TEST PAPER 3 Flashcards
(120 cards)
1.A 77-year-old man presents with a progressively enlarging pulsatile mass in the leftgroin corresponding to the puncture site of a previous coronary angiography.
Urgent outpatient ultrasound shows a large anechoic lesion with peripheral filling defectand arterial pattern intraluminal flow. What is most appropriate management plan?
A. Covered stent
B. Ultrasound-guided compression
C. Ultrasound-guided thrombin injection
D. Open surgery
E. CT angiogram first for treatment planning
1.C. Ultrasound-guided thrombin injection
Pseudoaneurysms are common vascular abnormalities that represent a disruption in arterial wallcontinuity.
Surgical repair was the treatment of choice for superficial extremity pseudoaneurysms untilUS-guided compression was introduced. US-guided percutaneous thrombin injection has replaced US-guided compression as the therapeutic method of choice for treatment of post catheterisation pseudoaneurysms. Endovascular techniques like stent placement (indispensable artery) or embolisation (dispensable artery) have a lower complication rate in the treatment of visceral pseudoaneurysms than does surgical management.
@# 2.All of the following are associations of Chiari II malformation, except
A. Dysgenesis of corpus callosum
B. Klippel-Feil deformity
C. Syringomyelia
D. Meningomyelocele
E. Tectal beaking
2.B. Klippel-Feil deformity
The hallmarks of Chiari II malformation include caudally displaced fourth ventricle (the fourthventricle is in normal position in Chiari 1 malformation), caudally displaced brain stem, and tonsillar or vermian herniation through the foramen magnum.
Associations include lumbar myelomeningocele, syringohydromyelia, dysgenesis of the corpus callosum, obstructive hydrocephalus, absent septum pellucidum and excess cortical gyration.
It is not associated with any of the bony abnormalities described in Chiari I malformation, like basilar impression, occipitalization of the atlas, platybasia and Klippel-Feil anomaly.
3.A 17-year-old girl is newly diagnosed with Crohn’s disease and an MR enterography has beenrequested for evaluation of the disease extent and distribution. All of the following arc latefindings of Crohn’s disease on MRE, except
A. Fistulae
B.Skip lesions
C. Strictures
D. Small bowel obstruction
E. Ulcers
3.E. Ulcers
At pathologic analysis, active inflammation is characterised by varying degrees of neutrophiliccrypt injury. In mildly active Crohn’s disease, a small fraction of crypts arc infiltrated by neutrophils (cryptitis), with associated crypt destruction and mucin depletion.
As the degree of activity increases, there is a corresponding increase in the proportion of involved crypts and the severity of crypt injury, including crypt epithelial necrosis, intraluminal exudates (crypt abscess), and eventual ulcer formation. Two types of ulcers are seen in Crohn’s disease: superficial aphthous ulcers and deep fissuring ulcers. Deep fissuring ulcers are more problematic than superficial aphthous ulcers; they break through the mucosa and into the deeper layers of the bowel wall, initially resulting in submucosal inflammation and oedema. Some investigators have reported that deep ulcers may be seen in MR enterography, whereas superficial ulcers defy detection.
4.A 76-year-old woman with one previous history of admission in the medical ward withacute renal failure 6 years ago has now been referred to the radiology department for aCT angiography of the lower limb arteries subject to symptoms of claudication. Therenal team were again involved when she developed acute worsening of renal functionafter the CT study. All the following are features of contrast-induced nephropathy,except
A. Alternative major insults to kidneys ruled out.
B.Increase in serum level of creatinine of 0.5 mg/dL.
C. Rise in serum creatinine level by >50% above baseline.
D. Increase in serum creatinine occurs 48-72 hours after administration of contrast.
E. Raised serum creatinine persists for 2-5 days.
- C. Rise in serum creatinine level by >50% above baseline.
Diagnosis of CIN (contrast-induced nephropathy) is most often based on an increase in the serum level of creatinine after exposure to a contrast agent. Diagnostic criteria for CIN include exposure to contrast agent, increase in serum level of creatinine of 0.5 mg/dL or 25% greater than baseline, increase in serum level of creatinine occurring 48-72 hours after administration of contrast agent and persisting for 2-5 days, and alternative major injuries being ruled out.
