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Paediatric airway management in mucopolysaccharidosis 1: a retrospective case review

Osthaus, Wilhlem A.*; Harendza, Thomas*; Witt, Lars H.; Jüttner, Björn; Dieck, Thorben; Grigull, Lorenz; Raymondos, Konstantinos; Sümpelmann, Robert

European Journal of Anaesthesiology: April 2012 - Volume 29 - Issue 4 - p 204–207
doi: 10.1097/EJA.0b013e328350677b
Airway management
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Context Airway management in children suffering from mucopolysaccharidosis 1 (Hurler syndrome) remains challenging despite advances in both treatment and airway management techniques.

Objectives Forty-one anaesthetic charts following ten children over a 6-year period (2004–2010) were reviewed with emphasis on airway problems.

Results All children had early stem cell transplantation at the age of 2 years or earlier. Mean (SD) age was 5 (4.3) years. Mask ventilation was difficult in five of 41 (12%) anaesthetics or in three of ten children. There were 29 intubations. Direct laryngoscopy was described as difficult (Cormack and Lehane ≥3) on 11 occasions in five of ten children. There were three of 26 (12%) failed intubations with direct laryngoscopy. These situations were resolved by a fibre-optic procedure, by laryngeal mask airway (LMA) insertion or by use of a videolaryngoscope. A laryngeal mask airway was used 11 times to avoid invasive airway management and once when direct laryngoscopy was impossible.

Conclusion The airway management of children with mucopolysaccharidosis 1 remains critical, despite advances in both treatment and airway management techniques. Problems did not seem to increase as children grew older. We assume that technical improvements such as standardised use of the laryngeal mask airway or attached tube channel videolaryngoscopes as well as a stem cell transplantation treatment of the disease helped the management of older children with mucopolysaccharidosis 1.

From the Department of Anaesthesiology and Intensive Care Medicine (WAO, TH, LHW, BJ, TD, KR, RS) and Department of Paediatric Haematology and Oncology (LG), Hannover Medical School, Hannover, Germany

*Wilhlem A. Osthaus and Thomas Harendza contributed equally to the writing of this article.

Correspondence to Thomas Harendza, Department of Anaesthesiology and Intensive Care Medicine, Hannover Medical School, Carl Neuberg Str. 1, 30625, Hannover, Germany E-mail: harendza.thomas@mh-hannover.de

Published online 22 February 2012

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Introduction

Hurler syndrome (MPS1H) is a form of mucopolysaccharidosis with an estimated prevalence of 0.7–1.6 per 100 000 people. It is genetically determined, generating skeletal deformities, corneal clouding, organomegaly, heart disease, short stature, hernias, facial dysmorphism, hirsutism and hydrocephaly. The underlying mechanism is a defect of lysosomal storage, resulting in accumulation of acid mucopolysaccharides in the central nervous system and peripheral tissues. MPS1H has been described as the ‘worst airway problem in paediatric anaesthesia’.1 Children have a narrowed nasal airway, adenotonsillar hypertrophy, an enlarged tongue, a short and immobile neck, stiff peri-laryngeal tissue and a thickened supraglottic and glottic area. Because this is a rare disorder, experience is small, especially following early stem cell transplantation and case series are few. The largest series was published in1992 and included 22 MPS1H patients.2 The most recent retrospective review was published in 1996.3 In recent years, drugs, monitoring, airway management strategies and devices have all improved, as indeed has stem cell transplantation treatment of this disorder.4 It was appropriate to perform a retrospective chart review within our department to see how advances might have influenced airway management in children suffering from MPS1H.

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Methods

This retrospective review of anaesthesia charts was carried out at a paediatric university hospital specialising in the treatment of inherited metabolic disorders by stem cell transplantation. In keeping with the Declaration of Helsinki, local Ethics Committee approval was considered unnecessary. All anaesthesia charts of MPS1H children undergoing elective general anaesthesia over a 6-year period (2004–2010) were reviewed. We evaluated the mode of anaesthesia, the airway devices used and the nature and frequency of intraoperative and postoperative complications. Data are presented as mean (SD) or median (range) where appropriate.

