The LMA was used 12 times in six patients either to avoid conventional intubation or when conventional intubation was not possible, generating one case of difficult but adequate ventilation. A paediatric videolaryngoscope was successfully used five times, three times as first choice, once after failed direct laryngoscopy with Cormack and Lehane grade 3–4 and once for urgent re-intubation in the recovery room. We did not include this case in our results because it was an emergency situation and not elective. The median age of the children treated with this device was 6.9 (18 months–12 years) years.
Postoperatively, five children had to be admitted to the ICU and four were mechanically ventilated. Three had to be ventilated for reasons such as following intracranial surgery or prolonged anaesthesia of more than 5 h and one child after urgent re-intubation in the recovery room due to respiratory failure following an extensive bronchoalveolar lavage. In this case, re-intubation was performed with a videolaryngoscope 90 min after arrival in the recovery room and failed at the first attempt because the appropriate size for this child was not available in Germany that time. Unfortunately, the child aspirated gastric fluid prior to the successful second attempt and so had to be transferred to the ICU. The fifth patient was admitted to ICU breathing spontaneously for close surveillance and general care. The remaining 36 cases stayed in the recovery room for a median duration of 45 (15–140) min.
The main challenge for anaesthesiologists when dealing with children with MPS1H remains the airway, although the incidences of difficult and failed conventional intubation in our series (43 and 12%, respectively) are slightly better than those reported earlier.5 In 1996, Moores et al.3 reported seven children with MPS1H after stem cell transplantation undergoing 16 anaesthetics. Conventional intubation was difficult in 72% and failed in 29%. Walker et al.1 published a larger trial, including 13 children with MPS1H without referring to the total number of anaesthetics in this group in which intubation was described as difficult in 54% and failed in 23%. Another series from 1993 describes perioperative experiences gathered in 14 children with MPS1H. The airway was difficult in 60% of the intubations.6 The largest series to date was from Mahoney et al. in 1992, analysing 22 children with MPS1H who received 110 general anaesthetics. Difficult airways occurred in 41% of the children or 34% of the conventional intubations.2
It has been suggested that tracheal intubation becomes more difficult as children with mucopolysaccharidoses grow older, independent of prior stem cell transplantation treatment.5,6 We have collected our data against a background of new improved early stem cell transplantation protocols and are unable to confirm this observation (Table 4). Successful stem cell transplantation initiates a regression in specific soft tissue changes 6,7 and this has beneficial effects on upper airway obstruction.8 These improvements are more pronounced after early stem cell transplantation and are reflected, for example, by significantly enhanced pulmonary function.9 Although this might be expected to make anaesthesia safer, to date there has been no significant reduction in anaesthesia-related perioperative complications. Published reports indicate that the incidence of severe complications varies from 4 to 14% and includes urgent reintubation,6 intraoperative death,2 urgent tracheostomy1 and emergency tracheostomy with secondary cardiac arrest.3 There were no serious life-threatening complications in our series except one urgent re-intubation in the recovery room mentioned above. While in the 1990s, 53–75% of Hurler syndrome patients survived after stem cell transplantation,10 all our children now have this treatment, with 100% survival.11 This has the obvious consequence of more surgical and diagnostic procedures requiring anaesthesia in this group which may explain the minor changes in anaesthesia in recent years. Another difficulty interpreting the information is that the age of MPS1H children is not mentioned in most reports. In one series the mean age was 2 yr and 11 months (range 2 months–8 yr 2 months) and in another it was 3.4 (2.9) yr.2,6 Our children with MPS1H were older, with a mean age of 5.0 (4.3) years. The reduced incidence of anaesthesia complications suggests that our airway outcomes seemed to be improved after early stem cell transplantation.
None of the authors had assistance, financial support or sponsorship. There were no conflicts of interest.
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