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Publication
Hidropisia Fetal Patologia do Passado, Presente e… Futuro? Estudo Retrospectivo
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Advisor(s)
Abstract(s)
RESUMO
Introdução: A hidropisia fetal define-se por uma acumulação anormal de
fluído intersticial no feto. Descrita inicialmente
como uma entidade patológica
única, Ballantyne em 1892 levantou a
hipótese de representar um estado final
comum a uma variedade de processos
patológicos diferentes. Potter distingue
2 tipos: imune (mais frequente na era
prévia à utilização da RhoGAM) e não
imune (implicada actualmente em cerca
de 90% dos casos). Apesar de todos os
avanços de diagnóstico, a hidropisia fetal
continua a ser uma entidade desafiante
na medicina perinatal. O objectivo deste
estudo foi analisar os casos de hidropisia
fetal numa Unidade de Cuidados Intensivos
Neonatais (UCIN), para avaliação de
etiologia, morbilidade e mortalidade.
Pacientes e métodos: Os autores
apresentam cinco casos de hidropisia fetal
tratados numa Unidade de Cuidados
Intensivos Neonatais entre Setembro de
2005 a Janeiro de 2009, ilustrativos de
diferentes etiologias.
O estudo baseou-se na análise retrospectiva
dos processos das mães e
recém-nascidos, com avaliação dos dados
obstétricos e neonatais.
Resultados: De Setembro de 2005
a Janeiro de 2009 foram internados na
Unidade 1923 recém-nascidos dos quais
cinco com diagnóstico de hidropisia fetal.
Destes, três casos foram de causa não
imune (fibromatose generalizada congénita;
diabetes materna e cardiomiopatia
dilatada; quilotórax congénito), um caso
por iso-imunização Rh e o outro de causa
idiopática.
Só um dos casos tinha diagnóstico
pré-natal. A mortalidade na nossa série
foi de 40%.
Conclusão: Apesar da melhoria no
diagnóstico e abordagem terapêutica, a
elevada taxa de morbilidade e mortalidade
permanece, sendo de extrema importância
o diagnóstico pré-natal precoce.
Este permite determinar a etiologia, antecipar
problemas, tomar decisões clínicas
de acordo com o prognóstico esperado
e também o aconselhamento genético/
DPN. Uma boa coordenação multidisciplinar,
envolvendo neonatologistas, obstetras
e patologistas é essencial.
ABSTRACT Introduction: Hydrops fetalis is defined by an abnormal accumulation of interstitial fluid in fetuses. Initially described as a unique pathologic entity, in 1892 Ballantyne suggested it might represent a final state common to a variety of different pathologic processes. Potter distinguishes 2 types: immune (more frequent till the discovering of RhoGAM) and non immune (90% of the cases nowadays, with an estimated incidence of 1 in 1500 to 3800 births). Besides all advances in diagnosis, hydrops fetalis still remains a defying entity in perinatal medicine. The aim of this review was to analyze the cases of hydrops fetalis in a Neonatal Intensive Care Unit (NICU) to assess etiology, morbidity and mortality. Patients and methods: The authors present five cases of hydrops fetalis treated in a NICU, from September 2005 to January 2009, illustrating different aetiologies. The study was based on the retrospective analysis of mothers and newborns’ medical files evaluating obstetric and perinatal parameters, diagnosis, treatment and evolution. Results: From September 2005 to January 2009, 1923 newborns were admitted in NICU. Five had the diagnosis of hydrops fetalis. Three of these were of non immune cause (congenital generalized fibromatosis, maternal diabetes and dilated cardiomyopathy and congenital chylothorax), one of Rh iso-immunization and the last of unknown aetiology. Only one of the cases had prenatal diagnosis. The group mortality was of 2/5 cases (40%). Conclusion: Besides the improvement in diagnosis and therapeutic approach, the high mortality and morbidity rates still remain, being extremely important an early prenatal diagnosis. This allows the determination of the aetiology, anticipating problems, making clinical decision based on the expected prognosis and also the genetic counselling. An excellent multidisciplinary coordination, involving neonatologists, obstetricians and pathologists is essential.
ABSTRACT Introduction: Hydrops fetalis is defined by an abnormal accumulation of interstitial fluid in fetuses. Initially described as a unique pathologic entity, in 1892 Ballantyne suggested it might represent a final state common to a variety of different pathologic processes. Potter distinguishes 2 types: immune (more frequent till the discovering of RhoGAM) and non immune (90% of the cases nowadays, with an estimated incidence of 1 in 1500 to 3800 births). Besides all advances in diagnosis, hydrops fetalis still remains a defying entity in perinatal medicine. The aim of this review was to analyze the cases of hydrops fetalis in a Neonatal Intensive Care Unit (NICU) to assess etiology, morbidity and mortality. Patients and methods: The authors present five cases of hydrops fetalis treated in a NICU, from September 2005 to January 2009, illustrating different aetiologies. The study was based on the retrospective analysis of mothers and newborns’ medical files evaluating obstetric and perinatal parameters, diagnosis, treatment and evolution. Results: From September 2005 to January 2009, 1923 newborns were admitted in NICU. Five had the diagnosis of hydrops fetalis. Three of these were of non immune cause (congenital generalized fibromatosis, maternal diabetes and dilated cardiomyopathy and congenital chylothorax), one of Rh iso-immunization and the last of unknown aetiology. Only one of the cases had prenatal diagnosis. The group mortality was of 2/5 cases (40%). Conclusion: Besides the improvement in diagnosis and therapeutic approach, the high mortality and morbidity rates still remain, being extremely important an early prenatal diagnosis. This allows the determination of the aetiology, anticipating problems, making clinical decision based on the expected prognosis and also the genetic counselling. An excellent multidisciplinary coordination, involving neonatologists, obstetricians and pathologists is essential.
Description
Keywords
Hidropisia fetal hidropisia imune hidropisia não imune isoimunização quilotórax fibromatose generalizada congénita Hydrops fetalis hydrops immune hydrops non immune iso-immunization chylothorax congenital generalized fibromatosis
Citation
Nascer e Crescer 2009; 18(4): 261-266