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Biomedical subjects

A Chrispin

Publications and source records attributed to A Chrispin.

12 recordsLinked to original sources

Omodysplasia.

Three cases of a new congenital bone disorder associating facial anomalies (depressed nasal bridge, broad base of the nose, long philtrum) with short humeri. The complex skeletal abnormalities consist of a defect of growth of the distal end of the humerus, a hypoplastic everted condyle, an upper radioulnar diastasis, and a anterolateral dislocation of the head of the radius. The condition is dominantly inherited. Two other cases with the same facial anomalies and osteoarticular abnormalities of the upper limbs are described. These cases also showed a severe micromelic dwarfism due to shortness of the long bones, particularly the femora. The present authors consider that these represent variable expressivity of the same disorder and propose that this condition be called omodysplasia (from the Greek term for humerus).

Abnormalities, Multiple↗

Echo planar imaging of normal and abnormal connections of the heart and great arteries.

Echo planar imaging (EPI) is that form of magnetic resonance imaging which provides very short image acquisition times. EPI also provides very rapid sequential imaging. The EPI method is ideal for imaging the heart and thoracic content because images are devoid of cardio-respiratory motion artefact. Previously an analysis of transectional images has been presented. This paper is concerned with the study of the heart by the use of EPI constructions in the sagittal and coronal planes. Defining connections between ventricle and great artery is of cardinal importance in paediatric cardiology. EPI constructions in the normal heart, transposition, truncus arteriosus and right heart hypoplasia are presented and discussed.

Heart Defects, Congenital↗

Transectional echo planar imaging of the heart in cyanotic congenital heart disease.

Echo planar imaging is that form of magnetic resonance imaging which gives very short image acquisition times. The method has been used to produce images of the infant heart which are free of cardiorespiratory motion artefact, despite tachypnoea and tachycardia. EPI transections of the normal heart are compared with transections in truncus arteriosus, tetralogy of Fallot, right heart hypoplasia and transposition of the great arteries. The diagnosis of the cause of cyanosis in these infants was established by the noninvasive EPI method and validation of the findings may be found in transectional postmortem analyses reported in the literature.

Heart Defects, Congenital↗

The diagnosis of airway obstruction in children.

Airway obstruction in children has many causes. Although vascular rings may have early onset of symptoms there was considerable delay in establishing the correct diagnosis. Barium swallow is diagnostic. Other tests, such as angiocardiography, bronchoscopy, bronchography, and lung scan are usually unnecessary. Pulmonary sling is a less common cause of vascular obstruction. Barium swallow showing anterior indentation at the level of the pulmonary hilum is diagnostic. Cysts and tumors are other causes of severe airway obstruction. The diagnostic and operative problems in three patients are discussed as examples. It is concluded that barium swallow is the most important single investigation in the evaluation of airway obstruction.

Airway Obstruction↗

Pulmonary artery sling in identical twins--report of two cases.

Pulmonary artery sling was successfully treated by division and reimplantation of the left pulmonary artery in identical twins. Long-term patency of the reimplanted pulmonary artery in both twins as well as in 4 other operated children was confirmed by lung perfusion scan. Early operative treatment of pulmonary artery sling is advocated.

Diseases in Twins↗

Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery: the ring-sling complex.

The persistent high mortality in infants with pulmonary artery "sling" (retrotracheal anomalous left pulmonary artery) is primarily due to the coexistence in such patients of long-segment tracheal stenosis due to complete cartilage rings. Five such patients are reported. Airway studies (by filtered high-kV radiography, bronchography, and/or CT) showed low carina, horizontal equal-length right and left mainstem bronchi, and long-segment tracheal stenosis. The length of the stenosis far exceeded the contact with the pulmonary sling. The suggested term "ring-sling complex" for such patients correctly places emphasis on detection of the tracheal malformation, which currently has no satisfactory surgical treatment.

Bronchography↗

Real-time nuclear magnetic resonance clinical imaging in paediatrics.

Echo-planar imaging (EPI), a distinctive variant of nuclear magnetic resonance, needs only a fraction of a second for an image to be acquired and so is free from movement artifacts caused by respiration or heart beat. Clinical findings in the lungs, heart, and mediastinum of three children with high respiratory and heart rates who were examined by EPI are described.

Bronchopulmonary Dysplasia↗

Surgical management of vascular ring.

Between 1968 and 1980, 51 children had an operation for various forms of vascular ring. Additional cardiac malformations were present in five patients, and six had noncardiac congenital anomalies. Although symptoms started within the first month of life in 39 infants, only 16 came to operation under three months of age, and a delay of more than six months occurred in 15. Stridor, often life-threatening, and recurrent infections were the most common symptoms, but dysphagia was also important. The reasons for delay in diagnosis are discussed. Barium swallow provided the diagnosis in 44 patients and suggested it in a further four patients. Innominate artery compression of the trachea was not diagnosed by barium swallow. Operations of various types were performed. Accuracy in diagnosis is important because not all patients could be treated through the classic left thoracotomy. Severe tracheomalacia was responsible for the only two deaths in the series; one of these had tracheostomy performed in the referring hospital, the second child also had Fallot's tetralogy. The large majority of the patients have done well, 76% being asymptomatic at follow-up. Minimal to moderate stridor persists among the remainder to the present time.

Aorta, Thoracic↗