1.Congenital Defect in the Pericardium 2.Aspirated Foreign Body 3.Wormian Bones 4.Retroperitoneal Fibrosis

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

11. Case 2
 16 month-old with wheezing

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

20. Treatment
 Bronchoscopic removal

21. Case 3
 28 year-old hit on the head with a brick

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

25. Diseases Associated With Wormian Bones
 Cleidocranial Dysplasia
 Pyknodysostosis
 Osteogenesis Imperfecta
 Hypothyroidism
 Hypophosphatasia
 Acro-osteolysis
 Down Syndrome

26. Case 4
 61 year-old with chronic back pain

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

29.  Rheumatoid arthritis
 Ankylosing spondylitis
 Polyarteritis nodosa
 Systemic lupus erythematosus (SLE)
 Hashimoto thyroiditis

30. Secondary Retroperitoneal Fibrosis
 Has been found associated with certain drugs,
such as
 Methylsergicide
 Beta-adrenergic blockers

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)

36. Prognosis
 Satisfactory if renal impairment is not too
severe

37. THANK YOU