Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease. We report a case of a term boy presenting with tachypnea at birth requiring supplemental oxygen. Chest radiographs followed by high-resolution CT (HRCT) demonstrated hyperinflation and diffuse interstitial markings interspersed with multiple cystic spaces. An open lung biopsy demonstrated a minor component of PIG superimposed upon poor alveolarization. PIG in the setting of lung growth abnormality might be more common than previously described. Additionally, radiographic findings associated with most pediatric interstitial lung diseases are nonspecific, and histopathologic correlation is essential for diagnosis.

High-quality cancer registry data are essential for assessing trends in incidence rates. This study evaluated the consistency of brain tumor surveillance data using a random sample of cases from the Connecticut Tumor Registry. Three neuropathologists independently and blindly reviewed tumor slides from 204 cases and a nosologist blindly reviewed and assigned International Classification of Diseases for Oncology (ICD-O) codes to 326 cases. For the pathology review, absolute concordance was as high as 81% for all primary brain tumors. Absolute concordance rates were high for nerve sheath (89%), meningioma (95%), and pituitary (95%) tumors. Rates were much lower for malignant tumors. ICD-O coding of malignant brain tumors is of relatively high quality with the exception of mixed gliomas and unspecified tumors. A high level of consistency for nonmalignant brain tumor diagnoses suggests that rates for these tumors, when actively reported to a surveillance system, can be of high quality.

Background and Relevance: Intracranial atherosclerosis is responsible for 70,000 ischemic strokes each year in the USA. Noninvasive testingsuch as transcranial Doppler ultrasound (TCD) and magnetic resonance angiography (MRA) to identify intracranial atherosclerosis is in widespread use, but has not been rigorously validated against the gold standard, catheter angiography. The recently NIH-funded Warfarin-Aspirin Symptomatic Intracranial Disease (WASID) trial will compare warfarin with aspirin for stroke prevention in patients with intracranial atherosclerosis. WASID requires performance of angiography along with TCD and MRA, providing an opportunity to critically evaluate these noninvasive tests. Main Objective: The purpose of the Stroke Outcomes and Neuroimaging of Intracranial Atherosclerosis (SONIA) study is to develop the noninvasive diagnosis of intracranial atherosclerosis. The primary aim of SONIA is to define velocity values on TCD and anatomic abnormalities on MRA that identify severe (50–99%) intracranial stenosis of large, proximal arteries seen on catheter angiography. SONIA will define the criteria, or ‘cutpoints’, for an abnormal TCD or MRA and show that they perform with a reliable positive predictive value (PPV). Study Design: SONIA will be conducted in collaboration with WASID. Study-wide cutpoints defining positive TCD and MRA have been developed and reviewed by the site investigators of WASID. Hard copy angiography, TCD and MRA generated in WASID will be centrally read in SONIA. TCD and MRA cutpoints seek to achieve a target PPV of 80% for the identification of severe intracranial stenosis on angiography. Conclusions: Central readings will be used to validate the cutpoints and to develop measures of negative predictive value, and inter- and intra-observer variability. Sensitivity and specificity will be determined after adjustment for verification bias and employed in receiver-operator characteristic analyses. SONIA will use these techniques to develop TCD and MRA cutpoints that minimize the clinical consequences of test errors occurring in the noninvasive evaluation of patients with suspected intracranial atherosclerosis.

Uterine arteriovenous malformation (AVM) is a rare but potentially lifethreatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manor are essential because instrumentation that is often used for other sources of uterine bleeding can lead to massive hemorrhage. Although angiography remains the gold standard for diagnosis, ultrasound (US) and magnetic resonance imaging (MRI) are the modalities of choice for the evaluation of a suspected AVM. US and MRI cannot only accurately define a uterine AVM, but they also have the ability to assess the extent of pelvic involvement noninvasively. The definitive treatment of uterine AVM is hysterectomy. However, most women diagnosed with the condition are of childbearing age. Transcatheter uterine artery embolization offers a safe and effective alternative to surgery, with the major advantage of retaining childbearing capacity.