Ultrasonographical features of perinatal testicular torsion: an assessment of interobserver variability among radiologists and pediatric urologists.

PURPOSE: To retrospectively compare interpretations of Doppler ultrasound (US) in newborns with confirmed perinatal testicular torsion (PTT) by an experienced faculty (staff) pediatric radiologist (SPR), pediatric radiology fellow (PRF), pediatric urology fellow (PUF) and staff pediatric urologist (SPU). METHODS: US images of 27 consecutive males with PTT between May 2000 and July 2020 were retrieved. The testicles were classified as affected or non-affected by PTT. We performed a blinded comparison of interpretation by four assessors (SPR, PRF, PUF, SPU), with respect to the US features of PTT. Paired inter-rater agreement was calculated using Cohen's Kappa (κ) and overall agreement was assessed using Fleiss' kappa. RESULTS: Overall comparison using Fleiss' kappa found fair agreement for most features except testicular echogenicity and echogenic foci at interface for which there was poor agreement. Paired comparisons revealed better agreement between the SPR and PRF compared to the remaining two pairs, suggesting a need for the pediatric urologists (PUF and SPU) to acquaint themselves with testicular ultrasonography as this may have an impact on patient risk stratification and the quality of information given to parents. CONCLUSION: This study highlights the need for focused training program for pediatric urologists to attain similar agreement as the radiologists, suggesting a need for the pediatric urologists (PUF and SPU) to acquaint themselves with testicular ultrasonography as this may have an impact on patient risk stratification and the quality of information given to parents.

Volumetric assessment of unaffected parenchyma and Wilms' tumours: analysis of response to chemotherapy and surgery using a semi-automated segmentation algorithm in children with renal neoplasms.

OBJECTIVE: To present our proof of concept with semi-automatic image recognition/segmentation technology for calculation of tumour/parenchyma volume. METHODS: We reviewed Wilms' tumours (WTs) between 2000 and 2018, capturing computed tomography images at baseline, after neoadjuvant chemotherapy (NaC) and postoperatively. Images were uploaded into MATLAB-3-D volumetric image processing software. The program was trained by two clinicians who supervised the demarcation of tumour and parenchyma, followed by automatic recognition and delineation of tumour margins on serial imaging, and differentiation from uninvolved renal parenchyma. Volume was automatically calculated for both. RESULTS: During the study period, 98 patients were identified. Of these, based on image quality and availability, 32 (38 affected moieties) were selected. Most patients (65%) were girls, diagnosed at age 50 ± 37 months of age. NaC was employed in 64% of patients. Surgical management included 27 radical and 11 partial nephrectomies. Automated volume assessment demonstrated objective response to NaC for unilateral and bilateral tumours (68 ± 20% and 53 ± 39%, respectively), as well as preservation on uninvolved parenchyma with partial nephrectomy (70 ± 46 cm3 at presentation to 57 ± 41 cm3 post-surgery). CONCLUSION: Volumetric analysis is feasible and allows objective assessment of tumour and parenchyma volume in response to chemotherapy and surgery. Our data show changes after therapy that may be otherwise difficult to quantify. Use of such technology may improve surgical planning and quantification of response to treatment, as well as serving as a tool to predict renal reserve and long-term changes in renal function.

Utility of Soft Tissue Lateral Neck Radiographs in the Emergency Department: The 5-Year Experience of a Large Tertiary Care Pediatric Hospital.

INTRODUCTION: Although retropharyngeal infection (RPI) may present with voice change, drooling, fever, and a toxic appearance, diagnosis based on symptoms alone is unreliable. As incidence is increasing in children and drug-resistant bacterial strains such as methicillin-resistant Staphylococcus aureus are becoming more common, we decided to assess the clinical utility of lateral neck radiography. OBJECTIVE: The aim of this study was to review the experience of a large tertiary care pediatric emergency department (ED) in using lateral soft tissue neck radiographs in the diagnosis of suspected RPI. METHODS: A retrospective analysis of all lateral soft tissue neck radiograph reports from 2011 to 2015 in conjunction with a review of patients' charts to describe clinical and laboratory findings, disposition, and final diagnosis was performed. Patients aged 31 days to 18 years who presented to the ED with suspicion of RPI were included. RESULTS: Review of 366 radiographic reports revealed that 46 were positive for RPI, 286 were negative, and 34 indeterminate. A final discharge diagnosis of RPI was made in 38 patients. Lateral neck radiographs had a sensitivity of 84.3% and a specificity of 93.7% for diagnosing RPI. In triage, most patients had no fever (264, 72.1%), stridor (356, 97%), drooling (348, 95%), or voice change (342, 93%). Surgical intervention occurred in 15 patients (39.5%) with a final diagnosis of RPI. CONCLUSIONS: Lateral neck radiography is useful for diagnosis of RPI in the ED with good sensitivity and specificity. Additional imaging is to be considered at the behest of physician's clinical judgment.

