Anesthetic Approach For a Patient With 1q21.1 Microdeletion Syndrome: a Case Report
Gül Cansever, Cansu Uzuntürk, Nur Canbolat, Mehmet I. Büget
INTRODUCTION
1q21.1 microdeletion syndrome is a rare disease. While some carriers of this microdeletion show no phenotype, some have craniofacial dysmorphism, cardiac, genitourinary and neurological abnormalities.
CASE REPORT
The orthopedic clinic planned a posterior spinal instrumentation for a 3-year-old, 12 kg male patient diagnosed with 1q21.1 microdeletion syndrome. In the preoperative anesthesia examination, it was predicted that the patient, with Mallampati score of 3, might encounter difficult airway. Anesthesia induction was performed with 8% sevoflurane. The mask ventilation was successful and he received fentanyl 1 mcg/kg and rocuronium 0.6 mg/kg intravenously. The patient with Cormack-Lehane score 1 was intubated with a 4.0-cufted endotracheal tube. Ventilation was started in volume control ventilation with positive end-expiratory pressure and pressure above positive end-expiratory pressure to create sufficient tidal volume. The anesthesia was maintained with oxygen/air and infusions of remifentanil and propofol. The patient was given a prone position. When the peak pressure was high and ETCO2 was low, pressure control ventilation was initiated. Somatosensory and motor evoked potentials were monitored throughout the surgery. The estimated blood loss of 130 ml was replaced with 750 ml of crystalloid solution. The urine output was 33 cc. The operation lasted 200 minutes. Postoperative analgesia was achieved with paracetamol 15 mg/kg and tramadol 1 mg/kg intravenously. At the end of the operation, the supine position was restored. He was extubated. Transport to the intensive care unit was carried out. Pediatric erythrocyte suspensions of 25ml/kg and fresh frozen plasmas of 15 ml/kg were administered. He was discharged after 5 days.
DISCUSSION
This article presents a first case in the literature that involves an anesthetic approach to a patient with 1q21.1 microdeletion syndrome that involves many systems and should be investigated more. It requires a comprehensive preoperative anesthesia examination and preparation against possible complications in the operation room.
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