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294 Clinical Guides


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Short Rib-Polydactyly Syndromes (SRPS)
The short rib-polydactyly syndromes (SRPS) consist of micromelia, polydactyly and severe rib shortening. The SRPS are classified into types I, ll, lll and IV, although some authors recognize up to 7 types. It has been suggested that SRPS types I,III and t...
Dr Karl Gloning   01 January 2001   

Schizencephaly
Porencephaly implies replacement of cerebral cortex by cystic cavities or clefts (schizencephaly). These may represent extensions of the subarachnoid space in regions where there has been maldevelopment of cerebral tissue or where traumatic or ischaemic episod...
Dr Alan Cameron, Dr Margaret McNay   01 January 2001   

Sacrococcygeal Teratoma
Sacrococcygeal teratoma (SCT) is the most common congenital tumour with an incidence of approximately 1:35,000. It is a tumour of pluripotential embryonic cells arising from the tip of the coccyx. Teratomas are usually benign, however malignant cases have...
Dr Murray Miskin; Dr Mary E Furness   01 January 2001   

Roberts Syndrome
Roberts syndrome is characterised by the association of tetraphocomelia and facial dysmorphism. Low birth weight is often present. Perinatal mortality is high. Survivors have a short life span. However, milder forms with better prognosis have been reported....
01 January 2001   

Right Ventricle Muscle Function
In this condition, there are muscle bundles in the right ventricle which obstruct the right ventricular outflow tract. There is usually a ventricular septal defect (VSD) in association. It is unusual in infants but the child may present in early life with...
01 January 2001   

Right Atrial Isomerism
In right atrial isomerism, there are two morphologically right atria. There is often asplenia, bilaterally trilobed lungs, bilateral right (short) bronchi and dextrocardia. There is complex heart disease associated with this syndrome which usually...
Professor Lindsey Allan   01 January 2001   

Right Atrial - Idiopathic Dilation
In this rare condition, the right atrium is dilated in the absence of tricuspid valve disease or tricuspid regurgitation. This causes an increased cardio-thoracic ratio. The underlying aetiology is unknown. There is a theoretical risk of secondary lung c...
01 January 2001   

Tetralogy of Fallot with Pulmonary Atresia
This is a more severe and less common form of tetralogy of Fallot where, instead of pulmonary stenosis, the pulmonary artery is atretic or completely obstructed. There is a ventricular septal defect and anterior displacement of the aorta as in the simpler form...
Dr Gurleen Sharland   01 January 2001   

Tetralogy of Fallot with Pulmonary Stenosis
This condition is made up of four components: anterior deviation of the aorta, a ventricular septal defect, infundibular subpulmonary stenosis, and right ventricular hypertrophy. The infant will present with cyanosis which tends to increase in the first mon...
Dr Gurleen Sharland   01 January 2001   

Tricuspid Valve - Regurgitation Isolated
In this condition, there is tricuspid regurgitation in the absence of tricuspid valve abnormality. It may be mild, moderate or severe. Mild transient tricuspid regurgitation of no pathological significance is probably not uncommon, although less common than po...
Professoar Lindsey Allan   01 January 2001   

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The ASUM Online Clinical Handbook is presented as an educational aid for experienced practitioners. It is the responsibility of the individual practitioner to determine how the information should be applied to individual cases.


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