{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T18:31:50Z","timestamp":1792175510013,"version":"4.4.0"},"reference-count":47,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2024,6,4]],"date-time":"2024-06-04T00:00:00Z","timestamp":1717459200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>The early management of neonates with meconium ileus (MI) and cystic fibrosis (CF) is highly variable across countries and is not standardized. We conducted a systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. The protocol was registered in PROSPERO (CRD42024522838). Studies from three providers of academic search engines were checked for inclusion criteria, using the following search terms: meconium ileus AND cystic fibrosis OR mucoviscidosis. Regarding the patient population studied, the inclusion criteria were defined using our predefined PICOT framework: studies on neonates with simple or complicated meconium which were confirmed to have cystic fibrosis and were conservatively managed or surgically treated. Results: A total of 566 publications from the last 10 years were verified by the authors of this review to find the most recent and relevant data, and only 8 met the inclusion criteria. Prenatally diagnosed meconium pseudocysts, bowel dilation, and ascites on ultrasound are predictors of neonatal surgery and risk factor for negative 12-month clinical outcomes in MI-CF newborns. For simple MI, conservative treatment with hypertonic solutions enemas can be effective in more than 25% of cases. If repeated enemas fail to disimpact the bowels, the Bishop\u2013Koop stoma is a safe option. No comprehensive research has been conducted so far to determine the ideal surgical protocol for complicated MI. We only found three studies that reported the types of stomas performed and another study comparing the outcomes of patients depending on the surgical management; the conclusions are contradictory especially since the number of cases analyzed in each study was small. Between 18% and 38% of patients with complicated MI will require reoperation for various complications and the mortality rate varies between 0% and 8%. Conclusion: This study reveals a lack of strong data to support management decisions, unequivocally shows that the care of infants with MI is not standardized, and suggests a great need for international collaborative studies.<\/jats:p>","DOI":"10.3390\/diagnostics14111179","type":"journal-article","created":{"date-parts":[[2024,6,5]],"date-time":"2024-06-05T08:43:54Z","timestamp":1717577034000},"page":"1179","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Diagnosis and Management of Simple and Complicated Meconium Ileus in Cystic Fibrosis, a Systematic Review"],"prefix":"10.3390","volume":"14","author":[{"given":"M\u0103d\u0103lina Andreea","family":"Donos","sequence":"first","affiliation":[{"name":"Saint Mary Emergency Hospital for Children, 700309 Iasi, Romania"},{"name":"Pediatrics Department, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela","family":"Ghiga","sequence":"additional","affiliation":[{"name":"Pediatrics Department, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura Mihaela","family":"Trandafir","sequence":"additional","affiliation":[{"name":"Pediatrics Department, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7859-840X","authenticated-orcid":false,"given":"Elena","family":"Cojocaru","sequence":"additional","affiliation":[{"name":"Department of Morphofunctional Sciences I\u2014Pathology, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viorel","family":"\u021aarc\u0103","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine and Interdisciplinarity, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6713-4244","authenticated-orcid":false,"given":"L\u0103cr\u0103mioara Ionela","family":"Butnariu","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Faculty of Medicine, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentin","family":"Bernic","sequence":"additional","affiliation":[{"name":"Department of Surgery II, \u201cSaint Spiridon\u201d Hospital, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugenia","family":"Moro\u0219an","sequence":"additional","affiliation":[{"name":"Department of Morphofunctional Sciences I\u2014Pathology, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iulia Cristina","family":"Roca","sequence":"additional","affiliation":[{"name":"Department of Surgery II, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana Elena","family":"M\u00eendru","sequence":"additional","affiliation":[{"name":"Pediatrics Department, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3018-8011","authenticated-orcid":false,"given":"Elena","family":"\u021aarc\u0103","sequence":"additional","affiliation":[{"name":"Department of Surgery II\u2014Pediatric Surgery, \u201cGrigore T. Popa\u201d University of Medicine and Pharmacy, 700115 Iasi, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1055\/s-0037-1609027","article-title":"Meconium Ileus","volume":"31","author":"Richards","year":"2018","journal-title":"Clin. Colon Rectal Surg."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1016\/j.jpedsurg.2012.02.019","article-title":"A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon","volume":"47","author":"Carlyle","year":"2012","journal-title":"J. Pediatr. Surg."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"S32","DOI":"10.1016\/j.jcf.2017.06.007","article-title":"Meconium ileus in Cystic Fibrosis","volume":"16","author":"Sathe","year":"2017","journal-title":"J. Cyst. Fibros."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1038\/ng.2221","article-title":"Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis","volume":"44","author":"Sun","year":"2012","journal-title":"Nat. Genet."