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Results

Patients

We identified ten children (three male, seven female) suffering from MPS1H who received 41 elective general anaesthetics between 2004 and 2010 (Fig. 1 and Table 1). Each child underwent early stem cell transplantation at the age of 2 years or younger. The mean age of the children at each anaesthetic episode was 5.0 (4.3) years, with a range from 6 months to 12 years. Each child had a median of 4.1 (2–8) general anaesthetics. All were conducted by experienced paediatric consultants.

Figure

Figure

Table 1

Table 1

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Indications for anaesthesia

The most frequent indications for anaesthesia were for diagnostic imaging such as MRI or computerised tomography (CT). For MRI, all children were given muscle relaxants, provided initial mask ventilation was satisfactory, intubated and ventilated. This was routine in our department because the average duration of the procedure was at least 40 min and we were anxious to avoid movement artefact associated with spontaneous breathing. In contrast, children undergoing CT were allowed to breathe spontaneously via facemask or laryngeal mask airway (LMA). The commonest surgical procedures were carpal tunnel release and adenotomy followed by neurosurgical interventions. Where surgical procedures allowed, patients were breathing spontaneously via LMA. The median duration of the elective surgical or diagnostic procedures was 77 (10–312) min. In order to reduce the risks associated with exposure to multiple anaesthetics, wherever possible two or more diagnostic and surgical interventions were combined during one episode (e.g. MRI and subsequent adenotomy).

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Anaesthesia

In 71% of children the anaesthesia was induced with propofol and 29% were induced with sevoflurane. Maintenance of anaesthesia was uneventful in all children included in this series.

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Facemask ventilation

Facemasks were used in all cases during induction. Difficulties in mask ventilation were recorded on five occasions in three of ten children and were remedied by insertion of an oral airway such as a Guedel's. In relation to the total number of anaesthetics, difficulties in mask ventilation were noted in 12% (five of 41). This did not change, as the children grew older. The three difficult airways remained difficult, whereas the others continued to pose little problem.

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Tracheal intubation

Tracheal intubation was attempted in 30 cases and direct laryngoscopy was successfully performed 23 times, but failed in three cases (12%). The alternative techniques employed were fibre-optic intubation on one occasion, attached tube channel videolaryngoscopes (Airtraq, Prodol Meditec, Vizcaya, Spain) on one occasion and an LMA. Details of these three failed attempts including age, time elapsed after stem cell transplantation and prior Cormack and Lehane grades are listed in Table 2. Fibre-optic intubation was performed successfully twice as the procedure of first choice.

Table 2

Table 2

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Cormack and Lehane grades

Our definition of difficult intubation was a Cormack and Lehane grade of at least 3 at direct laryngoscopy. Some 43% of direct laryngoscopies met this definition (Table 3). In seven children, the Cormack and Lehane grade did not change with age, but for one it improved and in two it became worse (Table 1). The incidence of difficult intubation was similar between the three age groups: 0–2, 2–4 and >4 years (Table 4). The distribution of the Cormack and Lehane laryngoscopy grade results is shown in Table 3.

Table 3

Table 3

Table 4

Table 4

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Alternative airway

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.

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Postoperative management

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.

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Discussion

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

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The impact of early stem cell transplantation

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.

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Alternative airway

Alternative airway management devices have a place in the management of children with MPS1H,12 particularly the LMA, which can be used instead of conventional intubation. Our experience with 12 LMA procedures is corroborated by Walker, 13 who, in 2000, reported 11 LMA insertions in eight children with MPS1H with no complications either alone or prior to fibre-optic intubation. We found only one case in which ventilation was difficult. Due to the abnormal anatomy, with large floppy arytenoids acting as ball valves under positive pressure,14 ventilation via supraglottic airway devices can be challenging. We also evaluated the role of the videolaryngoscope ATV. It has already been used successfully in children with Robin Sequence, Goldenhar syndrome or Treacher Collins syndrome, indicating the promising nature of these devices.15–18 We are the first to show preliminary results of videolaryngoscope use in children with MPS1H. An additional benefit of these devices is that neck extension can be avoided. Apart from airway problems, these children also suffer from a narrowed cervical spinal canal, leading to progressive myelopathy. Neck extension during conventional laryngoscopy may induce cervical cord damage which can be avoided by intubation in a neutral head position. We used our videolaryngoscope successfully on five occasions in four children; once in an urgent situation, once after failed conventional intubation and three times as the first choice technique.

