Detailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography.

INTRODUCTION: Magnetic resonance urography (MRU) has proven to be useful in the setting of complex urologic anatomy. Prune belly syndrome (PBS) patients are known to have malformed and highly variable urinary tract anatomy due to significant dilation and renal dysplasia. OBJECTIVE: To further characterize the renal and ureteral anatomy and renal function in patients with PBS via MRU. STUDY DESIGN: Children with PBS undergoing MRU (2006-2011) were identified. Studies were performed to evaluate severe hydronephrosis in all patients. Demographics, previous imaging, and MRU findings were collected. A single radiologist reviewed all studies. RESULTS: MRU was performed on 13 boys, with a median age of 29.3 months (IQR 6-97). Two patients underwent >1 study for ureteropelvic junction obstruction (UPJ obstruction) and calyceal diverticulum with a solitary kidney, respectively. Hydroureteronephrosis (HUN) was identified in 12 boys (92%), while one (8%) did not have ureteral dilation. All patients demonstrated morphologic abnormalities beyond HUN as follows: five (38%) renal dysplasia; five (38%) scarring; four (31%) calyceal diverticula; and three (23%) thickened bladder. The median renal transit time (RTT) was 6 min (IQR 3.5-10.5), and >8 min (range 8.5-35) in six patients; one patient was ultimately diagnosed with obstruction. The mean serum creatinine was 0.5 ± 0.3 mg/dl. This summary figure is a coronal excretory phase T1 MRU image demonstrating absence of well-defined calyces and a 5-cm calyceal diverticulum (white arrow). DISCUSSION: This study reports significant anatomic and functional findings on MRU that were not readily apparent when using standard imaging for children with PBS. The high-resolution images and functional data obtained with MRU allowed for visualization of calyceal diverticula and abnormal renal pelvic anatomy not previously described in PBS. In addition, renal dysplasia could be identified with MRU, which is badly characterized in the PBS population outside of renal biopsy studies. Potential limitations of the study included its nature as a small retrospective case series, which limited the ability to compare imaging modalities. Imaging modalities were based on individual clinical needs; therefore, comparison with diuretic renal scintigraphy was limited. CONCLUSION: MRU provided anatomic and functional details of the urinary tract in children with PBS that allowed for characterization of new renal anatomic abnormalities, including the incidence of calyceal diverticula and renal dysplasia, which have not been previously described. While renal scarring, dysplasia and calyceal diverticula were easily discerned on MRU in ten patients, their clinical significance requires longer follow-up in a larger patient population.

Comparison of magnetic resonance urography to dimercaptosuccinic acid scan for the identification of renal parenchyma defects in children with vesicoureteral reflux.

OBJECTIVE: The objective of this study was to compare the accuracy of dimercaptosuccinic acid (DMSA) renal scan to magnetic resonance urography (MRU) in the identification of renal parenchyma defects (RPD). MATERIALS AND METHODS: Twenty-five children with history of acute pyelonephritis and vesicoureteral reflux underwent DMSA scan and MRU to determine the presence of RPD. DMSA scans and MRUs were each evaluated by two radiologists and agreement achieved by consensus. Discordant DMSA-MRU findings were re-evaluated in a side-by-side comparison and an ultimate consensus reached. RESULTS: The ultimate consensus diagnosis was 18 kidneys with RPDs in 15 patients, of which five were classified as mild RPDs, six as moderate RPDs, and seven as severe RPDs. Although DMSA scan and MRU were similar in their ability to diagnose RPDs, MRU was considered to represent the true diagnosis in 11 of the 12 discordant cases in consensus review by four pediatric radiologists. MRU showed a much higher inter-observer agreement with a weighted kappa of 0.96 for both kidneys compared to 0.71 for the right kidney and 0.86 for the left kidney by DMSA scan. CONCLUSIONS: Our results suggest that MRU is superior to DMSA scan in the identification of renal parenchyma defects.

Clinicopathologic features of nephrogenic rests and nephroblastomatosis.

