Congenital Obstructive Megaureter Research Articles

Telocytes, c-Kit positive cells, Smooth muscles, and collagen in the ureter of pediatric patients with congenital primary obstructive megaureter: elucidation of etiopathology

ABSTRACT Congenital primary obstructive megaureter (POM) is an uncommon pediatric anomaly that is due to obstructive distal ureter leading to the loss of peristalsis with consequent ureterohydronephrosis causing loss of kidney function. The objectives are to elucidate the etiology of POM by demonstrating the presence of interstitial cells of Cajal (ICC), telocytes, smooth muscles, and collage in the obstructive and dilated ureteral segments. The study was carried out on 15 surgical specimens of congenital POM in pediatric patients, age range was 4 to 24 months, they were operated upon by excision of the obstructed segment, tailoring the dilated ureter, and anastomosing it to the bladder. Specimens included the obstructed ureteral segment and part of the dilated ureter. Specimens were examined with hematoxylin and eosin (H&E) stain, Modified Gomori trichrome stain, immunohistochemistry (IHC) with α-muscle actin, and c-kit (CD117), and transmission electron microscopy (TEM). Obstructed segment showed excess collagen intervening between smooth muscles, excess c-Kit positive cells, and presence of telocytes. The dilated segment of the ureteral wall is formed of smooth muscle bundles with scanty collagen. Staining with c-Kit did not demonstrate positive cells. TEM showed myofibroblasts and close adherence of smooth muscle cells to each other with absence of telocytes. The pathophysiology of POM is multifactorial. Loss of interstitial cells and rarity of collagen result in loss of elasticity of dilated segment leading to massive dilatation. While the obstructed segment had no muscle conductivity due to excess collagen irrespective of presence of telocytes.

Endoscopic balloon dilatation of primary obstructive megaureter: method standardization and predictive prognostic factors.

The management of congenital primary obstructive megaureter (POM) is usually conservative, especially during the first year of life. Endoscopic high-pressure balloon dilatation (EHPBD) is indicated when symptoms, increasing dilatation and progressive renal damage are recorded, particularly in children younger than one year of age. We identified and described endoscopic prognostic factors predicting the success or failure of endoscopic dilatation. Thirty-eight patients (33 M;5 F) with POM from 2005-2018 were included. Diagnosis was based on US distal ureter dilatation (>7 mm), obstructive MAG-3 diuretic renogram and absence of vesicoureteral reflux (cystography). 24 patients were under 1 year of age. All patients underwent cystoscopy and high-pressure balloon dilatation with 3,5 Fr dilating balloon, after ascending pyelography. Median follow-up was of 6.5 years. We identified characteristics with poor prognosis: stenotic punctiform ureteral ostium and/or ostium located in a bladder diverticulum (9 pts) and stenotic tract longer than 1 cm (5 pts). The patients with a stenotic tract shorter than 1 cm (18 pts) were divided into two groups: <5 mm (5 pts) and between 5 and 10 mm (13 pts) showed a good response to dilatation. Endoscopic evaluation of ureteral ostium with pneumatic dilatation when possible is a useful diagnostic and therapeutic solution for POM treatment, especially under one year of age. EHPBD is effective in short stenotic tracts (<5 mm). It may also be repeated with good results in intermediate stenotic sections (5 mm-1 cm). According to our preliminary results, the procedure is more effective if performed earlier (3-7 months of life). Greater cohort and longer follow-up are needed to verify the stability of these results.

Dilatação Endoscópica de Balão para Tratamento de Megaureter Obstrutivo Primário: Experiência de um Centro

