Dykie Adam, Katzman Philip, Chaturvedi Apeksha
Final Pr. ID: Poster #: EDU-037
Wilms tumors, also known as nephroblastomas, are overwhelmingly the most common renal tumors in children. While radiologists are abundantly familiar with the imaging appearances of these tumors, their knowledge of underlying histologic features, prognostic variables, and treatment approaches may not be equally robust. Histologically typically triphasic and containing differing proportions of blastemal, epithelial, and stromal components, the prognosis of individual subtypes differs. The “teratoid” variant of Wilms tumor contains components of differentiated tissues such as muscle, bone, cartilage, and fat. Focal and diffuse anaplasia are important histological features with diffuse anaplasia recognized as the most important prognostically unfavorable feature. In addition, there is an intermediate category of “nuclear unrest” which carries some but not all features of anaplasia. Clinical approaches differ between the two major renal tumor clinical research groups: the International Society of Paediatric Oncology (SIOP) Renal Tumor Study Group and the Children's Oncology Group (COG) Renal Tumor Group. This exhibit uses a case-based template of radiologic-pathologic correlation of different Wilms subtypes encountered at our institution, outlining treatment for individual tumor subtypes. We discuss differential diagnoses which may overlap with Wilms tumor on imaging, including nephrogenic rests, mesoblastic nephroma, malignant rhabdoid tumor of the kidney, clear cell sarcoma, and pediatric cystic nephroma (as a mimic of cystic Wilms tumor). We review Wilms tumor classification with known syndromic associations. We provide information on staging through the National Wilms Tumor Study system covering imaging and post-surgical findings. The ultimate goal is to make radiologists more effective members of the interdisciplinary tumor boards through enhancing a holistic understanding of Wilms tumor. Read More
Authors: Dykie Adam , Katzman Philip , Chaturvedi Apeksha
Keywords: Wilm's Tumor, Kidneys, Abdominal Imaging
Goldwasser Bernard, Kazmi Faaiza, Urbine Jacqueline, Poletto Erica, Malik Archana, Mallon Mary
Final Pr. ID: Poster #: EDU-048
There is a wide array of pathologies which can cause diffuse changes in the pediatric kidney. The purpose of the presentation is to show a multimodality pictorial review of characteristic findings of a wide variety of diffuse renal abnormalities in the pediatric patient. Read More
Authors: Goldwasser Bernard , Kazmi Faaiza , Urbine Jacqueline , Poletto Erica , Malik Archana , Mallon Mary
Keywords: renal mass, congenital renal abnormalities, syndromes affecting the kidney
Chew Lay Ee, Tan Timothy Shao Ern
Final Pr. ID: Poster #: SCI-046
Nutcracker syndrome (NCS) results from left renal vein (LRV) compression, typically between the abdominal aorta and superior mesenteric artery. The use of ultrasonography (US) to aid in diagnosis can be challenging due to the dynamic nature of the nutcracker phenomenon (NCP) and variability of symptoms, which can fluctuate with changes in patient posture and respiration. Moreover, defining a reliable Doppler threshold in children remains underexplored. This study explores the added utility of Doppler peak systolic velocity (PSV) ratio to LRV diameters in the evaluation of suspected NCS. Read More
Authors: Chew Lay Ee , Tan Timothy Shao Ern
Keywords: Nutcracker Syndrome, Doppler US, Kidneys
Heintzelman Briana, Khrichenko Dmitry, Carson Robert, Darge Kassa, Serai Suraj, Hartung Erum
Final Pr. ID: Paper #: 090
ARPKD causes diffuse microcysts in the kidney parenchyma, and cyst burden cannot be quantified by standard image segmentation methods. We sought to assess whether apparent diffusion coefficient (ADC) measured by DWI could serve as a non-invasive biomarker of ARPKD severity, with the hypothesis that ADC would be higher in cystic vs. non-cystic parenchyma due to higher extracellular water content. We examined whether ADC could distinguish healthy kidneys from those affected by ARPKD, and whether a threshold ADC value could be established to differentiate cystic vs. non-cystic parenchyma. Read More
