considerations. J Pediatr Urol 2008; 4(1):2-7.

[2] Chertin B, Pollack A, Koulikov D, et al. Conservative treatment of ureteropelvic junction obstruction in children with antenatal diagnosis of hydronephrosis: Lessons learned after 16 years of follow-up. Eur Urol 2006; 49(4):734-738.

[3] Chertin B, Rabinowitz R, Pollack A, et al. Does prenatal diagnosis influence the morbidity associated with left in situ nonfunctioning or poorly functioning renal moiety after endoscopic puncture of ureterocele? J Urol 2005; 173(4):1349-1352.

[4] Natsheh A, Shenfeld OZ, Farkas A, Chertin B. Endoscopic treatment of vesicoureteral reflux in an adult population: Can we teach our adult urology colleagues? J Pediatr Urol 2010;6:600-604.

[5] Shankar KR, Vishwanath N, Rickwood AM. Outcome of patients with prenatally detected duplex system ureterocele; Natural history of those managed expectantly. J Urol 2001; 165(4):1226-1228.

[6] Andrioli V, Guerra L, Keays M, et al. Active surveillance for antenatally detected ureteroceles: Predictors of success. J Pediatr Urol 2018; 14(3):243.e1-243.e6.

[7] Neheman A, Kord E, Koucherov S, et al. A novel surgical technique for obstructed megaureter: Robot-assisted laparoscopic dismembered extravesical cross-trigonal ureteral reimplantation-short-term assessment. J Endourol 2020; 34(3):249-254.

[8] Dothan D, Kocherov S, Jaber J, Chertin B. Endoscopic correction of reflux utilizing polyacrylate polyalcohol bulking copolymer (Vantris) as a tissue augmenting substance: Lessons learned over the 10 years of experience. J Laparoendosc Adv Surg Tech A 2021; 31(9):1073-1078.

[9] Chertin B, de Caluwé D, Puri P. Is primary endoscopic puncture of ureterocele a long-term effective procedure? J Pediatr Surg 2003; 38(1):116-119; discussion116-119.

[10] Herz D, Smith J, McLeod D, Schober M, Preece J, Merguerian P. Robot-assisted laparoscopic management of duplex renal anomaly: Comparison of surgical outcomes to traditional pure laparoscopic and open surgery. J Pediatr Urol 2016; 12(1):44.e1-44.e7.

[11] Dindo D, Demartines N, Clavien PA. Classification of surgical complications: A new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 2004; 240(2):205-213.

[12] Elbaset MA, Zahran MH, Sharaf MA, et al. Long term functional success after pyeloplasty for pelvi-ureteral junction obstruction in unilateral poorly functioning kidney in exclusively adults population. Urology 2019;131:234-239.

[13] Nascimento B, Andrade HS, Miranda EP, et al. Laparoscopic pyeloplasty as an alternative to nephrectomy in adults with poorly functioning kidneys due to ureteropelvic junction obstruction. Int Urol Nephrol 2021; 53(2):269-273.

[14] Freitas PFS, Barbosa JABA, Andrade HS, et al. Pyeloplasty in adults with ureteropelvic junction obstruction in poorly functioning kidneys: A systematic review. Urology 2021;156:e66-e73.

[15] Seibold J, Alloussi SH, Schilling D, et al. Minimally-invasive treatment of complicated ureteroceles in adults avoiding vesico-ureteric reflux. Cent Eur J Urol 2010; 63(2):74-76.

[16] Shah HN, Sodha H, Khandkar AA, Kharodawala S, Hegde SS, Bansal M. Endoscopic management of adult orthotopic ureterocele and associated calculi with holmium laser: Experience with 16 patients over 4 years and review of literature. J Endourol 2008; 22(3):489-496.

[17] Sahadev R, Rodriguez MV, Kawal T, et al. Upper or lower tract approach for duplex anomalies? A bi-institutional comparative analysis of robot-assisted approaches. J Robot Surg 2022; 16(6):1321-1328.

[18] Kawal T, Srinivasan AK, Talwar R, et al. Ipsilateral ureteroureterostomy: Does function of the obstructed moiety matter? J Pediatr Urol 2019; 15(1):50.e1-50.e6.

[19] Dothan D, Raisin G, Jaber J, Kocherov S, Chertin B. Learning curve of robotic-assisted laparoscopic pyeloplasty (RALP) in children: How to reach a level of excellence? J Robot Surg 2021; 15(1):93-97.

[20] Cohen S, Raisin G, Dothan D, Jaber J, Kocherov S, Chertin B. Robotic-assisted laparoscopic pyeloplasty (RALP), for ureteropelvic junction obstruction (UPJO), is an alternative to open pyeloplasty in the pediatric population. J Robot Surg 2022; 16(5):1117-1122.