Comparison of pediatric post-reduction fluoroscopic- and ultrasound forearm fracture images.

OBJECTIVE: Emergency department (ED) reduction of pediatric fractures occurs most commonly in the forearm and can be challenging if fluoroscopy is not available. We sought to assess the ability of point of-care ultrasonography (POCUS) to predict adequacy of reduction by fluoroscopy. METHODS: We prospectively enrolled ED patients 0-17 years of age with radial and/or ulnar fractures requiring reduction under fluoroscopic guidance. Post-reduction POCUS (probe dorsal, volar, and coronal) and fluoroscopic (AP and lateral) fracture images were recorded. Fracture angles were compared between blinded POCUS and fluoroscopic measurements and between POCUS measurements by a blinded emergency physician and a blinded radiologist, reporting mean differences and 95% confidence intervals. We calculated sensitivity, specificity, and likelihood ratios of POCUS in the prediction of fluoroscopically detected post-reduction malalignment, as interpreted by a blinded pediatric orthopaedist. RESULTS: The 58 patients were 7.9 ±â€¯3.5 years of age and had 21 radial (36%), 1 ulnar (2%), and 36 radioulnar (62%) fractures. Fluoroscopy and POCUS angles were within a mean of 0.1°-3.2°, depending on the site and surface measured. Radiologist- and emergency physician-interpreted POCUS measurements were within a mean of 1° in all dimensions. POCUS identified inadequate reductions with 100% sensitivity and 92-93% specificity. CONCLUSIONS: Blinded emergency medicine and radiology interpretations of post-reduction POCUS fracture images agree closely. Post-reduction POCUS measurements are comparable to those obtained by fluoroscopy and accurately predict adequacy of reduction. POCUS can be used to guide pediatric fracture reduction when bedside fluoroscopy is not available in the ED.

Does abdominal pain duration affect the accuracy of first-line MRI for pediatric appendicitis?

PURPOSE: To compare the accuracy of first-line MRI for pediatric appendicitis between children with different durations of abdominal pain. MATERIALS AND METHODS: Between November 2012 and September 2013, 364 pediatric patients underwent MRI for clinically suspected appendicitis. Patients were stratified into one group with pain lasting less than or equal to 1 day and pain longer than 1 day. RESULTS: In patients with early abdominal pain, appendicitis occurred in 83 of 208 patients (39.9%) vs. 49 of 156 patients with pain longer than 1 day (31.4%, p = 0.09). Sensitivity and specificity of MRI in patients with early abdominal pain were 97.6% (95% CI [91.6%-99.3%]) and 94.4% (95% CI [88.9%-97.3%]), respectively, vs. 93.9% (95% CI [83.5%-97.9%]) and 97.2% (95% CI [92.1%-99.4%]), respectively, in patients with pain longer than 1 day in duration (p = 0.36 and p = 0.35 for sensitivity and specificity, respectively). CONCLUSIONS: First-line MRI is accurate for acute appendicitis in children presenting with early abdominal pain, and may be appropriate as the initial examination in children. Further prospective studies are needed to compare MRI vs. ultrasound as the initial examination in children with early abdominal pain to help further delineate a diagnostic imaging algorithm.

An idiopathic case of pediatric retroperitoneal fibrosis with extensive magnetic resonance imaging and follow-up.

Retroperitoneal fibrosis has seldom been described in the pediatric literature. We present a child who presented with retroperitoneal fibrosis without any associated underlying vascular or inflammatory conditions. This is one of only a few cases of primary idiopathic retroperitoneal fibrosis in a pediatric patient to have been imaged extensively by MRI.

Diagnostic performance of contrast-enhanced MR for acute appendicitis and alternative causes of abdominal pain in children.

OBJECTIVE: Unenhanced MRI has emerged as a useful tool for diagnosing pediatric acute appendicitis. The use of contrast-enhanced MRI for diagnosing pediatric appendicitis has not been documented. The purpose of this study is to examine the diagnostic performance of contrast-enhanced MRI for acute appendicitis and alternative entities in the pediatric population presenting with acute abdominal pain. MATERIALS AND METHODS: A retrospective review was conducted of 364 consecutive pediatric patients undergoing contrast-enhanced MRI for the evaluation of possible appendicitis at a single institution between November 2012 and September 2013. RESULTS: There were 132 cases of pathologically confirmed appendicitis out of 364 pediatric patients (36.3%) included in the study. Overall sensitivity and specificity were 96.2% (95% CI [91.4-98.4%]) and 95.7% (95% CI [92.3-97.6%]), respectively. Positive predictive value and negative predictive value were 92.7% (95% CI [86.6-96.3%]) and 97.8% (95% CI [94.7-99.1%]), respectively. The appendix was visualized in 243 cases (66.8%). Imaging confirmed alternative diagnoses in 75 patients, including most commonly colitis, enteritis or terminal ileitis (n = 25, 6.9%), adnexal cysts (n = 25, 6.9%) and mesenteric adenitis (n = 7, 1.9%). CONCLUSION: Contrast-enhanced MRI is capable of accurately diagnosing acute appendicitis while detecting many alternative entities of abdominal pain, and it allows good visualization of the appendix. Further evaluation is needed to determine whether contrast-enhanced MRI provides an advantage over non-enhanced MRI for imaging evaluation of acute abdominal pain in the pediatric population.

