Special Imaging Casebook: Neonate With Osteogenesis Imperfecta and Asplenia Syndrome With Horseshoe Adrenal

Te, Herman

doi:10.1038/sj.jp.7200065

Cited by 3 publications

(1 citation statement)

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“…Asplenia syndrome, also known as right isomerism, is characterized by bilateral trilobed lungs (Fig 3a), right atria, a horizontal midline liver, an absent spleen, malrotation, and congenital heart disease (8). Associated congenital heart anomalies in asplenia syndrome include total anomalous pulmonary venous return (most common), endocardial cushion defect, transposition of the great arteries, and pulmonic stenosis or atresia (10). The renal anomalies associated with a horseshoe adrenal gland are bilateral renal agenesis and horseshoe kidney.…”

Section: Horseshoe Adrenal Glandmentioning

confidence: 99%

Imaging of Nonmalignant Adrenal Lesions in Children

2017

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The adrenal glands in children can be affected by a variety of benign lesions. The diagnosis of adrenal lesions can be challenging, but assessment of morphologic changes in correlation with the clinical presentation can lead to an accurate diagnosis. These lesions can be classified by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar sequestration and extramedullary hematopoiesis). Multimodality cross-sectional imaging helps to define the origin, extent, and relationship of these lesions to adjacent structures, as well as to guide treatment management. The anatomic and functional imaging modalities used to evaluate pediatric adrenal lesions include ultrasonography, computed tomography (CT), magnetic resonance imaging, and iodine 123 metaiodobenzylguanidine scintigraphy. Identifying the imaging features of nonmalignant adrenal lesions is helpful to distinguish these lesions from malignant adrenal neoplasms. Identifying characteristic imaging findings (eg, enlarged adrenal glands, with cerebriform surface, and stippled echogenicity in CAH; a T2-hyperintense mass with avid contrast enhancement in pheochromocytoma; low CT attenuation [<10 HU] and signal intensity drop on opposed-phase chemical shift images in adenoma; and enhancing suprarenal mass supplied by a systemic feeding artery in extralobar sequestration) can aid in making the correct diagnosis. In addition, clinical features (eg, ambiguous genitalia in CAH and hypertension in pheochromocytoma) can also guide the radiologist toward the correct diagnosis. RSNA, 2017.

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Section: Horseshoe Adrenal Glandmentioning

confidence: 99%