@# 5. Skeletal survey is indicated as an investigation for all of the following, except
A. Eosinophilic granuloma
B. Multiple myeloma
C. Non-accidental injury
D. Skeletal metastasis
E. Suspected skeletal dysplasia
- D. Skeletal metastasis
Eosinophilic granuloma is associated with Langerhans cell histiocytosis and is an indication for a skeletal survey, along with multiple myeloma and non-accidental injury. Skeletal surveys are never performed for skeletal metastasis.
Radiological evaluation of skeletal dysplasia often starts with a skeletal survey for several non-lethal skeletal dysplasia.
- A central mass with homogeneous signal intensity is identified on MRI in the fourth ventricle of a 4-year-old child. Which of the following is the most likely diagnosis?
A. Astrocytoma
B. Medulloblastoma
C. Ependymoma
D. Pontine glioma
E. Tectal plate glioma
- B. Medulloblastoma
Medulloblastoma is the most common infratentorial paediatric brain tumour. It typically presents as a midline, non-calcified solid vcrmal mass, obstructing the fourth ventricle.
Classic medulloblastoma typically arises from the roof of the fourth ventricle and is midline in location in 75% 90% of cases. Classic medulloblastoma is a highly cellular, densely packed tumour, which is reflected on imaging; it appears hyperdense relative to brain on CT (89% of cases) and shows restricted diffusion on DWI. This feature of medulloblastoma allows differentiation from JPA, ependymoma and brainstem glioma. Almost all medulloblastomas enhance post-contrast; the degree of enhancement varies from diffuse homogenous to heterogeneous.
- A young woman with positional headache shows a well-defined round mass at the anterior margin of the third ventricle with high signal on both T1W and T2W images. Asymmetrical lateral ventricular enlargement is evident. What is the diagnosis?
A. Colloid cyst
B. Choroid plexus cyst
C. Porencephalic cyst
D. ACA aneurysm
E. Haemorrhagic contusion
- A. Colloid cyst
The most common true mass of the foramen of Monro is a colloid cyst, a benign lesion that occurs in adult patients. This well defined round cyst may be from several millimeters to 3 cm in size and attaches to the anterior superior aspect of the third ventricle roof. Often hyperattenuating at non-enhanced CT, it has variable signal intensity at MR imaging and is often hyperintense on T1-weighted and FLAIR images. Peripheral gadolinium enhancement is rarely seen. Ninety percent of colloid cysts are asymptomatic and stable, whereas 10% are reported to enlarge or cause hydrocephalus. Rapid enlargement has been associated with coma and death.
- A CT scan of a 25-year old woman on OCP shows a focal area of low attenuation that demonstrates homogenous enhancement in the arterial phase with rapid washout on the portal venous and delayed phases. She presents a week later with an acute abdomen and hemodynamic instability. What is the most appropriate management?
A. Gel foam embolisation
B. Liver resection
C. Conservative management
D. Coil embolisation
E. Radiofrequency ablation
- D. Coil embolisation
Ruptured hepatic adenoma is a clinical emergency. Many adenomas are first diagnosed in symptomatic patients presenting with acute abdominal pain, hemodynamic instability, or other signs of rupture, most of whom are on OCP. The gold standard has been to perform emergency laparotomy with gauze packing or partial liver resection. Laparoscopic resection is also possible in theory, but it is technically difficult. Recently, less invasive procedures such as transarterial embolisation have been developed that may also lead to adequate haemostasis without the need for urgent laparotomy, and they are considered the first treatment option. RFA is used in non-ruptured adenomas and its use after haemorrhage may be irrelevant.
@# 9. A 43-year-old woman currently on treatment for Crohn’s disease needs to have her medication reviewed following the recommendation of the MDT. The gastroenterologist wants to perform a CT enterography to assess her disease status and response to treatment prior to the medication review. Which one of the following CT signs suggests inactive Crohn’s disease?
A. Increased mesenteric fat attenuation
B. Mesenteric fibro-fatty proliferation
C. Target sign
D. A non-enhancing thickened bowel wall
E. Comb sign
- B. Mesenteric fibro-fatty proliferation
The main diagnostic purpose of CT enterography in the setting of Crohn’s disease is to differentiate active inflammatory strictures from fibrotic strictures in order to guide therapy.