Feasibility and accuracy of laryngeal ultrasound for the assessment of vocal cord mobility in children.

OBJECTIVE: Transcutaneous laryngeal ultrasonography has been utilized to document vocal cord mobility in the adult perioperative thyroidectomy setting with variable success. The aims of this study were to assess the feasibility of using transcutaneous ultrasound in vocal cord visualization in children, to determine the accuracy in detecting vocal cord immobility compare to flexible laryngoscopy, and to identify any barriers that may affect its utility. METHODS: This is a prospective blinded-assessor study. Transcutaneous laryngeal ultrasound and flexible laryngoscopy were performed in two pediatric settings: perioperative thyroid surgery and inpatient consultation for airway, voice, or swallowing concerns. The video recordings of ultrasound and laryngoscopy were assessed by two fellowship-trained pediatric otolaryngologists independently. RESULTS: A total of 83 paired laryngoscopy and ultrasound assessments were performed, 39 for perioperative thyroidectomy and 44 for inpatient consultation. The majority had normal vocal cord mobility (65/83, 78%), while 16 had unilateral cord palsy and 2 had bilateral palsy. The vocal cords were successfully visualized on ultrasound in 82/83 (99%) evaluations. Compared to gold standard laryngoscopy, the sensitivity for diagnosing vocal cord palsy on ultrasound was 53-83%, and the specificity was 97-98%. The sensitivity for detecting asymmetric vocal cord movement was 56-88%, and the specificity was 99%. The agreement between ultrasound and laryngoscopy diagnoses were 87-94% (κ = 0.58-0.83, moderate to near perfect agreement). The intra-rater agreement was 95-100% (κ = 0.64-1.0), and inter-rater agreement was 90% (κ = 0.66). Both subjects with bilateral vocal cord palsy had incorrect ultrasound diagnoses by both observers. CONCLUSION: We were able to utilize transcutaneous laryngeal ultrasound to successfully visualize vocal cord mobility in almost all pediatric patients with a high degree of specificity. The modality has limitations in the assessment of bilateral vocal cord palsy, and its sensitivity was observer-dependent.

Activated phosphoinositide 3-kinase δ syndrome associated with nephromegaly, growth hormone deficiency, bronchiectasis: a case report.

BACKGROUND: Activated phosphoinositide 3-kinase (PI3K) δ syndrome (APDS) is a rare form of primary immunodeficiency with 243 known cases reported in the literature. Known findings associated with the condition include recurrent sinusitis and bronchitis, bronchiectasis, immune cytopenias, mild developmental delay, splenomegaly, and lymphadenopathy. We report the case of a child with APDS accompanied by unique clinical features: nephromegaly and growth hormone deficiency with associated pituitary anatomic abnormality. CASE PRESENTATION: The patient is a nine-year-old boy with a heterozygous de novo variant in phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit δ (p.E1021K), previously reported in association with APDS. Our patient, who had no family history of immunodeficiency, exhibits classic findings of this syndrome but also has unique features that extend the phenotypic spectrum of this disorder. At 5 years of age, the patient showed marked growth deceleration and was demonstrated to have growth hormone (GH) deficiency with associated pituitary anatomic abnormality. He started GH therapy with an excellent response. He additionally has bilateral nephromegaly of unclear etiology, microscopic hematuria and proteinuria, asthma, and has developed left hip pain with arthrocentesis consistent with oligoarticular juvenile idiopathic arthritis. At age nine, the patient was referred to genetics and whole exome sequencing revealed APDS. Though there was initial concern that GH may increase risk for malignancy as GH signals through the PI3K pathway, he was allowed to continue treatment as the PI3K pathway was considered constitutively active at baseline. CONCLUSIONS: Our patient's unique presentation adds to the clinical information regarding APDS, demonstrates the utility of genetic testing and illustrates the importance of a multidisciplinary collaborative approach in managing this complex syndrome.

