Anesthetic management of a child with congenital Steiner syndrome scheduled for laparoscopic bilateral orchidopexy

Abstract

Congenital Steinert syndrome is a severe form of myotonic dystrophy, characterized by general hypotonia, facial diplegia, respiratory difficulty and sucking and swallowing problems presented since birth. This syndrome has an estimated incidence of 1 per 10.000 births. Mortality is close to 50% during the neonatal period. It represents a challenge for the anesthesiologist due to the great variety of intraoperative and postoperative adverse events, given by both the evolution of the disease and the susceptibility to the vast majority of the anesthetic agents. A report of a 3-year-old boy with congenital myotonic dystrophy is presented, scheduled for laparoscopic bilateral orchidopexy, performed under general anesthesia without muscular relaxation, combined with ecoguide bilateral TAP block.

Keywords: Bloqueo TAP; Congenital Steinert syndrome; Congenital myotonic dystrophy; Distrofia miotónica congénita; Intubación traqueal sin relajación muscular; Síndrome de Steinert congénito; TAP block; Traqueal intubation without muscle relaxants.