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A-1442
2004
Anesthesia Risks before and after Hematopoietic Stem Cell Transplant (HSCT) in Hurler's Syndrome
Lipi M. Ramchandani, M.B.B.S., Satkiran S. Grewal, M.D., David S. Beebe, M.D., Charles Peters, M.D., Kumar G. Belani, M.D.
Anesthesiology & Pediatric Blood and Marrow Transplantation, University of Minnesota Medical School, Minneapolis, Minnesota, United States.
Introduction:

Type 1 (H) Mucopolysaccharidosis (Hurler's syndrome, 1H MPS) is an autosomal recessive storage disorder due to a deficiency of enzyme α-L iduronidase. This enzyme degrades glycosaminoglycans (GAGS). Its absence leads to deposition of dermatan and heparan sulfate substrates in body tissues. Infiltration of GAGS in the oropharynx, joints, and soft tissues results in significant airway and cardiovascular abnormalities creating major risks during anesthesia care1. HSCT has been used for treatment since 1980 and has shown to improve neuropsychological, cardiac functions, and long term survival1. This review examines airway related changes following HSCT in a large group of Hurler patients.

Methods:

A cohort review was done on 29 males and 28 females with Type 1H MPS. All underwent HSCT between 1992 and 2002 at our institution. Anesthesia related difficulties encountered during non-emergent procedures were evaluated before and after successful HSCT. The various parameters taken into account included difficulty in intubation, method of intubation, number of attempts at intubation, and extubation related complications

Results:

There were 302 anesthetic procedures performed in 57 patients. Anesthesia providers were skilled in airway care and had equipment available to handle a difficult airway. Average number of procedures performed on each patient was 5.8 (Pre HSCT = 2.8 and Post HSCT = 2.6). Pre and post HSCT data was available in 35 patients, to make valid comparison. Among 35 patients the number of attempts at performing intubations became fewer in 13 (38%), remained the same as before transplant in 12 (35%) and increased in 8 (22%). The median age of patients in whom the number of attempts became fewer after transplant was 18 months (Range 6 -31 months), in patients where it remained same was 14 months (Range 8-24 months), and in patients in whom it increased was 22 months (Range 17- 216 months). Using Wilcoxon rank test for analysis, the correlation between the age at transplant and the number of attempts at intubations was significant (p= 0.03). Figure1 shows that there is a statistical difference in age between group A (older) in whom more attempts were required for endotracheal intubation following HSCT and group B (in whom the attempts either decreased or stayed the same). Extubation related complications did not show any change (before versus after transplant). Conclusion:

Successful HSCT when performed before 22 months of age may result in improvement of the upper airway in children with Type 1H MPS.

Reference:

1. Belani KG, Krivit W, Carpenter BL, Braunlin E, Buckley JJ.et al. Children with mucopolysaccharidosis: perioperative care, morbidity, mortality, and new findings. Journal of Pediatric Surgery 28(3): 403-410; 1993.Mar

Anesthesiology 2004; 101: A1442
Figure 1