AN 8-YEAR-OLD CALIFORNIA GIRL WITH ASYMPTOMATIC HEPATIC CYSTS.

Echinococcus infections are rare in the United States but may present a growing public health threat. We present the case of an 8-year-old female patient from Southern California who was diagnosed with hepatic echinococcosis after the incidental discovery of large hepatic cysts.

Balloons for kids: Anatomic candidacy and optimal catheter size for pediatric resuscitative endovascular balloon occlusion of the aorta.

BACKGROUND: Resuscitative endovascular balloon occlusion of the aorta (REBOA) is a potential adjunct in pediatric trauma patients with noncompressible truncal and pelvic hemorrhage; however, there are little data evaluating the anatomic considerations of REBOA in children. We evaluated the vascular dimensions and anatomic limitations of using REBOA in children. METHODS: Computed tomography scans of pediatric patients performed between February 2016 and October 2019 were retrospectively reviewed by two investigators. Vascular measurements included diameters of aorta zones I and III, common iliac arteries, external iliac arteries, and common femoral arteries (CFAs), and distances between access site (CFA) and aorta zones I and III. Measurements were grouped within Broselow categories, based upon patient height. Interrater reliability for measurements was determined using intraclass correlation coefficients. Vascular dimensions were correlated with the patient's height, weight, and body mass index using linear regression analysis. RESULTS: A total of 557 computed tomography scans met the inclusion criteria and were reviewed. Measurements of vessel diameter and distance from the CFA to aorta zones I and III were determined and grouped by Broselow category. Patient age ranged from 0 to 18 years, with a male to female ratio of 1:1. Overall interrater reliability of vessel measurements was good (average intraclass correlation coefficient, 0.90). Vessel diameter had greatest correlation with height (R2 = 0.665, aorta zone I; R2 = 0.611, aorta zone III) and poorly correlated with body mass index (R2 = 0.318 and R2 = 0.290, respectively). CONCLUSION: This study represents the largest compilation of REBOA-related pediatric vessel diameter measurements and the first to provide data on distance between access site and balloon deployment zones. Based on our findings, the 7-Fr REBOA catheter would be appropriate for the Black, Green, and Orange Broselow categories, and a 4-Fr REBOA catheter would be warranted for Yellow, White, and Blue Broselow categories. LEVEL OF EVIDENCE: Prognostic and epidemiological, level III.

Diagnostic utility of fine needle aspiration cytology in pediatric thyroid nodules based on Bethesda Classification.

OBJECTIVES: The Bethesda system for reporting cytopathology (TBSRTC) has been widely adopted in the management of thyroid nodules. Based on the limited pediatric data available, the implied malignancy risk for each of the categories may be significantly different in pediatrics vs. adults, especially in the indeterminate categories (Bethesda Class III or IV). We report the diagnostic utility of fine needle aspiration (FNA) biopsy at our institution based on the Bethesda system and the risk of malignancy in each category. METHODS: We retrospectively reviewed all patients who underwent a thyroid FNA at our tertiary pediatric hospital from 12/1/2002 to 11/30/2018. FNA results were classified according to TBSRTC. Patient demographics, cytology, histopathology, radiological and clinical follow-ups were examined. RESULTS: A total of 171 patients were included with 203 cytological samples. Average age at initial FNA was 14.7 years (range 6.9-18.6 years). The numbers of nodules reported for Bethesda categories I-VI were 29, 106, 22, 14, 6 and 26, respectively, and the rate of malignancy was: 13.8, 4.7, 22.7, 35.7, 83.3 and 100%, respectively. Use of ultrasound guidance reduced the non-diagnostic rate from 38.1 to 11.5%. Introduction of on-site adequacy testing further reduced the non-diagnostic rate to 6.5% since 2014. CONCLUSIONS: The risk of malignancy for thyroid nodules in this pediatric cohort is higher than reported in adults. However, rates described here are much closer to adult ranges than previously published pediatric cohorts. The addition of adequacy testing improved the non-diagnostic rate of FNA procedures performed with ultrasound guidance.

Nusinersen treatment of older children and adults with spinal muscular atrophy.

