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Journal club cases
1. Journal club cases
1.Congenital Defect in the Pericardium
2.Aspirated Foreign Body
3.Wormian Bones
4.Retroperitoneal Fibrosis
2. Case 1
What is the most likely diagnosis?
41 year-old male, pre-op for hernia surgery
3. Frontal and lateral chest radiographs demonstrate an unusually-shaped (yellow arrow)
levopositioned (green arrow) heart. The heart is displaced upward from the left
hemidiaphragm (white arrow) and there is a clear space between the sternum and the
heart (blue arrow) on the lateral image.
4. Congenital Defect in the Pericardium
Rare absence of a part or all of the pericardium
Due to failure of pericardial development
secondary to premature atrophy of the left duct
of Cuvier (cardinal vein) which then fails to
nourish the left pleuropericardial membrane
Male:female ratio of 3:1
May be detected at any age but most commonly
in low 20’s
5. Location
Foraminal defect on left side 35%
Complete absence of left side 35%
levoposition of heart
Diaphragmatic surface 17%
Total bilateral absence 9%
Right sided 4%
6. Clinical findings
Mostly asymptomatic
May have:
Tachycardia
Palpitations
Right bundle block
Positional discomfort lying on left side
Chest pain
7. Imaging Findings
Focal bulge in area of main pulmonary artery
or left atrium in partial defects
In complete form, heart rotates up and to the
left
Lung may interpose between heart and left
hemidiaphragm
Increased distance between sternum and
heart 2° absence of sternopericardial ligament
8. Lung may interpose between aorta and main
pulmonary artery on axial CT scans
Levoposition of heart
9. Treatment
Asymptomatic, complete absence of the
pericardium and very small defects present no
danger to the patient and require no intervention
Symptomatic foraminal defect may require
surgery because of herniation and strangulation
of left atrial appendage or herniation of LA/LV
Surgery can be enlargement of the defect to prevent
strangulation or closure of the defect
10. Complications and associations
Associated congenital anomalies occur in about
30 per cent of the reported cases
Bronchogenic cysts
ASD, VSD, PDA, Tetralogy of Fallot, Mitral
stenosis
Diaphragmatic hernia
Pulmonary sequestration
13. Aspirated Foreign Body
Children between 1-3 are most at risk
Up until age 15, both right and left main
bronchi arise at about the same angle from the
trachea so that objects may be aspirated into
either side
Afterwards, the right main bronchus arises in a
less acute, more straight path than the left
14. The most frequently aspirated foreign bodies
are food (especially nuts), teeth, dental
devices and medical instruments
Some nuts, such as peanuts, have an oil that
leads to inflammation and edema making them
more difficult to expel
15. Clinical Findings
Many go undiscovered delaying diagnosis
Cough
Wheeze
Stridor
Dyspnea
Cyanosis
Asphyxia if the object obstructs the trachea or
larynx
16. Imaging Findings
A normal chest radiograph does not exclude
an aspirated foreign body
Children will more often display signs of air-trapping
while adults will more often show
atelectasis
80% of aspirated foreign bodies will be non-opaque
on conventional radiography
17. Hyperinflation of one lung or lobe may occur
(obstructive emphysema)
Lobar or segmental atelectasis
Mediastinal shift
Pneumomediastinum
CT may demonstrate the foreign body or better
show the narrowing of the bronchus
18. Differential Diagnosis
Asthma
In an adult, a large pulmonary embolus may
appear to cause increased lucency of one
hemithorax
Swyer-James syndrome
Lack of soft tissue on one side, such as from a
mastectomy or Poland Syndrome
19. complications
Mediastinitis or tracheoesophageal fistulas
Air trapping leading
Obstructive emphysema
Atelectasis
Post-obstructive pneumonia
Abscess
Bronchiectasis
21. Case 3
28 year-old hit on the head with a brick
22.
23. Wormian Bones
Accessory bones within a suture of the skull
Most often the lambdoid suture
Usually a normal variant occurring in as many
as 80% of Asian population
Males more frequently affected than females
Pathological only when greater than 10 in
number or large
24. A larger, single, centrally located intrasutural
bone at the junction of the lambdoid suture
and sagittal suture is called the os inca
27. Retroperitoneal Fibrosis
Relatively uncommon
More common in males than in females
Predominantly patients aged 40-60 years
In almost 70% of patients, no cause is found
28. Primary or Idiopathic Retroperitoneal Fibrosis
(RPF)
May be an autoimmune response.
Primary biliary cirrhosis
Fibrosing mediastinitis
Glomerulonephritis
Panhypopituitarism
31. Desmoplastic response to malignancy
Lymphoma
Carcinoid
Retroperitoneal metastases (breast, lung,
thyroid, GI tract, GU organs)
32. Retroperitoneal fluid collection as in trauma,
surgery or infection
Aneurysm of the aorta or iliac arteries
(desmoplastic response)
Radiation therapy
33. Clinical findings
Most common presentation is flank, back,
scrotal or lower abdominal pain
Fever
Weight loss
Nausea and vomiting
Symptoms relating to renal impairment and
hypertension are common clinical features
34. Imaging Findings
US
Hypoechoic homogeneous mass
On CT scans
Rind of soft tissue around aorta and inferior
vena cava between level of kidney and sacrum
Spreads to involve the ureters, causing
varying degrees of obstruction.
Fat plane between the mass and the psoas
muscle may be obliterated
35. MRI
Low to medium homogeneous signal intensity
Heterogeneous high signal intensity on T2
(inflammatory stage)
Low signal intensity on T2 (dense fibrotic
stage)