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Ectopic kidneys, with a prevalence of 1/1000-4000, often manifest in the pelvic region, can be complicated with problems such as ureteropelvic junction (UPJ) obstruction. This video article presents a case of open pyeloplasty in a 15-month-old infant with a pelvic kidney, emphasizing technical details for educational purposes. The patient was prenatally diagnosed with pelvic kidney hydronephrosis at 22 weeks of gestation, progressing to grade 4 postnatally. MAG-3 scintigraphy confirmed UPJ obstruction, warranting open pyeloplasty. A Pfannenstiel incision provided access to the Retzius space. The ureter was dissected, revealing the adhered renal pelvis. Stay sutures facilitated dissection, and 5/0 polyglactin sutures were strategically placed due to anatomical anomalies. Ureteropelvic anastomosis was performed using 6/0 PDS sutures. A 3-Fr Double J catheter preceded the closure of the renal pelvis. The procedure was concluded with meticulous layer closure. The operation lasted for 50 min, with minimal blood loss (10 mL). Drain was removed on postoperative day 2, and the patient was discharged. Ureteral stent was removed at 4 weeks. A 3-month follow-up ultrasound revealed a notable reduction in hydronephrosis, with an anteroposterior diameter of 6 mm. This video article elucidates the nuances of open pyeloplasty in pelvic kidneys and serves as a valuable resource for residents and fellows. The concise procedure, with a brief operative time and minimal blood loss, indicates the efficacy of the surgery. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to determine whether patients with isolated microtia or aural atresia have an increased prevalence of renal or cervical vertebral anomalies. Design: The study design was a retrospective medical record review. Setting: The setting was the following four distinct institutions: an urban tertiary care children’s hospital, two urban academic medical centers, and a staff-model health maintenance organization. Participants: Patients diagnosed with microtia, aural atresia, or oculoauriculovertebral spectrum were identified. Patients with facial asymmetry, craniofacial microsomia, and other craniofacial abnormalities or syndromes were excluded. Main Outcome Measures: Main outcome measures were the number of patients with isolated microtia or aural atresia who underwent a renal ultrasound or cervical spine X-ray, the results of those studies, and further evaluation or treatment for any abnormalities found. Statistical Analysis: A binomial analysis using a one-sided 95% confidence level was performed. Results: A total of 514 patients with isolated microtia and/or aural atresia were identified. Of these patients, 145 (28%) had undergone a renal ultrasound and 81 (16%) had undergone cervical spine X-rays. A total of 3 patients (2%) had minimal renal pelviectasis, all of which had resolved on repeat ultrasound and required no treatment. There were no structural renal abnormalities identified, and there were no cervical spine abnormalities identified. Conclusions: The data suggest that there is no increased prevalence of structural renal or cervical vertebral anomalies in patients with isolated microtia and/or aural atresia. Therefore, these patients do not require routine screening renal ultrasound or cervical spine X-rays. [ABSTRACT FROM AUTHOR]

Background: Joubert Syndrome (JS) is a rare genetic developmental disorder, first identified in 1969. In patients with JS, certain regions of the brain (mainly cerebellar vermis and brainstem) are underdeveloped or malformed. This can lead to impaired attention, visual, spatial, motor, language and social functional skills. JS is characterized by a host of features, many of which do not occur in every patient. Aim of the Study: To spotlight and increase awareness of clinical profile and neuroimaging findings of children with Joubert syndrome. Methods: This is a retrospective case series study of patients with JS who attended the Pediatric Neurology Clinic in Aladan and Alfarawanya Hospitals in Kuwait, from September 2007 to September 2012. Clinical and radiological data were obtained from the patient medical records. Results: Cerebellar vermis hypoplasia/aplasia and apnea were present in all patients, polydactly in 3 of 16, renal problems with cysts in 5 patients and 11 of 16 had abnormal electroretinograms (ERGs). Blood investigations of organic acids, amino acids and very-long-chain fatty acid, were normal in the all the nine patients. Conclusion: JS is a rare genetic brain malformation with association of retinal dystrophy and renal abnormalities. The retinal dystrophy may be progressive. The prognosis of patients depends mainly on the degree of brain malformation. [ABSTRACT FROM AUTHOR]