The purpose of this study was to determine how effective administration of nusinersen was at improving motor function in older adolescent and adult patients with spinal muscular atrophy, using standardized motor outcome measures. Data were gathered through a retrospective chart review of older spinal muscular atrophy patients (ages 5-58) being treated at Rady Children's Hospital and the University of California, San Diego with nusinersen from April 2017-June 2019. Linear mixed effects analyses found that, for older children and adult patients with SMA 1, 2, and 3, motor scores as measured by the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders for non-sitters improved by 6 points (p = .01) and the Hammersmith Infant Neurological Examination-2 by 2.6% (p = .008) over the 22-month study period. Over the same period, sitters improved on the Revised Upper Limb Module by 4.4 points (p = .02) and on the Hammersmith Functional Motor Scale-Expanded by 3.3% (p = .00005) post treatment with nusinersen. Older spinal muscular atrophy patients (5-58 years) being treated with nusinersen at our institutions are improving. Not only have symptoms stabilized, but their motor function has shown incremental improvements. Based on the results of this study, we suggested that nusinersen is well-tolerated and efficacious when treating older children and adult patients with spinal muscular atrophy 1, 2, and 3.

Ovarian volume ratio is a reliable predictor of ovarian torsion in girls without an adnexal mass.

PURPOSE: The aims of this study were to identify ultrasound-based predictors of ovarian torsion in girls without an adnexal mass and establish a set of normal values for ovarian volume ratio (OVR). METHODS: A retrospective review was performed of all premenarchal patients ≥3 years of age with a normal pelvic ultrasound between January 2016 and January 2019. A comparison group of premenarchal girls presenting between 2011 and 2019 with torsion in the absence of an adnexal mass was utilized. RESULTS: Five-hundred and four premenarchal girls underwent pelvic ultrasound evaluation with a normal examination. The mean OVR was 1.6 ±â€¯0.7 (range 1.0-6.5). OVR did not vary with age (r = -0.06) as compared to ovarian width which increased steadily with age (r = 0.53, p < 0.001). OVR was increased in girls with torsion (7.6 vs 1.4, p < 0.0001), and by receiver operating characteristic (ROC) analysis a cutoff value of >2.5 demonstrated the best diagnostic accuracy of any predictive variable (sensitivity 100%, specificity 94%, AUC 0.991, p < 0.001). CONCLUSIONS: OVR is an excellent predictor of ovarian torsion in premenarchal girls without an adnexal mass. Unlike ovarian width, OVR does not increase with age, and a cutoff OVR > 2.5 demonstrates high sensitivity and specificity for identifying ovarian torsion in this population. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: Level III.

Indeterminate orbital masses: restricted diffusion at MR imaging with echo-planar diffusion-weighted imaging predicts malignancy.

PURPOSE: To determine whether magnetic resonance (MR) imaging with diffusion-weighted (DW) imaging can help discriminate between radiologically indeterminate benign and malignant orbital masses and to identify optimal apparent diffusion coefficient (ADC) thresholds for such discrimination. MATERIALS AND METHODS: Informed consent was waived for this HIPAA-compliant institutional review board-approved retrospective study. Forty-seven orbital masses imaged with echo-planar DW imaging were identified in 47 patients (25 female patients, 22 male patients; average age, 35 years). A fellowship-trained orbital surgeon determined reference-standard diagnoses on the basis of chart review, and a neuroradiology fellow and senior neuroradiologist who were blinded to the diagnoses selected a region of interest for each lesion by consensus. ADC was calculated from signal intensity on DW images obtained with b = 1000 and b = 0 sec/mm(2). Lesion ADC was also compared with that of normal-appearing white matter (ADC ratio). The Student t test was used to compare groups. Receiver operating characteristic analysis was performed. Intraobserver agreement was assessed with a repeat data collection. RESULTS: Malignant lesions had lower ADCs than benign lesions, irrespective of patient age (P < .02) and in adults specifically (P < .05). Lymphomas had lower ADCs than pseudotumors (P < .001). An ADC of less than 1.0 x 10(-3) mm(2)/sec and an ADC ratio of less than 1.2 were optimal for predicting malignancy (sensitivity, 63% for both; specificity, 84% and 90%, respectively; and accuracy, 77% and 81%, respectively). Lymphoma was differentiated from pseudotumor with 100% accuracy (in 16 of 16 cases) by using these values. Infiltrative lesions that were hypointense on T2-weighted images were better characterized with DW imaging than lesions that were hyperintense or well defined. CONCLUSION: Echo-planar DW MR imaging can help characterize indeterminate orbital masses.

Skeletal and Brain Abnormalities in Fucosidosis, a Rare Lysosomal Storage Disorder.