CT features of active Crohn’s disease include mucosal hyperenhancement, wall thickening (thickness >3 mm), mural stratification with a prominent vasa recta (comb sign) and mesenteric fat stranding, all of which are exquisitely demonstrated at CT enterography.
The capability of CT enterography to depict extra-enteric disease allows the simultaneous diagnosis of complications associated with Crohn’s disease, such as obstruction, sinus tract, fistula and abscess formation.
Mural enhancement is the most sensitive indicator of active Crohn’s disease.
Care should be taken to compare bowel loops with similar distention, since both the jejunum and normal collapsed loops may demonstrate regions of higher attenuation, simulating enhancement.
Inadequately distended bowel loops may be difficult to assess, and secondary signs of active disease, such as mesenteric fat stranding, vasa recta prominence or complications such as fistulas and abscesses should be sought to maximise the accuracy of a diagnosis of active disease.
Prominence of the vasa recta adjacent to the inflamed loop of bowel (comb sign), along with increased mesenteric fat attenuation, is (he most specific CT feature of active Crohn’s disease.
Findings that might be seen in inactive long standing Crohn’s disease include submucosal fat deposition, pseudosacculation, surrounding fibro-fatty proliferation and fibrotic strictures.
- A 36-year-old woman with small cystic lesions identified in the liver when she had an ultrasound assessment a year ago underwent a CT urogram for renal colic. The CT urogram revealed speckled calcification in the medulla of both kidneys. Review of an old IVU showed a striated nephrogram and filling defects in the proximal right ureter. What is the most likely diagnosis?
A. Hyperparathyroidism
B. Medullary sponge kidney
C. Medullary cystic disease
D. Acquired renal cystic disease
E. PCKD
- B. Medullary sponge kidney
Medullary sponge kidney involves dysplastic dilatation of medullary and papillary collecting ducts. It is known to be associated with Ehlers-Danlos syndrome, parathyroid adenoma and Caroli’s disease. Recognised features include medullary nephrocalcinosis, ‘bunch of flower appearance’ and dense, striated nephrogram; it can affect a single kidney (25%) or single pyramids.
Note that papillary blush on IVU without dense streaks is a normal variant, especially when not associated with nephrocalcinosis.
@# 11. A 30-year-old woman is struck over the right cheek during an altercation with another woman. There is bruising and swelling, and a fracture is suspected. What view is the best for demonstrating a zygomatic arch fracture?
A. Townes view
B. Submentovertex view
C. Swimmer’s view
D. Occipitomental view 45 degrees
E. Occipitomental view 30 degrees
- B. Submentovertex view’
The submentovertex (SMV) view, also called the bucket-handle view, shows fractures of the zygomatic arch best.
Townes and occipitomental views are for skull and facial bone fractures.
Swimmer’s view is for viewing the cervico-thoracic junction
- A 1 -year-old infant is admitted with acute stridor. A viral cause is suspected. On AP chest radiography no foreign body is identified, but there is an inverted V appearance of the subglottic trachea. Which of the following is the most likely diagnosis?
A. Foreign body
B. Acute laryngotracheobronchitis
C. Whooping cough
D. Tracheobronchomalacia
E. Epiglottitis
- B. Acute laryngotracheobronchitis
Croup (laryngotracheobronchitis) most commonly affects children between 6 months and 3 years and presents with acute stridor, usually following viral infection. A subglottic inverted V sign is seen on plain film, but the epiglottis and aryepiglottic folds are usually normal.
In contrast, epiglottitis is a life-threatening condition affecting 3-6-year-olds, with a lateral soft-tissue neck radiograph showing thickening of the epiglottis and aryepiglottic folds described as the ‘thumb sign’.
@# 13. When can you see the radiographic changes of fat embolism on a chest X-ray?
- A. No Yes No
The chest radiographic appearance of fat embolism syndrome is non specific. Normal radiographs can also be seen. Most patients presenting with a normal initial radiograph develop radiographic- evident abnormalities within 72 hours of injury, and most cases show radiographic resolution within 2 weeks of hospitalisation.