Objective: This prospective study was designed to detect the role of magnetic resonance imaging (MRI) in refining the diagnosis of suspected fetal renal anomalies detected during screening sonography. Material and Methods: 54 pregnant women, with suspected fetal renal anomalies detected during routine ultrasound screening, were rescanned by MRI to refine the diagnosis of the suspected renal anomalies. The pregnancy outcome was examined externally and by postnatal ultrasonography. Results: Fifty-four cases of suspected renal anomalies detected during screening sonography of 8400 pregnant women (0.6%), were rescanned by MRI in this study. The MRI gave a similar diagnosis to postnatal ultrasound in 46 cases (16 cases of hydronephrosis, 14 cases of Polycystic Kidney Disease (PCKD), 9 cases of Multicystic Kidney Disease (MCKD), 2 cases of Renal Agensis (RA), 3 cases of single renal cyst and 2 cases of megacystis+hydroureter), while it gave a different diagnosis (false positive) in 6 cases (4 cases of hydronephrosis diagnosed by MRI confirmed to be PCKD by postnatal ultrasound, also, 1 case of MCKD diagnosed by MRI confirmed to be hydronephrosis by postnatal ultrasound and 1 case of RA diagnosed by MRI confirmed to be normal by postnatal ultrasound). The prenatal ultrasound gave a similar diagnosis to postnatal ultrasound in 43 cases (14 cases of hydronephrosis, 13 case of PCKD, 9 cases of MCKD, 2 cases of RA, 3 cases of single renal cyst and 2 case of megacystis+hydroureter), while it gave a different diagnosis (false positive) in 9 cases; 4 cases of hydronephrosis diagnosed by prenatal sonography confirmed to be PCKD by postnatal ultrasound, one case of PCKD+one case of MCKD, and one case of megacystis+hydroureter confirmed to be hydronephrosis by postnatal ultrasound, while one case of MCKD diagnosed by prenatal sonography was confirmed to be PCKD by postnatal ultrasound and one case of RA diagnosed by prenatal ultrasound was confirmed to be normal by postnatal ultrasound. Conclusion: The MRI can be used as a complementary adjunctive modality with excellent tissue contrast, especially in equivocal cases or inconclusive sonographic findings. [ABSTRACT FROM AUTHOR]

Beckwith-Wiedemann syndrome (BWS), an overgrowth disorder with several congenital abnormalities, encompasses nephrourological anomalies. The objective of the report is to analyze the latter and related genotype-phenotype correlations. The study was a retrospective review of nephrourological investigations and genotype in 67 BWS patients. Imaging and laboratory studies have been correlated with the molecular anomalies typical of BWS. Thirty-eight (56.7%) patients had a total of 61 nonmalignant nephrourological findings, including nephromegaly ( n = 24), collecting system abnormalities ( n = 14), cryptorchidism ( n = 11), nephrolithiasis ( n = 5), cysts ( n = 5), and dysplasia ( n = 1). Four patients had Wilms' tumor, all associated with renal hyperplasia. Renal findings were almost consistent in the BWS group, with nephromegaly in all patients and collecting system abnormalities in half of them. BWS and negative patients also had frequent anomalies (63.6% and 61.9% respectively), whereas only 36.0% of BWS had renal findings ( p = 0.003). Cryptorchidism was associated with abdominal wall defects ( p < 0.001) appearing more frequently in BWS ( p = 0.028). Urinary tract infections were observed in 17.9% of patients, with two resulting in life-threatening sepsis. Hypercalciuria was present in 10% of cases. 55.5% of BWS patients have renal findings. Although variegate, these anomalies disclose a genotype-phenotype correlation. [ABSTRACT FROM AUTHOR]

Objective: To document the types of congenital renal anomalies detected in adulthood, the clinical presentation and complications of these renal anomalies, and the most useful imaging modality in detecting a renal anomaly. Materials and methods: This study was approved by the institutional review board and informed consent was waived. Between January 2007 and January 2011, the clinical data and imaging studies of 28 patients older than 18 years diagnosed with renal anomaly at the authors' institution were retrospectively reviewed. Renal anomalies in this study included only those with abnormality in position and in form. Results: Of these 28 patients, 22 underwent imaging studies and their results constituted the material of this study. Of the 22 patients, 14 had horseshoe kidneys (HSK), four had crossed renal ectopia and four had malrotation. Sixteen patients were men and six were women. The patients ranged in age from 19 to 74 years (mean age 51.1 years). Clinical presentations were abdominal pain (13), fever (13), haematuria (4), palpable mass (2), asymptomatic (2), polyuria (1) dysuria (1), blurred vision (1), and headache with weakness of left extremities (1). Imaging studies included abdominal radiograph (15), intravenous pyelography (IVP) (8), retrograde pyelography (RP) (4), ultrasonography (US) (7), and computed tomography (CT) (9). Associated complications included urinary tract stones (17), urinary tract infection (16), hydronephrosis (12), and tumours (2). Abdominal radiograph suggested renal anomalies in nine out of 15 studies. IVP, RP, US and CT suggested anomalies in all patients who had these studies performed. However, CT was the best imaging modality to evaluate anatomy, function and complications of patients with renal anomalies. Conclusion: HSK was the most common renal anomaly, with abdominal pain and fever being the most common presentations. UTI and stones were the most common complications. IVP, RP, US and CT can be used to diagnose renal anomalies but CT is the best imaging modality to evaluate renal anatomy, function and its complications. [ABSTRACT FROM AUTHOR]

Abstract: There is a wide range of congenital renal anomalies that can present in adulthood. These include unilateral agenesis, ectopia, as well as malrotation and fusion anomalies. These may have clinically relevant associated anomalies. Alternatively, these may present due to related sequela of urinary stasis or malposition. We will discuss the imaging findings of a spectrum of congenital renal anomalies presenting in adults. [Copyright &y& Elsevier]