[Rett syndrome: double epidural catheter for the control of postoperative pain after scoliosis surgery. A literature review]. / Síndrome de Rett: doble catéter epidural para control del dolor postoperatorio tras cirugía de escoliosis. Revisión de la bibliografía.

Rett syndrome is a severe and incapacitating neurological disease caused by a structural defect in the short arm of the X chromosome (Xq28). It affects females and consists of multiple and progressive neurological impairments that start from a young age, leading to lifelong disability and dependency. Scoliosis appears in more than 50% of patients and requires surgical correction in cases where the curvature is severe. Pre-anaesthetic assessment is essential in order to identify the risk factors and thus reduce the morbidity and mortality associated with the surgical procedure. We present the case of a patient affected by this syndrome and scoliosis, who was scheduled to have an instrumented thoracolumbar spine arthrodesis with general anaesthesia, which passed without incident. We evaluate the specific details of this syndrome, its potential complications, and its management from an anaesthetic point of view, emphasising the control of postoperative pain using a double epidural catheter with an infusion of local anaesthetics and fentanyl.

[Conradi-Hünermann Chondrodysplasia punctata: anaesthetic implications]. / Condrodisplasia punctata de Conradi-Hünermann: implicaciones anestésicas.

Chondrodysplasia punctata syndrome is a group of rare congenital diseases that give rise to malformations, mainly skeletal, linked to the premature calcification of structures. There is little in the literature in the field of Anaesthesiology on this despite there being some clinical manifestations, particularly the possibility of difficult airway, which may be of importance during the peri-operative period. During the first years of life, the patients affected are often subjected to repeated surgery to correct their malformations. We describe the anaesthetic treatment during orthopaedic surgery of a paediatric patient with Conradi-Hünermann type chondrodysplasia punctata, highlighting the managementof the airway using a laryngeal mask in combination with balanced general anaesthesia with remifentanil. We also present a review of the characteristics of the different entities of these syndromes, with special emphasis on the aspects most relevant to the anaesthesiologist.