- A 46-year old woman with constant headache undergoes a CT brain for preliminary evaluation. CT brain reveals a suprasellar solid cystic lesion with some calcification.
MRI confirms the suprasellar mass with high signal in both T1W and T2W images. Marginal enhancement of the peripheral solid rim portion of the lesion is also noted.
What is the diagnosis?
A. Craniopharyngioma
B. Pituitary adenoma
C. Pineoblastoma
D. Meningioma
E. Dermoid cyst
- A. Craniopharyngioma
Craniopharyngiomas account for about 3% of all primary intracranial tumours. Two types of craniopharyngiomas have been described: a childhood type, with frequent occurrence of cyst formation and calcifications and generally a poor prognosis, and an adult type, generally without calcifications or cyst formation and generally a good prognosis.
The cystic areas may be iso-, hyper- or hypointense relative to brain tissue with T1 -weighted sequences. The short T1 relaxation times are the result of very high protein content. With T2-weighted sequences, both the cystic and solid components tend to have high signal intensity. After the administration of contrast material, the solid portions enhance heterogeneously. The thin walls of the cystic areas nearly always enhance. The characteristic calcifications in paediatric craniopharyngiomas may not be discernible, although gradient-echo images may show susceptibility effects from calcified components. Occasionally, craniopharyngiomas are predominantly solid, typically without calcification.
@# 15. A 53-year-old woman with a long-standing history of known Crohn’s disease is referred for a CT enterography for assessment of disease status. Which one of the following statements regarding the CT evaluation of Crohn’s disease is true?
A. Perianal disease is uncommon
B. A thickened hyperenhancing bowel wall is a sign of active disease
C. Mural stratification implies perforation in the bowel wall
D. The comb sign is a specific sign
E. Perienteric stranding is a specific sign
- B. A thickened hyperenhancing bowel wall is a sign of active disease.
The main diagnostic purpose of CT enterography in the setting of Crohn’s disease is to differentiate active inflammatory strictures from fibrotic strictures in order to guide therapy.
CT features of active Crohn’s disease include mucosal hyperenhancement, wall thickening (thickness >3 mm), mural stratification with a prominent vasa recta (comb sign) and mesenteric fat stranding, all of which are exquisitely demonstrated at CT enterography.
The capability of CT enterography to depict extra-enteric disease allows the simultaneous diagnosis of complications associated with Crohn’s disease, such as obstruction, sinus tract, fistula and abscess formation.
Mural enhancement is the most sensitive indicator of active Crohn’s disease.
The term mural stratification denotes the visualisation of bowel wall layers at CT. At CT enterography, the oedematous bowel wall has a trilaminar appearance, with enhanced outer serosal and inner mucosal layers and an interposed submucosal layer of lower attenuation. However, this feature is not specific to Crohn’s disease; it is seen also in other inflammatory bowel diseases and even in some cases of bowel ischaemia.
Prominence of the vasa recta adjacent to the inflamed loop of bowel (comb sign) along with increased mesenteric fat attenuation, is the most specific CT feature of active Crohn’s disease.
Findings that might be seen in inactive long standing Crohn’s disease include submucosal fat deposition, pseudosacculation, surrounding fibro-fatty proliferation and fibrotic strictures.
Perianal disease is common in Crohn’s disease.
@#1 16. A young woman with polycystic ovarian disease is currently under the transplant team, being reviewed and worked up for potential renal transplantation. Regarding renal transplantation, all of the following are true, except
A. US kidney, CT angiography and plain abdominal film provide similar information to MR angiography and MR urography.
B. Pelvicalyceal duplication precludes kidney donation.
C. Twenty percent of kidneys have accessory arterial supply.
D. Right kidney is placed in the left iliac fossa because it is easier technically.
E. Carrel patch is used only for cadaveric kidney.
- B. Pelvicalyceal duplication precludes kidney donation.
The aims of preoperative evaluation of living related donors are to show that the donor will retain a normal kidney after unilateral nephrectomy, to demonstrate that the kidney to be transplanted has no major abnormality, and to outline the vascular anatomy.
US assesses the parenchyma, CT angiogram shows the arterial and venous anatomy, and the plain film demonstrates the pelvicalyceal system. MR angiography and MR urography provide similar information.
