Sir,Rubinstein-Taybi syndrome (RTS) is a rare autosomal dominant disorder characterised by mental and growth retardation, craniofacial, vertebral, ocular and cardiac anomalies, gastro-oesophageal reflux and difficult airway [Table 1].[12]
Table 1
Systemic involvement in Rubinstein-Taybi syndrome
Systemic involvement in Rubinstein-Taybi syndromeA 4-years-old 14 kg boy with bilateral ptosis was scheduled for corrective ocular surgery. He had a history of recurrent regurgitation and respiratory tract infection. However, his effort tolerance and developmental milestones were normal. He had broad thumbs, pectus excavatum, hyperteleorism, micrognathia, retrognathia, prominent incisors and high arched palate. The modified Mallampatti class was III and neck movements were normal. Vital parameters were stable. Haematological and biochemical investigations, chest X-ray, electrocardiography (ECG) and echocardiography were normal.He was made to fast for 8 hours for solids and 2 hours for clear liquids and premedicated with intravenous ranitidine 20 mg and metoclopramide 2.5 mg. In the operating room, he was preoxygenated for 3 minutes and propofol 50 mg and Rocuronium 15 mg were injected as cricoid pressure was applied. Direct laryngoscopy revealed Cormack-Lehane grade IIIb; gum elastic bougie could not be negotiated. Gentle mask ventilation with 100% oxygen and sevoflurane was initiated maintaining cricoid pressure. Second attempt using McCoy laryngoscope also failed. Subsequently, air-Q intubating laryngeal airway (ILA) #2 was inserted and adequate ventilation was achieved. Uncuffed endotracheal tube #5 mm was successfully inserted through the air-Q ILA in the first attempt and the latter was removed. Pharyngeal packing was done. Cricoid pressure was maintained throughout the entire procedure till intubation.Anaesthesia was maintained with 50% oxygen-air mixture and sevoflurane. Fentanyl 30 mcg and intravenous paracetamol 200 mg were administered for analgesia. At the end of surgery, residual neuromuscular blockade was reversed with neostigmine and glycopyrrolate and trachea was extubated after the child was fully awake. Postoperative course was uneventful and the child was discharged home after 3 days.In view of severe gastro-oesophageal reflux and recurrent aspiration, rapid sequence induction of anaesthesia with endotracheal intubation was planned. Muscle relaxant was deemed necessary for rapid sequence intubation and rocuronium was used as succinylcholine is known to cause serious arrhythmia in RTSpatients.[3] Air-Q ILA was chosen as it is easy to insert and provides good conduit for intubation. Air-Q ILA has been successfully used as a rescue airway device following failed rapid sequence intubation in two children with airway haemorrhage.[4] In the present case, air-Q could be placed easily and endotracheal tube was passed successfully. Spontaneous inhalational induction with fibre optic bronchoscope (FOB) guided intubation could be safer option in such cases of difficult airway, but due to risk of aspiration this was not done. However, FOB was kept ready. Abnormal narrowing of upper and lower airway and tracheomalacia may be present in patients with RTS and relatively smaller size tube should be chosen.[5] Anticholinesterase and anticholinergic drugs can precipitate arrhythmia in RTSpatients with associated cardiac anomalies.[1] This patient did not have any cardiac anomaly and neostigmine was used under continuous ECG monitoring. Postoperative oxygen therapy and vigilant monitoring is important as postoperative respiratory distress with copious secretion and recurrent desaturation episodes have been reported even after uneventful anaesthesia in these patients.[35]To conclude, difficult airway along with risk of pulmonary complications and cardiac involvement are main concerns in children with RTS. Air-Q intubating laryngeal airway is a useful airway device in children with difficult airway and it should be readily available in theatres where high-risk children with difficult airway are regularly anaesthetized.