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Resistance to rocuronium in a child with Schwartz-Jampel syndrome type 1 B
M Eikermann1, M Bredendiek, J Schaper
1Abteilung für Anästhesiologie und Intensivmedizin, Universität Essen, Germany. matthias.eikermann@uni-essen.de
Insights
Schwartz-Jampel syndrome patients require significantly higher doses of rocuronium for tracheal intubation due to muscle relaxant resistance. Careful monitoring is crucial for determining optimal neuromuscular blocking agent dosage in these rare cases.
Area of Science:
- Anesthesiology
- Genetics
- Pharmacology
Background:
- Schwartz-Jampel syndrome (SJS) presents with micrognathia and jaw muscle rigidity, complicating tracheal intubation.
- The neuromuscular response to muscle relaxants in SJS is not well-documented, necessitating further investigation.
Observation:
- Mechanomyography assessed the neuromuscular response to rocuronium in a pediatric patient with SJS Type 1 B.
- The study observed a 3.5-fold rightward shift in the rocuronium dose-response curve compared to healthy children.
Findings:
- Patients with SJS exhibit resistance to non-depolarizing neuromuscular blocking agents (NDMRs).
- Higher rocuronium doses improved intubation conditions, suggesting altered acetylcholine metabolism due to the HSPG2 gene mutation.
Implications:
- Anesthesiologists may need considerably higher NDMR doses for tracheal intubation in SJS patients.
- Continuous neuromuscular monitoring and incremental dosing are recommended due to SJS genetic heterogeneity and to establish optimal relaxant levels.
Abstract:
In Schwartz-Jampel syndrome micrognathia and jaw muscle rigidity may result in difficult or impossible tracheal intubation. Since the dose-response relationship to muscle relaxants is unknown in this rare disease we assessed by mechanomyography the neuromuscular response to the rocuronium in a two-year-old child with Schwartz-Jampel syndrome (SJS) Type 1 B. Rocuronium's dose-response curve was markedly shifted (3.5-fold dose) to the right when compared to healthy children and intubation conditions were improved. This resistance to NDMR may result from a lower acetylcholine degradation rate suggested as being the consequence of mutation of the gene encoding perlecan (HSPG2) in SJS. Thus, considerably higher doses of NDMR than usual may be required for facilitation of tracheal intubation in patients with SJS. Since evidence for genetic heterogeneity of SJS exists we also recommend incremental doses of a rapidly acting NDMR with continuous monitoring of neuromuscular function so as to assess the optimum relaxant dose.