Authors: Heintzelman Briana , Khrichenko Dmitry , Carson Robert , Darge Kassa , Serai Suraj , Hartung Erum
Heintzelman Briana, Khrichenko Dmitry, Carson Robert, Darge Kassa, Serai Suraj, Hartung Erum
Final Pr. ID: Poster #: SCI-017
ARPKD causes diffuse microcysts in the kidney parenchyma, and cyst burden cannot be quantified by standard image segmentation methods. We sought to assess whether apparent diffusion coefficient (ADC) measured by DWI could serve as a non-invasive biomarker of ARPKD severity, with the hypothesis that ADC would be higher in cystic vs. non-cystic parenchyma due to higher extracellular water content. We examined whether ADC could distinguish healthy kidneys from those affected by ARPKD, and whether a threshold ADC value could be established to differentiate cystic vs. non-cystic parenchyma. Read More
Authors: Heintzelman Briana , Khrichenko Dmitry , Carson Robert , Darge Kassa , Serai Suraj , Hartung Erum
Final Pr. ID: Poster #: EDU-063
Ultrasound is the current modality of choice in assessing appropriate renal growth in the pediatric population, and hence establishing accurate and reliable normograms is important. Many sonographic sets of normative data on renal length and normal range limits have been created since the early 1980’s. We evaluate the consistency of mean renal length and standard deviation between these normograms. In addition, we assess the potential role of CT/MR in evaluating renal growth. Read More
Authors: La Quan , Isaacson Jared
Keywords: kidney, size, ultrasound
Gibson Samantha, Koberlein George, Neville Kucera Jennifer
Final Pr. ID: Poster #: EDU-038
This review aims to provide a comprehensive visual guide to the imaging features of fetal genitourinary obstructive anomalies. The development of the fetal genitourinary tract is a complex process and, in turn, there are a variety of points in time when the tract may be at risk for the development of malformations. Early diagnosis is crucial for optimizing prenatal care, preventing oligohydramnios, and improving the prognosis and management of affected neonates. Read More
Authors: Gibson Samantha , Koberlein George , Neville Kucera Jennifer
Keywords: Kidneys, Bladder, Congenital
Ahn Ju Hee, Hook Marcus, Swenson Zachary, King Cody
Final Pr. ID: Poster #: EDU-037
Ultrasound is the most accessible and reliable modality to evaluate the kidneys in neonates and young infants. The sonographic appearance of the kidneys in the immediate postnatal period can differ significantly from that in older children and adults. In clinical practice, radiologists encounter a full spectrum of renal pathology, with some appearing as hyperechogenicity on ultrasound. The purpose of this case-based educational exhibit is to provide a review of normal and abnormal causes of increased echogenicity of the kidneys in neonates and young infants, and common sonographic pitfalls that can artificially increase renal echogenicity Read More
Authors: Ahn Ju Hee , Hook Marcus , Swenson Zachary , King Cody
Keywords: Ultrasound, Neonate, Kidney
Llorens-salvador Roberto, Veiga-canuto Diana, Ortega-lopez Pedro, Navarro Oscar
Final Pr. ID: Poster #: EDU-029
HNF1B nephropathy is a hereditary renal disease caused by mutations in the HNF1B gene, transmitted in an autosomal dominant manner but frequently occurring as de novo mutations. This genetic variability complicates diagnosis, as family history of renal disease may be absent. Clinical presentation is heterogeneous, with onset in both children and adults, and may include renal dysfunction, metabolic abnormalities, and in some cases early-onset diabetes.
In the pediatric setting, imaging plays a crucial role in raising suspicion of HNF1B nephropathy. Prenatal ultrasound and MRI may reveal echogenic kidneys or renal hypoplasia in the third trimester, while postnatal ultrasound may show variable findings such as increased renal echogenicity, renal cysts, and reduced renal size. These abnormalities can mimic more common entities, highlighting the importance of a systematic imaging approach.