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1053\/j.gastro.2006.07.016","article-title":"Relative contribution of genetic and nongenetic modifiers to intestinal obstruction in cystic fibrosis","volume":"131","author":"Blackman","year":"2006","journal-title":"Gastroenterology"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Butnariu, L.I., \u021aarc\u0103, E., Cojocaru, E., Rusu, C., Mois\u0103, M., Constantin, M.-M.L., Gorduza, E.V., and Trandafir, L.M. (2021). Genetic Modifying Factors of Cystic Fibrosis Phenotype: A Challenge for Modern Medicine. J. Clin. Med., 10.","DOI":"10.3390\/jcm10245821"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1080\/17476348.2023.2228194","article-title":"Thinking outside the box: A review of gastrointestinal symptoms and complications in cystic fibrosis","volume":"17","author":"Yule","year":"2023","journal-title":"Expert Rev. Respir. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1016\/j.jpedsurg.2021.02.047","article-title":"Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study","volume":"56","author":"Long","year":"2021","journal-title":"J. Pediatr. Surg."},{"key":"ref_9","first-page":"32","article-title":"Meconium ileus in newborns with cystic fibrosis\u2014Results of treatment in the group of patients operated on in the years 2000\u20132014","volume":"19","author":"Boczar","year":"2015","journal-title":"Dev. Period Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1016\/j.jpedsurg.2013.11.038","article-title":"Gastrointestinal surgery in cystic fibrosis: A 20-year review","volume":"49","author":"Farrelly","year":"2014","journal-title":"J. Pediatr. Surg."},{"key":"ref_11","first-page":"339","article-title":"The PRISMA statement for reporting systematic 426 reviews and meta-analyses of studies that evaluate healthcare interventions: Explanation and elaboration","volume":"21","author":"Liberati","year":"2009","journal-title":"BMJ"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1055\/a-1194-4363","article-title":"Fetal Meconium Peritonitis\u2014Prenatal Findings and Postnatal Outcome: A Case Series, Systematic Review, and Meta-Analysis. Fetale Mekoniumperitonitis\u2014Pr\u00e4natale Befunde und postnatales Outcome: Eine Fallserie, systematische \u00dcbersicht und Metaanalyse","volume":"43","author":"Shinar","year":"2022","journal-title":"Ultraschall Med. Eur. J. Ultrasound"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1016\/j.jcf.2019.07.003","article-title":"Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study","volume":"18","author":"Padoan","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1755","DOI":"10.1007\/s00247-018-4198-5","article-title":"Meconium peritonitis: The role of postnatal radiographic and sonographic findings in predicting the need for surgery","volume":"48","author":"Zani","year":"2018","journal-title":"Pediatr. Radiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1016\/j.jpedsurg.2018.05.016","article-title":"Predictors for surgical intervention and surgical outcomes in neonates with cystic fibrosis","volume":"53","author":"Jessula","year":"2018","journal-title":"J. Pediatr. Surg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e1485","DOI":"10.1590\/0102-672020190001e1485","article-title":"A comparative study between Santulli ileostomy and loop ileostomy in neonates with meconium ileus","volume":"33","author":"Askarpour","year":"2020","journal-title":"Abcd-Arquivos Bras. Cir. Dig. Arch. Dig. Surg."},{"key":"ref_17","unstructured":"Parikh, N.S., Ibrahim, S., and Ahlawat, R. (2024, March 17). Meconium Ileus, StatPearls [Internet], Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK537008\/."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1136\/thoraxjnl-2018-211706","article-title":"Impact of cystic fibrosis on birthweight: A population based study of children in Denmark and Wales","volume":"74","author":"Griffiths","year":"2019","journal-title":"Thorax"},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Belu, A., Trandafir, L.M., \u021aarc\u0103, E., Cojocaru, E., Fr\u0103sinariu, O., St\u00e2rcea, M., Moscalu, M., Tiutiuca, R.C., Luca, A.C., and Galaction, A. (2022). Variations in Biochemical Values under Stress in Children with SARS-CoV-2 Infection. Diagnostics, 12.","DOI":"10.3390\/diagnostics12051213"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1590\/s1806-37562017000000065","article-title":"Brazilian guidelines for the diagnosis and treatment of cystic fibrosis","volume":"43","author":"Athanazio","year":"2017","journal-title":"J. Bras. Pneumol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1016\/S0140-6736(09)60327-5","article-title":"Cystic fibrosis","volume":"373","author":"Freedman","year":"2009","journal-title":"Lancet"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2622","DOI":"10.37358\/RC.19.7.7392","article-title":"Antibiotic Molecules Involved in Increasing Microbial Resistance","volume":"70","author":"Duceac","year":"2019","journal-title":"Rev. Chim."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2130","DOI":"10.1016\/j.jpedsurg.2009.06.005","article-title":"Diminishing role of contrast enema in simple meconium ileus","volume":"44","author":"Copeland","year":"2009","journal-title":"J. Pediatr. Surg."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/0022-3468(69)90390-X","article-title":"Treatment of uncomplicated meconium ileus by gastrografin enema: A preliminary report","volume":"4","author":"Noblett","year":"1969","journal-title":"J. Pediatr. Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1053\/jpsu.2002.32272","article-title":"New strategies in nonoperative management of meconium ileus","volume":"37","author":"Burke","year":"2002","journal-title":"J. Pediatr. Surg."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"120","DOI":"10.4103\/jiaps.JIAPS_41_18","article-title":"Management Strategy of Meconium Ileus-Outcome Analysis","volume":"24","author":"Pandey","year":"2019","journal-title":"J. Indian Assoc. Pediatr. Surg."