diagnosis and perinatal outcome of 38 cases with congenital diaphragmatic hernia: 8-year experience of a tertiary Brazilian center. Clinics. 2006;61(3):197-202. PURPOSE:To evaluate the perinatal results for neonates with congenital diaphragmatic hernia diagnosed prenatally. METHOD: We reviewed data from 38 cases of congenital diaphragmatic hernia diagnosed prenatally from January 1995 to December 2003 in the Fetal Medicine Unit of the Department of Obstetrics and Gynecology, São Paulo University Medical School. The main data analyzed were gestational age at diagnosis, fetal karyotyping, side of diaphragmatic defect, presence of associated structural malformations, hepatic herniation, and severe mediastinal shift. Perinatal outcomes were obtained by reviewing hospital documents or by directly calling the patients' immediate relatives. RESULTS: Mean gestational age at diagnosis was 29 weeks (range, 16-37 weeks).Thirty (79%) cases had a left diaphragmatic defect and 8 (21%) had a right lesion. Associated structural malformations were observed in 21 (55%) cases, in which 12 fetuses had a normal karyotype and 9 had chromosomal abnormalities. Isolated congenital diaphragmatic hernia was confirmed in 17 (45%) cases. The overall perinatal mortality rate was 92%. Rates of fetal deaths, early neonatal deaths, late neonatal deaths, and survival were 42%, 50%, 0%, and 8%, respectively, in cases with associated structural malformations but normal karyotyping; 56%, 44%, 0%, and 0% for cases with chromosomal abnormalities; and, 0%, 76%, 12%, and 12% in cases with isolated congenital diaphragmatic hernia. The neonatal mortality rate was 89% in cases with isolated congenital diaphragmatic hernia. CONCLUSION: Perinatal mortality was very high in prenatally diagnosed cases of congenital diaphragmatic hernia. Earlier perinatal deaths are associated with the presence of other structural defects or chromosomal abnormalities. In cases of isolated congenital diaphragmatic hernia, mortality is related to the presence of herniated liver, right-sided lesion, and major mediastinal shift.
RESUMO Objetivo: avaliar a evolução de 24 casos de gastrosquise, em relação aos fatores prognósticos pré-natais que interferiram na sobrevida pós-natal. Pacientes e Métodos: foram avaliados 24 casos de gastrosquise diagnosticados no Setor de Medicina Fetal do Hospital das Clínicas da FMUSP, durante um período de 8 anos. Foram classificados em gastrosquise associada, quando presentes outras malformações, e isolada. Nos dois grupos foram analisados parâmetros referentes às alças intestinais dilatadas na avaliação ultra-sonográfica (dilatação >18 mm), complicações obstétricas e resultados pós-natal. Resultados: foram observados 9 casos de gastrosquise associada (37,5%) e 15 casos de gastrosquise isolada (62,5%). Todos os casos de gastrosquise associada foram de prognóstico letal, levando a uma alta taxa de mortalidade geral de 60,8%. Do grupo de gastrosquises isoladas, todos nasceram vivos e foram submetidos a cirurgia, com taxa de sobrevida de 60% e mortalidade pós-natal de 40%. A mediana da idade gestacional foi de 35 semanas e o peso no nascimento de 2.365 gramas no grupo geral. Nas gastrosquises isoladas, o parto prematuro ocorreu em 10 casos, principalmente decorrente de complicações obstétricas. Dois recém-nascidos foram considerados pequenos para a idade gestacional e apenas 3 apresentaram peso no nascimento >2.500 gramas. O oligoidrâmnio foi um achado comum (46,6%), sendo mais freqüente no grupo que evoluiu para óbito neonatal (66,7%). A avaliação ultra-sonográfica das alças intestinais demonstrou que em 13 de 15 casos (86,6%) as alças eram dilatadas, mas sem relação significativa com o prognóstico e achados pós-natais. Não houve diferença significativa em relação a idade gestacional e peso no nascimento, comparando os grupos de vivos e óbitos neonatais. Conclusões: as gastrosquises isoladas apresentam um melhor prognóstico quando comparadas às associadas, sendo de suma importância a sua diferenciação pré-natal. As gastrosquises isoladas estão associadas a complicações obstétricas (60%), prematuridade e baixo peso ao nascimento. O diagnóstico pré-natal permite uma melhor monitorização das condições fetais. O parto destas gestações deve ser no termo, a menos que complicações obstétricas se apresentem. PALAVRAS-CHAVE: Gastrosquise. Diagnóstico pré-natal. Malformação fetal.
OBJECTIVE:The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy.METHODS:We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016.RESULTS:During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%.CONCLUSION:Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.
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