SCIN - Serviço de Cuidados Intensivos Neonatais
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- [Chorioamnionitis and neonatal morbidity]Publication . Rocha, G.; Proença, E.; Quintas, C.; Rodrigues, T.; Guimarães, H.CHORIOAMNIONITIS AND NEONATAL MORBIDITY Introduction - Several studies highlight the association between perinatal infection/ inflammation and neonatal morbidity, mainly bronchopulmonary dysplasia and periventricular leukomalacia. Aim – To evaluate the role of histological chorioamnionitis on the overall morbidity of preterm newborns. Methods – A retrospective study on preterm newborns less than 34 weeks gestational age at birth, and respective mothers, at three tertiary medical centers (Hospital de São João, Maternidade Júlio Dinis and Centro Hospitalar de Vila Nova de Gaia) in the north of Portugal, between January 2001 and December 2002. We evaluated the association between histological chorioamnionitis and the overall neonatal morbidity. The association between histological chorioamnionitis and acute (respiratory distress syndrome) and chronic (bronchopulmonary dysplasia) lung damage was also evaluated in the subgroup of less than 1000 g birthweight preterm neonates. Results – 452 [ M 253 / F 217; birthweight 1440 (515 – 2620) g; gestational age 31 (23 – 33) weeks] preterm newborns were included. The association between histological chorioamnionitis and the overall neonatal morbidity was: respiratory distress syndrome OR 1,5 (95% CI 0,94 – 2,31); bronchopulmonary dysplasia OR 2,6 (95% CI 1,16 – 6,03); patent ductus arteriosus OR 2,5 (95% CI 1,17 – 5,44); sepsis OR 1,2 (95% CI 0,9 – 2,13); necrotizing enterocolitis OR 1,4 (95% CI 0,9 – 1,76); intraventricular hemorrhage grades III-IV OR 2,5 (1,20 – 5,11); cystic periventricular leukomalacia OR 3,0 (1,5 – 6,07); retinopathy of prematurity OR 1,4 (95% CI 0,8 – 1,35). The association adjusted to birthweight and gestational age was: bronchopulmonary dysplasia OR 1,2 (95% CI 0,51 – 2,95); patent ductus arteriosus OR 0,9 (95% CI 0,4 – 2,35); intraventricular hemorrhage grades III-IV OR 0,9 (95% CI 0,39 – 2,28); cystic periventricular leukomalacia OR 2,2 (95% CI 1,03 – 4,61). The association between histological chorioamnionitis and lung damage in the subgroup of less than 1000 g birthweight preterm neonates was: respiratory distress syndrome OR 0,23 (95% CI 0,01 – 2,51); bronchopulmonary dysplasia OR 1,61 (95% CI 0,38 – 6,97). Conclusion – This study confirms the association between histological chorioamnionitis and cystic periventricular leukomalacia of the preterm newborn.
