Top

BMC Anesthesiology

Published in:

Open Access 01-12-2024 | Rett Syndrome | Case Report

An ambulatory dental treatment of a child with Rett syndrome and limited mouth opening under muscle relaxant-free general anesthesia: a case report

Authors: Xiao Tan, Bo Zhu, Yanchen Li, Yuguang Huang

Published in: BMC Anesthesiology | Issue 1/2024

Abstract

Background

Rett Syndrome (RTT) is a rare, severe, and progressive developmental disorder with intellectual disability. Anesthesia in RTT patients presents a range of challenges. We report a child with RTT who received dental treatment under muscle relaxant-free general anesthesia in our ambulatory center.

Case presentation

A 15-year-old girl with RTT was admitted to our dental clinic with multiple dental caries and residual roots. Dental treatment was scheduled under ambulatory general anesthesia. After anesthesia induction, a nasal tube was initiated under the guidance of a fiberoptic bronchoscope. Multimodal analgesia, body temperature monitoring, and postoperative nausea and vomiting prevention were applied. No muscle relaxants were used throughout the process. The endotracheal tube was successfully removed after the operation and the patient was discharged home the same day.

Conclusion

An individualized anesthesia strategy enabled a quick and safe recovery for this RTT patient after dental treatment under muscle relaxant-free general anesthesia.

Available only for authorised users

Pejhan S, Rastegar M. Role of DNA Methyl-CpG-Binding protein MeCP2 in Rett Syndrome Pathobiology and mechanism of Disease. Biomolecules. 2021;11(1):75. https://doi.org/10.3390/biom11010075.CrossRefPubMedPubMedCentral

Liyanage VR, Rastegar M. Rett syndrome and MeCP2. Neuromolecular Med. 2014;16(2):231–64. https://doi.org/10.1007/s12017-014-8295-9.CrossRefPubMedPubMedCentral

Kyle SM, Vashi N, Justice MJ. Rett syndrome: a neurological disorder with metabolic components. Open Biol. 2018;8(2):170216. https://doi.org/10.1098/rsob.170216.CrossRefPubMedPubMedCentral

Ben Zeev Ghidoni B, Clin. N Am. 2007;16(3):723–43. https://doi.org/10.1016/j.chc.2007.03.004.CrossRef

Lai YYL, Downs J, Zafar S, Wong K, Walsh L, Leonard H. Oral health care and service utilisation in individuals with Rett syndrome: an international cross-sectional study. J Intellect Disabil Res. 2021;65(6):561–76. https://doi.org/10.1111/jir.12834.CrossRefPubMed

Som A, Baidya DK, Arora MK, Maitra S, Gupta S. Rett syndrome: a concern for the anesthesiologists. J Clin Anesth. 2016;31:247–8. https://doi.org/10.1016/j.jclinane.2016.01.017.CrossRefPubMed

Al-Hathlol W, Bokhari R, Alzahrani N, Alkuwaiti E. Challenges in the Dental Management of Rett Syndrome under General Anesthesia: a rare Disease. Case Rep Dent. 2022;2022:4038221. https://doi.org/10.1155/2022/4038221.CrossRefPubMedPubMedCentral

Ponde V, Desai A, Ekambaram K, Thakur S. Dental procedure under opioid-free balanced anaesthesia in a child with Rett syndrome who convulsed on every attempt to feed: Case report. Indian J Anaesth. 2020;64(4):325–7. https://doi.org/10.4103/ija.IJA_812_19.CrossRefPubMedPubMedCentral

Jakobsson JG. Recovery and discharge criteria after ambulatory anesthesia: can we improve them? Curr Opin Anaesthesiol. 2019;32(6):698–702. https://doi.org/10.1097/ACO.0000000000000784.CrossRefPubMed

10.

Home page UK Day surgery guidelines. https://anaesthetists.org/Portals/0/Images/Guidelines%20cover%20images/Guideline_day_case_surgery_2019.pdf?ver=2019-05-05-075731-563.

11.

Hinkelbein J, Lamperti M, Akeson J, Santos J, Costa J, De Robertis E, Longrois D, Novak-Jankovic V, Petrini F, Struys MMRF, Veyckemans F, Fuchs-Buder T, Fitzgerald R. European Society of Anaesthesiology and European Board of Anaesthesiology guidelines for procedural sedation and analgesia in adults. Eur J Anaesthesiol. 2018;35(1):6–24. https://doi.org/10.1097/EJA.0000000000000683.CrossRefPubMed

12.

Konarzewski WH, Misso S. Rett syndrome and delayed recovery from anaesthesia. Anaesthesia. 1994;49(4):357. https://doi.org/10.1111/j.1365-2044.1994.tb14208.x.CrossRefPubMed

13.

Chung F, Clin Anesth. Are discharge criteria changing? J. 1993;5(6 Suppl 1):64S-68S. https://doi.org/10.1016/0952-8180(93)90011-3.

14.

Nho JS, Shin DS, Moon JY, Yi JW, Kang JM, Lee BJ, Kim DO, Chung JY. Anesthetic management of an adult patient with Rett syndrome and limited mouth opening -A case report-. Korean J Anesthesiol. 2011;61(5):428–30. https://doi.org/10.4097/kjae.2011.61.5.428.CrossRefPubMedPubMedCentral

15.

Motomura Y, Idei M, Sato H, Goto T. Anesthetic management of a Rett syndrome patient with apnea and Epilepsy: a case report. JA Clin Rep. 2018;4(1):32. https://doi.org/10.1186/s40981-018-0169-y.CrossRefPubMedPubMedCentral

16.

