CASE PRESENTATION
MARIAM MUKHTAR
CASE:
24 year old male patient
k/C of asthma and bronchiectasis.
c/o:
worsening SOB and C/P x 1m .
HISTORY
Neonatal history:
• Situs inversus & dextrocardia
• repeated URTI :
Recurrent OM & coryza
Chronic ,thick , muciod rhinorrhea
• recurrent LRTI :
Pneumonia ,bronchiolitis
HISTORY
At age of 3 years:
Diagnosed to have asthma:
• SOB , chronic cough
• Nasal polyps
• obstructive pattern of PFT
• improvement on BD
HISTORY:
On asthma meds:
• Multiple admissions
• Symptoms of sinusitis
• Worsening of productive cough
• recurrent pleural effusions
• Thoracotomy & decortication
IMAGING REPORTS
Early childhood:
• Situs inversus totalis
• Hyper-infilated lungs
• Areas of consolidation
Last few years:
• Bronchiectatic changes ( progressive)
• Opacified sinus cavities
• pleural effusion
EXAMINATION
• Normal vitals
• Finger clubbing
• Chest: basal Coarse crackles, reduced breath
sounds on left side
• nose: Pale, edematous mucosa & polyps
• Tenderness over sinuses
IMAGING
IMAGING
IMAGING
IMAGING
IMAGING
KARTAGENER
SYNDROME
TRIAD OF BRONCHIECTASIS , CHRONIC SINUSITIS
AND SITUS INVERSUS
KARTAGENER SYNDROME
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AR inheritance
defective ciliary motility
1 /3200 live births
Situs inversus in 50%
CLINICAL MANIFESTATIONS
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Chronic URTI,LRTI
Bronchiectasis
Corneal abnormalities
poor sense of smell
Male Infertility
female sub-fertility
DIAGNOSIS
• 50 visits
• Average age 10-14 at diagnosis
• Clinical/ tests:
nitric oxide test , mucociliary transport
test , semen analysis & PFT
• Imaging
• Biopsy
OTHER ASSOCIATIONS
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transposition of great vessels
Trilocular /bilocular heart
Pyloric stenosis
Polysplenia
Hepatic statosis
Hydrocephalus
IMAGING
Plain films:
• Bronchial wall thickening
• Bronchial dilatation + loss of normal tapering
• Finger in glove sign ( mucus plug)
• Situs inversus
KARTAGENER SYNDROME
CT SCANS
• Bronchiectasis
Non specific
tubular or cystic
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Consolidation
Mucocele
Tree in bud or centrolobular nodules
Scarring
Situs inversus
Opacified sinus cavities , hypoplastic frontal sinuses
KARTAGENER SYNDROME
DIFFERENTIAL DIAGNOSES
• Hereditary impaired muco-cilliary clearance : e.g. CF
, Young syndrome
• Impaired immunity:
1ry immunodeficiency syndrome, AIDS
• Hyper-sensitvity and immune reaction: e.g. ABPA
• Alpha 1 antitrypsin deficiency
MANAGEMENT
• Medical care:
Immunizations , A/B ,inhaled BD ,
Steriod , PT
• Surgical care:
Tympanostomy tubes ,sinus surgeries
• Consultations: ENT , chest ,OB/ GYNE and urology
physicians , social workers
THANK YOU
References:
Medscape
Radiopaedia.org.
Radiographics journal
Wikipedia