Case Presentation: Kartagener Syndrome in a 24-Year-Old Male
24-year-old male patient with a history of asthma and bronchiectasis presents with worsening shortness of breath and chest pain. Neonatal history reveals Situs inversus and dextrocardia, recurrent upper and lower respiratory tract infections, while childhood history includes asthma diagnosis and chronic symptoms. Imaging reports show bronchiectatic changes and opacified sinus cavities. Examination findings include finger clubbing, chest crackles, reduced breath sounds, and nasal polyps. Diagnosis of Kartagener Syndrome is made due to the triad of bronchiectasis, chronic sinusitis, and Situs inversus. Inheritance is linked to defective ciliary motility.
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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
KARTAGENER SYNDROME TR I A D O F B R O NC HI EC TA SI S , C HR O NI C SI NU SI TI S A ND SI TU S I NVE R SU S
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
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
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
