Stephen F. Simoneaux

What physicians think about the need for informed consent for communicating the risk of cancer from low-dose radiation

BackgroundThe National Institute of Environmental Health Sciences, a subsidiary of the Food and Drug Administration, has declared that X-ray radiation at low doses is a human carcinogen.ObjectiveThe purpose of our study was to determine if informed consent should be obtained for communicating the risk of radiation-induced cancer from radiation-based imaging.Materials and methodsInstitutional review board approval was obtained for the prospective survey of 456 physicians affiliated with three tertiary hospitals by means of a written questionnaire. Physicians were asked to state their subspecialty, number of years in practice, frequency of referral for CT scanning, level of awareness about the risk of radiation-induced cancer associated with CT, knowledge of whether such information is provided to patients undergoing CT, and opinions about the need for obtaining informed consent as well as who should provide information about the radiation-induced cancer risk to patients. Physicians were also asked to specify their preference among different formats of informed consent for communicating the potential risk of radiation-induced cancer. Statistical analyses were performed using the chi-squared test.ResultsMost physicians stated that informed consent should be obtained from patients undergoing radiation-based imaging (71.3%, 325/456) and the radiology department should provide information about the risk of radiation-induced cancer to these patients (54.6%, 249/456). The informed consent format that most physicians agreed with included modifications to the National Institute of Environmental Health Services report on cancer risk from low-dose radiation (20.2%, 92/456) or included information on the risk of cancer from background radiation compared to that from low-dose radiation (39.5%, 180/456).ConclusionMost physicians do not know if patients are informed about cancer risk from radiation-based imaging in their institutions. However, they believe that informed consent for communicating the risk of radiation-induced cancer should be obtained from patients undergoing radiation-based imaging.

Cystic dysplasia of the testis associated with multicystic dysplastic kidney.

Cystic dysplasia of the testis (CDT) is a rare congenital defect characterized by the formation of multiple irregular cystic spaces in the mediastinum testis. Co-existent genitourinary lesions have commonly been associated with this lesion and have included absence of the ipsilateral kidney, duplication anomalies, and cryptorchidism. We describe the first case in which multicystic dysplastic kidney (MCDK) is associated with CDT.

Role of PET/CT in congenital histiocytosis

Congenital Langerhans cell histiocytosis (LCH) is a rare, but often severe, form of LCH. Although a more benign single-system congenital LCH has been described, most cases present as multisystem disease with poorer prognosis and are often treated with systemic chemotherapy. Imaging plays a central role in diagnosis, initial staging and assessment of treatment response. PET/CT is increasingly utilized for pediatric LCH. We report a unique case with PET/CT used as an imaging tool in staging and evaluating treatment response in congenital multisystem LCH.

Violating traditional NPO guidelines with PO contrast before sedation for computed tomography.

Background Administration of PO contrast within 2 hours before sedation for abdominal computed tomography (CT) is controversial because it violates American Society of Anesthesiologists guidelines that recommend 2 hours of fasting for clear fluids before sedation. Objective This study aimed to review the administration of PO contrast for patients undergoing propofol sedation for abdominal CT and to evaluate the impact of violation of traditional 2-hour NPO guidelines on care. Methods Sedation records were reviewed from January 2010 to October 2011 from all patients who received PO contrast within 2 hours of propofol sedation for abdominal CT. A control group of patients receiving intravenous contrast only before propofol sedation and abdominal CT was reviewed. Demographics, time between PO contrast administration and sedation, and amount of PO contrast were recorded. Outcome measures including success of sedation and adverse events (vomiting, suctioning, use of O2 or advanced airway, unplanned admissions) were recorded. Results Eighty-five patients received PO contrast within 2 hours before sedation for abdominal CT; 21 controls were identified. No differences in demographics or outcome measures were seen. No significant differences were seen in rates of interventions or adverse outcomes between groups. Median time between the successive PO contrast doses and propofol administration was 1.6 hours and 0.6 hours. Conclusions Administering oral contrast material within 2 hours of propofol sedation for abdominal CT in children seems to be relatively safe compared with those sedated after traditional NPO time frames.

