CT of congenital malformations of the lung.

We reviewed 40 cases of congenital malformations of the lung that were studied with both plain radiography and computed tomography (CT). We compared the CT findings with those of radiography. We found that CT was helpful in the management of these cases because it helped confirm the diagnosis, demonstrated unsuspected findings, and better depicted anatomic extent of anomalies, thus allowing better planning for surgery. We believe that CT is the method of choice for the study of congenital lung malformations and that it should be used before more invasive procedures such as bronchography or aortography.

Idiopathic infantile arterial calcification: unusual features.

Idiopathic infantile arterial calcification (IIAC) is a rare disease of unknown etiology, which causes widespread arterial calcification and usually leads to early death from coronary arterial occlusion. Periarticular calcification has been reported in some cases. Two new cases are reported. In addition to the usual features of the disease, one was found to have ear-lobe calcification and the other an aortic aneurysm and coarctation of the aorta. Therapy with diphosphonate was apparently successful in one patient.

Pulmonary sequestration associated with unilateral pulmonary hypoplasia and massive pleural effusion. A case report and review of the literature.

Pulmonary sequestration associated with pulmonary hypoplasia and pleural effusion is very uncommon. Only three cases have been previously reported in the literature. We add one more case and describe its interesting clinical, radiological and pathological findings.

[Jeune syndrome and fungal bronchial asthma]. / Síndrome de Jeune y asma bronquial fúngico.

A young girl suffering from asphyxiating thoracic dystrophy secondary to bronchial asthma was submitted to an allergologic evaluation. We have not found these processes associated in what some authors have called the "minor forms" of the Jeune syndrome. Osteochondrodysplasia, called in recent papers thoracic-pelvic-phalangeal dysplasia, is characterised by marked retraction of the thoracic cage associated with changes in the bones of the pelvis and extremities. We placed special emphasis on the classification of the different forms of presentation as being of greater or lesser severity from a clinical point of view, and stressed the importance of associated abnormalities (such as renal complications, as in the case of our patient) with respect to the eventual prognosis. The diagnosis of allergy is based on a detailed clinical history, positive results to cutaneous testing and the presence of specific IgE, together with the results of nasal provocation. The recording by rhinomanometre, of the temperature and pressure, and the assessment of the nasal mucosa following contact with the suspected allergen was also of help. Finally, having established the diagnosis, we advised the corresponding treatment, both from the allergic and renal viewpoints and concluded with an evaluation of the prognosis as conditioned by the renal pathology.

The Coffin-Siris syndrome. A report of four cases and review of the literature.

Four additional cases of the Coffin-Siris syndrome bring the number of reported cases to 16. This disorder is characterized by the absence or hypoplasia of the nails, especially those of the fifth fingers and toes, growth retardation and mental deficiency, microcephaly, coarse facial appearance, sparse scalp hair and lax joints. Feeding difficulties and respiratory problems are common in infancy. Absence or hypoplasia of the distal and middle phalanges, especially those of the fifth finger and toes and retarded bone maturation are the most common radiological features.