The objective of the study was to study the magnetic resonance imaging (MRI) features of Hirayama disease on a 3 Tesla MRI scanner. Nine patients with clinically suspected Hirayama disease were evaluated with neutral position, flexion, contrast-enhanced MRI and fast imaging employing steady-state acquisition (FIESTA) sequences. The spectrum of MRI features was evaluated and correlated with the clinical and electromyography findings. MRI findings of localized lower cervical cord atrophy (C5-C7), abnormal curvature, asymmetric cord flattening, loss of attachment of the dorsal dural sac and subjacent laminae in the neutral position, anterior displacement of the dorsal dura on flexion and a prominent epidural space were revealed in all patients on conventional MRI as well as with the dynamic 3D-FIESTA sequence. Intramedullary hyperintensity was seen in four patients on conventional MRI and on the 3D-FIESTA sequence. Flow voids were seen in four patients on conventional MRI sequences and in all patients with the 3D-FIESTA sequence. Contrast enhancement of the epidural component was noted in all the five patients with thoracic extensions. The time taken for conventional and contrast-enhanced MRI was about 30–40 min, while that for the 3D-FIESTA sequence was 6 min. Neutral and flexion position MRI and the 3D-FIESTA sequence compliment each other in displaying the spectrum of findings in Hirayama disease. A flexion study should form an essential part of the screening protocol in patients with suspected Hirayama disease. Newer sequences such as the 3D-FIESTA may help in reducing imaging time and obviating the need for contrast.
The radiological imaging plays a vital role in the evaluation of patients with congenital anomalies of the gastrointestinal tract. The evaluation of these patients, most of which present early after birth, frequently requires the use of various imaging modalities for making the correct diagnosis and planning surgical correction. This article reviews the common congenital anomalies of the gastrointestinal tract including obstructive lesions, anomalies of rotation and fixation, anorectal anomalies, and intestinal duplications. The plain radiograph is often diagnostic in neonates with complete gastric of upper intestinal obstruction and further radiologic evaluation may be unnecessary. An upper gastrointestinal series should be performed in all patients with incomplete intestinal obstruction. Sonography is useful in the evaluation of many congenital anomalies affecting pediatric gastrointestinal tract especially hypertrophic pyloric stenosis, enteric duplication cysts, midgut malrotation, meconium ileus and meconium peritonitis. Moreover, CT and MRI has assumed a greater importance as these provide excellent anatomic details which may be necessary for correct diagnosis as well as treatment planning. This is particularly true in evaluation of congenital anomalies such as esophageal/enteric duplications, vascular rings and anorectal anomalies. It is important to be familiar with the role nad usefulness of the various imaging modalities so that these can be used judiciously to avoid unnecessary radiation exposure while minimizing the patient discomfort.
Congenital cystic eye is a rare cause of cystic orbital lesion. The condition is recognized at birth as a large orbital mass in place of a normal eye. Only 29 cases have been reported previously. We report a case of unilateral congenital cystic eye with multiple brain anomalies in the form of agenesis of corpus callosum and grey matter heterotopias. In this case report we highlight the MRI features of this entity, which have not been described previously in the literature.
Extramedullary hematopoiesis (EMH) is a response to erythropoiesis failure in bone marrow, and is mostly encountered in the liver and spleen. Renal involvement is rare and the radiological literature on renal EMH is scant. We report the computed tomography (CT) and magnetic resonance imaging (MRI) findings of one such rare case of renal EMH in a young boy, appearing as a unilateral renal hilar mass.
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