Periventricular Hemorrhagic Infarction in Very Preterm Infants: Characteristic Sonographic Findings and Association with Neurodevelopmental Outcome at Age 2 Years.

OBJECTIVE: To describe the sonographic characteristics of periventricular hemorrhagic infarction (PVHI) and their association with mortality and neurodevelopmental disability in very preterm infants born in 2008-2013. STUDY DESIGN: Retrospective multicenter observational cohort study. Diagonal PVHI size was measured and severity score assessed. PVHI characteristics were scored and temporal trends were assessed. Neurodevelopmental outcome at 2 years of corrected age was assessed using either the Bayley Scales of Infant and Toddler Development, Third Edition or the Griffiths Mental Development Scales. Multigroup analyses were applied as appropriate. RESULTS: We enrolled 160 infants with median gestational age of 26.6 weeks. PVHI was mostly unilateral (90%), associated with an ipsilateral grade III intraventricular hemorrhage (84%), and located in the parietal lobe (51%). Sixty-four (40%) infants with PVHI died in the neonatal period. Of the survivors assessed at 2 years of corrected age, 65% had normal cognitive and 69% had normal motor outcomes. The cerebral palsy rate was 42%. The composite outcome of death or severe neurodevelopmental disability was observed in 58%, with no trends over the study period (P = .6). Increasing PVHI severity score was associated with death (P < .001). Increasing PVHI size and severity score were negatively associated with gross motor scores (P = .01 and .03, respectively). Trigone involvement was associated with cerebral palsy (41% vs 14%; P = .004). Associated posthemorrhagic ventricular dilation (36%) was an independent risk factor for poorer cognitive and motor outcomes (P < .001 for both). CONCLUSIONS: Increasing PVHI size and severity score were predictive of less optimal gross motor outcome and death in very preterm infants.

Prevalence, Doppler Ultrasound Findings, and Clinical Implications of the Nutcracker Phenomenon in Pediatric Varicoceles.

OBJECTIVE: To examine the implications of varicoceles and nutcracker phenomenon (NcP) in a large cohort of adolescent patients. Varicoceles are common in adolescent males, generating concerns regarding etiology and management. At our institution, Doppler ultrasound (US) of the renal vessels is routinely obtained with the goal of assessing for an associated nutcracker phenomenon. METHODS: Between 1/2000 and 3/2017, 182 patients with clinical varicoceles were evaluated with US. Retrospective assessment provided complete data in 137, including maximum varicose vein diameter, testicular measurements, left renal vein velocities at the hilum and impingement point by the superior mesenteric artery, and procedural interventions. RESULTS: NcP was detected in 77 patients (56.2%), who experienced higher venous velocity ratios (8.33 vs 2.87; P < 0.001) than those without. Overall, 39 patients (28.5%) had a testicular volume discrepancy >20%, without a significant difference based on the presence or absence of NcP (27.3 vs 30.0%, respectively; P = 0.36). Both groups had similar ages at diagnosis, bilateral volume parameters, volume difference, maximum varicose vein sizes, and follow-up duration (P ≥ 0.05 for all). Intervention was more likely in patients with volume difference >20% (P = 0.014). Having NcP was not associated with a higher incidence of initial (P = 0.59) or reoperative surgery (P = 0.73). CONCLUSION: NcP is common in adolescent patients with a varicocele, but it is not associated with differences in testicular parameters or an increased frequency of initial or reoperative surgery. As such, NcP may have few clinical ramifications as an isolated finding in this patient population, calling into question routine assessment for its presence.

Management of ovarian lesions diagnosed during infancy.

