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Background and Aims Cornelia de Lange syndrome is a rare congenital disease characterized by multiple malformations. Anesthetic management can be a challenge due craniofacial malformation (low-set ears, wide nasal bridge, cleft palate, narrow inter-incisor distance, micrognathia, short neck and limited neck extension) that makes it a difficult airway. Regional anesthesia ultrasound guided is a safe option in these patients since allows adequate anesthetic condition for performing the surgical procedure and postsurgical pain relief. However, its use in this population has not been reported.
Methods A 15-year-old female with diagnoses of Cornelia De Lange Syndrome, neuromuscular scoliosis treated with T9-L2 spinal instrumentation, patent ductus arteriosus with spontaneous closure, delayed intellectual development, gastroesophageal reflux disease and tubular acidosis renal. Scheduled for arthrodesis of the scaphoid talus and lengthening of the calcaneus of right foot. Physical examination showed weight 32.8 kg, short neck, narrow inter-incisor distance, limited neck extension, low-set ears, residual scoliosis. Premedication with dexmedetomidine 50 μg intranasal was performed. Sedation was achieved through of infusion´s Propofol at 3-4 μg/ml and Fentanyl 35 μg intravenous. Spontaneous ventilation with supplemental O2. An ultrasound guided popliteal sciatic nerve block was performed, administering ropivacaine 78.5 mg (0.4%), 20 ml of volume. During surgery she remains hemodynamically stable. In recovery, the patient is calm, no pain data.
Conclusions In patients with difficult airway like our patient with Cornelia De Lange syndrome, regional anesthesia plus sedation intravenous was the best choice to offer and it was successfully used as an anesthetic and analgesic management in the orthopedic surgery.
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