Fucosidosis is a rare genetic lysosomal storage disorder caused by a deficiency in alpha- L-fucosidase. We present a case of a 4-year, 11-month-old girl with developmental delay, as well as skeletal and brain abnormalities as shown on X-ray and MRI. Her spinal X- rays demonstrated lumbar kyphosis and anterior beaking of lumbar vertebral bodies. Lower iliac segment constriction, increased angulation of the acetabular roof, and widening of the ribs were apparent on abdominal X-ray. Her brain MRI illustrated symmetric T1 hyperintensity and T2 hypointensity of the bilateral globi pallidi. The case report highlights clinical and imaging findings of this rare disease.

Carotid artery pseudoaneurysm: A rare complication following tonsillectomy.

Tonsillectomy is one of the most common surgical procedures performed in children. The most frequent complications are dehydration and bleeding. We present the case of a 6 year old child who developed an internal carotid artery pseudoaneurysm following elective tonsillectomy, necessitating urgent coil embolization and stenting. This is the first reported case in the pediatric population of a vascular injury that manifested in a delayed fashion (6 months) after routine tonsillectomy, and is also one of the youngest reported cases. It is imperative for the otolaryngologist to be aware of this rare complication.

Imaging Pediatric Vascular Lesions.

Vascular anomalies are commonly encountered in pediatric and dermatology practices. Most of these lesions are benign and easy to diagnose based on history and clinical exam alone. However, in some cases the diagnosis may not be clear. This may be of particular concern given that vascular anomalies may occasionally be associated with an underlying syndrome, congenital disease, or serious, life-threatening condition. Defining the type of vascular lesion early and correctly is particularly important to determine the optimal approach to management and treatment of each patient. The care of pediatric patients often requires collaboration from a multitude of specialties including pediatrics, dermatology, plastic surgery, radiology, ophthalmology, and neurology. Although early characterization of vascular lesions is important, consensus guidelines regarding the evaluation and imaging of vascular anomalies does not exist to date. Here, the authors provide an overview of pediatric vascular lesions, current classification systems for characterizing these lesions, the various imaging modalities available, and recommendations for appropriate imaging evaluation.

A clinical update and radiologic review of pediatric orbital and ocular tumors.

While pediatric orbital tumors are most often managed in tertiary care centers, clinicians should be aware of the signs of intraocular and orbital neoplasms. In the pediatric population, a delay in diagnosis of orbital and intraocular lesions, even if benign, can lead to vision loss and deformity. Intraocular lesions reviewed are retinoblastoma, medulloepithelioma, and retinal astrocytic hamartoma. Orbital neoplasms reviewed are rhabdomyosarcoma, neuroblastoma metastases, optic pathway glioma, plexiform neurofibroma, leukemia, lymphoprolipherative disease, orbital inflammatory syndrome, dermoid and epidermoid inclusion cysts, and Langerhans' cell histiocytosis. Vascular lesions reviewed are infantile hemangioma and venous lymphatic malformation. In conjunction with clinical examination, high-resolution ophthalmic imaging and radiologic imaging play an important role in making a diagnosis and differentiating between benign and likely malignant processes. The radiologic imaging characteristics of these lesions will be discussed to facilitate prompt diagnosis and treatment. The current treatment modalities and management of tumors will also be reviewed.

Primary tuberculosis in a malnourished adolescent.

Although the overall prevalence of tuberculosis has decreased in the United States, with the increasing prevalence of tuberculosis globally, higher rates of tuberculosis in some states and localities have been reported, with some component probably related to immigrant populations. We report a case of primary pulmonary tuberculosis in a malnourished adolescent.

Delayed presentation of traumatic infrapopliteal arteriovenous fistula and pseudoaneursym in a 10-year-old boy managed by coil embolization.

Blunt traumatic arteriovenous fistulae of the extremities are rare in children. We report the case of a 10-year-old boy with a traumatic infrapopliteal arteriovenous fistula and concomitant pseudoaneurysm diagnosed 2 months after he was struck by a car. It was definitively managed with coil embolization. Postprocedure, the vascular anatomy of the patient's right calf was well preserved. He had complete resolution of his symptoms immediately after the intervention and continues to do well 6 months later.

Multifocal epithelioid hemangioendothelioma in a 16-year-old boy.

We report on a 16-year-old white male presenting with a 4-month history of syncopal episodes and occasional headaches. The CT and MRI studies revealed numerous lytic lesions of the skull base and cervical spine; subsequently, similar lesions were demonstrated in all areas of the skeleton, and CT showed numerous lesions in the lungs, liver, and kidneys. Excisional biopsy from several sites confirmed the diagnosis of epithelioid hemangioendothelioma. We conclude that epithelioid hemangioendothelioma should be added to the differential diagnosis for lytic lesions of bone that are clustered in the same anatomic region and that might also present with visceral involvement.