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Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome

BACKGROUND: Potocki -Lupski syndrome is an uncommon disorder caused by a micro-duplication in chromosome 17p11.2. Variable clinical manifestations bring troubles to the general and neuraxial anesthesia, including mental retardation, facial dysmorphisms, structural cardiovascular anomalies, scoliosis...

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Autores principales: Li, Junfeng, Bao, Jie, Zhang, Di, Zhou, Shuzhen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8422621/
https://www.ncbi.nlm.nih.gov/pubmed/34493221
http://dx.doi.org/10.1186/s12871-021-01433-3
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author Li, Junfeng
Bao, Jie
Zhang, Di
Zhou, Shuzhen
author_facet Li, Junfeng
Bao, Jie
Zhang, Di
Zhou, Shuzhen
author_sort Li, Junfeng
collection PubMed
description BACKGROUND: Potocki -Lupski syndrome is an uncommon disorder caused by a micro-duplication in chromosome 17p11.2. Variable clinical manifestations bring troubles to the general and neuraxial anesthesia, including mental retardation, facial dysmorphisms, structural cardiovascular anomalies, scoliosis, and malignant hyperthermia. Until now, the anesthesia management for cesarean section in these patients has not been reported yet. CASE PRESENTATION: Here we present a 23-year-old Chinese parturient with Potocki -Lupski syndrome who underwent elective cesarean section under spinal anesthesia. She was transferred to our hospital in her 40th week of gestation. She had a history of IgA nephropathy for more than three years and was diagnosed with Potocki -Lupski syndrome (17p12p11.2 segment 3.1 Mb repeat) in the 29th week of pregnancy. Amniocentesis showed the fetus had no abnormal autosomes. Preoperative multidisciplinary consultation suggested that she should terminate the pregnancy as soon as possible. She was ASA II. Her BMI was 26.43 kg/m(2). Her airway evaluation was normal. Her spine could bend well and her spinal interspace could be touched clearly. We did the single spinal anesthesia at L2-3 interspace and gave 0.5% bupivacaine 1.7 ml. The absolute anesthesia level reached T8. The Apgar score for the newborn infant was 10 for 1st minute, 5th minute, and 10th minute. The vital signs were steady without using any vasoactive drugs. The patient had a good prognosis, and was subsequently discharged from hospital. CONCLUSION: To date, the case may be the first reported spinal anesthesia for the parturient with Potocki -Lupski syndrome. Although its manifestations are variable, the spinal anesthesia is feasible under careful and comprehensive preoperative evaluation.
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spelling pubmed-84226212021-09-09 Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome Li, Junfeng Bao, Jie Zhang, Di Zhou, Shuzhen BMC Anesthesiol Case Report BACKGROUND: Potocki -Lupski syndrome is an uncommon disorder caused by a micro-duplication in chromosome 17p11.2. Variable clinical manifestations bring troubles to the general and neuraxial anesthesia, including mental retardation, facial dysmorphisms, structural cardiovascular anomalies, scoliosis, and malignant hyperthermia. Until now, the anesthesia management for cesarean section in these patients has not been reported yet. CASE PRESENTATION: Here we present a 23-year-old Chinese parturient with Potocki -Lupski syndrome who underwent elective cesarean section under spinal anesthesia. She was transferred to our hospital in her 40th week of gestation. She had a history of IgA nephropathy for more than three years and was diagnosed with Potocki -Lupski syndrome (17p12p11.2 segment 3.1 Mb repeat) in the 29th week of pregnancy. Amniocentesis showed the fetus had no abnormal autosomes. Preoperative multidisciplinary consultation suggested that she should terminate the pregnancy as soon as possible. She was ASA II. Her BMI was 26.43 kg/m(2). Her airway evaluation was normal. Her spine could bend well and her spinal interspace could be touched clearly. We did the single spinal anesthesia at L2-3 interspace and gave 0.5% bupivacaine 1.7 ml. The absolute anesthesia level reached T8. The Apgar score for the newborn infant was 10 for 1st minute, 5th minute, and 10th minute. The vital signs were steady without using any vasoactive drugs. The patient had a good prognosis, and was subsequently discharged from hospital. CONCLUSION: To date, the case may be the first reported spinal anesthesia for the parturient with Potocki -Lupski syndrome. Although its manifestations are variable, the spinal anesthesia is feasible under careful and comprehensive preoperative evaluation. BioMed Central 2021-09-07 /pmc/articles/PMC8422621/ /pubmed/34493221 http://dx.doi.org/10.1186/s12871-021-01433-3 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Li, Junfeng
Bao, Jie
Zhang, Di
Zhou, Shuzhen
Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome
title Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome
title_full Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome
title_fullStr Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome
title_full_unstemmed Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome
title_short Case report: Spinal anesthesia for cesarean section in a parturient with Potocki-Lupski syndrome
title_sort case report: spinal anesthesia for cesarean section in a parturient with potocki-lupski syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8422621/
https://www.ncbi.nlm.nih.gov/pubmed/34493221
http://dx.doi.org/10.1186/s12871-021-01433-3
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