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Background and Aims Lamb-Shaffer syndrome is a genetic intellectual disability reported in less than 100 patients worldwide. Most patients show facial dysmorphia, including depressed nasal bridge, micrognathia and crowded teeth. We could not find any reports on anesthetic management in these patients. Halo traction before scoliosis surgery (the most common in this syndrome) provides gradual correction, minimizing complications. Although scalp block is widely described in neurosurgery, we have not found reports on its use in this orthopedic procedure.
Methods A 8 year-old ASA III status girl with Lamb-Shaffer syndrome was proposed for application of a halo fixator for traction before scoliosis correction. The patient presented with a difficult airway, due to scoliosis, mandibular hypoplasia and crowded shark teeth. To avoid airway approach, we opted for combining sedation with regional anesthesia. Nonetheless, we prepared difficult airway material and discussed the possibility of an emergent airway with the surgical team. We inserted a nasal cannula with oxygen and a capnography line, with additional basic ASA monitoring. For sedation with spontaneous ventilation, we combined ketamine and dexmedetomidine. With the patient sedated, we did a bilateral scalp block using anatomical references.
Results The case was uneventful, and the patient maintained spontaneous ventilation the entire 45-minute procedure. There were no postoperative complications.
Conclusions Lamb-Shaffer syndrome is an extremely rare disease, posing a great challenge on airway approach. Sedation with ketamine and dexmedetomidine associated with scalp block is a safe and feasible anesthetic option for application of a halo fixator. This strategy allows for spontaneous ventilation, especially relevant in difficult airway scenarios.
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