- Congenital cystic adenomatoid malformation of the lung - the experience of five medical centresPublication . Rocha, G.; Fernandes, P.; Proença, E.; Quintas, C.; Martins, T.; Azevedo, I.; Guimarães, H.Background: The clinical spectrum of congenital cystic adenomatoid malformation of the lung to the neonatal intensive care units of five tertiary medical centres in the north of Portugal between 1996 and 2005. Results: Fifteen neonates with CCAML were identified, 9F/6M, birth weight 3100 g (645-3975), gestational age 38 weeks (24-40). The incidence of CCAML was 1: 9300 births. There were 11 (73%) cases of cystic lung lesion diagnosed during pregnancy, median age 22 weeks (19-30). The lesion was right sided in six (40%) and left sided in nine (60%) cases. In utero spontaneous regression of the lesion was observed in two cases. Antenatal intervention (pleurocentesis and thoracoamniotic shunting) was performed in one foetus with impending hydrops. Normal lung radiographic findings at birth were present in five cases, with an abnormal CT scan. Three (20%) neonates became symptomatic during the neonatal period (respiratory distress) and one (70%) after the neonatal period (spontaneous pneumothorax). Two neonates (13%) died. Six (40%) patients underwent thoracotomy and appropriate excisional surgery. Histological examination showed definitive features of CCAML (Stocker classification: type I = 4; type II = 1; type III = 2). Eight (53%) patients remain asymptomatic and did not undergo surgery. Conclusions: Antenatally diagnosed CCAML has a good prognosis in the absence of severe foetal distress; normal radiographic findings at birth do not rule out CCAML; treatment of asymptomatic CCAML is controversial; surgery may be advocated because of its low morbidity and the prevention of late complications such as malignancy. (CCAML) ranges from asymptomatic lesions to neonatal respiratory distress and hydrops fetalis. Aim: To review our experience with CCAML, emphasising natural history, management and outcome. Material and methods: A retrospective chart review of all CCAML-diagnosed neonates admitted
- Displasia Broncopulmonar - Análise de 5 anos na Maternidade de Júlio DinisPublication . Miguez, M.; Mesquita, S.; Almeida, A.; Proença, E.A displasia broncopulmonar continua a ser uma patologia actual, com morbilidade e mortalidade importantes, no entanto, o conceito original criado por Northway et al tem sofrido algumas alterações ao longo do tempo. O presente trabalho pretende fazer uma análise retrospectiva dos casos de displasia broncopulmonar na Unidade de Cuidados Intensivos Neonatais da Maternidade Júlio Dinis, referente aos anos de 1997 a 2001, tentando relacionar alguns factores com o desenvolvimento desta patologia.
- Isoimunização Rh a Múltiplos AntigéniosPublication . Mesquita, S.; Proença, E.; Alexandrino, A.RESUMO A doença hemolítica perinatal isoimune (DHP) resulta da destruição dos eritrócitos fetais e do recém-nascido por anticorpos maternos dirigidos especificamente contra os antigénios de membrana dessas células. Os autores apresentam um caso de isoimunização Rh a antigénios múltiplos, anti-C, D e E, num 2º filho de um casal com antecedentes de abortamento espontâneo e um 1º filho com DHP anti- CDE e necessidade de transfusão permuta. Apesar da gravidade, com necessidade de várias transfusões- permuta e concentrados de plaquetas, a doença acabou por evoluir favoravelmente. O evento sensibilizante desta isoimunização terá sido o abortamento, sete anos antes, em que não foi feita profilaxia anti-D. O principal objectivo na DHP é, sempre que possível, a prevenção da isoimunização primária e a identificação precoce dos casos em que a isoimunização já ocorreu. A propósito deste caso, são discutidos os factores envolvidos na falência da prevenção e os responsáveis pela sua gravidade. Os autores concluem que o erro humano constitui um dos principais factores implicados no aparecimento e gravidade da maioria dos casos de DHP. ABSTRACT The haemolytic perinatal isoimune disease (HPD) results from the destruction of fetal and newborn erythrocytes by maternal antibodies directed specifically against membrane antigens of those cells. The authors present a case of Rh isoimmunization by multiple antigens, anti-C, D and E, in a second offspring of a couple which previously had had a spontaneous abortion, and then a first offspring with HPD anti-CDE who needed an exchange transfusion. Despite the severity of the disease, requiring several exchange transfusions and platelet transfusions, the outcome was favourable. The event responsible for this isoimmunization was the previous spontaneous abortion, which was not followed by anti-D prophylaxis. In the management of the HPD the main goals are the prevention of primary isoimmunization and the early identification of those cases in which isoimmunization already occurred. In the reference case we discuss the factors responsible for the failure in the prevention and for its severity. The authors conclude that human error remains one of the main factors responsible for the majority of the DHP cases and its severity.
- Neonatal Hemophilia: A Rare PresentationPublication . Ferreira, N.; Proença, E.; Godinho, C.; Oliveira, D.; Guedes, A.; Morais, S.; Carvalho, C.Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.