This educational exhibit reviews the prenatal and postnatal imaging findings of HNF1B nephropathy in children emphasizing key imaging clues that should raise diagnostic suspicion when encountering cystic or dysplastic renal patterns. The exhibit also includes discussion of the differential diagnosis with other conditions associated with renal cysts, such as autosomal dominant polycystic kidney disease (ADPKD), autosomal recessive polycystic kidney disease (ARPKD), multicystic renal dysplasia, Meckel–Gruber syndrome and other ciliopathies. Recognizing the imaging spectrum of HNF1B nephropathy is essential to avoid misclassification as nonspecific chronic interstitial nephropathy and to ensure timely genetic testing, counseling, and long-term multidisciplinary management.
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Authors: Llorens-salvador Roberto , Veiga-canuto Diana , Ortega-lopez Pedro , Navarro Oscar
Sosin Scott, Handler Marci, Murphy Robyn
Final Pr. ID: Poster #: EDU-050
Teaching Points
1. Review normal renal anatomy and normal renal and urinary imaging findings in the pediatric population.
2. Discuss the imaging characteristics of the different causes of congenital pediatric renal and urinary disorders, predominantly utilizing ultrasonography and fluoroscopy.
3. Learn the epidemiology, appropriate workup, and management of congenital pediatric renal and urinary disorders.
Introduction
There is a large spectrum of frequently encountered congenital renal and urinary disorders, ranging from incidental findings to severe, life-threatening pathologies. Recognizing the multi-modality imaging characteristics of these anomalies is an important tool in any radiologists’ arsenal. The purpose of this presentation is to perform a case-based imaging review of congenital renal and urinary disorders and discuss their etiologies and clinical importance. After completing this educational exhibit, the reader will be able to recognize the various causes and imaging features of congenital pediatric renal disorders. The following topics will be discussed:
Vesicoureteral reflux
Autosomal recessive polycystic disease (ARPKD)
Autosomal dominant polycystic disease (ADPKD)
Renal anomalies - agenesis/ectopia/horseshoe/duplicated
UPJ obstruction
Posterior urethral valves
Megaureter
Ureterocele
Multicystic dysplastic kidney
Mesoblastic nephroma
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Authors: Sosin Scott , Handler Marci , Murphy Robyn
El-jalbout Ramy, Coulombe Edouard
Final Pr. ID: Poster #: SCI-026
Chronic kidney disease (CKD) has a prevalence of 18% among adults born preterm. The standard diagnostic method is renal biopsy combined with Glomerular Filtration Rate (GFR) estimation. However, these methods are limited by their invasiveness and lack of sensitivity in the early stages. The lack of guidelines for assessing kidney function in young adults born prematurely highlights the need to develop screening methods for early CKD detection. The objectives are to evaluate feasibility and reproducibility of multiparametric MRI (MpMRI) sequences and explore associations with anthropometric and renal function. Read More
Authors: El-jalbout Ramy , Coulombe Edouard
Keywords: Kidneys, Multimodality, Predisposition
Matos Rojas Irma, Katekaru Tokeshi Doris
Final Pr. ID: Poster #: CR-06 (R)
OEIS complex is the most severe end of the expectrum of the exstrophy - epispadias complex . It is characterized by omphalocele, extrophy, imperforate anus and spinal defects and is often associated with other malformations on chest, abdomen genitourinary, skeletal and neurologic. The incidence of OEIS complex is very rare, estimated to occur in 0, 5 – 1 per 200 000 live births.
We present a case of OEIS complex associated with horseshoe kidney. The baby was delivered by vaginal delivery (GA 39 weeks). Present normal male kariotype.
On physical examination there were omphalocele with herniation of a segment of the large bowel, cloacal exstrophy with two hemi bladders, imperforate anus and spina bifida. No external genitalia were identified on physical examination but bilateral cryptorchidism was observed in pelvic MRI
Renal ultrasound show hoershoe kidney with left pelvic ectasia that was confirmed on abdominal MRI and urotomography. Spinal ultrasound and MRI show lipomyelomenigocele and low spinal cord insertion. Also the baby has sacral segmentation defects and congenital hip subluxation.
