Melanoma in infants, caused by a gene fusion involving the anaplastic lymphoma kinase (ALK).

We describe the first cases of pediatric melanoma with ALK fusion gene arising within giant congenital melanocytic nevi. Two newborn boys presented with large pigmented nodular plaques and numerous smaller satellite nevi. Additional expansile nodules developed within both nevi and invasive melanomas were diagnosed before 10 months of age in both boys. Oncogenic driver mutations in NRAS and BRAF were absent in both cases. Instead, oncogenic ZEB2::ALK fusion genes were identified in both the nevus and melanoma developing within the nevus. In both cases, tumors were noted by ultrasound in utero, demonstrated significant nodularity at birth, and progressed to melanoma in the first year of life suggesting that congenital nevi with ALK fusion genes may behave more aggressively than those with other mutations. As ALK kinase inhibitors are effective against a range of tumors with similar ALK fusion kinases, identifying ALK fusion genes in congenital melanocytic nevi may provide an opportunity for targeted therapy.

Nonparasitic Splenic Cysts in Children: Outcomes after Sclerotherapy or Surgical Resection as Initial Management.

Management of splenic cysts in children remains undefined. Sclerotherapy is an innovative, less invasive treatment. This study examined the safety and preliminary effectiveness of sclerotherapy for splenic cysts in children compared with those of surgical treatment. A retrospective review of pediatric patients treated for nonparasitic splenic cysts from 2007 to 2021 was performed at a single institution. Posttreatment outcomes for patients who underwent either expectant management, sclerotherapy, or surgery were reviewed. Thirty patients aged between 0 and 18 years met the inclusion criteria. Cysts in 3 of 8 patients who underwent sclerotherapy were either unresolved or recurred. Patients who underwent sclerotherapy and required surgery for residual symptomatic cyst had an initial cyst diameter of >8 cm. Symptoms resolved in 5 of 8 patients who underwent sclerotherapy, with a significantly reduced cyst size compared with that in patients with continued symptoms who underwent sclerotherapy (61.4% vs 7.0%, P = .01). Sclerotherapy is an effective treatment for splenic cysts, particularly those measuring <8 cm. However, surgical excision may be preferable for large cysts.

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children.

Importance: The ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear. Objective: To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children. Design, Setting, and Participants: This retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020. Main Outcomes and Measures: Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. Results: Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%. Conclusions and Relevance: This study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

Weight gain and resource utilization in infants after fundoplication versus gastrojejunostomy.

PURPOSE: There is wide practice variation in the use of laparoscopic fundoplication (LF) versus gastrojejunostomy (GJ) tube insertion for children who do not tolerate gastric feeds. Using weight gain as an objective proxy of adequate nutrition, we sought to evaluate the difference in weight gain between LF and GJ. METHODS: A retrospective, cohort study was conducted of patients ≤ 2 years who underwent LF or GJ between 2014 and 2019 at a single institution. Patient characteristics, change in weight 1-year post-procedure and frequency of unplanned healthcare utilization encounters were collected and examined. RESULTS: A total of 125 patients (50.4%LF, 49.6%GJ) were identified. Adjusted modeling demonstrated that on average, there was an additional 0.85-unit increase in weight-for-age Z scores in the LF compared to the GJ cohort (p = 0.01). The GJ cohort had significantly more unplanned healthcare utilization encounters (4.2, SD 3.4) compared to LF (3.0, SD 3.1) (p = 0.03). Furthermore, the GJ cohort underwent an average of 3.3 planned GJ exchanges within 1-year post-procedure. CONCLUSION: In the first year post-operatively, LF is associated with increased weight gain and fewer unplanned and overall healthcare encounters compared to GJ. Long-term outcomes including weight gain and quality-of-life measures should be studied to develop standardized guidelines for this common clinical scenario.

Pediatric Vascular Malformations: Imaging Guidelines and Recommendations.

Vascular malformations are commonly encountered in the pediatric population. This article reviews the imaging appearances of simple and syndromic vascular malformations in infants and children that radiologists should know and provides imaging guidelines based on an evidence-based approach. Malformations are discussed within the framework of the International Society for the Study of Vascular Anomalies classification system.

Midline venous catheters as an alternative to central line catheter placement: a product evaluation.

Midline catheters have emerged as a successful alternative to peripheral venous catheters (PVCs) and central venous catheters (CVCs) in select patients. Midline catheters allow for greater duration of access when compared with PVCs and avoid the critical complications associated with CVCs. This article describes the implementation of the Powerwand® midline at a large paediatric tertiary care facility in acute and intensive care settings, and presents illustrative cases of the catheter in use. Product evaluation was performed by a paediatric vascular access team of registered nurses and included information on patient outcomes, inserter satisfaction, patient satisfaction, and cost effectiveness.

Spectrum of Imaging Manifestations of Vascular Malformations and Tumors Beyond Childhood: What General Radiologists Need to Know.