PURPOSE: Prior studies of infant ovarian cysts have recommended intervention for those larger than 4 cm. We reviewed the natural history and features of those managed operatively versus nonoperatively. METHODS: A retrospective study was performed of ovarian lesions in children <1 year-old from 2000 to 2014. RESULTS: Forty patients were identified. Twenty-eight (70%) underwent operative management, while 12 (30%) were managed conservatively, including one undergoing aspiration. The mean age at surgery was 125 days. All but one patient (96%) had evidence of antenatal torsion intraoperatively or on final pathology. All resected lesions were benign. Ultrasound findings more common in those undergoing surgery included intracystic debris (p < 0.001), fluid-fluid or fluid-debris levels (p = 0.002), absence of Doppler flow (p = 0.014), solid components (p = 0.04), and calcifications (p = 0.001). Cysts managed nonoperatively had an average diameter of 2.5 cm, compared to 5.1 cm in the operative group (p < 0.001). Three of the lesions managed nonoperatively had a diameter greater than 3.5 cm (mean 5.1 cm) and were followed for an average of 153 days until resolution. CONCLUSIONS: The majority of infant ovarian lesions were excised, although none were malignant. Aspiration or observation of larger cysts was feasible and safe. Ovarian cysts in this age group should be considered for nonoperative management and closely followed. LEVEL OF EVIDENCE: IIC.

Posthemorrhagic ventricular dilatation in preterm infants: When best to intervene?

OBJECTIVE: To compare neurodevelopmental outcomes of preterm infants with and without intervention for posthemorrhagic ventricular dilatation (PHVD) managed with an "early approach" (EA), based on ventricular measurements exceeding normal (ventricular index [VI] <+2 SD/anterior horn width <6 mm) with initial temporizing procedures, followed, if needed, by permanent shunt placement, and a "late approach" (LA), based on signs of increased intracranial pressure with mostly immediate permanent intervention. METHODS: Observational cohort study of 127 preterm infants (gestation <30 weeks) with PHVD managed with EA (n = 78) or LA (n = 49). Ventricular size was measured on cranial ultrasound. Outcome was assessed at 18-24 months. RESULTS: Forty-nine of 78 (63%) EA and 24 of 49 (49%) LA infants received intervention. LA infants were slightly younger at birth, but did not differ from EA infants for other clinical measures. Initial intervention in the EA group occurred at younger age (29.4/33.1 week postmenstrual age; p < 0.001) with smaller ventricles (VI 2.4/14 mm >+2 SD; p < 0.01), and consisted predominantly of lumbar punctures or reservoir taps. Maximum VI in infants with/without intervention was similar in EA (3/1.5 mm >+2 SD; p = 0.3) but differed in the LA group (14/2.1 mm >+2 SD; p < 0.001). Shunt rate (20/92%; p < 0.001) and complications were lower in EA than LA group. Most EA infants had normal outcomes (>-1 SD), despite intervention. LA infants with intervention had poorer outcomes than those without (p < 0.003), with scores <-2 SD in 81%. CONCLUSION: In preterm infants with PHVD, those with early intervention, even when eventually requiring a shunt, had outcomes indistinguishable from those without intervention, all being within the normal range. In contrast, in infants managed with LA, need for intervention predicted worse outcomes. Benefits of EA appear to outweigh potential risks. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that for preterm infants with PHVD, an EA to management results in better neurodevelopmental outcomes than a LA.

Applying Radiomics to Predict Pathology of Postchemotherapy Retroperitoneal Nodal Masses in Germ Cell Tumors.