- A Novel Noonan Syndrome RAF1 Mutation: Lethal Course in a Preterm InfantPublication . Ratola, A.; Silva, H.; Guedes, A.; Mota, C.; Braga, A.; Oliveira, D.; Alegria, A.; Carvalho, C.; Álvares, S.; Proença, E.Noonan syndrome is a relatively common and heterogeneous genetic disorder, associated with congenital heart defect in about 50% of the cases. If the defect is not severe, life expectancy is normal. We report a case of Noonan syndrome in a preterm infant with hypertrophic cardiomyopathy and lethal outcome associated to acute respiratory distress syndrome caused by Adenovirus pneumonia. A novel mutation in the RAF1 gene was identified: c.782C>G (p.Pro261Arg) in heterozygosity, not described previously in the literature. Consequently, the common clinical course in this mutation and its respective contribution to the early fatal outcome is unknown. No conclusion can be established regarding genotype/phenotype correlation.
- Prática ventilatória em recém-nascidos de extremo baixo pesoPublication . Ratola, A.; Silva, H.; Oliveira, D.; Carvalho, C.; Almeida, A.; Proença, E.Introdução: As práticas ventilatórias nos recém-nascidos de extremo baixo peso (RNEBP) têm vindo a alterar-se ao longo dos anos, havendo uma preocupação crescente em implementar estratégias protetoras do pulmão. O presente trabalho pretendeu caracterizar a prática ventilatória nos RNEBP numa unidade de cuidados intensivos neonatais. Métodos: Análise retrospetiva dos registos clínicos dos RNEBP, admitidos entre 01/09/2010 e 31/08/2013. Resultados: Foram admitidos 94 RNEBP (mediana peso 790 g), com idade gestacional de 23-32 semanas (mediana 27 semanas). Foi administrado pelo menos um ciclo de corticoide pré-natal em 65% e 69% receberam no mínimo uma dose de surfatante. Realizaram ventilação invasiva 74%, a maioria desde o nascimento, e 15% necessitaram de ventilação de alta frequência como resgate. Efetuaram ventilação não invasiva 70%, de forma exclusiva em 23% dos casos. Não precisaram de qualquer suporte ventilatório 3%. A mortalidade atingiu 31% (mediana idade gestacional 25 semanas), ocorrendo quase metade dos óbitos no primeiro dia de vida. Desenvolveram hemorragia pulmonar 7%, fuga aérea 5%, hemorragia intraperiventricular de grau III 22%, canal arterial hemodinamicamente significativo 23% e enterocolite necrosante 3%. O grupo que não realizou ventilação invasiva não apresentou hemorragia pulmonar, fuga aérea ou hemorragia intraperiventricular. Dos 65 sobreviventes, 20% desenvolveram displasia broncopulmonar moderada ou grave e 14% retinopatia da prematuridade (≥3/ doença plus). Discussão: O suporte ventilatório dos RNEBP tende a ser progressivamente mais protetor. No grupo analisado, a quase totalidade dos recém-nascidos necessitou de apoio ventilatório, mas cerca de um quarto realizou apenas ventilação não invasiva. A mortalidade global foi elevada, atingindo sobretudo os mais imaturos e em cerca de metade ocorreu no primeiro dia.