Conditions that do not preclude donation include pelvicalyceal duplication, solitary renal cyst, unilateral mild reflux nephropathy and only one scar.
In the case of unilateral duplication, the contralateral kidney is donated.
Twenty percent of kidneys have accessory arterial supply.
Kidneys with multiple arterial supply are more likely to have vascular complications.
The transplanted kidney is placed extraperitoneally in the iliac fossa; usually the right kidney is placed in the left iliac fossa because the vascular anastomosis is easier.
When a cadaveric kidney is used, an aortic patch (Carrel patch) is removed with the renal artery.
@#1 17. A 25-year-old man presents with progressive increase in knee pain. Plain films show features of osteoarthrosis with increased soft-tissue swelling and density. An MRI shows diffuse low signal on T2W images to the synovium of the knee on the background of large joint effusion. What is the likely diagnosis?
A. Amyloid arthropathy
B. Haemophilic arthropathy
C. PVNS
D. Primary synovial osteochondromatosis
E. Rapidly destructive articular disease
- B. Haemophilic arthropathy
Radiographic findings vary greatly with the different stages of haemophilic arthropathy (acute, subacute or chronic haemarthrosis) and reflect the presence of haemarthrosis (joint effusion), synovial inflammation and hyperaemia (osteoporosis and epiphyseal overgrowth), chondral erosions and subchondral resorption (osseous erosions and cysts), cartilaginous denudation (joint space narrowing), intraosseous or subperiosteal haemorrhage (pseudotumours) and osseous proliferation (sclerosis and osteophytosis).
Some abnormalities of osseous shape, such as widening of the intercondylar notch, flattening of the condylar surface or squaring of the patella, are very characteristic of chronic haemarthrosis of the knee.
At MR imaging, hypertrophied synovial membrane resulting from repetitive haemarthrosis has characteristic low signal intensity with all pulse sequences, especially with gradient echo sequences, due to the magnetic susceptibility effect caused by haemosiderin.
As in pigmented villonodular synovitis, the signal intensity of the subarticular defects varies and may indicate the presence of fluid (high signal intensity on T2-weighted images), soft tissue (intermediate signal intensity) or synovial tissue with haemosiderin (low signal intensity).
Rapidly destructive articular disease is an unusual form of osteoarthritis that typically involves the hip. The disease is almost always unilateral, but bilateral lesions and involvement of shoulder have also been reported. Serial radiographs show progressive loss of joint space and loss of subchondral bone in the femoral head and acetabulum, resulting in marked flattening and deformity of the femoral head (‘hatchet’ deformity). Superolateral subluxation of the femoral head or intrusion deformity within the ilium can be observed. Most cases demonstrate subchondral defects and mild sclerosis. However, osteophytes are small or absent.
- A 10-year old girl is diagnosed with pilocytic astrocytoma. Which of the following are the most likely findings on MRI?
A. Hyperintense to brain on T1WI and hypointense on T2WI
B. Isointense to brain on T1WI and hypointense on T2WI
C. Isointense to brain on T1WI and isointense on T2WI
D. Hypointense to brain on T1WI and hyperintense on T2WI
E. Hyperintense to brain on T1WI and hyperintense on T2WI
- D. Hypointense to brain on T1WI and hyperintense on T2WI
Pilocytic astrocytoma typically presents in first two decades, with peak age from birth to 9 years of age. It is the most common paediatric glioma, with the majority located in the cerebellum. Pilocytic astrocytomas are predominantly cystic with an intensely enhancing mural nodule; half show enhancement of the cyst wall. The magnetic resonance signal pattern is as for cysts: hypointense on T1-weighted images and hyperintense on T2-weighted images.
Post contrast T1-weighted images shows enhancement of the mural nodule.
- A 53-year-old woman with chronic renal failure and polycystic renal disease had renal transplantation surgery 5 weeks ago. Initial recovery’ was uneventful and she is regularly being followed up by the transplant and the renal team. A follow-up graft ultrasound done at 5 weeks post-surgery reveals a large simple fluid collection in relation to the graft. What is the most likely explanation?