Blastic plasmacytoid dendritic cell neoplasm presenting as a subcutaneous mass in an 8-year-old boy

Blastic plasmacytoid dendritic cell neoplasm (also referred to as CD4+/CD56+ hematodermic neoplasm) is a rare hematological malignancy typically seen in older adults. The disease presents with nonspecific cutaneous lesions and advances toward a fatal leukemic phase despite an often favorable initial response to chemotherapy. Fewer than 200 cases have been documented, and it is exceedingly rare in children. We report a case of histopathologically proven blastic plasmacytoid dendritic cell neoplasm arising in an otherwise healthy and asymptomatic 8-year-old boy who noticed a painless mass within the subcutaneous tissues below the left calf. In addition to contrast-enhanced MRI, the imaging work-up included PET/CT, which showed metastatic involvement within left inguinal and retroperitoneal lymph nodes. The case is notable both for the young age of the patient and for the absence of characteristic cutaneous manifestations of this disease.

Laryngeal and tracheal anomalies in an infant with oral-facial-digital syndrome type VI (Váradi-Papp): report of a transitional type

The oral-facial-digital syndromes (OFDS) comprise a group of disorders involving malformations of the mouth, face, and digits. There are 13 subtypes of the OFDS, and much overlap exists among OFDS patients. Distinct syndromes such as Joubert and Pallister-Hall display many of the same features. This report describes an infant with abnormalities including a hypoplastic/absent cerebellar vermis and forked third metacarpals, consistent with a diagnosis of OFDS type VI (Váradi-Papp). The girl’s abnormalities also included malformations of the larynx and trachea, findings never before described in type VI but described in other OFDS subtypes and similar syndromes. Our patient represents a transitional OFDS type, further supporting evidence of a common molecular pathway among these disorders. This report highlights the importance of the radiologist’s role in diagnosis.

Quality measures and pediatric radiology: suggestions for the transition to value-based payment

Recent political and economic factors have contributed to a meaningful change in the way that quality in health care, and by extension value, are viewed. While quality is often evaluated on the basis of subjective criteria, pay-for-performance programs that link reimbursement to various measures of quality require use of objective and quantifiable measures. This evolution to value-based payment was accelerated by the 2015 passage of the Medicare Access and CHIP (Children’s Health Insurance Program) Reauthorization Act (MACRA). While many of the drivers of these changes are rooted in federal policy and programs such as Medicare and aimed at adult patients, the practice of pediatrics and pediatric radiology will be increasingly impacted. This article addresses issues related to the use of quantitative measures to evaluate the quality of services provided by the pediatric radiology department or sub-specialty section, particularly as seen from the viewpoint of a payer that may be considering ways to link payment to performance. The paper concludes by suggesting a metric categorization strategy to frame future work on the subject.

Risk factors and surveillance for reduced bone mineral density in pediatric cancer survivors

Pediatric cancer survivors are at increased risk of developing low bone mineral density (BMD) due to cancer treatment. This study assessed the yield of screening for low BMD in pediatric‐aged cancer survivors as per the Childrens Oncology Group Long‐Term Follow‐Up (COG‐LTFU) Guidelines, which recommend screening survivors who received steroids, methotrexate, or hematopoietic cell transplant (HCT).

Brown fat necrosis with calcifications in the newborn: Risk factors, radiographic findings, and clinical course

Objective: To describe the radiographic appearance of subclinical calcified brown fat necrosis and the associated clinical and laboratory findings. Materials and Methods: Picture Archiving and Communications Sytem (PACS) was searched using keywords “soft tissue calcification” and “chest.” The clinical record was searched for prior cardiac surgery, bypass, Extracorporeal Membrane Oxygentation (ECMO) and prostaglandin use. Age when calcifications were first detected, location, resolution, and associated laboratory abnormalities were recorded. Results: Nine patients were identified. None had skin lesions. All patients had congenital heart disease and had experienced cardiac/respiratory arrest and/or severe hypotension 1–6 weeks before soft tissue calcifications occurred. Calcifications resolved by 9 weeks to 5 months in 3 patients. The remaining were either deceased or lacked follow-up imaging. Renal ultrasound was performed in all but 1 patient. Nephrocalcinosis was only seen in 1 patient. Conclusion: Brown fat necrosis is subclinical, diagnosed on plain film, and likely self-limited. It occurs in term and preterm infants who have undergone significant systemic stress and carries a poor prognosis.

Malakoplakia and Primary Immunodeficiency

Malakoplakia, a rare granulomatous disease caused by impaired macrophage response, has been reported only rarely in children. We report 3 unique cases, with lesions occurring in unusual locations in children with primary immune deficiencies.