PURPOSE: After chemotherapy, approximately 50% of patients with metastatic testicular germ cell tumors (GCTs) who undergo retroperitoneal lymph node dissections (RPNLDs) for residual masses have fibrosis. Radiomics uses image processing techniques to extract quantitative textures/features from regions of interest (ROIs) to train a classifier that predicts outcomes. We hypothesized that radiomics would identify patients with a high likelihood of fibrosis who may avoid RPLND. PATIENTS AND METHODS: Patients with GCT who had an RPLND for nodal masses > 1 cm after first-line platinum chemotherapy were included. Preoperative contrast-enhanced axial computed tomography images of retroperitoneal ROIs were manually contoured. Radiomics features (n = 153) were used to train a radial basis function support vector machine classifier to discriminate between viable GCT/mature teratoma versus fibrosis. A nested 10-fold cross-validation protocol was used to determine classifier accuracy. Clinical variables/restricted size criteria were used to optimize the classifier. RESULTS: Seventy-seven patients with 102 ROIs were analyzed (GCT, 21; teratoma, 41; fibrosis, 40). The discriminative accuracy of radiomics to identify GCT/teratoma versus fibrosis was 72 ± 2.2% (area under the curve [AUC], 0.74 ± 0.028); sensitivity was 56.2 ± 15.0%, and specificity was 81.9 ± 9.0% ( P = .001). No major predictive differences were identified when data were restricted by varying maximal axial diameters (AUC range, 0.58 ± 0.05 to 0.74 ± 0.03). The prediction algorithm using clinical variables alone identified an AUC of 0.76. When these variables were added to the radiomics signature, the best performing classifier was identified when axial masses were limited to diameter < 2 cm (accuracy, 88.2 ± 4.4; AUC, 0.80 ± 0.05; P = .02). CONCLUSION: A predictive radiomics algorithm had a discriminative accuracy of 72% that improved to 88% when combined with clinical predictors. Additional independent validation is required to assess whether radiomics allows patients with a high predicted likelihood of fibrosis to avoid RPLND.

Contemporary Practice Patterns of Voiding Cystourethrography Use at a Large Tertiary Care Center in a Single Payer Health Care System.

PURPOSE: Voiding cystourethrogram involves radiation exposure and is invasive. Several guidelines, including the 2011 AAP (American Academy of Pediatrics) guidelines, no longer recommend routine voiding cystourethrogram after the initial urinary tract infection in children. The recent trend in voiding cystourethrogram use remains largely unknown. We examined practice patterns of voiding cystourethrogram use and explored the impact of these guidelines in a single payer system in the past 8 years. MATERIALS AND METHODS: We identified all voiding cystourethrograms performed at a large pediatric referral center between January 2008 and December 2015. Patients 2 to 24 months old who underwent an initial voiding cystourethrogram for the diagnosis of a urinary tract infection in the first 6 months of 2009 and 2014 were identified. Medical records were retrospectively reviewed. RESULTS: During the study period 8,422 voiding cystourethrograms were performed and the annual number declined over time. In the pre-AAP and post-AAP cohorts 233 and 95 initial voiding cystourethrograms were performed, respectively. While there was no statistically significant difference in the vesicoureteral reflux detection rate between 2009 and 2014 (37.3% vs 43.0%, p = 0.45), there was a threefold increase in high grade vesicoureteral reflux in 2014 (2.6% vs 8.4%, p = 0.03). CONCLUSIONS: A clear trend toward fewer voiding cystourethrograms was noted at our institution. This decrease started before 2011 and cannot be attributed to the AAP guidelines alone. While most detected vesicoureteral reflux remains low grade, there was a greater detection rate of high grade vesicoureteral reflux in 2014 compared to 2009. This may reflect a favorable impact of a more selective approach to obtaining voiding cystourethrograms.

Anti-Müllerian Hormone in Female Adolescent Cancer Patients Before, During, and After Completion of Therapy: A Pilot Feasibility Study.

