Long-term follow-up and liver outcomes in children with cystic fibrosis and nodular liver on ultrasound in a multi-center study.

BACKGROUND: Nodular liver (NOD) in cystic fibrosis (CF) suggests advanced CF liver disease (aCFLD); little is known about progression of liver disease (LD) after detection of sonographic NOD. METHODS: Clinical, laboratory, and ultrasound (US) data from Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in CFLD Study participants with NOD at screening or follow-up were compared with normal (NL). Linear mixed effects models were used for risk factors for LD progression and Kaplan-Meier estimator for time-to-event. RESULTS: 54 children with NOD (22 screening, 32 follow-up) and 112 NL were evaluated. Baseline (BL) and trajectory of forced expiratory volume, forced vital capacity, height/BMI z-scores were similar in NOD vs NL. Platelets were lower in NOD at BL (250 vs 331×103/microL; p < 0.001) and decreased by 8600/year vs 2500 in NL. Mean AST to Platelet Ratio Index (1.1 vs 0.4; p < 0.001), Fibrosis-4 Index (0.4 vs 0.2, p < 0.001), and spleen size z-score (SSZ) [1.5 vs 0.02; p < 0.001] were higher in NOD at BL; SSZ increased by 0.5 unit/year in NOD vs 0.1 unit/year in NL. Median liver stiffness (LSM) by transient elastography was higher in NOD (8.2 kPa, IQR 6-11.8) vs NL (5.3, 4.2-7, p < 0.0001). Over 6.3 years follow-up (1.3-10.3), 6 NOD had esophageal varices (cumulative incidence in 10 years: 20%; 95% CI: 0.0%, 40.0%), 2 had variceal bleeding, and 2 underwent liver transplantation; none had ascites or hepatic encephalopathy. No NL experienced liver-related events. CONCLUSIONS: NOD developed clinically evident portal hypertension faster than NL without worse growth or lung disease.

Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis.

Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (rs =  - 0.28, P = 0.0071) and positive correlation with aspartate aminotransferase-to-platelet ratio index (rs = 0.38, P = 0.0002), Fibrosis-4 index (rs = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; rs = 0.35, P = 0.0017), GGT-to-platelet ratio (rs = 0.35, P = 0.003), and US spleen size z-score (rs = 0.27, P = 0.0073). Conclusion: VCTE is associated with US patterns and conventional markers in patients with liver disease with CF.

Challenges and Opportunities for Continued Success and Growth of Pediatric Interventional Radiology: A Communiqué From the Society for Pediatric Interventional Radiology.

OBJECTIVE: Pediatric interventional radiology (PIR) is a dynamic and growing subspecialty. We will detail our need to evolve like the emerging therapies and innovative imaging options that we provide to children. CONCLUSION: New interventional radiology training pathways, maintaining competency with small volumes of complex procedures, limited availability of pediatric-specific equipment, questions about the safety of sedation in the developing brain, and the dearth of PIR opportunities outside of North America provide challenges and opportunities for the vibrant community of PIR practitioners.

Pediatric interventional radiology workforce survey: 10-year follow-up.

BACKGROUND: Pediatric interventional radiology is a distinct subspecialty differing from both pediatric diagnostic radiology and adult interventional radiology. We conducted a workforce survey in 2005 to evaluate the state of pediatric interventional radiology at that time. Since then there have been many advancements to the subspecialty, including the founding of the Society for Pediatric Interventional Radiology (SPIR). OBJECTIVE: To evaluate the current state of the pediatric interventional radiology workforce and compare findings with those of the initial 2005 workforce survey. MATERIALS AND METHODS: We sent a two-part survey electronically to members of SPIR, the Society for Pediatric Radiology (SPR), the Society of Chairmen of Radiology in Children's Hospitals (SCORCH) and the Society of Interventional Radiology (SIR). Part 1 focused on individual practitioners (n=177), while part 2 focused on group practices and was answered by a leader from each group (n=88). We examined descriptive statistics and, when possible, compared the results to the study from 2005. RESULTS: A total of 177 individuals replied (a 331% increase over the first study) and 88 pediatric interventional radiology (IR) service sites responded (a 131.6% increase). Pediatric IR has become a more clinically oriented specialty, with a statistically significant increase in services with admitting privileges, clinics and performance of daily rounds. Pediatric IR remains diverse in training and practice. Many challenges still exist, including anesthesia/hospital support, and the unknown impact of the new IR residency on pediatric IR training, although the workforce shortage has been somewhat alleviated, as demonstrated by the decreased mean call from 165 days/year to 67.2 days/year. CONCLUSION: Pediatric interventional radiology practitioners and services have grown significantly since 2005, although the profile of this small subspecialty has changed and some challenges remain.

CT outperforms radiographs at a comparable radiation dose in the assessment for spondylolysis.

