Congenital ureteropelvic junction (UPJ) obstruction is a well recognized pathology. It typically occurs due to the presence of an aperistaltic segment of the ureter, very frequently aggravated by a strangling vessel defined by different authors as "anomalous", "aberrant" or "crossing". An embryologic review of the development of the kidney, ureter and renal vessels reveals that such definitions are etiologically inadequate since such vessels almost always run anterior to the UPJ and therefore in reality are not anomalous or aberrant. It would be, therefore, more appropriate to speak of a "vascular bar" rather that congenital vascular anomaly. A series of 23 patients treated by a laparoscopic technique (15 using the Anderson-Hynes pyeloplasty, 4 Fenger's technique, 3 a simple ureterolysis, 1 necessitating a conversion to open procedure (due to bleeding) are presented. An inferior polar vessel was found in 15 cases and this was running anterior to the UPJ in 14. The mean operative time was 235 min., while the mean hospital stay 7 (3-15) days. The mean follow-up was 47.7 months. Twenty-one pts. (95.5%) were symptom-free with imaging studies confirming relief from obstruction.
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J Pediatr Urol
December 2024
Department of Urology, University of Iowa, Iowa City, IA, 52242, USA. Electronic address:
Introduction: Prior analysis of children with grade 3 and 4 congenital hydronephrosis demonstrated that renal medullary pyramidal thickness (PT) is predictive of subsequent pyeloplasty (area under the curve [AUC] = 0.78). The objective of this study was to further analyze the utility of sonographic measurements including PT, anteroposterior pelvic diameter (APD), and renal length with an expansion of the number of infants with hydronephrotic kidneys including grades 2, 3, and 4 hydronephrosis.
View Article and Find Full Text PDFJ Med Genet
December 2024
John T. Macdonald Foundation Department of Human Genetics, University of Miami Miller School of Medicine, Miami, Florida, USA
Introduction: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder classically associated with multiple basal cell carcinomas, odontogenic keratocysts and skeletal anomalies. However, its significant phenotypic heterogeneity often delays the diagnosis. Here, we undertake the first comprehensive characterisation of NBCCS and congenital urinary tract anomalies.
View Article and Find Full Text PDFJ Pediatr Urol
November 2024
Department of Urology, Ankara City Hospital, Ankara, Türkiye. Electronic address:
Background: The study aimed at evaluating the role of thiol-disulphide balance parameters [native thiol (SH), total thiol (SH + SS), disulphide (SS), disulphide/native thiol ratio (% SS/SH), disulphide/total thiol ratio (% SS/Total Thiol) and native thiol/total thiol ratio (%SH/Total Thiol)], which are important oxidative stress markers in the congenital ureteropelvic junction (UPJ) stenosis, in the diagnosis of the disease, and its role in determining the need for surgery and follow-up.
Materials And Methods: This prospective study included 30 children diagnosed with congenital intrinsic ureteropelvic junction obstruction (UPJO) and a control group of 30 healthy children admitted to the pediatric clinic between January 2016 and February 2017. The children with UPJO underwent laparoscopic dismembered pyeloplasty.
Prenatal diagnosis of left multicystic dysplastic kidney (MCDK) and ureterocele was made in a male fetus at 24-week gestation. The initial postnatal course was uneventful. At 4 weeks of age, he developed urosepsis and respiratory distress.
View Article and Find Full Text PDFEur J Hum Genet
January 2025
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
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