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Authors: Matos Rojas Irma , Katekaru Tokeshi Doris
Keywords: OEIS complex, horseshoe kidney, extrophy - epispadias complex, magnetic resonance imaging, ultrasound
Farhat Ahmed, Huang Pauline, Servaes Sabah, Hailemichael Eyassu
Final Pr. ID: Poster #: CR-020
A “pancake” kidney is a rare subtype of cross fused renal ectopia, in which there is both fusion of the upper and lower poles of the kidneys in the pelvis, resulting in a disc or cake shaped appearance of the fused kidneys. The cross fused renal ectopia is a rare congenital anomaly caused by abnormal growth of the ureteric bud and separation failure of metanephric tissues with an estimated incidence of 1 in 2000, with a male predominance. Patients are often asymptomatic, but urinary tract infections, ureteropelvic junction obstruction, vesicoureteric reflux, and renal stones can be associated with renal ectopia. We present a case of a 7 year old boy who initially presented to the ED with back pain and clinical concerns of hydronephrosis, with an incidental finding of a “pancake” kidney. Read More
Authors: Farhat Ahmed , Huang Pauline , Servaes Sabah , Hailemichael Eyassu
Keywords: Renal Ectopia, Pancake kidney
El-hayani Reem, Malik Ammara, Manickam Sivakumar
Final Pr. ID: Poster #: CR-023
Introduction:
Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive metabolic disorder caused by deficiency of 7-dehydrocholesterol reductase, leading to impaired cholesterol biosynthesis (Kelley & Hennekam, 2000). It has associations with multiple congenital anomalies, including craniofacial, cardiac, and genitourinary malformations. Renal abnormalities, including renal agenesis, affect around 25% of individuals with SLOS (Nowaczyk, 2020), yet little literature is available on how this manifests clinically.
Case:
We report a 6-month-old infant with a background of known SLOS admitted with vomiting, fever, and persistent electrolyte derangements despite IV fluids. Initial ultrasound of the urinary tract demonstrated increased echogenicity and moderate pelvicalyceal dilatation of the right kidney with mild ureteric dilatation. The left kidney was not visualised in the renal fossa, and a small elongated echogenic structure adjacent to the spleen was suggestive of an atrophic kidney. Follow-up ultrasound at 7 months confirmed absence of the left kidney, with compensatory hypertrophy of the right kidney and mild hydronephrosis. Serial chest radiographs revealed concurrent cardiomegaly and recurrent respiratory infections. The case was discussed in a multidisciplinary setting, and urology input confirmed the diagnosis of unilateral renal agenesis in the context of SLOS.
Discussion:
While renal anomalies such as hypoplasia, duplication, and vesicoureteric reflux have been reported in SLOS (Nowaczyk & Irons, 2012), more data is needed to explore how this may clinically present. This case highlights the importance of systematic renal evaluation in children with SLOS, particularly when presenting with acute kidney injury or electrolyte imbalance (Porter, 2008) as a result of unilateral or bilateral kidney agenesis. This report builds the case for early urological screening of all children with SLOS, allowing for early detection and longitudinal monitoring, which is essential for guiding management, anticipating complications, and counselling families.
References:
Kelley RI, Hennekam RC. Smith-Lemli-Opitz syndrome. J Med Genet. 2000;37(5):321–335.