Introdução: O megauretero obstrutivo congénito pode ser tratado por dilatação endoscópica com balão, especialmente nas crianças com menos de um ano de idade. Este trabalho revela a nossa experiência num período de seis anos.&#x0D; Doentes e métodos: Todos os doentes com o diagnóstico de megauretero obstrutivo congénito submetidos a dilatação endoscópica com balão foram incluídos, no período de tempo compreendido entre 2009 e 2014 (seis anos). O diagnóstico baseou-se na identificação de dilatação do uretero distal superior a 7 mm, padrão obstrutivo no renograma MAG3 e ausência de refluxo vesico-ureteral. Após o diagnóstico, adoptou-se uma atitude conservadora com início de profilaxia antibiótica e vigilância ecográfica em todos os doentes. A indicação cirúrgica resultou de um conjunto de achados clínicos, ecográficos e renográficos. Sob anestesia geral, e após pielografia retrógrada, foi realizada dilatação endoscópica da junção uretero-vesical sob visão directa e controlo fluoroscópico até ao desaparecimento do anel estenótico, colocando-se no final um stent duplo J. O seguimento foi feito com controlo ecográfico e renograma. O sucesso da intervenção cirúrgica foi definido como uma melhoria no grau de hidroureteronefrose (pelo menos 2 graus).&#x0D; Resultados: No total, 9 doentes foram intervencionados num só uretero, duas meninas e sete meninos, com idade média de 7.6 meses (entre 1-14) na data da cirurgia. Cinco foram no lado esquerdo e quatro no lado direito. Todos os doentes tinham diagnóstico pré-natal de hidroureteronefrose. Nenhum doente foi perdido no seguimento (média 46.7 meses). Todos tinham hidroureteronefrose de grau superior ou igual a 3 e padrão obstrutivo no renograma MAG3. A função relativa média do rim homolateral foi 46.2% (entre 40-53%). A principal indicação cirúrgica foi o agravamento da hidroureteronefrose durante a vigilância ecográfica. Não existiram complicações intra-operatórias. A ecografia mostrou melhoria da hidroureteronefrose em 6 doentes (66.7%). Três doentes foram re-implantados (33.3%) A função relativa média após a cirurgia foi 47.4% (entre 41-53%). Na consulta de seguimento mais recente, todos os doentes estavam assintomáticos.&#x0D; Discussão: A dilatação endoscópica com balão é uma opção na abordagem do megauretero obstrutivo primário com indicação operatória e pode ser considerado como tratamento de primeira linha nas crianças com menos de um ano de idade.

Pregnancy and foetal outcomes following anti-tumor necrosis factor alpha therapy: A prospective multicentre study

ObjectiveAs many inflammatory rheumatic diseases affect patients in childbearing age, some concern has been expressed about the safety of biologic drugs during pregnancy. This study evaluated the effects of anti-tumor necrosis factor alpha (TNFα) agents on pregnancy/foetal outcomes. MethodsThirty-eight pregnancies were followed prospectively from November 2008 to February 2015. Information about the patients’ exposure to anti-TNFα, disease activity, DMARD therapy, pregnancy/foetal outcomes were registered. ResultsTwenty-four/38 (71.1%) pregnancies were exposed to anti-TNFα at conception/I trimester, 11/38 (28.9%) prior to conception and 3 (11.1%) following paternal exposure. There were two congenital malformations: one infant (4.2%) was diagnosed with congenital diaphragmatic hernia and obstructive megaureter; the mother was exposed to adalimumab at conception/I trimester. While one foetus (9.1%) showed a trisomy 16, the mother 38 year-old had suspended etanercept 4 weeks before conception. There was no significant difference in pregnancy/foetal outcome between the two groups. Nor were there any significant differences in pregnancy/foetal outcomes in the various groups being treated with different anti-TNFα antagonists. No congenital malformations were found in connection to paternal exposure. ConclusionStudy results suggest that anti-TNFα drugs could be safe when administered during conception/I trimester and following paternal exposure.

Late results of ureteric reimplantations for congenital obstructive megaureter: Using the method developed by József Tóth

Late results of ureteric reimplantations for congenital obstructive megaureter: Using the method developed by József Tóth

Treatment of Symptomatic Primary Obstructive Megaureter by Laparoscopic Intracorporeal or Extracorporeal Ureteral Tapering and Ureteroneocystostomy: Experience on 11 Patients

