Retrospective Review of Management of Antenatally Diagnosed Ovarian Cysts at the Hospital for Sick Children.

INTRODUCTION: Postnatal management of antenatally diagnosed ovarian cysts is not well-defined. The clinical course, management, and outcomes of patients with antenatally diagnosed ovarian cysts were reviewed. METHODS: Infants <1 year of age with antenatally diagnosed ovarian cyst managed at The Hospital for Sick Children between January 2017 and December 2021 were included. Patient charts were reviewed for postnatal ultrasound (US) images, management, clinical course and complications. Mixed linear regression analysis was used to model the change in cyst size over time. RESULTS: In total, 52 patients were included and 10 patients had no cyst identified at their first postnatal US. Of the remaining cases, 36% were simple/physiologic and 64% had complex features. Two underwent percutaneous aspiration while 40 patients were managed expectantly with most cysts (62%) resolving. The rate of resolution was significantly higher and faster for simple compared to complex cysts (84% versus 52%, p < 0.05). Cysts that persisted at the end of the study period (n = 14) had all decreased in size, with a rate of resolution similar to resolved cysts. Only one patient managed expectantly required urgent laparoscopy for salpingoophorectomy. CONCLUSION: Antenatally diagnosed ovarian cysts exhibit high rates of resolution with expectant management, supporting the safety and efficacy for expectant management for these patients. LEVEL OF EVIDENCE: III.

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.

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.

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.

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.

Gastrointestinal complications in children with acute myeloid leukemia.

Gastrointestinal complications in pediatric acute myeloid leukemia (AML) have not been systematically described in the literature. Our objective was to describe complications related to the small and large bowel in children with AML. Literature searches were conducted of Ovid Medline from 1950 to November 2009 and EMBASE from 1980 to November 2009. We included any study design that described gastrointestinal complications in children and/or adults with AML. Common gastrointestinal complications were typhlitis and enterocolitis. Less common complications included appendicitis, pneumatosis intestinalis, and perianal infections. Both leukemia infiltration and intensive chemotherapy likely play a role in the etiology of these conditions. There is a paucity of carefully conducted studies that describe the natural history of typhlitis and enterocolitis and evidence is needed to help guide the management of gastrointestinal complications. Gastrointestinal complications are relatively common in children with AML. Conduction of carefully performed cohort studies is needed to better understand the spectrum of symptoms and expected consequences of gastrointestinal complications. Randomized trials are required to develop evidence-based guidelines for the management of gastrointestinal complications in pediatric AML.

Sonographic assessment of renal growth in patients with Beckwith-Wiedemann syndrome: the Beckwith-Wiedemann syndrome renal nomogram.

BACKGROUND: Beckwith-Wiedemann syndrome is a disorder of somatic overgrowth. Evidence of kidney overgrowth is a diagnostic criterion that may be used to help identify those patients who are at the greatest risk of developing Wilms tumors. In such subjects, kidney size is typically larger than that of age-matched normal controls. OBJECTIVE: The purpose of our study was to generate a nomogram that could be used to measure renal dimensions in children with Beckwith-Wiedemann syndrome in a clinical setting. MATERIALS & METHODS: All of the Beckwith-Wiedemann syndrome patients followed at our institution from 1996 to 2004 were eligible for inclusion in our study. Renal length was measured with a curvilinear transducer and with the patient supine. Renal lengths were measured for both kidneys using real-time ultrasound for all patients. Their data were compared with those of age-matched controls reported in the 1984 study by Rosenbaum et al. RESULTS: Ninety-six children with Beckwith-Wiedemann syndrome were followed from 1996 to 2004. Forty-three of these patients met our criteria for inclusion in the study: 28 girls (65%) and 15 boys (35%). We identified a linear relationship between kidney length and patient age. No statistically significant differences in renal length were found between boys and girls (p=0.2153) or between the kidneys on either side of the body (p=0.9613). CONCLUSION: Our study provides a practical, simple renal growth chart that offers a reasonable, sensitive method for evaluating kidney size in children with Beckwith-Wiedemann syndrome.

Sonographic assessment of renal growth in patients with Beckwith-Wiedemann syndrome: the Beckwith-Wiedemann syndrome renal nomogram

BACKGROUND: Beckwith-Wiedemann syndrome is a disorder of somatic overgrowth. Evidence of kidney overgrowth is a diagnostic criterion that may be used to help identify those patients who are at the greatest risk of developing Wilms tumors. In such subjects, kidney size is typically larger than that of age-matched normal controls. OBJECTIVE: The purpose of our study was to generate a nomogram that could be used to measure renal dimensions in children with Beckwith-Wiedemann syndrome in a clinical setting. MATERIALS & METHODS: All of the Beckwith-Wiedemann syndrome patients followed at our institution from 1996 to 2004 were eligible for inclusion in our study. Renal length was measured with a curvilinear transducer and with the patient supine. Renal lengths were measured for both kidneys using real-time ultrasound for all patients. Their data were compared with those of age-matched controls reported in the 1984 study by Rosenbaum et al. RESULTS: Ninety-six children with Beckwith-Wiedemann syndrome were followed from 1996 to 2004. Forty-three of these patients met our criteria for inclusion in the study: 28 girls (65 percent) and 15 boys (35 percent). We identified a linear relationship between kidney length and patient age. No statistically significant differences in renal length were found between boys and girls (p=0.2153) or between the kidneys on either side of the body (p=0.9613). CONCLUSION: Our study provides a practical, simple renal growth chart that offers a reasonable, sensitive method for evaluating kidney size in children with Beckwith-Wiedemann syndrome.