ABSTRACT
El síndrome de Steinert congénito es una forma severa de distrofia miotónica, caracterizada por hipotonía generalizada, diplejía facial, dificultad respiratoria y anomalías en la succión y deglución presentes desde el nacimiento. Este síndrome tiene una incidencia de 1 caso por cada 10.000 recién nacidos y una mortalidad del 50% en el período neonatal. Se considera un reto para el anestesiólogo debido a la gran variedad de complicaciones que se pueden presentar en el intraoperatorio y postoperatorio, tanto por la evolución de la enfermedad como por la susceptibilidad a la gran mayoría de los anestésicos. Describimos el caso de un niño de 3 años con distrofia miotónica congénita, en el que se realiza una orquidopexia bilateral por laparoscopia con anestesia general sin relajación muscular, combinada con un bloqueo TAP bilateral ecoguiado
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
Subject(s)
Humans , Male , Child, Preschool , Myotonic Dystrophy/complications , Orchiopexy/methods , Cryptorchidism/surgery , Anesthesia, Conduction/methods , Laparoscopy/methods , Nerve Block/methods , Anesthetics, Local/administration & dosage , Sleep Apnea Syndromes/complications , Intubation, Intratracheal/methodsABSTRACT
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.
