Rothmund–Thomson syndrome: anaesthesia considerations

Rothmund–Thomson syndrome (RTS) or poikiloderma congenitale is a rare autosomal recessive disorder. Approximately 300 cases of this syndrome have been reported in the scientific literature worldwide. This study reports the case of an 11-yearold female child with RTS undergoing diagnostic oesophago-gastro-duodeno (OGD) scopy as a result of dysphagia to solids. Adequate knowledge of the condition is needed when planning anaesthesia in such a case as associated anomalies can interfere with anaesthesia management.Keywords: anaesthesia management, Rothmund–Thomson syndrom

Gastroschisis repair under caudal anesthesia: a series of three cases

Gastroschisis is a congenital anomaly characterized by a defect in the anterior abdominal wall with protrusion of abdominal viscera. Perioperative mortality is very high in these patients. Traditionally gastroschisis repair has been performed under general anesthesia with endotracheal intubation, requiring postoperative intensive care admission and mechanical ventilation. Caudalblock is an attractive alternative to general anesthesia. We present a series of three neonates with gastroschisis, repaired solely under caudal anesthesia. Resumo: Gastrosquise é uma anomalia congênita caracterizada por um defeito da parede abdominal anterior com protrusão de vísceras abdominais. A mortalidade no período perioperatório é muito elevada nesses pacientes. Tradicionalmente, a correção de gastrosquise tem sido realizada sob anestesia geral com intubação orotraqueal, o que requer internação em unidade de terapia intensiva e ventilação mecânica no pós-operatório. O bloqueio caudal é uma alternativa atraente à anestesia geral. Apresentamos uma série de três casos de recém-nascidos com gastrosquise corrigida unicamente sob anestesia caudal. Keywords: Gastroschisis, Anesthesia, caudal, Infant, newborn, Palavras-chave: Gastrosquise, Anestesia caudal, Lactentes, recém-nascid