A. Abscess
B. Resolving haematoma
C. Lymphocele
D. Urinoma
E. Seroma
- C. Lymphocele
Urine leaks and urinomas are relatively rare complications and are usually found in the first 2 weeks post-operative between the transplanted kidney and the bladder. They appear as a well-defined, anechoic fluid collection with no septations that increases in size rapidly. Antegrade pyelography is necessary to provide detailed information about the site of origin of the urinoma and in planning appropriate intervention.
Haematomas are common in the immediate post-operative period, but they may also develop spontaneously or as a consequence of trauma or biopsy. At US, haematomas demonstrate a complex appearance. Acute haematomas are echogenic and become less echogenic with time. Older haematomas even appear anechoic, more closely resembling fluid, and septations may develop.
Lymphoceles are the most common peritransplant fluid collections that may develop at any time, from weeks to years after transplantation. However, they usually occur within 1-2 months after transplantation. At US, lymphoceles are anechoic and may have septations. Similar to other peritransplant fluid collections, they can become infected and can develop a more complex appearance.
Abscesses have a complex, cystic, non-specific appearance at US. Peritransplant abscesses are an uncommon complication and usually develop within the first few weeks after transplantation.
@#e 20. All of the following are causes of lower zone fibrosis, except
A. Amiodarone
B. Idiopathic pulmonary fibrosis
C. Asbestosis
D. Ankylosing spondylitis
E. Neurofibromatosis I
- D. Ankylosing spondylitis
Causes of lower zone fibrosis include asbestosis, aspiration, cryptogenic alveolitis (IPF), neurofibromatosis I and tuberous sclerosis; connective tissue diseases like RA, scleroderma and SLE; and drug toxicity to substances like amiodarone and nitrofurantoin.
@# 21. All of the following are true for neurocysticercosis, except
A. There arc four recognised stages on CT/MR.
B. The granular nodular stage is not associated with oedema.
C. The vesicular stage does not show any oedema.
D. The vesicular stage shows a nodule with a ‘hole with dot’ appearance.
E. Colloidal stage shows an avid ring-enhancing capsule.
- B. The granular nodular stage is not associated with oedema.
Neurocysticercosis has been classified into active and non active forms on the basis of clinical presentation, results of CSF analysis and imaging findings.
The active forms include arachnoiditis with or without ventricular obstruction and vasculitis with or without infarction.
On the basis of radiologic findings, neurocysticercosis is divided into five stages: non-cystic, vesicular, colloidal vesicular, granular nodular and calcified nodular. Of these, all the stages apart from the first (non cystic stage) arc visible on CT/MRI.
Vesicular stage: Cyst signal intensity similar to that of CSF on T1-weighted and T2-weighted images; cyst wall is well defined and thin, with little or no enhancement on gadolinium enhanced images; scolex (hole with dot appearance); iso- or hypointense relative to white matter on T1-weighted images; iso- to hyperintense relative to white matter on T2-weighted images; best seen on PD-weighted images.
Colloidal vesicular stage: Cyst contents are hyperintense on T1-weighted and T2-weighted images (proteinaceous fluid), cyst wall is thick and hypointense, pericystic oedema (best seen on FLAIR), pericystic enhancement on gadolinium-enhanced images.
Granular nodular stage: Similar to the colloidal vesicular stage but with more oedema, thicker ring enhancement.
Calcified nodular stage: Hypointense nodules, no oedema, no enhancement.
- A 43-year-old woman currently on treatment for known Crohn’s disease has recently been unwell again with a mildly raised CRP. MRI could not be performed because she wras claustrophobic, so a CT was done instead. Which one of the following statements concerning CT enterography for evaluation of small bowel Crohn’s disease is not true?
A. Patients should ideally ingest 1 L of positive oral contrast medium before the start of the examination.
B. Small bowel distension is a key factor in the examination.
C. Patients should ideally ingest 1 L of negative oral contrast medium before the start of the examination.
D. In CT enterography, CT scanning should start 65 seconds after the start of IV administration of contrast infusion.
E. Patients should fast for 4 hours before the exam.
- A. Patients should ideally ingest 1 L of positive oral contrast medium before the start of the
examination.