Vascular anomalies encompass a collection of diagnoses that differ greatly in terms of clinical presentation, natural history, imaging findings, and management. The purpose of this article is to review diagnostic imaging findings of vascular malformations and vascular tumors, excluding the central nervous system, that occur beyond childhood. A widely accepted classification system created by the International Society for the Study of Vascular Anomalies provides a framework for this review, focusing on the entities most likely to be encountered by general radiologists, although several rare but clinically important entities are also reviewed.

Adherence to the 2011 American Academy of Pediatrics Urinary Tract Infection Guidelines for Voiding Cystourethrogram Ordering by Clinician Specialty.

OBJECTIVE: To evaluate rates of guideline adherence and associations with voiding cystourethrogram result. The American Academy of Pediatrics guidelines recommend voiding cystourethrogram after abnormal renal ultrasound or 2 febrile urinary tract infections. It is unclear whether guideline adherence increases vesicoureteral reflux detection. Additionally, guidelines targeting children 2-24 months are often applied to other ages. METHODS: Children undergoing voiding cystourethrogram from January 2012 to December 2013 at 1 institution were retrospectively reviewed. Children with known genitourinary abnormalities were excluded. The primary outcome was guideline adherence. Univariate and multivariate analyses were performed. Subgroup analysis of children 2-24 months was completed. RESULTS: Voiding cystourethrograms from 365 children were included in the primary analysis, including 187 (51.2%) aged 2-24 months. Overall, 60.3% of voiding cystourethrograms were ordered in accordance with the guidelines. Urologists/nephrologists were more likely to adhere to ordering guidelines than pediatricians/others (76.4% vs 51.7%, odds ratio 3.0 [1.9-4.9], P <.001). Subgroup analysis in children 2-24 months revealed similar findings (76.4% vs 51.5%, odds ratio 3.0 [1.5-6.2], P = .002). Voiding cystourethrograms were abnormal in 31.8% overall and 26.2% aged 2-24 months. Guideline adherence was associated with increased likelihood of abnormal voiding cystourethrogram among all children (P = .02), but not among children 2-24 months (P = .95). Older age, white race, and guideline adherence remained significantly associated with abnormal voiding cystourethrogram in a multiple logistic regression model. CONCLUSIONS: Guideline adherence was more likely among urologists/nephrologists than pediatricians/others and was not associated with abnormal voiding cystourethrogram among children 2-24 months. Multicenter evaluation is necessary to determine if ordering recommendations should be revised.

Urinary tract infection after retrograde urethrogram in children: A multicenter study.

INTRODUCTION: The risk of post-procedural urinary tract infection (ppUTI) after retrograde urethrogram (RUG) has not been well quantified. Prophylactic antibiotics may reduce the risk, but must be weighed against the risks of antibiotic resistance. Prior research has shown that this risk is variable after voiding cystourethrogram (0-42% reported ppUTI rate) and appears to be low after urodynamics (0-4.8%) but this risk has not been well documented for patients undergoing RUG. OBJECTIVE: We aimed to (1) describe the rate of ppUTI after RUG, and (2) examine factors associated with use of antibiotics at the time of RUG. STUDY DESIGN: We conducted a retrospective cohort study of children undergoing RUG at two hospitals January 1, 2004, to December 31, 2014. ppUTI within 7 days was measured. Antibiotic prophylaxis was determined. Relationships between clinical characteristics and receipt of pre-procedure antibiotics were evaluated using univariate statistics. RESULTS: Forty-two patients (100% male, median age 11.7 years) underwent 47 RUGs. Indications included trauma (27%), hypospadias (17%), and non-hypospadias (27%) stricture. Study indication and antibiotic administration practices are illustrated in the Figure. Three patients (6.4%) had a history of posterior urethral valves (PUV); one had neurogenic bladder (NGB) (2.4%). Two (4%) studies were performed within 30 days of a clinical UTI and 11% had a positive urinalysis or culture within 30 days pre-RUG. UTI in the 30 days before RUG was not associated with antibiotic prescription (p > 0.99). One child (2.1%; 95% CI 0-6.3%) had a ppUTI: a 7-year-old, uncircumcised male with a history of PUV and voiding dysfunction who was asymptomatic at the time of RUG. DISCUSSION: Although the risk of ppUTI after RUG appears to be low, the rare occurrence of an iatrogenic UTI can lead to high cost and patient morbidity, particularly in cases similar to our patients who required inpatient admission for intravenous antibiotics. To help balance the risk of UTI with the associated healthcare costs and morbidity with the concerns about antibiotic overuse and potential exposure to drug reaction, data from this study may inform future development of evidence-based guidelines targeting only patients at highest risk for ppUTI with antibiotic prophylaxis. CONCLUSIONS: The risk of ppUTI after RUG is low. Antibiotic prophylaxis was unrelated pre-RUG UTI in our population. These results indicate the need for pre-procedural antibiotic prescribing guidelines, and suggest that routine antibiotics prior to RUG are not indicated unless the patient has another indication for antibiotics.