Nowaczyk MJ, Wassif CA. Smith-Lemli-Opitz Syndrome. In: Adam MP, Feldman J, Mirzaa GM, et al., eds. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1998
Nowaczyk MJ, Irons MB. Smith-Lemli-Opitz syndrome: phenotype, natural history, and epidemiology. Am J Med Genet C Semin Med Genet. 2012
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Authors: El-hayani Reem , Malik Ammara , Manickam Sivakumar
Keywords: Kidneys, Genetics, Genitourinary
Gilligan Leah, Davenport Matthew, Trout Andrew, Su Weizhe, Zhang Bin, Goldstein Stuart, Dillman Jonathan
Final Pr. ID: Paper #: 089
Acute kidney injury (AKI) remains a concern in hospitalized children undergoing computed tomography (CT) examinations with intravenous (IV) iodinated contrast material (ICM). Adult studies have shown frequencies of AKI after CT with IV ICM to be similar to propensity score-matched ICM unexposed cohorts. The purpose of this study is to evaluate the association between IV ICM exposure and AKI in hospitalized pediatric patients with stable kidney function undergoing computed tomography (CT). Read More
Authors: Gilligan Leah , Davenport Matthew , Trout Andrew , Su Weizhe , Zhang Bin , Goldstein Stuart , Dillman Jonathan
Keywords: contrast induced nephropathy, acute kidney injury, iodinated contrast material
Final Pr. ID: Poster #: EDU-030
The purpose of these educational exhibit is to illustrate the tomographic evaluation of living renal donors for pediatric patients
Renal transplantation has become the treatment of choice for end stage renal disease in children. Living donor renal transplantation is a better option for recipient and graft survival in comparison with cadaveric donor. Laparoscopic nephrectomy is preferred for removing the kidney from living donors, therefore the surgeons need accurate anatomic information
We present an approach for the evaluation of living renal donors by tomography. Selected cases illustrating the evaluation of renal parenchyma, renal arteries, renal veins and collecting system are shown, including the key points of anatomic information that are valuable for the surgeons
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Authors: Valdez Quintana Melissa
Keywords: kidney transplant, CT, donor
Final Pr. ID: Poster #: EDU-028
In a child the abdominal organs are more at risk from blunt injury, the reasons for this include the fact that the organs are relatively more anterior and inferior and lie inferior to the ribs rather than behind them as in an adult. Additionally, paediatric ribs are cartilaginous and so although rib fractures are less likely, compressive injuries secondary to the relative elasticity of the rib cage are far more common. The computed tomography (CT) features of abdominal visceral injury include lacerations, subcapsular or parenchymal haematomas, active haemorrhage, and vascular injuries, all of which the radiologists must be familiar with. Although there is an increasing trend toward non-operative management of abdominal solid organ injury, radiologists must also be aware of the key imaging features that suggest the need for surgical or interventional radiology input.
Based on our experience as one of the largest Level 1 trauma centres in Europe, the aims of this educational exhibit are to:
(i) Provide a pictorial review the spectrum of findings in blunt injury to the liver, kidneys and spleen as demonstrated on CT.
(ii) Discuss the role of Interventional Radiology in cases of associated vascular injury.
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Authors: Adu John , Mcdonald Kirsteen
Keywords: trauma, liver, kidney, spleen, laceration
Sala Simone, Cirillo Marco, Raimondi Edoardo, Bassi Matteo
Final Pr. ID: Poster #: CR-016
The incidence of upper genitourinary anomalies in children has been reported up to 4-6% and the association of cryptorchidism, hypospadias, double collecting system or fused kidney with Wilms’ tumor is well known. Horseshoe kidney is a rare anomaly of fusion occurring between 4th and 6th weeks of gestation. The association of Wilms’ tumor with horseshoe kidney is uncommon, with limited cases described. The incidence varies from 0.4% to 0.9% of all Wilms’ tumor. Rare variants of Wilms’ tumor like extrarenal teratoid tumor have also been described in children with horseshoe kidney. We describe two cases of Wilms’ tumor arising from horseshoe kidney, detected on US and later confirmed on CE-CT with low-dose imaging protocol. Both cases underwent surgical resection of the abnormal kidney and the diagnosis of Wilms’tumor was confirmed on microscopic examination of the excised tumor. In presence of kidney abonormalities imaging surveillance and follow-up evaluation is mandatory in order to grant an early detection of neoplastic evolution.
In our experience, multislice CT allows complete preoperative evaluation of this rare condition mainly using multiplanar reconstructions and may facilitate surgery.
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Authors: Sala Simone , Cirillo Marco , Raimondi Edoardo , Bassi Matteo
Keywords: wilms, horseshoe kidney