1. MARIAM MUKHTAR CASE PRESENTATION

2. CASE: 24 year old male patient k/C of asthma and bronchiectasis. c/o: worsening SOB and C/P x 1m.

3. HISTORY Neonatal history: Situs inversus & dextrocardia repeated URTI : Recurrent OM & coryza Chronic,thick, muciod rhinorrhea recurrent LRTI : Pneumonia,bronchiolitis

4. HISTORY At age of 3 years: Diagnosed to have asthma: SOB, chronic cough Nasal polyps obstructive pattern of PFT improvement on BD

5. HISTORY: On asthma meds: Multiple admissions Symptoms of sinusitis Worsening of productive cough recurrent pleural effusions Thoracotomy & decortication

6. IMAGING REPORTS Early childhood: Situs inversus totalis Hyper-infilated lungs Areas of consolidation Last few years: Bronchiectatic changes ( progressive) Opacified sinus cavities pleural effusion

7. EXAMINATION Normal vitals Finger clubbing Chest: basal Coarse crackles, reduced breath sounds on left side nose: Pale, edematous mucosa & polyps Tenderness over sinuses

8. IMAGING

13. TRIAD OF BRONCHIECTASIS, CHRONIC SINUSITIS AND SITUS INVERSUS KARTAGENER SYNDROME

14. AR inheritance defective ciliary motility 1 /3200 live births Situs inversus in 50%

15. CLINICAL MANIFESTATIONS Chronic URTI,LRTI Bronchiectasis Corneal abnormalities poor sense of smell Male Infertility female sub-fertility

16. DIAGNOSIS 50 visits Average age 10-14 at diagnosis Clinical/ tests: nitric oxide test, mucociliary transport test, semen analysis & PFT Imaging Biopsy

17. OTHER ASSOCIATIONS transposition of great vessels Trilocular /bilocular heart Pyloric stenosis Polysplenia Hepatic statosis Hydrocephalus

18. IMAGING Plain films: Bronchial wall thickening Bronchial dilatation + loss of normal tapering Finger in glove sign ( mucus plug) Situs inversus

19. KARTAGENER SYNDROME

20. CT SCANS Bronchiectasis Non specific tubular or cystic Consolidation Mucocele Tree in bud or centrolobular nodules Scarring Situs inversus Opacified sinus cavities, hypoplastic frontal sinuses

21. KARTAGENER SYNDROME

22. 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

23. 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

24. THANK YOU References: Medscape Radiopaedia.org. Radiographics journal Wikipedia