66yo male for colonoscopy for polypectomy.
- Kartagener’s Syndrome
- Chronic SOB, ok on flat
- FEV1 1.65 (50%), FVC 2.71 (63%) ratio 61% TLCO 54%
- Situs inversus totalis
- HTN & Dyslipidaemia
- 2019 critical illness
- Life-threatening pulmonary haemorrhage
- Failed intubation due to bleeding -> surgical cricothyroidotomy
- 2/12 ICU stay, 17 days ECMO, DVT, IVC filter.
- Multiple tracheal/bronchial clot retrievals and bronchial artery embolization.
- Recent Colonoscopy/ICU stay
- Failed colonoscopy in private hospital due to difficulty passing scope.
- Patient reported anaesthesia complication and ICU stay post-procedure
- Anaesthetic chart – THRIVE and sedation, nil concerns
- ICU d/c summary – precautionary admission, nil adverse events.
- What is Kartagener’s Syndrome?
- Autosomal recessive, multiple possible genetic pathways known, some unidentified.
- Primary ciliary dyskinesia leads to:
- Neonatal distress syndrome
- Frequent sinus and middle ear infections, hearing loss
- Frequent resp infections, leading to bronchiectasis
- Situs inversus totalis (but organs unaffected in other ways)
- Proceed to colonoscopy
- Suggest right lateral position to aid scope passage.
- If more major surgery required consider pulmonary rehab, nutrition optimization and respiratory review for bronchiectasis.