STUDY OBJECTIVE: Alkylating agents are implicated in premature ovarian insufficiency. To optimize counseling regarding future ovarian function in survivors of adolescent cancer, we describe anti-Müllerian hormone (AMH) levels in female adolescents at diagnosis, during, and shortly after completion of chemotherapy. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: This was a prospective single-institution study. Participants were a mixed population of newly diagnosed postmenarchal female adolescents with malignancy. AMH was performed at diagnosis (T1), 6 months from diagnosis (T2), at end of therapy or 12 months [T3, whichever came first], 1 year after the end of therapy or 24 months from diagnosis [T4, whichever came first], and 18 months from the time of diagnosis (T5). All patients had baseline pelvic ultrasound examinations. Presence of menses and hot flashes were recorded at each time point. RESULTS: Sixteen participants with a median age at diagnosis of 14.3 years (range 12-17 years) were followed for 18.2 months (range, 14-24 months). Oncology diagnoses included leukemia, lymphoma, and sarcoma. Ten patients (62.5%) received alkylating agents with a median cumulative dose of 3041 mg/m2 (range, 2639-6478 mg/m2) of cyclophosphamide. Almost half (n = 7; 44%) experienced amenorrhea during treatment with resumption of menses in 6 of 7 patients (85%). Fifteen of 16 (94%) participants showed a decline in mean AMH levels by 6 months (T2) from diagnosis (15.8 IU/mL at T1 vs 6.5 IU/mL at T2; P = .003) and 12 of 15 (80%) showed at least some recovery of AMH (mean AMH at T4 = 13.2 IU/mL compared with 6.5 IU/mL at T2; P = .02). There was no difference in the mean decline nor recovery of AMH in those who did, vs did not receive cyclophosphamide. CONCLUSION: To our knowledge, this is the largest series to date in adolescents showing that AMH is uniformly suppressed during cancer therapy and short-term recovery occurs in just more than half of the patients by 18-24 months. The contribution of short-term AMH measurements in predicting long-term ovarian function remains to be defined. Long-term follow-up with serial AMH levels is required to help predict those at risk for premature ovarian insufficiency.

Evolution of Renal Cysts to Anaplastic Sarcoma of Kidney in a Child With DICER1 Syndrome.

Anaplastic sarcoma of kidney (ASK) is a rare neoplasm recently associated with DICER1 mutations. We report a child with germline DICER1 mutation who developed ASK in preexisting septated renal cysts, which were likely cystic nephroma. From age 2.5 to 6 years, sonographic imaging illustrated changes in the size and number of renal cysts, followed at age 8.8 years by a mass, pathologically an ASK. Lung cysts resected in infancy were diagnosed retrospectively as pleuropulmonary blastoma. Both tumors had acquired somatic DICER1 mutations. Ultrasonographic evolution of renal cysts to ASK has not previously been documented. Children with both pulmonary and renal cysts are candidates for DICER1 mutation testing.

Ovarian embryonal rhabdomyosarcoma is a rare manifestation of the DICER1 syndrome.

Embryonal rhabdomyosarcoma (ERMS), a soft tissue sarcoma, is one of the most common pediatric cancers. Certain ERMSs are associated with the DICER1 syndrome, a tumor predisposition syndrome caused by germ-line DICER1 mutations. Characteristic somatic mutations have also been identified in DICER1-associated tumor types. These "hotspot" mutations affect the catalytic activity of the DICER1 ribonuclease IIIb domain. Primary ovarian ERMS (oERMS) is extremely rare. We present a case of a 6-year-old girl with an oERMS harboring 2 DICER1 mutations. The girl also exhibited other DICER1 phenotypes: cystic nephroma (CN) and multinodular goiter. Somatic investigations of the CN identified a hotspot DICER1 mutation different from that in the oERMS. Significantly, the CN presented at 12 years of age, which is much older than the previously reported age range of susceptibility. This report documents the occurrence of DICER1 mutations in a case of oERMS, expanding the spectrum of DICER1-associated tumors.

Incidence, risk factors, and outcomes of enteritis, typhlitis, and colitis in children with acute leukemia.

This retrospective chart review describes pediatric patients with acute lymphoblastic leukemia or acute myeloid leukemia diagnosed between January 1999 and January 2008, who were identified with enteritis, typhlitis, or colitis. Among the acute leukemia patients, 33/449 (7.3%) with acute lymphoblastic leukemia and 13/89 (14.6%) with acute myeloid leukemia experienced 51 episodes of enteritis (n=8), typhlitis (n=15), colitis (n=19), or enterocolitis (n=9). Twenty-five (49%) patients were exposed to corticosteroids within 14 days of the episode and 35 (68.6%) had fever and neutropenia concurrent with the episode. Forty-eight (94%) patients were treated with complete bowel rest and broad-spectrum antibiotics. However, 3 patients received no therapy and had uneventful courses. Complications included sepsis in 7/51 (13.7%) and intestinal obstruction in 3/51 (5.9%). One child required surgery for abscess drainage and 2 children died of causes unrelated to their colitis. Enteritis, typhlitis, or colitis occurred in 8.6% of children treated for leukemia. The optimal management approach is uncertain.

Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital.

