MARIAM MUKHTAR CASE PRESENTATION. CASE: 24 year old male patient k/C of asthma and bronchiectasis....

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