Paediatric Syndromes, Neuromuscular Diseases, and Haemoglobinopathies — WACS Viva & Clinical Scenarios (Paediatrics & Day Care)

Exam-style paediatric syndromes, neuromuscular diseases, and haemoglobinopathies viva scenarios with examiner probes and model answers for ANAESTHESIA…

Scenarios covered

• SCENARIO 1: A 6-year-old child weighing 18 kg with known Sickle Cell Anaemia (HbSS, steady-state Hb 6.8 g/dL) presents with acute right iliac fossa pain and is scheduled for an emergency appendicectomy. Detail your complete perioperative management, addressing the role of preoperative blood transfusion, fluid therapy, oxygenation, pain control, and prevention of Acute Chest Syndrome (ACS).
• SCENARIO 2: A 3-month-old infant with Pierre Robin Sequence is scheduled for bilateral cleft palate and lip reconstruction. Preoperative examination reveals extreme micrognathia and glossoptosis with intercostal retractions when supine. Describe your airway management strategy from pre-induction to extubation, detailing your rescue algorithms for cannot intubate, cannot oxygenate (CICO) scenarios.
• SCENARIO 3: A 4-year-old child with Down Syndrome (Trisomy 21) is scheduled for elective cardiac catheterisation and repair of an inguinal hernia. Preoperative echocardiography demonstrates an unrepaired ostium primum atrial septal defect with mild pulmonary hypertension. Outline your anaesthetic plan, specifically addressing airway difficulties, subglottic stenosis risk, atlantoaxial instability, and hemodynamic stability.
• SCENARIO 4: A 7-year-old boy with suspected Duchenne Muscular Dystrophy (DMD) presents for open reduction and internal fixation of a fractured femur. Explain why standard inhalational anaesthesia and succinylcholine are strictly contraindicated, what pathological phenomenon occurs if they are mistakenly administered, and how you execute a safe trigger-free anaesthetic technique.