BACKGROUND: Lumbar spondylolysis, a unilateral or bilateral fracture at pars interarticularis, is a common cause of low back pain in children. The initial imaging study in the diagnosis of lumbar spondylolysis has historically been lumbar spine radiographs; however, radiographs can be equivocal or false-negative. Definitive diagnosis can be achieved with computed tomography (CT), but its use has been limited due to the dose of ionizing radiation to the patient. OBJECTIVE: By limiting the z-axis coverage to the relevant anatomy and optimizing the CT protocol, we are able to provide a definitive diagnosis of fractures of the pars interarticularis at comparable or lower radiation dose than commonly performed lumbar spine radiographs. As there is no gold standard for the diagnosis of spondylolysis besides surgery, we compared interobserver agreement and degree of confidence to determine which modality is preferable. MATERIALS AND METHODS: Sixty-two patients with low back pain ages 5-18 years were assessed for the presence of spondylolyis. Forty-seven patients were evaluated by radiography and 15 patients were evaluated by limited field-of-view CT. Both radiographic and CT examinations were assessed anonymously in random order for the presence or absence of spondylolyisis by six raters. Agreement was assessed among raters using a Fleiss Kappa statistic for multiple raters. RESULTS: CT provided a significantly higher level of agreement among raters than radiographs (P < 0.001). The overall Kappa for rater agreement with radiographs was 0.24, 0.34 and 0.40 for 2, 3 or 4 views, respectively, and 0.88 with CT. CONCLUSION: Interobserver agreement is significantly greater using limited z-axis coverage CT when compared with radiographs. Radiologist confidence improved significantly with CT compared to radiographs regardless of the number of views.

Familiarity with current practices of granting and maintaining privileges in pediatric interventional radiology--a worldwide survey of the members of the Society for Pediatric Interventional Radiology (SPIR).

BACKGROUND: Physician credentialing is a complex process driven by the demand for quality improvement in health care. In the U.S., the Joint Commission Standard of 2007 has tied hospital accreditation to credentialing through mandated use of the Focused Professional Practice Evaluation (FPPE) and Ongoing Professional Practice Evaluation (OPPE). OBJECTIVE: To assess pediatric interventional radiologists' knowledge of how institutions grant them privileges. MATERIALS AND METHODS: Members of the Society for Pediatric Interventional Radiology (SPIR) were sent a web-based survey regarding credentialing. RESULTS: Of 122 members from 19 countries, 81 (66%) responded, and of these 81, 59 (73%) were familiar with their hospital's privileging process. Of 49 U.S. respondents and 32 non-U.S. respondents, 37 (76%) and 17 (53%), respectively, stated that interventional radiology credentialing was different from diagnostic radiology credentialing. Of the 49 U.S. respondents, 24 (49%) reported an OPPE, and of the 32 non-U.S. respondents, 8 (25%) reported an ongoing evaluation. The U.S. OPPE is performed at shorter intervals than its international equivalent. CONCLUSION: Four years after the Joint Commission defined the FPPE and OPPE, separate credentialing of pediatric interventional radiology from pediatric diagnostic radiology is more likely in the U.S. than internationally, and U.S. pediatric interventional radiologists are more likely to have a defined ongoing professional evaluation and to be evaluated every 6 months or more frequently. There are many SPIR members who do not know how they obtain privileges and/or are not knowingly subject to an OPPE. This lack of knowledge may affect future education of interventional radiologists as well as the definition of pediatric interventional radiology practices within individual institutions.

An 18-year-old man with fenestrated vertebral arteries, recurrent stroke and successful angiographic coiling.

Fenestration of vertebral arteries has been reported in association with thromboembolic brain infarctions. However, few cases have been reported in which recurrent infarction occurred in spite of adequate anticoagulation. We report a young man with fenestrated vertebral arteries and stroke who failed to respond to standard anticoagulation therapy but did well with angiographic coil obliteration of an abnormal vertebral segment. An 18-year-old left-handed man presented with acute onset of dizziness and headache. No trauma or other stroke risk factors were identified. Left cerebellar infarction was seen on CT, but the cause could not be identified by brain and neck MRI, MRA, or CTA. Bilateral fenestrated vertebral arteries were identified with conventional angiography. Although the patient recovered fully and was treated with anticoagulation, he suffered a recurrent stroke 1 month later involving the right cerebellum while he was on a therapeutic dose of warfarin. Repeat arteriography showed a spontaneous dissection within one of the fenestrated vertebral segments. Since receiving angiographic coil obliteration of the pathologic segment, he has been free of all symptoms. We conclude that the patient sustained recurrent thromboembolic events in his posterior circulation due to spontaneous dissection within a fenestrated vertebral artery segment. Conventional angiography and emergent interventional embolization were essential to his diagnostic evaluation and therapeutic intervention.

Giant cystic abdominal masses in children.

In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis.

Successful thoracoscopic lung biopsy in children utilizing preoperative CT-guided localization.

BACKGROUND/PURPOSE: Although thoracoscopy in children is a well-accepted technique, there are limitations to this approach. Small lung nodules or those not located on the pleural surface often require an open thoracotomy. The purpose of this report is to evaluate the utility of computed tomography (CT) guided needle localization of lung nodules in children followed by thoracoscopic resection. METHODS: From 1997 to 2000, 13 thoracoscopic procedures were performed on 12 children who presented with small pulmonary nodules (<1 cm) or nodules deep to the pleural surface. Preoperative needle localization was performed by injecting methylene blue under CT guidance. Patients then were taken directly to the operating room for thoracoscopic resection. RESULTS: All 13 procedures were completed successfully thoracoscopically. Twelve yielded diagnostic pathologic findings as well as therapeutic resections in 7. All patients underwent extubation in the operating room, and chest tubes were removed within 36 hours. No thoracotomies were performed, and there were no complications. CONCLUSIONS: Preoperative localization utilizing CT guidance is safe and beneficial in children with pulmonary nodules less than 1 cm in size and those located deep to the pleural surface. This technique allows the surgeon to perform thoracoscopy for diagnosis and therapeutic resection of these lesions rather than submitting patients to the morbidity of a thoracotomy.