Roy G. K. McCauley

Observer variability assessing US scans of the preterm brain: the ELGAN study

BackgroundNeurosonography can assist clinicians and can provide researchers with documentation of brain lesions. Unfortunately, we know little about the reliability of sonographically derived diagnoses.ObjectiveWe sought to evaluate observer variability among experienced neurosonologists.Materials and methodsWe collected all protocol US scans of 1,450 infants born before the 28th postmenstrual week. Each set of scans was read by two independent sonologists for the presence of intraventricular hemorrhage (IVH) and moderate/severe ventriculomegaly, as well as hyperechoic and hypoechoic lesions in the cerebral white matter. Scans read discordantly for any of these four characteristics were sent to a tie-breaking third sonologist.ResultsVentriculomegaly, hypoechoic lesions and IVH had similar rates of positive agreement (68–76%), negative agreement (92–97%), and kappa values (0.62 to 0.68). Hyperechoic lesions, however, had considerably lower values of positive agreement (48%), negative agreement (84%), and kappa (0.32). No sonologist identified all abnormalities more or less often than his/her peers. Approximately 40% of the time, the tie-breaking reader agreed with the reader who identified IVH, ventriculomegaly, or a hypoechoic lesion in the white matter. Only about 25% of the time did the third party agree with the reader who reported a white matter hyperechoic lesion.ConclusionObtaining concordance seems to be an acceptable way to assure reasonably high-quality of images needed for clinical research.

Gomez-Lopez-Hernandez syndrome : Expansion of the phenotype

Gomez-Lopez-Hernandez syndrome (cerebello-trigeminal-dermal dysplasia) is a condition that includes abnormalities of the cerebellum (rhombencephalosynapsis), cranial nerves (trigeminal anesthesia), and scalp (alopecia). Seven patients with this condition have been documented since 1979. We now report a male with Gomez-Lopez-Hernandez syndrome who, at the age of 19 years, is the oldest patient identified to date. He has been followed since birth, allowing us to report on the progression of his physical findings and psychiatric problems including hyperactivity, depression, self-injurious behavior and bipolar disorder. In addition, he has short stature and growth hormone deficiency.

The development of hypertrophic pyloric stenosis in a patient with prostaglandin-induced foveolar hyperplasia.

Background. Hypertrophic pyloric stenosis (HPS) has been described in association with several obstructive antropyloric lesions including idiopathic foveolar hyperplasia (gastric mucosal hypertrophy), feeding tubes, eosinophilic gastroenteritis, and hypertrophic antral polyps. Non obstructive antral webs have also been described with HPS. Patient and methods. We present a case of gastric-outlet obstruction in association with HPS, namely, prostaglandin-induced foveolar hyperplasia. This entity has been previously described, but rarely in association with HPS. We report a female infant requiring prostaglandin therapy for pulmonary atresia who developed dose-related prostaglandin-induced foveolar hyperplasia and symptoms of progressive non-bilious vomiting. Results. Intially, ultrasonography demonstrated evidence of antral mucosal hypertrophy as the cause for gastric-outlet obstruction. The patient subsequently developed progressive thickening of the antropyloric muscle, resulting in sonographic appearances of hypertrophic pyloric stenosis. Pyloromyotomy was eventually required for treatment of HPS. Conclusion. A common denominator of most of the above-described entities is thickening and/or hypertrophy of the antral mucosa. We suggest that the antropyloric musculature may hypertrophy in an effort to overcome the gastric-outlet obstruction caused by the adjacent thickened antral mucosa. In other words, these entities may represent examples of “secondary” hypertrophic pyloric stenosis.

Blue Rubber Bleb Nevus Syndrome1

Two cases of pediatric blue rubber bleb nevus syndrome are reported. The main features of the disease are rubbery blue cutaneous nevi and hemangiomatous, frequently hemorrhagic malformations of the...

Hypertrophic bursopathy of the subacromial-subdeltoid bursa in juvenile rheumatoid arthritis: Sonographic appearance

Hypertrophic bursopathy is a term used to describe the massive synovial proliferation occasionally seen in the bursae of patients with arthritis. Involvement of the subacromial-subdeltoid (SA-SD) bursa in adults in uncommon, and it is still rarer in children. It may simulate synovial proliferation or fluid within the adjacent shoulder joint. Sonography clearly shows the location and nature of the soft-tissue swelling. Two cases of this entity in children with juvenile rheumatoid arthritis are described, one with a uniquely severe sonographic picture.

Gastroesophageal Reflux in Infants and Children: Correlation of Radiological Severity and Pulmonary Pathology11

A review of the barium study of 507 children for the presence and severity of gastroesophageal reflux revealed 79 cases with coexisting pulmonary disease. Clinical-radiological analysis of the cases with respiratory disease showed good correlation between the radiographic demonstration of major reflux or no reflux and the presence or absence of pulmonary disease.

Primary idiopathic chylopericardium

Primary idiopathic chylopericardium is rare and represents a fraction of all pericardial effusions in the pediatric population. Most cases of chylopericardium occur as a complication of cardiac surgery or after trauma. They may also be caused by tuberculosis, neoplasm, or congenital lymphangiomatosis. Primary idiopathic chylopericardium is a rare disease with unknown etiology. In assessing pediatric patients with an enlarged cardiac silhouette one should be aware of this entity and its presentation. We report two cases of primary idiopathic chylopercardium, one of which at 6 weeks of age is the youngest case to our knowledge so far reported.

Mechanisms of injury to white matter adjacent to a large intraventricular hemorrhage in the preterm brain.

The purpose of this article is to investigate the hyperechoic lesion seen adjacent to a lateral ventricle that contains blood but is not distended. The literature on ependymal barrier dysfunction was reviewed in search of mechanisms of injury to the white matter adjacent to an intraventricular hemorrhage. The clinical literature on the clinical diagnosis of periventricular hemorrhagic infarction was also reviewed to find out how frequently this diagnosis was made. Support was found for the possibility that the ventricular wall does not always function as an efficient barrier, allowing ventricular contents to gain access to the white matter where they cause damage. Hemorrhagic infarction may not be the only or the most frequent mechanism of white matter damage adjacent to a large intraventricular hemorrhage.

Air embolism as a complication of hip arthrography

As an aid to correctly placing the needle tip within the hip joint during arthrography, injection of small amounts of air has been used to outline the joint space. Over a period of seven years, air embolus has been noted in three pediatric patients, twice with minor symptoms, and once with cardiac arrest.Even small amounts of air (less than 5 cc) injected accidentally intravenously may cause dangerous complications in small infants. If this technique is to be used, it is recommended that small amounts of carbon dioxide or oxygen be used instead of air.

Milk of calcium fluid collection in dermatomyositis: Ultrasound findings

Extensive subcutaneous and intermuscular calcium laden fluid collections (milk of calcium) were found in two young girls with severe dermatomyositis. Sonographic examination clearly showed the nature and extent of the collections. Knowledge of this new feature of dermatomyositis should help avoid confusing these fluid collections with soft tissue infection and deep abscess in these often steroid dependent children.