| Abstrakt: |
Background: Mucopolysaccharidosis (MPS) are a group of lysosomal storage diseases caused by a deficiency of enzymes in glycosaminoglycan (GAG) metabolism, resulting in GAG accumulation in tissues and organs. Anesthetic management of these patients can be challenging due to difficult airway and cardiopulmonary problems. In this case we aim to emphasize the anesthetic consideration of two different MPS syndrome patients undergoing different surgeries. Case 1: A 5-year-old boy weighting 21 kg with MPS type II (Hunter syndrome) was scheduled for adenoidectomy operation. His physical examination showed macrocephaly, short neck and coarse facial features (Fig.1). His mallampati grade was III. Oral and written consent was obtained from the patient’s family. For the potential risk for difficult airway; LMA, videolaryngoscope, and fiberoptic bronchoscope was kept ready. After preoxygenation, anesthesia induction was performed with sevoflurane via face mask avoiding hyperextension of the neck. After insertion of an intravenous access, anesthesia induction was maintained with lidocaine, fentanyl and rocuronium. Using direct laryngoscope; Cormack-Lehane grade IV was visualized at the first attempt. Immediately afterwards videolaryngoscopy has initiated (Fig.2). Intubation was unsuccessful despite using several size of endotracheal tubes with styles. Eventually, the patient was intubated with the tube angled 90° using a rigid stile. The child was extubated while awake and transferred to the recovery unit without any complications. Case 2: A 21-year-old, 155 cm, 86 kg male patient with MPS type VI (Maroteaux-Lamy syndrome) was scheduled for a right inguinal hernia operation. Preoperative evaluation revealed coarse facial features, short neck, flattened nose, macroglossia, and kyphoscoliosis (Fig 3). His Mallampati grade was IV. Oral and written consent was obtained from the patient. Although spinal anesthesia was planned, preparation for difficult airway management was performed (Fig 4). A single subarachnoid block was administered with 12.5 mg of heavy bupivacaine, and the operation was completed under spinal anesthesia without complications. The patient was then transferred to the service. Conclusion: MPS syndrome patients often have a difficult airway due to mucopolysaccharide accumulation, short neck, ankylosis, and dislocation risks. Regional anesthesia is preferable for suitable patients and surgeries even so preoperative airway evaluation and difficult intubation preparation are essential. [ABSTRACT FROM AUTHOR] |
