Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease

Detalhes bibliográficos
Autor(a) principal: Norcia, Luiz Fernando [UNESP]
Data de Publicação: 2022
Outros Autores: Watanabe, Erika Mayumi [UNESP], Hasimoto, Claudia Nishida [UNESP], Pelafsky, Leonardo [UNESP], Oliveira, Walmar Kerche de [UNESP], Sassaki, Ligia Yukie [UNESP]
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Institucional da UNESP
Texto Completo: http://dx.doi.org/10.1159/000523662
http://hdl.handle.net/11449/240896
Resumo: Autosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future.
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spelling Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver DiseaseCase reportHepatic cystsHepatomegalyLaparotomy fenestrationPolycystic liver diseaseAutosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future.Department of Internal Medicine São Paulo State University (Unesp) Medical SchoolDepartment of Radiology São Paulo State University (Unesp) Medical SchoolDepartment of Surgery São Paulo State University (Unesp) Medical SchoolDepartment of Internal Medicine São Paulo State University (Unesp) Medical SchoolDepartment of Radiology São Paulo State University (Unesp) Medical SchoolDepartment of Surgery São Paulo State University (Unesp) Medical SchoolUniversidade Estadual Paulista (UNESP)Norcia, Luiz Fernando [UNESP]Watanabe, Erika Mayumi [UNESP]Hasimoto, Claudia Nishida [UNESP]Pelafsky, Leonardo [UNESP]Oliveira, Walmar Kerche de [UNESP]Sassaki, Ligia Yukie [UNESP]2023-03-01T20:37:31Z2023-03-01T20:37:31Z2022-03-31info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article201-208http://dx.doi.org/10.1159/000523662Case Reports in Gastroenterology, v. 16, n. 1, p. 201-208, 2022.1662-0631http://hdl.handle.net/11449/24089610.1159/0005236622-s2.0-85129059661Scopusreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengCase Reports in Gastroenterologyinfo:eu-repo/semantics/openAccess2024-08-14T17:23:33Zoai:repositorio.unesp.br:11449/240896Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462024-08-14T17:23:33Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false
dc.title.none.fl_str_mv Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
title Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
spellingShingle Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
Norcia, Luiz Fernando [UNESP]
Case report
Hepatic cysts
Hepatomegaly
Laparotomy fenestration
Polycystic liver disease
title_short Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
title_full Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
title_fullStr Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
title_full_unstemmed Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
title_sort Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
author Norcia, Luiz Fernando [UNESP]
author_facet Norcia, Luiz Fernando [UNESP]
Watanabe, Erika Mayumi [UNESP]
Hasimoto, Claudia Nishida [UNESP]
Pelafsky, Leonardo [UNESP]
Oliveira, Walmar Kerche de [UNESP]
Sassaki, Ligia Yukie [UNESP]
author_role author
author2 Watanabe, Erika Mayumi [UNESP]
Hasimoto, Claudia Nishida [UNESP]
Pelafsky, Leonardo [UNESP]
Oliveira, Walmar Kerche de [UNESP]
Sassaki, Ligia Yukie [UNESP]
author2_role author
author
author
author
author
dc.contributor.none.fl_str_mv Universidade Estadual Paulista (UNESP)
dc.contributor.author.fl_str_mv Norcia, Luiz Fernando [UNESP]
Watanabe, Erika Mayumi [UNESP]
Hasimoto, Claudia Nishida [UNESP]
Pelafsky, Leonardo [UNESP]
Oliveira, Walmar Kerche de [UNESP]
Sassaki, Ligia Yukie [UNESP]
dc.subject.por.fl_str_mv Case report
Hepatic cysts
Hepatomegaly
Laparotomy fenestration
Polycystic liver disease
topic Case report
Hepatic cysts
Hepatomegaly
Laparotomy fenestration
Polycystic liver disease
description Autosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future.
publishDate 2022
dc.date.none.fl_str_mv 2022-03-31
2023-03-01T20:37:31Z
2023-03-01T20:37:31Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://dx.doi.org/10.1159/000523662
Case Reports in Gastroenterology, v. 16, n. 1, p. 201-208, 2022.
1662-0631
http://hdl.handle.net/11449/240896
10.1159/000523662
2-s2.0-85129059661
url http://dx.doi.org/10.1159/000523662
http://hdl.handle.net/11449/240896
identifier_str_mv Case Reports in Gastroenterology, v. 16, n. 1, p. 201-208, 2022.
1662-0631
10.1159/000523662
2-s2.0-85129059661
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Case Reports in Gastroenterology
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 201-208
dc.source.none.fl_str_mv Scopus
reponame:Repositório Institucional da UNESP
instname:Universidade Estadual Paulista (UNESP)
instacron:UNESP
instname_str Universidade Estadual Paulista (UNESP)
instacron_str UNESP
institution UNESP
reponame_str Repositório Institucional da UNESP
collection Repositório Institucional da UNESP
repository.name.fl_str_mv Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)
repository.mail.fl_str_mv
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