Valdés-Torres A, García-Cid S, Quintana-Santos N

Language: Spanish
References: 14
Page: 1-13
PDF size: 2531.31 Kb.

ABSTRACT

Introduction: myotonic dystrophy type 1 or Steinert's disease (OMIM 160900, ORPHA 273, ICD-9- MC359.21, ICD-10 G71.1, ICD-11 8C71.0) is the most prevalent myopathy in adults, although there are two forms of pediatric presentation: congenital myotonic dystrophy and the infantile onset variant. It constitutes a challenge for the anesthesiologist due to the complications that may arise during the surgical act.
Objective: to describe the anesthetic action in a patient with Steiner's myotonic disease.
Case presentation: 11-year-old white male patient weighing 58 kg, who was announced for laparoscopic exploration for undescended right testis. With a history of delayed dystocic delivery and cyanosis at birth, which required artificial ventilation in neonatology for a period of three days, with delayed psychomotor development diagnosed during the infant period, Steiner's myotonic disease. The anesthetic technique used, the drugs and control procedures in the preoperative period, during the surgical act and in the recovery period are described.
Conclusions: Steiner's disease is a challenge for the anesthesiologist due to the multisystemic effects that can lead to perioperative complications and even death during the surgical procedure or anesthetic recovery. Therapeutic conduct must be individualized and assessed during the perioperative period, a thorough anesthetic planning is recommended, anticipating complications.

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