- Prenatal Diagnosis and Management of a Fetal Goiter Hypothyroidism due to DyshormonogenesisPublication . Figueiredo, C.; Falcão, I.; Vilaverde, J.; Freitas, J.; Oliveira, M.; Godinho, C.; Dores, J.; Rodrigues, M.; Carvalho, C.; Borges, T.Fetal goiter is a rare disorder not expected to be found during a healthy woman's pregnancy. It can be a prenatal manifestation of congenital hypothyroidism due to thyroid dyshormonogenesis and it can lead to serious perinatal complications. A vascularized fetal neck mass was detected at 29 weeks' gestation of a healthy primigravida. Magnetic resonance was suggestive of goiter causing airway deviation without polyhydramnios. Maternal thyroid function was normal and thyroid antibodies were negative. Two intra-amniotic levothyroxine infusions were performed at 32 and 33 weeks. Serial imaging control showed no progression of the mass. Elective caesarean section was performed at 38 weeks. The male newborn was admitted to the intensive care unit due to cardiorespiratory insufficiency with pulmonary hypertension. Hormonal assays revealed primary congenital hypothyroidism and ultrasonography confirmed diffuse goiter. Levothyroxine was started. Currently, he is 6 years old with adequate growth and normal psychomotor development. Genetic study found a heterozygous mutation in the TPO gene.
- REANIMAÇÃO NEONATALPublication . Madeira, I.; Matias, S.
- Use of Therapeutic Hypothermia in Sudden Unexpected Postnatal Collapse: A Retrospective StudyPublication . Brito, Sara; Sampaio, Isabel; Dinis, Alexandra; Proença, Elisa; Vilan, Ana; Soares, Eunice; Pinto, Filomena; Tomé, Teresa; Graça, André M.Introduction: Sudden and unexpected postnatal collapse is a rare event with potentially dramatic consequences. Intervention approaches are limited, but hypothermia has been considered after postnatal collapse. The aim of this study was to analyse sudden and unexpected postnatal collapse cases that underwent therapeutic hypothermia in the five Portuguese hypothermia centres. Material and methods: In this multicentre, retrospective and descriptive study, clinical, ultrasonography, amplitude-integrated electroencephalography and brain magnetic resonance findings of newborns with postnatal collapse that underwent therapeutic hypothermia are reported (2010 - 2018). Statistical analysis was performed by using IBM SPSS Statistics version 21. Results: Twenty-two cases of sudden and unexpected postnatal collapse were referred for therapeutic hypothermia (82% outborn), all ≥ 36 weeks, with Apgar 5´ ≥ 8. Collapse occurred during the first two hours in 73% (all < 24 hours), 50% during skin-to-skin care, 55% related to feeding and 23% during co-bedding. Moderate-severe encephalopathy and severe acidosis were observed (median: Thompson score 16, pH 6.90, base deficit 22 mmol/L). Amplitude-integrated electroencephalogram was abnormal in 95% and magnetic resonance imaging showed severe brain injury in 46%. The mortality rate was 50%. A possible cause was identified in 27%. Discussion: The incidence rate of 2.7 sudden cases of postnatal collapse per 100 000 births, is possibly under-estimated. All infants suffered the collapse in the first day, mostly within the first two hours, as reported before. Possible causes were identified in less than a third of cases, but multiple predisposing conditions were identified, suggesting that prevention may be possible. Newborn positioning and skin-to-skin care have been the most discussed practices. A significant proportion of infants had poor outcomes. Lower Thompson score, electroencephalogram amplitude normalization and normal magnetic resonance imaging seemed to indicate better outcomes. Although conclusive trials on therapeutic hypothermia after postnatal collapse are not available, its use has been considered individually. No severe adverse effects directly related to hypothermia were registered in this study, but the results do not allow drawing meaningful conclusions. Conclusion: In our national sample of 22 infants who suffered sudden and unexpected postnatal collapse and underwent therapeutic hypothermia, a significant proportion had poor outcomes. Absolute conclusions from our experience with hypothermia in postnatal collapse cannot be drawn, but systematic reporting of cases and long-term clinical evaluation would facilitate understanding of the real benefits of hypothermia. As this procedure has not been validated with clinical trials for this indication, its use should be considered on a case-by-case approach. The potentially avoidable nature of unexpected postnatal collapse is evident from its association with certain behaviours and risk factors. Surveillance practices during the first hours should be implemented, whilst the benefits of breastfeeding and skin-to-skin care should continue to be widely promoted.