},{"key":"ref_27","first-page":"5312179","article-title":"Fetal Midgut Volvulus with Meconium Peritonitis Detected on Prenatal Ultrasound","volume":"2018","author":"Best","year":"2018","journal-title":"Case Rep. Obstet. Gynecol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s00247-011-2173-5","article-title":"US assessment of neonatal bowel (necrotizing enterocolitis excluded)","volume":"42","author":"Veyrac","year":"2012","journal-title":"Pediatr. Radiol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.radcr.2018.10.013","article-title":"Meconium pseudocyst presenting as massive ascites in a new-born","volume":"14","author":"Khan","year":"2018","journal-title":"Radiol. Case Rep."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1007\/s00383-005-1614-3","article-title":"Immunoreactive trypsin levels in neonates with meconium ileus","volume":"22","author":"Steven","year":"2006","journal-title":"Pediatr. Surg. Int."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"4150","DOI":"10.1097\/MS9.0000000000001048","article-title":"A unique presentation of hyponatremia and seizures in a 2-month-old child with cystic fibrosis: A case report","volume":"85","author":"Allouzi","year":"2023","journal-title":"Ann. Med. Surg."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Trandafir, L.M., Fr\u0103sinariu, O.E., \u021aarc\u0103, E., Butnariu, L.I., Constantin, M.M.L., Moscalu, M., Temneanu, O.R., Popescu, A.S.M., Popescu, M.G.M., and St\u00e2rcea, I.M. (2023). Can Bioactive Food Substances Contribute to Cystic Fibrosis-Related Cardiovascular Disease Prevention?. Nutrients, 15.","DOI":"10.3390\/nu15020314"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1136\/archdischild-2013-304159","article-title":"Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes","volume":"99","author":"Leeuwen","year":"2014","journal-title":"Arch. Dis. Child."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.jcmgh.2019.04.013","article-title":"Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota","volume":"8","author":"Fiorotto","year":"2019","journal-title":"Cell. Mol. Gastroenterol. Hepatol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"943649","DOI":"10.3389\/fped.2023.943649","article-title":"Nutrition in children with exocrine pancreatic insufficiency","volume":"11","author":"Zheng","year":"2023","journal-title":"Front. Pediatr."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1513\/AnnalsATS.202008-933OC","article-title":"Early-Life Height Attainment in Cystic Fibrosis Is Associated with Pulmonary Function at Age 6 Years","volume":"18","author":"Sanders","year":"2021","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_37","first-page":"8","article-title":"Association of nutritional status with lung function and morbidity in children and adolescents with cystic fibrosis: A 36-month cohort study","volume":"36","author":"Hauschild","year":"2018","journal-title":"Rev. Paul Pediatr."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.jcf.2019.09.008","article-title":"Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus","volume":"18","author":"Tan","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"e20152907","DOI":"10.1542\/peds.2015-2907","article-title":"Pubertal Height Growth and Adult Height in Cystic Fibrosis After Newborn Screening","volume":"137","author":"Zhang","year":"2016","journal-title":"Pediatrics"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1903","DOI":"10.1007\/s10620-015-3546-7","article-title":"Gastrointestinal Manifestations of Cystic Fibrosis","volume":"60","author":"Kelly","year":"2015","journal-title":"Dig. Dis. Sci."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/j.suc.2022.07.016","article-title":"Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Other Gastrointestinal Pathology in the Cystic Fibrosis Patient","volume":"102","author":"Tobias","year":"2022","journal-title":"Surg. Clin. N. Am."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1016\/j.jcf.2019.10.003","article-title":"Is meconium ileus associated with worse outcomes in cystic fibrosis?","volume":"18","author":"Sathe","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1007\/s11894-011-0185-9","article-title":"Intestinal obstruction syndromes in cystic fibrosis: Meconium ileus, distal intestinal obstruction syndrome, and constipation","volume":"13","author":"Kokke","year":"2011","journal-title":"Curr. Gastroenterol. Rep."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.jpeds.2006.04.059","article-title":"Potential impact of newborn screening for cystic fibrosis on child survival: A systematic review and analysis","volume":"149","author":"Grosse","year":"2006","journal-title":"J. Pediatr."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"S23","DOI":"10.1016\/j.jcf.2014.03.010","article-title":"European Cystic Fibrosis Society Standards of Care: Best Practice guidelines","volume":"13","author":"Smyth","year":"2014","journal-title":"J. Cyst. Fibros."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"S3","DOI":"10.1002\/ppul.23240","article-title":"Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors","volume":"50","author":"Solomon","year":"2015","journal-title":"Pediatr. Pulmonol."},{"key":"ref_47","first-page":"CD009841","article-title":"Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis","volume":"2019","author":"Skilton","year":"2019","journal-title":"Cochrane Database Syst. Rev."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/14\/11\/1179\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:53:19Z","timestamp":1760107999000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/14\/11\/1179"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,4]]},"references-count":47,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2024,6]]}},"alternative-id":["diagnostics14111179"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics14111179","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,4]]}}}