Items tagged “cases”

3,932 results found


Rhombencephalosynapsis (RS) is a congenital abnormality of the cerebellum characterised by the vermis absence and continuity of the cerebellar hemispheres, dentate nuclei, and superior cerebellar peduncles. It either occurs as an isolated anomaly (rare) or as part of wider cerebral malformation ...

Cowden syndrome

Cowden syndrome, also known as multiple hamartoma syndrome, is characterised by multiple hamartomas throughout the body and increased risk of several cancers. Terminology Type 2 segmental Cowden syndrome is the association of Cowden syndrome with a Cowden naevus, when it is considered a type o...

Lasajunias classification of vein of Galen aneurysmal malformations

The Lasjaunias classification, at the time of writing (mid 2016), is one of the two commonly used systems for classifying vein of Galen malformations. It relies on dividing the entity into choroidal or mural types, depending on the number and origin of feeding arteries.  Classification choroid...


A santorinicoele refers to a cystic dilatation of the end of the dorsal  pancreatic duct (duct of Santorini) 1,2 and is believed to be analogous to a dilatation of the most distal common bile duct, which is commonly known as a choledochocoele3.  It can occur in association with pancreas divisum...

Testicular epidermoid cyst

Testicular epidermoid cysts, also known as keratocysts, are rare benign tumours of germ cell origin that occur in the testis.  Epidemiology Testicular epidermoid cysts account for around 1-2% of all testicular masses and typically present in mid-adulthood (2nd to 4th decades) 1,2. They are the...

Hepatoblastoma (staging)

As expected there are a number of different staging systems for hepatoblastoma. Staging PRETEXT grouping system of paediatric liver tumours not specific to hepatoblastoma; used in all paediatric liver tumours Intergroup staging system specific for hepatoblastoma (see below) Intergroup stag...

Rhizomelic dwarfism

Rhizomelic dwarfism is a type of dwarfism where the dominant feature is proximal (i.e. femoral, humeral) limb shortening. Pathology The following conditions fall under the heading of rhizomelic dwarfism 3 metatropic dysplasia achondrogenesis rhizomelic chondrodysplasia punctata achondropla...

SAPHO syndrome

The SAPHO syndrome is an acronym that refers to a rare condition that is manifested by a combined occurrence of 2 S: synovitis A: acne P: pustulosis  H: hyperostosis O: osteitis Epidemiology It classically tends to present in young to middle-aged adults. Presentation in the paediatric pop...

Neonatal appendicitis

Neonatal appendicitis is rare, presumably in part due to the short funnel shape to the appendix at that age. Symptoms are non-specific and may mimic necrotising enterocolitis. 

Zuelzer-Wilson syndrome

The Zuelzer-Wilson syndrome (total colonic aganglionosis) is a subset of Hirschsprung disease, in which the whole colon is aganglionic. It is uncommon and accounts for 2-13% of cases of Hirschsprung disease 3. Multiple procedures have been devised to treat the condition, including proctocolectom...

Rectosigmoid ratio

The rectosigmoid ratio is a measurement of the diameter of the rectum divided by that of the sigmoid colon during contrast enema. It is of particular use in the diagnosis of Hirschsprung disease. Normal children have a rectum that is larger than the sigmoid (i.e. rectosigmoid ration >1). In fac...

Congenital heart disease chest x-ray (an approach)

With the advent of echocardiography, and cardiac CT and MRI, the role of chest radiographs in evaluating congenital heart disease has been largely been relegated to one of historical and academic interest, although they continue to crop up in radiology exams. In most instances a definite diagnos...

Pentalogy of Fallot

The pentalogy of Fallot is a variant of the more common tetralogy of Fallot, comprising the classical four features with the addition of an atrial septal defect or patent ductus arteriosus The five features therefore are: ventricular septal defect (VSD)  right ventricular outlfow tract narro...

Pink tetralogy of Fallot

Pink tetralogy of Fallot refers to a tetralogy of Fallot in which the degree of right ventricular outflow is minimal, resulting no significant right to left shunt, and therefore no cyanosis. Symptoms are mild and presentation may be delayed, even into adulthood. See also tetralogy of Fallot ...

Pott shunt

A Pott shunt is a form of palliative surgery performed in patients with tetralogy of Fallot prior to the ability to repair the defect. It consists of a shunt formed between the descending thoracic aorta and the left pulmonary artery. This does not relieve the right ventricular outflow obstructi...

Waterston shunt

A Waterston shunt is a form of palliative surgery performed in patients with tetralogy of Fallot prior to the ability to repair the defect. It consists of a shunt formed between the ascending aorta and the right pulmonary artery. This does not relieve the right ventricular outflow obstruction, ...

Blalock-Taussig shunt

Blalock-Taussig shunt, also known as Blalock-Thomas-Taussig shunt, is a palliative procedure performed in patients with tetralogy of Fallot (prior to the ability to repair the defect) to increase the pulmonary blood flow.  Originally the shunt sacrificed the subclavian artery (with a distal lig...

Multiple filling defects of the ureter (differential)

Multiple filling defects within a ureter, as seen on conventional IVU or CT IVU, have a relatively small differential including: spreading or multifocal transitional cell carcinoma (TCC) vascular indentations multiple ureteral stones (steinstrasse) blood clots ureteritis cystica Stevens-Jo...

Bowel obstruction

Bowel obstructions are common and account for 20% of admissions with "surgical abdomens". Radiology is important in confirming the diagnosis and identifying the underlying cause. Bowel obstructions are usually divided according to where the obstruction occurs, and since imaging appearances, und...

Obersteiner-Redlich zone

The Obersteiner-Redlich zone represents the transition point along a nerve where enveloping glial cells are replaced by Schwann cells. Vestibular schwannomas are believed to arise most commonly from this region 1.

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