The failure of embryonic vascular arches to fuse and regress in the usual manner during the formation of the aortic arch, pulmonary arteries and ductus arteriosus can cause a wide spectrum of vascular congenital abnormalities of the aortic arch and its branches. These abnormal vascular structures may cause variable compression of the trachea and/or oesophagus with symptoms ranging from none to severe stridor, dyspnoea, dysphagia and cyanosis. Diagnosis and possible treatment of affected patients require multiple imaging modalities. In the majority of cases, the underlying malformation can be detected by chest radiography and barium oesophagography, visualizing the location of the aortic arch and the presence of anomalous compressions of the trachea and/or oesophagus. However, in most cases the exact configuration of the vascular abnormality cannot be fully defined with conventional radiology alone. MRI is fundamental for evaluation of the thoracic vessels. Not only is it non-invasive, but it can also provide large-field-of-view images in any number of planes with three-dimensional reconstruction, adding valuable information about exact vascular configuration, tracheobronchial compression and brachiocephalic vessel branching. The aim of this review is to describe the imaging findings in children affected with special emphasis on MRI.

Two young children with renal artery stenosis and severe hypertension who presented with the so-called hyponatremic-hypertensive syndrome (HHS), with marked urine and solute loss during the acute phase, are described. Both children also presented with severe high molecular proteinuria, glycosuria, and hypercalciuria, only the first symptom having prompt remission after normalization of blood pressure. In children with renal artery stenosis, HHS is associated with severe proteinuria due to hyperfiltration and more extensive tubular functional alterations. Hyponatremia and acute tubulopathy may mask the presenting clinical picture of renal artery stenosis.

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Neonatal hemochromatosis is a rare congenital disorder which affects both fetuses and newborns. It is characterized by hepatocellular failure, often appearing on the first day of life in the form of coagulopathy, hypoalbuminemia, hypoglycemia, and jaundice. Most of the affected infants die early in life, and definitive diagnosis has often been made only by post-mortem evaluation. With the help of MRI, plus increasing awareness of the disorder, diagnosis is now often made early, even in utero. Duplex Doppler sonography does not provide information on siderosis but shows abnormalities in the liver or blood-flow patterns associated with liver disease.

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Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications.An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. Bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient. Eur Respir J 1997; 10: 494-496 Relapsing polychondritis is an uncommon inflammatory disease of unknown cause affecting cartilages in many organs and systems [1,2]. This disorder is manifested by recurrent and, generally, progressive inflammation of the cartilaginous portion of the external ear, joints, cardiovascular system and respiratory tract [2,3]. At the airway level, the extent of the disease may vary and all cartilage-containing structures can be affected [4]. Although onset of relapsing polychondritis may vary, the majority of cases appear between the ages of 40 and 60 yrs, with an equal sex distribution and no known familial predisposition [2][3][4].The most widely-accepted hypothesis for the pathogenesis of polychondritis is that a primary or secondary immune reaction against mucopolysaccharide components of cartilage ground substance results in severe inflammatory changes leading to the destruction of cartilage [4,5]. Histologically, the characteristic abnormalities are: 1) focal or diffuse loss of basophilic staining, indicating depletion of proteoglycans from the cartilage matrix; 2) inflammatory infiltrates, consisting predominantly of mononuclear cells and plasma cells; and 3) destruction of the cartilage with loss of chondrocytes [2][3][4][5].When untreated, this disorder is associated with a high mortality rate, usually from respiratory and cardiovascular complications, such as obstructive pneumonia, aortic valve disease and aortic aneurysm [4][5][6][7]. In addition to anti-inflammatory and immunosuppressive drugs, airway lesions may require a variety of interventions, including tracheostomy and bronchial resection [8]. Case reportAn 8 year old Caucasian girl was referred to our department for evaluation of worsening dyspnoea. At another institution, a diagnosis of relapsing polychondritis had been made 18 months previously, on the basis of the presence of: 1) nasal chondritis, resulting in collapse of the nasal bridge with a saddle deformity; 2) clinical, roentgenographic and endoscopic demonstration of inflammatory alteration of cartilages of the proximal trachea; 3) histological abnormality ...

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Relapsing polychondritis is very rare in children. The diagnosis must be based on a combination of clinical and pathologic features. CT is very useful for an accurate and rapid assessment of laryngo-tracheo-bronchial involvement and the typical finding is lumen narrowing by wall thickening and collapse of the supporting cartilaginous structures. The role of MR imaging should be complementary to CT.