Giant omphaloceles: surgical management and perinatal outcomes

Abstract Background The purpose of this study was to describe the current management and outcomes of infants with omphalocele. Methods The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classi...

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Veröffentlicht in:The Journal of surgical research 2015-10, Vol.198 (2), p.388-392
Hauptverfasser: Akinkuotu, Adesola C., MD, Sheikh, Fariha, MD, Olutoye, Oluyinka O., MD, PhD, Lee, Timothy C., MD, Fernandes, Cariciolo J., MD, Welty, Stephen E., MD, Ayres, Nancy A., MD, Cass, Darrell L., MD
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container_issue 2
container_start_page 388
container_title The Journal of surgical research
container_volume 198
creator Akinkuotu, Adesola C., MD
Sheikh, Fariha, MD
Olutoye, Oluyinka O., MD, PhD
Lee, Timothy C., MD
Fernandes, Cariciolo J., MD
Welty, Stephen E., MD
Ayres, Nancy A., MD
Cass, Darrell L., MD
description Abstract Background The purpose of this study was to describe the current management and outcomes of infants with omphalocele. Methods The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac. Results Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect ( n  = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively ( P  = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P  = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P  
doi_str_mv 10.1016/j.jss.2015.03.060
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Methods The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as &gt;50% of liver in the omphalocele sac. Results Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect ( n  = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively ( P  = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P  = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P  &lt; 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4–23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. Conclusions The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.</description><identifier>ISSN: 0022-4804</identifier><identifier>EISSN: 1095-8673</identifier><identifier>DOI: 10.1016/j.jss.2015.03.060</identifier><identifier>PMID: 25918004</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Giant omphalocele ; Hernia, Umbilical - diagnosis ; Hernia, Umbilical - mortality ; Hernia, Umbilical - surgery ; Humans ; Infant, Newborn ; Prenatal diagnosis ; Prenatal Diagnosis - statistics &amp; numerical data ; Retrospective Studies ; Surgery ; Surgical outcomes ; Texas - epidemiology</subject><ispartof>The Journal of surgical research, 2015-10, Vol.198 (2), p.388-392</ispartof><rights>Elsevier Inc.</rights><rights>2015 Elsevier Inc.</rights><rights>Copyright © 2015 Elsevier Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c478t-8c958e70c16c224d0cc8e0860ffb9dd275409f2c20fe9b1942b8174c152ce8413</citedby><cites>FETCH-LOGICAL-c478t-8c958e70c16c224d0cc8e0860ffb9dd275409f2c20fe9b1942b8174c152ce8413</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0022480415003236$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,65309</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25918004$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Akinkuotu, Adesola C., MD</creatorcontrib><creatorcontrib>Sheikh, Fariha, MD</creatorcontrib><creatorcontrib>Olutoye, Oluyinka O., MD, PhD</creatorcontrib><creatorcontrib>Lee, Timothy C., MD</creatorcontrib><creatorcontrib>Fernandes, Cariciolo J., MD</creatorcontrib><creatorcontrib>Welty, Stephen E., MD</creatorcontrib><creatorcontrib>Ayres, Nancy A., MD</creatorcontrib><creatorcontrib>Cass, Darrell L., MD</creatorcontrib><title>Giant omphaloceles: surgical management and perinatal outcomes</title><title>The Journal of surgical research</title><addtitle>J Surg Res</addtitle><description>Abstract Background The purpose of this study was to describe the current management and outcomes of infants with omphalocele. Methods The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as &gt;50% of liver in the omphalocele sac. Results Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect ( n  = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively ( P  = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P  = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P  &lt; 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4–23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. Conclusions The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.