Nephrogenic rests are the consequence of residual metanephric tissue in a fully developed kidney. They usually occur along the perimeter of a mature renal lobe (i.e., perilobar), within the lobe itself (i.e., intralobar), or both (i.e., combined). Nephrogenic rests can be grossly obvious or microscopically discrete. Nephroblastomatosis designates nephrogenic rests that are multifocal or diffuse, and implies more extensive disease. Universal (panlobar) nephroblastomatosis denotes complete replacement of the renal lobe by nephrogenic tissue. The fate of nephrogenic rests and nephroblastomatosis varies and includes obsolescence, sclerosis, dormancy, hyperplasia, or neoplasia. Evidence strongly suggests that neoplastic transformation of nephrogenic rests results in Wilms' tumor (nephroblastoma). Nephrogenic rests almost always occur in the setting of Wilms' tumor; perilobar rests show a strong association with synchronous bilateral Wilms' tumors, whereas intralobar rests are more strongly associated with metachronous tumors. Genetic studies have shown that nephrogenic rests often share many of the same chromosomal defects as Wilms' tumor, which provides further evidence that they are precursors to nephroblastoma. Thus, nephrogenic rests are recognized as clinically significant entities requiring adequate detection and close surveillance. Heightened awareness regarding the clinical relevance of nephrogenic rests and nephroblastomatosis (1) has led to improved detection of these precancerous lesions, (2) fostered more intensive investigation into their biologic behavior, and (3) initiated in-depth discussions about potentially new treatment regimens. The pathologists' ability to identify and detect nephrogenic rests has benefited from the more efficient Beckwith classification. Radiologists have deployed high-resolution radiologic/imaging modalities to improve detection of nephrogenic rests in situ. Clinicians and surgeons are more aware of the impact that nephrogenic rests have upon patient management. Despite this progress, more data is needed to further define these lesions.

MR of the brain in children.

MR imaging has firmly established its place as the cornerstone of pediatric neuroimaging. Recent advances in MR imaging have led to decreased imaging time, high resolution studies, and new methods for obtaining tissue contrast. Magnetic resonance angiography (MRA) now obviates the need for angiography in some children, although its extended role is still to be defined. Normal and abnormal development and myelination patterns have been further defined with MR imaging. The patterns of brain injury resulting from hypoxia and ischemia vary with the degree of the insult as well as the gestational age of the child. These patterns of hypoxic-ischemic encephalopathy can be analyzed to determine when the insult occurred. Neuronal migration disorders and phakomatoses can be diagnosed with confidence at an early age, thus facilitating genetic counseling. MR imaging can detect the most common lesions associated with childhood epilepsy, such as hippocampal sclerosis, focal cortical dysplasias, and low-grade tumors. Other areas, including pediatric AIDS, toxicity-related injury, metabolic/mitochondrial conditions, and disorders associated with iatrogenic injury, can be diagnosed with MR. Spectroscopy provides information that should prove useful in evaluating and monitoring neuronal and other brain tissue disorders in children.

Voiding cystourethrography in boys: does the presence of the catheter obscure the diagnosis of posterior urethral valves?

OBJECTIVE: In voiding cystourethrography, the urethral catheter may or may not be left in place during voiding. The main argument for removing the catheter is that the diagnosis of posterior urethral valves may be missed because the catheter can hold open the valve, efface it, and render it invisible. However, if the catheter does not prevent the diagnosis of urethral disease, it is preferable to leave it in place. The catheter makes it possible to repeat the procedure easily if necessary, and using it to drain the bladder provides information about ureteric obstruction in the presence of vesicoureteric reflux. Accordingly, the purpose of this study was to determine whether leaving the urethral catheter in place throughout voiding cystourethrography affects the efficacy of the procedure for the diagnosis of posterior urethral valves. MATERIALS AND METHODS: Three radiologists reviewed the preoperative voiding cystourethrograms obtained in 48 boys who ranged in age from 1 day to 10 years old (mean, 1.5 years). All patients had a diagnosis of posterior urethral valves made at cystoscopy, which was used as the gold standard. The voiding cystourethrogram was obtained with a catheter in place during voiding in 28 (58%) of the 48 boys, without a catheter in 17 (35%), and with and then without a urethral catheter during the voiding phase of the study in three (6%). RESULTS: Posterior urethral valves were detected on 25 (89%) of the 28 voiding cystourethrograms obtained with a urethral catheter in place and in 15 (88%) of the 17 voiding cystourethrograms done without a urethral catheter. The five children in whom posterior urethral valves had been diagnosed by cystoscopy but were not detected on voiding cystourethrography had no dilatation of the posterior urethra nor any other evidence of obstruction; these were possibly false-positive cystoscopic diagnoses. CONCLUSION: Our results show that a urethral catheter does not obscure posterior urethral valves in boys and need not be removed routinely during the voiding phase of voiding cystourethrography.