Patients undergoing CT enterography are asked to withhold all oral intake, starting 4 hours before the examination. To improve visualisation of the mucosa and achieve better bowel distension, a negative oral contrast agent is administered. A typical regimen with regard to the timing of administration of oral contrast agents involves the ingestion of a total of 1.35 L over 1 hour 450 mL at 60 minutes, 450 mL at 40 minutes, 225 mL at 20 minutes and 225 mL at 10 minutes before scanning. After the oral contrast agent is ingested, a bolus of intravenous contrast material followed by 50 mL of saline solution is administered with a power injector at a rate of 4 mL/sec.
Helical scanning is performed from the diaphragm to the symphysis pubis, beginning 65 seconds after the administration of intravenous contrast material; it includes a single (venous) phase for the evaluation of known or suspected Crohn’s disease or dual (arterial and venous) phases for the evaluation of mesenteric vessels, GI tract bleeding and suspected tumours. Scanning parameters include a section thickness of 0.625 mm and interval of 0.625 mm.
- A 5-year old boy with a large head has widened sutures and Wormian bones on a skull radiograph. Review of other examinations performed earlier shows bilateral hypoplastic clavicles and delayed ossification of symphysis pubis. A chest radiograph shows supernumerary ribs. What is your diagnosis?
A. Hypothyroidism
B. Primary hyperparathyroidism
C. Cleidocranial dysostosis
D. Ehlers-Danlos syndrome
F. Downs syndrome
- C. Cleidocranial dysostosis
Cleidocranial dysplasia (CCD) is characterised by aplasia or hypoplasia of the clavicles, characteristic craniofacial malformations, and the presence of numerous supernumerary and unerupted teeth. Cranial abnormalities include wide open sutures, patent fontanelles and the presence of Wormian bones. Delayed closure of cranial sutures and fontanelles leads to frontal, parietal and occipital bossing. Additionally, there may be poor or absent pneumatization of paranasal, frontal and mastoid, and sphenoid sinuses.
Pelvic features include delayed ossification with wide pubic symphysis, hypoplastic iliac wings, widened sacroiliac joints and a large femoral neck resulting in coxa vara.
The differential diagnosis of CCD includes Crane-Heise syndrome (CCD with cleft lip and agenesis of cervical vertebra), mandibuloacral dysplasia (CCD plus hypoplastic mandible), pyknodysostosis (CCD and osteopetrosis), Yunis-Varon syndrome (CCD with hypoplastic thumb and big toe), CDAGS syndrome (craniosynostosis, anal anomalies and genital hyplasia).
- A child presents following a witnessed first seizure. An MRI scan is arranged. This shows a well-demarcated, T1 hypointense, T2 hyperintense supratentorial mass with a bright rim on FLAIR. There was no enhancement post-contrast. Which of the following is the most likely diagnosis?
A. Ependymoma
B. Dysembryoplastic neuroepithelial tumour
C. Pilocytic astrocytoma
D. Oligodendroglioma
F. Ganglioglioma
- B. Dysembryoplastic neuroepithelial tumour
History of seizure that may be medically refractory makes dysembryoplastic neuroepithelial tumour (DNET) more likely.
DNET is a benign, supratentorial and predominantly cortical intra-axial lesion, characterised by a multinodular architecture. Although DNETs are usually located in the temporal lobe, any lobe within the brain lobes may be involved. They have a cortical base and an apex pointing towards the lateral ventricle. They are homogeneously hyperintense on T2-weighted images and hypointense on T1-weighted images. Some delicate septa like structures are visible within the lesions. Despite their size, neither mass effect nor surrounding parenchymal oedema is present. On FLAIR images, the lesions show a hyperintense ring. Susceptibility-weighted images do not depict any hypointense signal in the lesion, which indicates the absence of calcium or blood products. Very high ADC values are measured inside the mass. No contrast enhancement is noted and there is scalloping of overlying bone.
The differential diagnosis includes other brain tumours, such as ganglioglioma (cyst with a strongly enhancing mural nodule, frequent calcification), angiocentric glioma (hyperintense on Tl and star-like extension to ventricle), low grade astrocytoma (similar to DNET but no scalloping of bone or rim of FLAIR) and pleomorphic xanthoastrocytoma (cyst with mural nodule, enhancement and dural tail).