Malformación adenomatoidea quística: diagnóstico prenatal, resultados de la cirugía y seguimiento a largo plazo.

Translated title of the contribution: Congenital cystic adenomatoid malformation: prenatal diagnosis, surgical treatment results and long-term follow-up

J. L. Encinas, F. Pederiva, A. Luis, L. F. Avila, A. Fernández, C. Carrero, J. M. Mariño, A. Queizán, L. Lassaleta, J. A. Tovar

Research output: Contribution to journalArticle

Abstract

PURPOSE: (PD), possibility of regresion and hidden mortality are open questions in congenital cystic adenomatoid malformation (CCAM) treatment. METHODS: Children with CCAM were reviewed focused on: PD, postnatal diagnosis, clinic, radiology, histology and evolution. RESULTS: Seventeen fetus had PD of CCAM. Five gestations were electively finished (41%) with PD of CCAM previous to 20th week, 3/5 (60%) were type III and 2/5 (40%) type I; 4/5 (80%) presented mediastinal shift and 1/5 (20%) hidrops. Two fetuses (11%) suffered fetal demise in 20th and 32th week; 1 type 1 and 1 type III; 1/2 (50%) presented hidrops and 2/2 (100%) mediastinal shift. Two (12%) died before 24 hours after birth without intervention possibility due to respiratory instability, 1 type II and 1 type III, both with mediastinal shift (100%). In one fetus with a type III malformation the image disappeared completely in 32th week and no intervention was done. Fourteen patients were operated (8 girls and 6 boys); 7/14 (50%) had PD, average diagnosis week was 21.9 (range 19.1-35.5), 5/7 (71%) was type I, 1/7 (14%) type II and 1/7 (14%) type III. None had mediastinal shift or hidrops. Average postnatal diagnosis week was 7 months (range 0.1-29). In 10/14 (71.4%) there were not respiratory difficulty during neonatal period and 3/10 (30%) suffered respiratory infections afterwards. Average week of operation was 8 months (range 0.1-30). PD was according with histology in 6/7 (86%) patients. After an average follow-up period of 4.3 years (range 1-9.5) the only complication is a pectus excavatum. CONCLUSIONS: More than half of patients with PD of CCAM died without intervention. Half of cases of CCAM are diagnosed prenatally. Type of CCAM in PD is according to histology in 86% of the cases. Fetuses with hidrops present a worse prognosis. Surgical timing do not seem to influence on outcome.

Translated title of the contributionCongenital cystic adenomatoid malformation: prenatal diagnosis, surgical treatment results and long-term follow-up
Original languageSpanish
Pages (from-to)87-90
Number of pages4
JournalCirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
Volume20
Issue number2
Publication statusPublished - Apr 2007

ASJC Scopus subject areas

  • Medicine(all)

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    Encinas, J. L., Pederiva, F., Luis, A., Avila, L. F., Fernández, A., Carrero, C., Mariño, J. M., Queizán, A., Lassaleta, L., & Tovar, J. A. (2007). Malformación adenomatoidea quística: diagnóstico prenatal, resultados de la cirugía y seguimiento a largo plazo. Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica, 20(2), 87-90.