Anesthetic management of a child with congenital Steiner syndrome scheduled for laparoscopic bilateral orchidopexy. / Manejo anestésico en un niño con síndrome de Steinert congénito programado para orquidopexia bilateral por laparoscopia.

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.

[Mediastinal tumors in children: apropos of 2 cases]. / Tumores mediastínicos en niños: a propósito de 2 casos.

General anesthesia in patients with mediastinal tumors has specific problems that need careful evaluation before surgery. Sometimes, mediastinal masses may result in life threatening complications, such as upper airway obstruction, superior vena cava syndrome, cardiac or pulmonary artery compression and acute pulmonary edema. After a review of the literature related to death during general anesthesia in children with mediastinal tumors, we emphasize the importance of the agreement between oncologists, surgeons and anesthesiologists for a sound diagnosis of these patients. We report two patients which illustrate the possibility of airway obstruction during anesthesia. The first was a 13-year-old female with an anterior and mean mediastinal tumor who had severe respiratory complications during general anesthesia for biopsy of cervical lymphadenopathy . The second patient was a 2-year-old female with anterior mediastinal and paratracheal masses and severe respiratory compromise, who was operated under general inhalation anesthesia and spontaneous breathing for biopsy of supraclavicular lymphadenopathy, after a meticulous preanesthetic evaluation. In these patients, the anesthetic procedure is a challenge to the anesthesiologist. Inhalation induction in a half seated position is recommended, maintaining the patient with spontaneous ventilation with halogenated agents and avoiding muscle relaxants.

Manejo anestésico en un niño con síndrome de Steinert congénito programado para orquidopexia bilateral por laparoscopia / Anesthetic management of a child with congenital Steiner syndrome scheduled for laparoscopic bilateral orchidopexy

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