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Anesthetic management of a pediatric patient with Dravet syndrome: A case report

Dravet syndrome (DS) is a rare and severe myoclonic epilepsy, with onset commonly occurring in infancy. Seizures are triggered by various causes, including fever, bathing, and light stimulus. DS is refractory to drug treatment. Moreover, status epilepticus (SE) can cause serious encephalopathy and e...

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Autores principales: Hase, Yuri, Takuma, Shigeru, Hojo, Takayuki, Nitta, Yukie, Kamekura, Nobuhito
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9875994/
https://www.ncbi.nlm.nih.gov/pubmed/36705365
http://dx.doi.org/10.1097/MD.0000000000032709
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author Hase, Yuri
Takuma, Shigeru
Hojo, Takayuki
Nitta, Yukie
Kamekura, Nobuhito
author_facet Hase, Yuri
Takuma, Shigeru
Hojo, Takayuki
Nitta, Yukie
Kamekura, Nobuhito
author_sort Hase, Yuri
collection PubMed
description Dravet syndrome (DS) is a rare and severe myoclonic epilepsy, with onset commonly occurring in infancy. Seizures are triggered by various causes, including fever, bathing, and light stimulus. DS is refractory to drug treatment. Moreover, status epilepticus (SE) can cause serious encephalopathy and epilepsy-related deaths. There are very few reports of general anesthesia in DS patients. Herein, we report our experience with the anesthetic management of a pediatric patient with DS. PATIENT CONCERNS AND DIAGNOSES: A 5-year-old boy (height, 112 cm; weight, 19 kg) was diagnosed with DS through SCN1A genetic testing, which revealed a de novo novel missense mutation. His medical history included drug-resistant epilepsy, developmental delay, and hypotonia. His seizures tended to be triggered daily by a rise in body temperature (BT), bathing, and light stimulus. He could not receive adequate dental treatment due to DS, although he had previously undergone dental treatment under restraint at the pediatric dentistry department of our hospital. INTERVENTIONS AND OUTCOMES: The patient was scheduled for intensive dental treatment under general anesthesia due to noncooperation, and DS-related limitations. By considering the risk posed by elevated BT, seizure-inducing drugs were avoided, and general anesthesia was completed as planned, uneventfully. Although fluctuation of BT occurred during the procedure, it was finally controlled at the end of anesthesia at about the same level as at anesthesia induction. However, small seizures and a single generalized convulsion were observed accompanied by fever on postoperative day 1. The patient was discharged from the hospital without major problems on postoperative day 3, because of detailed planning and close preoperative cooperation with the attending pediatrician. CONCLUSION: It is essential to pay attention to managing BT and to avoid drugs that induce seizures during anesthesia for patients with DS. Cautious preoperative planning for anesthesia based on evaluation of the patient and rapid postoperative response in collaboration with the attending pediatrician is necessary in case an epileptic seizure occurs.
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spelling pubmed-98759942023-01-27 Anesthetic management of a pediatric patient with Dravet syndrome: A case report Hase, Yuri Takuma, Shigeru Hojo, Takayuki Nitta, Yukie Kamekura, Nobuhito Medicine (Baltimore) 3300 Dravet syndrome (DS) is a rare and severe myoclonic epilepsy, with onset commonly occurring in infancy. Seizures are triggered by various causes, including fever, bathing, and light stimulus. DS is refractory to drug treatment. Moreover, status epilepticus (SE) can cause serious encephalopathy and epilepsy-related deaths. There are very few reports of general anesthesia in DS patients. Herein, we report our experience with the anesthetic management of a pediatric patient with DS. PATIENT CONCERNS AND DIAGNOSES: A 5-year-old boy (height, 112 cm; weight, 19 kg) was diagnosed with DS through SCN1A genetic testing, which revealed a de novo novel missense mutation. His medical history included drug-resistant epilepsy, developmental delay, and hypotonia. His seizures tended to be triggered daily by a rise in body temperature (BT), bathing, and light stimulus. He could not receive adequate dental treatment due to DS, although he had previously undergone dental treatment under restraint at the pediatric dentistry department of our hospital. INTERVENTIONS AND OUTCOMES: The patient was scheduled for intensive dental treatment under general anesthesia due to noncooperation, and DS-related limitations. By considering the risk posed by elevated BT, seizure-inducing drugs were avoided, and general anesthesia was completed as planned, uneventfully. Although fluctuation of BT occurred during the procedure, it was finally controlled at the end of anesthesia at about the same level as at anesthesia induction. However, small seizures and a single generalized convulsion were observed accompanied by fever on postoperative day 1. The patient was discharged from the hospital without major problems on postoperative day 3, because of detailed planning and close preoperative cooperation with the attending pediatrician. CONCLUSION: It is essential to pay attention to managing BT and to avoid drugs that induce seizures during anesthesia for patients with DS. Cautious preoperative planning for anesthesia based on evaluation of the patient and rapid postoperative response in collaboration with the attending pediatrician is necessary in case an epileptic seizure occurs. Lippincott Williams & Wilkins 2023-01-27 /pmc/articles/PMC9875994/ /pubmed/36705365 http://dx.doi.org/10.1097/MD.0000000000032709 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle 3300
Hase, Yuri
Takuma, Shigeru
Hojo, Takayuki
Nitta, Yukie
Kamekura, Nobuhito
Anesthetic management of a pediatric patient with Dravet syndrome: A case report
title Anesthetic management of a pediatric patient with Dravet syndrome: A case report
title_full Anesthetic management of a pediatric patient with Dravet syndrome: A case report
title_fullStr Anesthetic management of a pediatric patient with Dravet syndrome: A case report
title_full_unstemmed Anesthetic management of a pediatric patient with Dravet syndrome: A case report
title_short Anesthetic management of a pediatric patient with Dravet syndrome: A case report
title_sort anesthetic management of a pediatric patient with dravet syndrome: a case report
topic 3300
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9875994/
https://www.ncbi.nlm.nih.gov/pubmed/36705365
http://dx.doi.org/10.1097/MD.0000000000032709
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