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Published on: May 26, 2023
General anesthesia in patients with syndrome of Poland
J Díaz-Crespo1, Y Vázquez-Mambrilla1, F García-Herrera1
1Servicio de Anestesiología y Reanimación, Hospital de SAS Jerez de la Frontera, Jerez de la Frontera, Cádiz, España.
Insights
This case study highlights anesthesia management for Poland Syndrome, a rare congenital muscle disorder. Total intravenous anesthesia with propofol was initially used but led to spasms, necessitating a switch to midazolam.
Area of Science:
- Anesthesiology
- Rare Diseases
- Congenital Disorders
Background:
- Poland Syndrome is a rare congenital condition affecting muscle development, potentially causing thoracic anomalies and increasing risks like malignant hyperthermia during surgery.
- Anesthetic management for patients with Poland Syndrome requires careful consideration due to potential airway complications and susceptibility to specific anesthetic agents.
- Surgical interventions, including breast prosthesis placement, are increasingly common, necessitating tailored anesthetic approaches for rare disease patients.
Observation:
- A patient with Poland Syndrome undergoing breast prosthesis surgery presented unique anesthetic challenges.
- Initial anesthetic approach involved avoiding halogenated agents and utilizing Total Intravenous Anesthesia (TIVA) with propofol.
- Muscle spasms occurred during propofol administration, indicating a potential adverse reaction in this patient.
Findings:
- The use of propofol in this Poland Syndrome patient resulted in unexpected muscle spasms.
- A second anesthetic procedure was required to manage the patient's condition.
- Total Intravenous Anesthesia (TIVA) with midazolam was successfully employed in the subsequent procedure.
Implications:
- This case underscores the importance of individualized anesthetic strategies for rare diseases like Poland Syndrome.
- Anesthesiologists must be prepared for potential adverse reactions to common anesthetic agents in patients with specific congenital conditions.
- Alternative anesthetic agents and techniques, such as TIVA with agents like midazolam, may be crucial for safe surgical management in susceptible patients.
Abstract:
The increased use of surgery as a treatment or as an alternative for improvement means that we have a larger number of patients in the operating theatre, including those who suffer from rare diseases. Poland Syndrome is a rare congenital disease associated with muscle development. These patients may have a broad spectrum of abnormalities, which include thoracic anomalies, which can alter the ventilatory management at the level of the airway; the possible onset of malignant hyperthermia. This leads the anaesthetist to take certain preventive measures. We report the case of a patient with Poland syndrome operated for the placement of a breast prosthesis. We avoid halogenated agents, and use a Total Intravenous Anaesthesia with propofol. The appearance of muscle spasms as a result of the use of propofol, forced us into a second anaesthesia to perform total intravenous anaesthesia with Midazolam.
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