Febrile seizures and hippocampal sclerosis: frequent and related findings in intractable temporal lobe epilepsy of childhood.

The relationship between hippocampal sclerosis, febrile seizures, and complex partial seizures in temporal lobe epilepsy continues to be the subject of great debate in the literature. Hippocampal sclerosis is reported infrequently in young children with temporal lobe epilepsy, a factor that has supported the theory that hippocampal sclerosis develops in later life during the course of recurrent complex partial seizures. In a blinded review of magnetic resonance imaging in 53 children, aged 2-17 years (mean: 10 years) with temporal lobe epilepsy, hippocampal sclerosis was diagnosed in 30 children (57%), concordant with ictal electroencephalographic lateralization in 93% and pathologic diagnosis in all children who had undergone surgery and had hippocampal tissue available for histologic examination. Fourteen of the children (47%) with hippocampal sclerosis were younger than 10 years of age, the youngest being 2 years. Thirty-four children (64%) had histories of neurologic insults prior to the onset of complex partial seizures, including idiopathic febrile seizures in 22. Hippocampal sclerosis was associated with a history of a neurologic insult prior to the onset of complex partial seizures (P < .001) and was not associated with age at onset of temporal lobe epilepsy, age at magnetic resonance imaging, duration of epilepsy, or presence of secondarily generalized seizures. These findings suggest that hippocampal sclerosis is underdiagnosed in children and is the cause and not the consequence of temporal lobe epilepsy.

Hippocampal sclerosis in children with intractable temporal lobe epilepsy: detection with MR imaging.

OBJECTIVE: Hippocampal sclerosis is the most common lesion associated with temporal lobe epilepsy. Temporal lobectomy is effective for the control of medically refractory seizures in these patients. Before the advent of MR imaging, hippocampal sclerosis was rarely diagnosed preoperatively. The purpose of this study was to determine the frequency of hippocampal sclerosis in children with intractable temporal lobe epilepsy and the accuracy and reliability with which hippocampal sclerosis can be diagnosed on the basis of MR findings in children. MATERIALS AND METHODS: We reviewed the MR images of 53 children (mean age, 10 years) with medically refractory temporal lobe epilepsy. The MR images were reviewed blindly and independently by two radiologists on two occasions, and were classified as showing hippocampal sclerosis, other lesions, or no abnormality. Hippocampal sclerosis was diagnosed when evidence of hippocampal atrophy was present or hippocampal signal intensity was abnormal without evidence of a mass lesion. RESULTS: MR images showed hippocampal sclerosis in 30 children (57%), other lesions in 10 (19%) (tumors in eight, cavernous angioma in one, and ectopic gray matter in one), and no abnormality in 13 (24%) (intraobserver agreement: kappa = 0.77 and 0.84, interobserver agreement: kappa = 0.76). MR lateralization was concordant with ictal EEG in 36 (92%) of 39 children. Hippocampal sclerosis was bilateral in one child and associated with extrahippocampal lesions in nine. Hippocampal sclerosis was detected on MR images of 11 (85%) of 13 children with pathologic confirmation of hippocampal sclerosis. Beneficial results were seen in 26 (90%) of 29 children who had temporal lobectomy. CONCLUSION: Hippocampal sclerosis is the most common lesion in children with intractable temporal lobe epilepsy, and it can be detected reliably and accurately on MR images. We suspect that hippocampal sclerosis is underdiagnosed in children, possibly leading to postponement of surgery in children with refractory seizures.

Osteomyelitis of the talus: an unusual cause of limping in childhood.

Hematogenous osteomyelitis of the talus is a rare and infrequently considered cause of limping in children. We describe five children younger than 2 years of age with osteomyelitis of the talus who had a limp and a paucity of constitutional symptoms. Conventional radiography and scintigraphy were the imaging techniques used. The usual radiologic features include soft-tissue swelling and an osteolytic defect in the talus without sequestrum formation of periosteal reaction. Bone scans were positive in all cases and led to the localization of the lesion in two patients. Definitive diagnosis was made in all cases only after needle aspiration or open biopsy and curettage. Increased awareness of this unusual lesion and its subtle early radiologic manifestations will allow prompt diagnosis and treatment.