España Fuente L, Méndez Redondo RE, González González JL. Use of Clarus Video System < sup>® in expected difficult airway in a patient with Rett syndrome. Rev Esp Anestesiol Reanim. 2017;64(1):50–4. https://doi.org/10.1016/j.redar.2016.09.003.CrossRefPubMed

17.

Kimura F, Wada M, Kudo T, Hashimoto H, Ishihara H, Hirota K. [Case of Rett syndrome monitored with BIS and neuromuscular monitor during total intravenous anesthesia]. Masui. 2011;60(6):700–2.PubMed

18.

Kawasaki E, Mishima Y, Ito T, Ito A, Takaseya H, Kameyama N, Fukugasako H, Ushijima K. [Anesthetic management of a patient with Rett syndrome associated with trismus and apnea Attacks]. Masui. 2012;61(1):96–9.PubMed

19.

Kim JT, Muntyan I, Bashkirov IL, Bloom M, Hartmannsgruber MW. The use of bispectral index monitoring in the anesthetic management of a patient with Rett syndrome undergoing scoliosis Surgery. J Clin Anesth. 2006;18(2):161–2. https://doi.org/10.1016/j.jclinane.2005.12.009.CrossRefPubMed

20.

Dahaba AA, Yin J, Xiao Z, Su J, Bornemann H, Dong H, Xiong L. Different propofol-remifentanil or sevoflurane-remifentanil bispectral index levels for electrocorticographic spike identification during Epilepsy Surgery. Anesthesiology. 2013;119(3):582–92. https://doi.org/10.1097/ALN.0b013e3182976036.CrossRefPubMed

21.

Dahaba AA. Different conditions that could result in the bispectral index indicating an incorrect hypnotic state. Anesth Analg. 2005;101(3):765–73. https://doi.org/10.1213/01.ane.0000167269.62966.af.CrossRefPubMed

22.

Bernard H. Patient warming in surgery and the enhanced recovery. Br J Nurs. 2013 Mar 28-Apr 10;22(6):319 – 20, 322-5. https://doi.org/10.12968/bjon.2013.22.6.319.

23.

Symons FJ, Byiers B, Hoch J, Dimian A, Barney C, Feyma T, Beisang A. Infrared thermal analysis and Individual Differences in skin temperature asymmetry in Rett Syndrome. Pediatr Neurol. 2015;53(2):169–72. https://doi.org/10.1016/j.pediatrneurol.2015.03.018.CrossRefPubMedPubMedCentral

24.

Dai Z, Zhang Y, Yi J, Huang Y. Validation of a prediction model for Intraoperative Hypothermia in patients receiving General Anesthesia. Int J Clin Pract. 2022;2022:6806225. https://doi.org/10.1155/2022/6806225.CrossRefPubMedPubMedCentral

25.

Kamekura N, Hojo T, Nitta Y, Hase Y, Fujisawa T. Anesthetic management of a Rett Syndrome patient at high risk for respiratory Complications. Anesth Prog. 2021;68(3):163–7. https://doi.org/10.2344/anpr-68-02-07.CrossRefPubMedPubMedCentral

26.

Meneely SJ, McFadzean J. Acute respiratory compromise due to bilateral pneumothoraces in a patient with Rett syndrome. Anaesth Rep. 2021;9(2):e12135. https://doi.org/10.1002/anr3.12135.CrossRefPubMedPubMedCentral

27.

Cohen JL, Klyce W, Kudchadkar SR, Kotian RN, Sponseller PD. Respiratory Complications after posterior spinal Fusion for Neuromuscular scoliosis: children with Rett Syndrome at Greater Risk Than those with cerebral palsy. Spine (Phila Pa 1976). 2019;44(19):1396–402. https://doi.org/10.1097/BRS.0000000000003075.CrossRefPubMed

Title: An ambulatory dental treatment of a child with Rett syndrome and limited mouth opening under muscle relaxant-free general anesthesia: a case report
Authors: Xiao Tan
Bo Zhu
Yanchen Li
Yuguang Huang
Publication date: 01-12-2024
Publisher: BioMed Central
Keywords: Rett Syndrome
General Anesthesia
Muscle Relaxant
Muscle Relaxant
Postoperative Nausea and Vomiting
Disorders of Intellectual Development
Intellectual Disability
Epilepsy
Published in: BMC Anesthesiology / Issue 1/2024
Electronic ISSN: 1471-2253
DOI: https://doi.org/10.1186/s12871-023-02379-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

An ambulatory dental treatment of a child with Rett syndrome and limited mouth opening under muscle relaxant-free general anesthesia: a case report

Abstract

Background

Case presentation

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusion

Please log in to get access to this content

Other articles of this Issue 1/2024

Fascia iliaca compartment block for postoperative pain after total hip arthroplasty: a systematic review and meta-analysis of randomized controlled trials

Machine learning approach for predicting post-intubation hemodynamic instability (PIHI) index values: towards enhanced perioperative anesthesia quality and safety

Association between serum calcium and prognosis in patients with acute ischemic stroke in ICU: analysis of the MIMIC-IV database

The effect of haloperidol’s perioperative application on postoperative delirium in elderly patients: a systematic review and meta-analysis

Mass intraoperative endothelial glycocalyx shedding affects postoperative systemic inflammation response

Role of preoperative zinc, magnesium and budesonide gargles in Postoperative Sore Throat (POST) - a randomised control trial