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MARIAM MUKHTAR
CASE PRESENTATION
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
T R I A D O F B R O N C H I E C T A S I S , C H R O N I C S I N U S I T I S A N D S I T U S I N V E R S U S
KARTAGENER SYNDROME
KARTAGENER SYNDROME
• AR inheritance• defective ciliary motility • 1 /3200 live births• Situs inversus in 50%
CLINICAL MANIFESTATIONS
• 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
• 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
• BronchiectasisNon specific tubular or cystic
• 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 journalWikipedia