</description><subject>Giant omphalocele</subject><subject>Hernia, Umbilical - diagnosis</subject><subject>Hernia, Umbilical - mortality</subject><subject>Hernia, Umbilical - surgery</subject><subject>Humans</subject><subject>Infant, Newborn</subject><subject>Prenatal diagnosis</subject><subject>Prenatal Diagnosis - statistics &amp; numerical data</subject><subject>Retrospective Studies</subject><subject>Surgery</subject><subject>Surgical outcomes</subject><subject>Texas - epidemiology</subject><issn>0022-4804</issn><issn>1095-8673</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kcFq3DAQhkVpabZpH6CXsMde7M7IsiUlEAihTQqBHpqehVYeJ3JsayvZhbx9ZDbpIYeehND3_2i-YewzQomAzde-7FMqOWBdQlVCA2_YBkHXhWpk9ZZtADgvhAJxxD6k1EO-a1m9Z0e81qgAxIadX3k7zdsw7u_tEBwNlE63aYl33tlhO9rJ3tFImbBTu91T9JOd80NYZhdGSh_Zu84OiT49n8fs9_dvt5fXxc3Pqx-XFzeFE1LNhXK6ViTBYeM4Fy04pwhUA123023LZS1Ad9xx6EjvUAu-UyiFw5o7UgKrY_bl0LuP4c9CaTajT_m3g50oLMmgBC1RoJQZxQPqYkgpUmf20Y82PhoEs2ozvcnazKrNQGWytpw5ea5fdiO1_xIvnjJwdgAoD_nXUzTJeZoctT6Sm00b_H_rz1-l3eCn1fADPVLqwxKnbM-gSdyA-bXubV0b1gAVr5rqCf48kYk</recordid><startdate>20151001</startdate><enddate>20151001</enddate><creator>Akinkuotu, Adesola C., MD</creator><creator>Sheikh, Fariha, MD</creator><creator>Olutoye, Oluyinka O., MD, PhD</creator><creator>Lee, Timothy C., MD</creator><creator>Fernandes, Cariciolo J., MD</creator><creator>Welty, Stephen E., MD</creator><creator>Ayres, Nancy A., MD</creator><creator>Cass, Darrell L., MD</creator><general>Elsevier Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20151001</creationdate><title>Giant omphaloceles: surgical management and perinatal outcomes</title><author>Akinkuotu, Adesola C., MD ; Sheikh, Fariha, MD ; Olutoye, Oluyinka O., MD, PhD ; Lee, Timothy C., MD ; Fernandes, Cariciolo J., MD ; Welty, Stephen E., MD ; Ayres, Nancy A., MD ; Cass, Darrell L., MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c478t-8c958e70c16c224d0cc8e0860ffb9dd275409f2c20fe9b1942b8174c152ce8413</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Giant omphalocele</topic><topic>Hernia, Umbilical - diagnosis</topic><topic>Hernia, Umbilical - mortality</topic><topic>Hernia, Umbilical - surgery</topic><topic>Humans</topic><topic>Infant, Newborn</topic><topic>Prenatal diagnosis</topic><topic>Prenatal Diagnosis - statistics &amp; numerical data</topic><topic>Retrospective Studies</topic><topic>Surgery</topic><topic>Surgical outcomes</topic><topic>Texas - epidemiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Akinkuotu, Adesola C., MD</creatorcontrib><creatorcontrib>Sheikh, Fariha, MD</creatorcontrib><creatorcontrib>Olutoye, Oluyinka O., MD, PhD</creatorcontrib><creatorcontrib>Lee, Timothy C., MD</creatorcontrib><creatorcontrib>Fernandes, Cariciolo J., MD</creatorcontrib><creatorcontrib>Welty, Stephen E., MD</creatorcontrib><creatorcontrib>Ayres, Nancy A., MD</creatorcontrib><creatorcontrib>Cass, Darrell L., MD</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>The Journal of surgical research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Akinkuotu, Adesola C., MD</au><au>Sheikh, Fariha, MD</au><au>Olutoye, Oluyinka O., MD, PhD</au><au>Lee, Timothy C., MD</au><au>Fernandes, Cariciolo J., MD</au><au>Welty, Stephen E., MD</au><au>Ayres, Nancy A., MD</au><au>Cass, Darrell L., MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Giant omphaloceles: surgical management and perinatal outcomes</atitle><jtitle>The Journal of surgical research</jtitle><addtitle>J Surg Res</addtitle><date>2015-10-01</date><risdate>2015</risdate><volume>198</volume><issue>2</issue><spage>388</spage><epage>392</epage><pages>388-392</pages><issn>0022-4804</issn><eissn>1095-8673</eissn><abstract>Abstract Background The purpose of this study was to describe the current management and outcomes of infants with omphalocele. Methods The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as &gt;50% of liver in the omphalocele sac. Results Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect ( n  = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively ( P  = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P  = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P  &lt; 0.001) compared with those with minor or no anomalies. Of 40 infants with GO, the majority (85%) were managed surgically by delayed closure with a median age at repair of 10 mo (range, 3.4–23.6 mo). Six-month survival was 80%. None of the delayed repair patients required a later operative revision, whereas 2 of 5 with early repair did. Conclusions The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele. In patients with GO, delayed closure is associated with good outcomes, but larger, prospective studies comparing delayed to early closure are needed to determine the optimal timing of repair.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>25918004</pmid><doi>10.1016/j.jss.2015.03.060</doi><tpages>5</tpages></addata></record>
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subjects Giant omphalocele
Hernia, Umbilical - diagnosis
Hernia, Umbilical - mortality
Hernia, Umbilical - surgery
Humans
Infant, Newborn
Prenatal diagnosis
Prenatal Diagnosis - statistics & numerical data
Retrospective Studies
Surgery
Surgical outcomes
Texas - epidemiology
title Giant omphaloceles: surgical management and perinatal outcomes
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