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[General anesthesia for a girl with Joubert syndrome].
Masui. 2010 Mar; 59(3):383-5.M

Abstract

Joubert syndrome is a rare autosomal recessive disorder, which is characterized by absence or underdevelopment of the cerebellar vermis and severe developmental delay. The other common features include ataxia, an abnormal breathing pattern, abnormal eye movements and hypotonia. We report the anesthetic management in a 13-year-old girl with Joubert syndrome, scheduled for cauterization of nasal mucosa under general anesthesia. She had episodes of tachypnea and apnea. Oral midazolam 10 mg and famotidine 20 mg were administered 30 min before surgery. Anesthesia was induced and maintained with sevoflurane and nitrous oxide in oxygen. Vecuronium 2 mg was used to facilitate tracheal intubation. Mechanical ventilation was performed with a low ventilation setting of respiratory rate 5 beats x min(-1) and peak inspiratory pressure 9 cm H2O to maintain normal end-tidal CO2. Flurbiprofen axetil 30 mg was administered intravenously for analgesia, because opioids are not recommended. After reversal of muscle relaxation by atropin 0.5 mg and neostigmine 1.5 mg, her trachea was extubated. She did not develop postoperative apnea. In this patient with Joubert syndrome, midazolam, sevoflurane, nitrous oxide and flurbiprofen axetil were used without any complications.

Authors+Show Affiliations

Department of Anesthesia and Intensive Care, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi 594-1101.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

20229761

Citation

Matsuura, Kokoro, et al. "[General Anesthesia for a Girl With Joubert Syndrome]." Masui. the Japanese Journal of Anesthesiology, vol. 59, no. 3, 2010, pp. 383-5.
Matsuura K, Takeuchi M, Tachibana K, et al. [General anesthesia for a girl with Joubert syndrome]. Masui. 2010;59(3):383-5.
Matsuura, K., Takeuchi, M., Tachibana, K., Matsunari, Y., & Kinouchi, K. (2010). [General anesthesia for a girl with Joubert syndrome]. Masui. the Japanese Journal of Anesthesiology, 59(3), 383-5.
Matsuura K, et al. [General Anesthesia for a Girl With Joubert Syndrome]. Masui. 2010;59(3):383-5. PubMed PMID: 20229761.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [General anesthesia for a girl with Joubert syndrome]. AU - Matsuura,Kokoro, AU - Takeuchi,Muneyuki, AU - Tachibana,Kazuya, AU - Matsunari,Yasunori, AU - Kinouchi,Keiko, PY - 2010/3/17/entrez PY - 2010/3/17/pubmed PY - 2010/4/30/medline SP - 383 EP - 5 JF - Masui. The Japanese journal of anesthesiology JO - Masui VL - 59 IS - 3 N2 - Joubert syndrome is a rare autosomal recessive disorder, which is characterized by absence or underdevelopment of the cerebellar vermis and severe developmental delay. The other common features include ataxia, an abnormal breathing pattern, abnormal eye movements and hypotonia. We report the anesthetic management in a 13-year-old girl with Joubert syndrome, scheduled for cauterization of nasal mucosa under general anesthesia. She had episodes of tachypnea and apnea. Oral midazolam 10 mg and famotidine 20 mg were administered 30 min before surgery. Anesthesia was induced and maintained with sevoflurane and nitrous oxide in oxygen. Vecuronium 2 mg was used to facilitate tracheal intubation. Mechanical ventilation was performed with a low ventilation setting of respiratory rate 5 beats x min(-1) and peak inspiratory pressure 9 cm H2O to maintain normal end-tidal CO2. Flurbiprofen axetil 30 mg was administered intravenously for analgesia, because opioids are not recommended. After reversal of muscle relaxation by atropin 0.5 mg and neostigmine 1.5 mg, her trachea was extubated. She did not develop postoperative apnea. In this patient with Joubert syndrome, midazolam, sevoflurane, nitrous oxide and flurbiprofen axetil were used without any complications. SN - 0021-4892 UR - https://www.unboundmedicine.com/medline/citation/20229761/[General_anesthesia_for_a_girl_with_Joubert_syndrome]_ L2 - http://www.diseaseinfosearch.org/result/3917 DB - PRIME DP - Unbound Medicine ER -