Pelizaeus-Merzbacher disease (PMD) is a progressive and degenerative chromosomal disorder of the central nervous system caused by defective myelin production. Few case reports have been issued on the anesthetic management of PMD, because of its extremely low incidence. We anesthetized a 13-year-old female patient diagnosed with PMD for ophthalmic surgery because of intermittent exotropia. General anesthesia was induced and maintained with propofol and sevoflurane in air and oxygen. Rocuronium was administered to facilitate orotracheal intubation, and residual neuromuscular blockage was reversed with pyridostigmine. Between emergence to 24 hours postoperatively, her muscle power completely recovered and no unpredictable events occurred. Summarizing, anesthesiologists should be concerned about the high possibility of aspiration, spasticity, and seizure during the perioperative period in patients with even mild PMD. Appropriate preoperative evaluation, intraoperative monitoring, and choice of proper anesthetic drugs enable safe anesthesia in patients with PMD.
Adolescent* ; Anesthesia ; Anesthesia, General* ; Anesthetics ; Central Nervous System ; Chromosome Disorders ; Exotropia ; Female ; Humans ; Incidence ; Intubation ; Monitoring, Intraoperative ; Muscle Spasticity ; Myelin Sheath ; Oxygen ; Pelizaeus-Merzbacher Disease* ; Perioperative Period ; Propofol ; Pyridostigmine Bromide ; Seizures

BACKGROUND: The authors have performed ultrasound-guided stellate ganglion block (SGB) in our clinic using a lateral paracarotid approach at the level of the 6th cervical vertebra (C6). Although SGB at C6 is a convenient and safe method, there are ongoing concerns about the weak effect of sympathetic blockade in the ipsilateral upper extremity. Therefore, ultrasound-guided SGB was attempted using a lateral paracarotid approach at the level of the 7th cervical vertebra (C7). This prospective study aimed to compare changes in skin temperature after SGB was performed at C6 and C7, and to introduce a lateral paracarotid approach for SGB.METHODS: Thirty patients underwent SGB twice: once at C6 and once at C7. For every SGB, the skin temperature of the patient's hypothenar area was measured for 15 min at 1-min intervals. Skin temperatures before and after SGB and side effects were compared between C6 and C7 groups.RESULTS: The temperature of the upper extremity increased after SGB was performed at C6 and C7. There were significant differences between mean pre-SGB and the largest increases in post-SGB temperatures (0.50±0.38℃ and 1.41±0.68℃ at C6 and C7, respectively; p < 0.05). Significantly increased post-SGB temperatures (difference >1℃) were found in 5/30 (16.7%) and 24/30 (80%) cases for C6 and C7, respectively (p < 0.05). There were no significant differences in side effects between SGB performed at C6 or C7 (p>0.05).CONCLUSION: The lateral paracarotid approach using out-of-plane needle insertion for ultrasound-guided SGB performed at C7 was feasible and more effective at elevating skin temperature in the upper extremity than SGB at C6.
Autonomic Nerve Block ; Cervical Vertebrae ; Female ; Humans ; Methods ; Needles ; Prospective Studies ; Skin Temperature ; Spine ; Stellate Ganglion ; Ultrasonography ; Upper Extremity

BACKGROUND: The authors have performed ultrasound-guided stellate ganglion block (SGB) in our clinic using a lateral paracarotid approach at the level of the 6th cervical vertebra (C6). Although SGB at C6 is a convenient and safe method, there are ongoing concerns about the weak effect of sympathetic blockade in the ipsilateral upper extremity. Therefore, ultrasound-guided SGB was attempted using a lateral paracarotid approach at the level of the 7th cervical vertebra (C7). This prospective study aimed to compare changes in skin temperature after SGB was performed at C6 and C7, and to introduce a lateral paracarotid approach for SGB. METHODS: Thirty patients underwent SGB twice: once at C6 and once at C7. For every SGB, the skin temperature of the patient's hypothenar area was measured for 15 min at 1-min intervals. Skin temperatures before and after SGB and side effects were compared between C6 and C7 groups. RESULTS: The temperature of the upper extremity increased after SGB was performed at C6 and C7. There were significant differences between mean pre-SGB and the largest increases in post-SGB temperatures (0.50±0.38℃ and 1.41±0.68℃ at C6 and C7, respectively; p < 0.05). Significantly increased post-SGB temperatures (difference >1℃) were found in 5/30 (16.7%) and 24/30 (80%) cases for C6 and C7, respectively (p < 0.05). There were no significant differences in side effects between SGB performed at C6 or C7 (p>0.05). CONCLUSION The lateral paracarotid approach using out-of-plane needle insertion for ultrasound-guided SGB performed at C7 was feasible and more effective at elevating skin temperature in the upper extremity than SGB at C6.