Thalassaemias are a group of hereditary disorders characterised by a reduction in the synthesis of globin chain (α or beta) in haemoglobin (Hb) molecule. Beta thalassaemia major has a defect in both alleles leading to a defect of the beta-globin chain of Hb A molecule with an autosomal recessive inheritance and is considered more dangerous.
We are reporting the anaesthetic management of a 26-year-old female, a known case of beta thalassaemia major, post-splenectomy at 5 years of age. She had presented with swelling over the right side of the neck and was posted for cervical lymph node biopsy. She gave a history of dyspnoea on exertion. On examination, the airway was Mallampati Grade 3, and multiple enlarged discrete cervical lymph nodes were present. She had Hb of 9.2 g/dL, the pulmonary function test showed a restrictive pattern and the echo showed moderate pulmonary hypertension. Liver function tests were normal.
The plan of anaesthesia was general anaesthesia with endotracheal intubation and positive pressure ventilation. She was pre-oxygenated, midazolam 1 mg and fentanyl 2 mcg/kg were given, induced with propofol 2 mg/kg and intubated following administration of suxamethonium 2 mg/kg using C MAC video laryngoscope. A well-lubricated cuffed flexo metallic 6.5 mm endotracheal tube was passed under vision. After confirming bilateral air entry and the appearance of end-tidal carbon dioxide waveforms, atracurium 0.5 mg/kg was given. Anaesthesia was maintained with oxygen air mixture (1:1) with sevoflurane 2%–2.5%.
Intraoperatively, blood pressure remained high which was managed with increasing sevoflurane and intravenous incremental boluses of labetalol 5 mg and propofol 0.5 mg/kg. At the end of the surgery, the neuromuscular blockade was reversed and extubated when fully awake after gentle oral suctioning. Post-operatively, her saturation remained 95%–96% and hence the patient was retained in the post-operative intensive care unit with oxygen supplementation. Deep-vein thrombosis (DVT) prophylaxis with enoxaparin 40 mg subcutaneously was initiated. She was shifted to the ward on the next day and had an unremarkable recovery. We used suxamethonium for intubation as we anticipated a difficult airway in view of Mallampati Grade 3 airway and also due to presumed difficulties with laryngoscopy and intubation in patients with thalassaemia. After securing the airway, we opted for atracurium, a longer-acting muscle relaxant, as the surgery was expected to last for 30–45 min.
The incidence of beta trait of thalassaemia is common in India with a frequency of 1%–17%. Anaesthetic challenges in these patients are unanticipated difficult airway due to facial skeletal deformities, peri-operative high blood pressure, endocrinological abnormalities, cardiac diseases, a restrictive respiratory disease with severe ventilation/perfusion mismatch, pulmonary hypertension, life-threatening bleeding and problems associated with anaemia and iron overload.[3,4] Splenectomy is a common surgery performed in children of beta thalassaemia major to reduce the frequency of transfusion.
Difficult intubation could be due to maxillary hypertrophy or orofacial malformations. They may have a high-arched palate. Organ dysfunction occurs due to iron overload requiring adequate chelating therapy. Hypercoagulability is common and hence preventive measures for DVT are mandatory. Positioning under anaesthesia should be done with caution as the risk of pathological fractures is high due to osteoporosis. Intraoperative blood salvage is considered useful as decreased membrane stability of thalassaemic red cells makes them prone to haemolysis. Infections, oxidant drugs such as prilocaine, nitroprusside, Vitamin K, aspirin, penicillin, sulphonamides and chloramphenicol, enhance haemolysis and induce oxidation and intracellular precipitation of Hb molecule.
A multidisciplinary team involving an anaesthesiologist, surgeon and haematologist are essential for the optimal management of patients with thalassaemia. Judicious selection of anaesthetic drugs and techniques ensures a safe outcome.
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