To present our experience and the feasibility with laparoscopic ureteral reimplantation using ureteral tapering (intracorporeal or extracorporeal) for symptomatic primary obstructive megaureter. Between June 2005 and September 2010, 11 patients (mean age: 33.2 years) with symptomatic congenital primary obstructive megaureter underwent laparoscopic reconstruction. All patients underwent laparoscopic intracorporeal or extracorporeal ureteral tailoring and ureteroneocystostomy by the same surgical team. The relevant perioperative details and complications were recorded. No open conversions or blood transfusions were necessary. Total mean operative time was 142 minutes (range 109-227 min). The mean operative time for the five patients with intracorporeal tailoring was 154 minutes (range 121-227 min), compared with 125 minutes (range 109-165 min) for the six patients with extracorporeal tailoring. The mean blood loss was 45 mL (range 30-85 mL) for all cases. Mean postoperative hospital stay was 6.4 days (range 5-8 days). In one patient, urinary leakage was noted immediately postoperatively; it disappeared spontaneously with conservative treatment by postoperative day 7. Average follow-up was 18 months (range 13-24 mos). Follow-up renal ultrasonography and intravenous urography confirmed decreased hydronephrosis with good drainage. Nonobstructed clearance was also demonstrated using diuretic renography in all cases. Laparoscopic intracorporeal or extracorporeal ureteral tailoring and ureteroneocystostomy is a feasible and reproducible procedure. Follow-up revealed satisfactory objective and subjective outcomes.

Robotic Repair of Primary Symptomatic Obstructive Megaureter with Intracorporeal or Extracorporeal Ureteric Tapering and Ureteroneocystostomy

To describe the technique, feasibility, and effectiveness of robotic ureteric tapering (intra- or extracorporeal) and ureteroneocystostomy with and without ureteric stones retrieval in patients with symptomatic primary obstructive megaureter. Seven patients (one bilateral) (mean age: 28.3 years) with symptomatic or complicated congenital primary obstructive megaureter were considered for robot-assisted laparoscopic reconstruction. All surgical steps were performed purely robotically via transperitoneal access by single surgeon including ureteric reimplantation and retrieval of ureteral stones, except in two patients where ureteral tapering was done extracorporeally. The relevant perioperative details, complications, and functional outcomes were analyzed. Besides clinical follow-up, objective evaluation was done with diuretic renogram and intravenous urography. Total mean operative time and surgeon's console time were 142.5 and 127.5 minutes (range: 115-230 and 100-210), respectively, with an estimated blood loss of less than 50 mL. Mean analgesic requirement was 175 mg of diclofenac sodium and oral feeds were started after 12 hours (range: 7-16). Average hospital stay was 3.2 days (range: 2-6). Complications included one case of perioperative urinary tract infection. Average follow-up period was 16 months (range: 11-20). Follow-up ultrasonography and intravenous urography confirmed reduction of hydronephrosis and good drainage. The mean split renal function of the salvaged kidney was 41.2% at last follow-up when compared with preop average value of 41.3%. Robotic repair and removal of ureteric stones in primary symptomatic obstructive megaureter is safe, feasible, and effective with either intracorporeal or extracorporeal ureteric tapering. It has minimal perioperative morbidity and durable success as demonstrated with subjective and objective evaluation.

Is primary obstructive megaureter repair at risk for contralateral reflux?

Contralateral vesicoureteral reflux occurs after successful unilateral reflux repair in a significant proportion of patients without correlation to the surgical approach. Unilateral congenital obstructive megaureter was compared to primary vesicoureteral reflux with regard to the risk of onset of contralateral reflux after unilateral ureteral reimplantation. Unilateral congenital obstructive megaureter was diagnosed in 58 consecutive patients 2 to 10 years old (mean age 3.2). Cross-trigonal ureteroneocystostomy was performed in 57 cases and longitudinal ureteral reimplantation, according to the Politano-Leadbetter technique was done in 1. Longitudinal tapering according to Hendren was performed in 44 ureters, and the Kalicinski folding was used to repair 11 ureters. All patients underwent serial renal ultrasound, diethylenetetraminepentaacetic acid nuclear scan, excretory urogram and voiding cystourethrogram. The control group was composed of 98 age matched children with unilateral vesicoureteral reflux who underwent unilateral reimplantation with or without tapering. Fisher's exact test and Student's t test were used for statistical analysis. Followup ranged from 1 to 5 years. All patients in both groups underwent a voiding cystourethrogram at 6 months, and renal ultrasound at 3, 6 and 12 months postoperatively. Grade 2 reflux developed in 1 study group patient after contralateral Kalicinski ureteral folding and cross-trigonal reimplantation (1.7%). In the control group new onset contralateral reflux developed in 11 cases (11.2%). The difference was statistically significant (p <0.005, Fisher's exact test p = 0. 033). Ureteral reimplantation for unilateral congenital obstructive megaureter is not correlated with the development of contralateral reflux. The occurrence of contralateral reflux after successful unilateral reflux repair is high (11.2%), and is not correlated with age, sex and technique of reimplantation or tapering. These results support the hypothesis that the functional anatomy of the trigone is preserved in congenital obstructive megaureter but is impaired on both sides in cases of unilateral vesicoureteral reflux. The surgical management of unilateral primary vesicoureteral reflux and congenital obstructive megaureter should be differentiated based on these results.