BACKGROUND: Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances. OBJECTIVE: To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings. MATERIALS AND METHODS: We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis. RESULTS: We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage. CONCLUSION: Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.

The long-term outcomes of pediatric pleural empyema: a prospective study.

OBJECTIVE: To describe the long-term outcomes of pediatric pleural empyema. DESIGN: Prospective observational study from October 2008 to October 2011. SETTING: Tertiary care children's hospital. PARTICIPANTS: Children with pleural empyema (loculations and/or septations identified on radiologic imaging or frank pus on thoracentesis). MAIN OUTCOME MEASURES: Children were seen 1, 6, and 12 months postdischarge. Outcome measures included symptoms and signs of respiratory disease, child and parental impact, radiographic resolution, spirometry, and health-related quality of life (Pediatric Quality of Life Inventory score). Analysis was based on the last observation carried forward for missing data. RESULTS: Eighty-two of 88 patients (93%) eligible were recruited. Fifty-four percent were male and mean (SD) age was 4.5 (3.4) years. Outcome data was obtained in 100% at 1 month, 90% at 6 months, and 72% at 1 year. Seventy-one percent had effusions occupying a quarter or more of the hemithorax and 62% of effusions were drained. Fever, cough, parental work loss, child school loss, radiographic abnormalities, and abnormal spirometry results were common in the first month and then declined. By the last observation, 2% of patients had abnormal radiographs (aside from pleural thickening), 6% had mild obstruction on spirometry, and Pediatric Quality of Life Inventory scores were better than for children with asthma (P < .001). Patients with abnormal outcomes in 1 measure had normal outcomes in all other clinical measures. CONCLUSIONS: Clinically important phenomena persist in the short-term, but virtually all children with pleural empyema have no long-term sequelae.

Association between corticosteroids and infection, sepsis, and infectious death in pediatric acute myeloid leukemia (AML): results from the Canadian infections in AML research group.

BACKGROUND: Infection continues to be a major problem for children with acute myeloid leukemia (AML). Objectives were to identify factors associated with infection, sepsis, and infectious deaths in children with newly diagnosed AML. METHODS: We conducted a retrospective, population-based cohort study that included children ≤ 18 years of age with de novo, non-M3 AML diagnosed between January 1995 and December 2004, treated at 15 Canadian centers. Patients were monitored for infection from initiation of AML treatment until recovery from the last cycle of chemotherapy, conditioning for hematopoietic stem cell transplantation, relapse, persistent disease, or death (whichever occurred first). Consistent trained research associates abstracted all information from each site. RESULTS: 341 patients were included. Median age was 7.1 years (interquartile range [IQR], 2.0-13.5) and 29 (8.5%) had Down syndrome. In sum, 26 (7.6%) experienced death as a first event. There were 1277 courses of chemotherapy administered in which sterile site microbiologically documented infection occurred in 313 courses (24.5%). Sepsis and infectious death occurred in 97 (7.6%) and 16 (1.3%) courses, respectively. The median days of corticosteroid administration was 2 per course (IQR, 0-6). In multiple regression analysis, duration of corticosteroid exposure was significantly associated with more microbiologically documented sterile site infection, bacteremia, fungal infection, and sepsis. The only factor significantly associated with infectious death was days of corticosteroid exposure (odds ratio, 1.05; 95% confidence interval, 1.02-1.08; P = .001). CONCLUSIONS: In pediatric AML, infection, sepsis, and infectious death were associated with duration of corticosteroid exposure. Corticosteroids should be avoided when possible for this population.

Color Doppler US of normal cerebral venous sinuses in neonates: a comparison with MR venography.

