Delayed presentation of a large congenital laryngeal cleft in an adult.

Laryngeal clefts are rare congenital malformations of the posterior laryngotracheal wall that lead to an abnormal communication between the airway and pharyngo-oesophageal tract. The condition is almost universally identified during infancy with minor laryngeal clefts very rarely diagnosed in adulthood. We present our tertiary centre's experience of a large laryngeal cleft presenting at an advanced age, with the aim of increasing awareness of this correctible cause of respiratory distress and aspiration in adults.

The windows of the inner ear.

The oval and round windows of the inner ear are important structures for the transmission of sound and may be affected by a variety of disease entities. The anatomy of this small area is one that often causes the radiology trainee some difficulty, but there are certain disease states that can be easily diagnosed when knowing where and how to look. As this area is very important to the otologist in a variety of preoperative settings, accurate assessment of the windows and recognition of important and potentially complex intra-operative anomalies, will greatly aid our surgical colleagues.

Imaging in pulsatile tinnitus.

Tinnitus may be continuous or pulsatile. Vascular lesions are the most frequent radiologically demonstrable cause of pulsatile tinnitus. These include congenital vascular anomalies (which may be arterial or venous), vascular tumours, and a variety of acquired vasculopathies. The choice of imaging depends on the clinical findings. If a mass is present at otoscopy, thin-section computed tomography (CT) is indicated. In the otoscopically normal patient, there is a range of possible imaging approaches. However, combined CT angiography and venography is particularly useful.

The role of radiology in the management of systemic sclerosis.

Systemic sclerosis is a multisystem connective tissue disorder. Radiology plays an integral part in its management, guiding the clinician concerning the onset and severity of visceral involvement. After skin involvement, the gastrointestinal tract is the most commonly affected system; contrast radiography and magnetic resonance imaging (MRI) play a role in diagnosis. Non-specific interstitial pneumonia is the most frequent respiratory disease and high-resolution computed tomography (CT) is the cornerstone of management. In common with other rheumatic disorders, the role of cardiac MRI is expanding. Radiography remains the main technique in the investigation of skeletal involvement, although MRI is useful as a problem-solving tool. Neurological involvement is increasingly recognized and the major role of radiology is the exclusion of coexistent pathology. We present a thorough review of the role of radiology in the management of systemic sclerosis.

The radiological manifestations of sickle cell disease.

Sickle cell disease (SCD) is an inherited abnormality of the beta-globin chain, which causes a spectrum of haemolytic anaemias. Clinical manifestations in SCD include anaemia, jaundice, recurrent vaso-occlusive crises, and infections (particularly by encapsulated bacteria) due to functional asplenia and cerebrovascular accidents. Radiological investigations play a critical role both in the diagnosis and in the primary prevention of the complications of SCD.

The radiological appearances of thalassaemia.

The skeletal changes of untreated thalassaemia result from ineffective erythropoiesis and expansion of the bone marrow and affect every part of the skeleton. These changes include osteoporosis, growth retardation, platyspondyly and kyphosis. Erythropoiesis occurs at extra-medullary sites, most commonly resulting in a paraspinal mass but occasionally affecting organs containing pluripotential stem cells. Repeated transfusion unaccompanied by iron chelation causes haemosiderosis; iron is deposited at various sites causing functional impairment. Iron-chelation therapy with desferrioxamine (DFX) prevents haemosiderosis but causes a skeletal dysplasia predominantly affecting the rapidly growing long bones, in particular the distal ulna, and causing irregularity and sclerosis of the physeal-metaphyseal junction and splaying of the metaphysis. DFX also exacerbates the observed growth retardation. DFX-induced skeletal dysplasia may herald toxicity, which is associated with visual and auditory impairment. Therefore, careful balancing of the transfusion regimen and iron-chelation therapy is required. Magnetic resonance imaging (MRI) is the most sensitive technique for the detection of DFX-induced dysplasia.