Role of Prenatal Diagnosis in the Treatment of Congenital Obstructive Megaureter in a Solitary Kidney

Congenital megaureter in a solitary kidney (CMSK) is a life-threatening disease. From January 1988 to December 1993, we treated 9 patients for CMSK (2 cases with unilateral renal dysplasia and 7 with unilateral renal agenesis). In 7 cases, the malformation was detected by prenatal ultrasonography and urological counseling. The ages at first postnatal observation ranged from 24 h to 5 years (mean = 8 months). The postnatal urological workup included: serum renal function screening, urinary system ultrasonography, a micturition cystogram, intravenous pyelography and a nuclear renal scan. The therapy of choice was decided on the basis of renal function. One emergency divertive procedure was performed after birth, in 3 cases of renal failure in the first 2 weeks of life. The remaining cases were operated electively at an average age of 20 months. In 2 cases that underwent delayed surgical elective correction and in 1 of the 3 cases that underwent an emergency diversion, some degree of renal failure still persists. In our opinion, prenatal diagnosis, accurate postnatal urological workup and strict postoperative follow-up are fundamental to avoid the devastating consequences of CMSK on the affected child.

Neonatal congenital megaureters: trends in diagnosis and management

This report describes the last 16 cases of congenital primary obstructive megaureter (CPOM) encountered at our institution, whereby antenatal diagnosis was an important method of detection (13 of 16 cases). In most cases, no precise diagnosis was established in utero (2 of 13 infants). This lack of accuracy is of little importance, since all babies with suspected uropathies undergo a uroradiological work-up after birth (ultrasound, voiding cystogram, and urogram). Our series showed a marked male predominance (80%). CPOM showed a high potential for spontaneous resolution after birth (40%). Therapeutic decisions should be based mainly on studies of renal function (quantitative isotopic nephrogram with diuretics). Therefore, as long as renal function has not deteriorated during follow-up, conservative management should be elected first. Only cases showing clinical symptoms or decreased renal function should be treated surgically.

976 - Renal Function After Surgery for Congenital Obstructive Mega-Ureter - Long Term Follow-Up

976 - Renal Function After Surgery for Congenital Obstructive Mega-Ureter - Long Term Follow-Up

Not in Our Own Image: Educating the Pediatrician for Practice

As many inflammatory rheumatic diseases affect patients in childbearing age, some concern has been expressed about the safety of biologic drugs during pregnancy. This study evaluated the effects of anti-tumor necrosis factor alpha (TNFα) agents on pregnancy/foetal outcomes.Thirty-eight pregnancies were followed prospectively from November 2008 to February 2015. Information about the patients’ exposure to anti-TNFα, disease activity, DMARD therapy, pregnancy/foetal outcomes were registered.Twenty-four/38 (71.1%) pregnancies were exposed to anti-TNFα at conception/I trimester, 11/38 (28.9%) prior to conception and 3 (11.1%) following paternal exposure. There were two congenital malformations: one infant (4.2%) was diagnosed with congenital diaphragmatic hernia and obstructive megaureter; the mother was exposed to adalimumab at conception/I trimester. While one foetus (9.1%) showed a trisomy 16, the mother 38 year-old had suspended etanercept 4 weeks before conception. There was no significant difference in pregnancy/foetal outcome between the two groups. Nor were there any significant differences in pregnancy/foetal outcomes in the various groups being treated with different anti-TNFα antagonists. No congenital malformations were found in connection to paternal exposure.Study results suggest that anti-TNFα drugs could be safe when administered during conception/I trimester and following paternal exposure.