BACKGROUND: Color Doppler US (CDUS) has been used for evaluation of cerebral venous sinuses in neonates. However, there is very limited information available regarding the appearance of superficial and deep normal cerebral venous sinuses using CDUS and the specificity of the technique to rule out disease. OBJECTIVE: To determine the specificity, inter-modality and inter-reader agreement of color Doppler US (CDUS). To evaluate normal cerebral venous sinuses in neonates in comparison to MR venography (MRV). MATERIALS AND METHODS: Newborns undergoing a clinically indicated brain MRI were prospectively evaluated. All underwent a dedicated CDUS of the cerebral venous sinuses within 10 h (mean, 3.5 h, range, and 2-7.6 h) of the MRI study using a standard protocol. RESULTS: Fifty consecutive neonates participated in the study (30 males [60%]; 25-41 weeks old; mean, 37 weeks). The mean time interval between the date of birth and the CDUS study was 19.1 days. No cases showed evidence of thrombosis. Overall agreement for US reading was 97% (range, 82-100%), for MRV reading, 99% (range, 96-100%) and for intermodality, 100% (range, 96-100%). Excellent US-MRI agreement was noted for superior sagittal sinus, cerebral veins, straight sinus, torcular Herophili, sigmoid sinus, superior jugular veins (94-98%) and transverse sinuses (82-86%). In 10 cases (20%), MRV showed flow gaps whereas normal flow was demonstrated with US. Visualization of the inferior sagittal sinus was limited with both imaging techniques. CONCLUSION: Excellent reading agreement was noted for US, MRV and intermodality. CDUS is highly specific to rule out cerebral venous thrombosis in neonates and holds potential for clinical application as part of clinical-laboratory-imaging algorithms of pre/post-test probabilities of disease.

Pneumatosis intestinalis in children with acute lymphoblastic leukemia and acute myeloid leukemia.

PURPOSE: To describe symptoms, diagnostic features, treatments, and outcomes of pneumatosis intestinalis (PI) in pediatric patients being treated for acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). METHODS: This retrospective chart review included 514 patients ≤ 18 years of age diagnosed with ALL or AML between January 1999 and December 2007. PI episodes were identified by radiology report reviews. RESULTS: Twenty patients with ALL and four patients with AML presented 31 PI episodes. The median time between diagnoses of leukemia and PI was 1.0 month (interquartile range 0.8-6.4 months). Plain radiographs diagnosed all PI episodes. Computerized tomography (CT) and ultrasound were performed in 6 and 13 episodes, respectively. All CT and three ultrasounds demonstrated PI. Thirty episodes occurred exclusively in the colon, most commonly in the ascending (n = 26) and transverse (n = 18) segments. Treatment included complete bowel rest in 27 (87.1%) and intravenous broad-spectrum antibiotics in 29 (93.5%). One case required colectomy. Two episodes were untreated. There were no fatalities associated with PI. CONCLUSIONS: PI is uncommon in children with ALL or AML. Ultrasound is less sensitive than plain radiographs for diagnosis. PI occurred almost exclusively in the colon. With conservative management, most patients had excellent outcome.

Utility of contrast echocardiography for pulmonary arteriovenous malformation screening in pediatric hereditary hemorrhagic telangiectasia.

OBJECTIVE: To evaluate the utility of transthoracic contrast echocardiography (TTCE) as a screening tool for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN: This was a single-center study of children who underwent baseline screening for PAVMs using both TTCE and chest computed tomography (CT) for evaluation of HHT. The CT and TTCE results were prospectively reviewed independently by 2 radiologists and 2 cardiologists blinded to the study results. RESULTS: Both intraobserver and interobserver agreement for interpreting TTCE results were excellent (κ = 0.97 and 0.92, respectively) and higher than the interobserver agreement for CT interpretation (κ = 0.75). The sensitivity and specificity of TTCE to predict PAVMs were 1 and 0.82, respectively, and the positive predictive and negative predictive values were 0.39 and 1, respectively. CONCLUSION: TTCE is a sensitive test for PAVMs in children with suspected HHT and can be a useful initial screening tool in pediatric HHT.

Pleuropulmonary blastoma in a child with autosomal-recessive polycystic kidney disease.

Autosomal-recessive polycystic kidney disease (ARPKD) is a developmental disorder known to affect both the kidneys and the liver. Renal involvement results in progressive renal insufficiency and hypertension, while hepatic involvement can result in portal hypertension and cholangitis. Pulmonary abnormalities relate mainly to pulmonary insufficiency in those patients who present as neonates. We present a unique case of a child with ARPKD found to have a cystic lesion of the lung. Upon surgical resection, a pathological diagnosis of pleuropulmonary blastoma (PPB) was made. There are no previous reports in the literature describing the association of these two entities. Knowledge of this potential association is important in the clinical management of these children and may open new avenues of genetic research.