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Conclusion

Our case series has shown a low incidence of serious anaesthesia-related perioperative complications despite difficult direct laryngoscopy in 43% of children with MPS1H, and despite the fact that our patients were older compared with previous studies. This finding could be influenced by the improved early stem cell transplantation strategy which slows the progress of the disease and improves the quality of life. Limitations due to the retrospective non-randomised and non-controlled design of our trial have to be considered.

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Acknowledgements

None of the authors had assistance, financial support or sponsorship. There were no conflicts of interest.

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References

1. Walker RW, Darowski M, Morris P, Wraith JE. Anaesthesia and mucopolysaccharidoses. A review of airway problems in children. Anaesthesia 1994; 49:1078–1084.
2. Mahoney A, Soni N, Vellodi A. Anaesthesia and the mucopolysaccharidoses: a review of patients treated by bone marrow transplantation. Paediatr Anaesth 1992; 2:317–324.
3. Moores C, Rogers JG, McKenzie IM, Brown TC. Anaesthesia for children with mucopolysaccharidoses. Anaesth Intensive Care 1996; 24:459–463.
4. Lucke T, Das AM, Hartmann H, et al. Developmental outcome in five children with Hurler syndrome after stem cell transplantation. A pilot study. Dev Med Child Neurol 2007; 49:693–696.
5. Kempthorne PM, Brown TC. Anaesthesia and the mucopolysaccharidoses: a survey of techniques and problems. Anaesth Intensive Care 1983; 11:203–207.
6. Belani KG, Krivit W, Carpenter BL, et al. Children with mucopolysaccharidosis: perioperative care, morbidity, mortality, and new findings. J Pediatr Surg 1993; 28:403–410.
7. Hobbs JR, Hugh-Jones K, Barrett AJ, et al. Reversal of clinical features of Hurler's disease and biochemical improvement after treatment by bone-marrow transplantation. Lancet 1981; 2:709–712.
8. Guffon N, Souillet G, Maire I, et al. Follow-up of nine patients with Hurler syndrome after bone marrow transplantation. J Pediatr 1998; 133:11–25.
9. Yeung A, Cowan M, Horn B, et al. Airway Management in children with mucopolysaccharidoses. Arch Otolaryngol Head Neck Surg 2009; 135:73–79.
10. Peters C, Balthazor M, Shapiro EG, et al. Outcome of unrelated donor bone marrow transplantation in 40 children with Hurler syndrome. Blood 1996; 87:4894–4902.
11. Sauer M, Meissner B, Fuchs D, et al. Allogeneic blood SCT for children with Hurler syndrome: results from the German multicenter approach MPS-HCT 2005. Bone Marrow Transplant 2009; 43:375–381.
12. Walker RW, Ellwood J. The management of difficult intubation in children. Paediatr Anaesth 2009; 19 (Suppl 1):77–87.
13. Walker RW. The laryngeal mask airway in the difficult paediatric airway: an assessment of positioning and use in fibreoptic intubation. Paediatr Anaesth 2000; 10:53–58.
14. Khan FA, Khan FH. Use of the laryngeal mask airway in mucopolysaccharidoses. Paediatr Anaesth 2002; 12:468.
15. Khalil S, Vinh B. Successful intubation of a child with Goldenhar syndrome, who previously failed intubation, using an Airtraq. Paediatr Anaesth 2010; 20:204–205.
16. Vlatten A, Soder C. Airtraq optical laryngoscope intubation in a 5-month-old infant with a difficult airway because of Robin sequence. Paediatr Anaesth 2009; 19:699–700.
17. Pean D, Desdoits A, Asehnoune K, Lejus C. Airtraq laryngoscope for intubation in Treacher Collins syndrome. Paediatr Anaesth 2009; 19:698–699.
18. Hirabayashi Y, Shimada N, Nagashima S. Tracheal intubation using pediatric Airtraq optical laryngoscope in a patient with Treacher Collins syndrome. Paediatr Anaesth 2009; 19:915–916.
Keywords:

airway management; anaesthesia; Hurler syndrome; mucopolysaccharidosis I; stem cell transplantation

© 2012 European Society of Anaesthesiology