{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T10:56:55Z","timestamp":1793271415904,"version":"4.5.0"},"reference-count":27,"publisher":"American Academy of Pediatrics (AAP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,4,1]]},"abstract":"<jats:sec>\n                    <jats:title>INTRODUCTION<\/jats:title>\n                    <jats:p>While severe macroglossia in Beckwith-Wiedemann syndrome (BWS) is often treated with tongue reduction surgery, most patients with mild to moderate macroglossia are nonsurgically managed. Given that outcomes for these conservatively managed patients are not well characterized, this study aims to describe the natural history of patients with macroglossia and BWS who did not undergo early surgery.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>Records of patients with BWS and macroglossia seen between 2004\u20132024 were reviewed. Conservative management was defined as not having had surgery during the first 3\u00a0years of life. Macroglossia was rated using the BWS Index of macroGlossia (BIG) scale to stratify phenotypic severity. Relationships among polysomnography data, surgical incidence, percentage mosaicism, and clinical scores were assessed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>320 patients with BWS and macroglossia were included, 231 (72.2%) of whom were conservatively managed. As BIG scores increased from BIG1 (mild) to BIG2 (moderate) to BIG3 (severe), both obstructive sleep apnea severity (P\u2009&amp;lt;\u2009.001) and surgical incidence increased (P\u2009&amp;lt;\u2009.001). Among conservatively managed patients, obstructive apnea-hypoxia index improved from 4.5 (interquartile range: 2.7\u201311.2) events per hour (age 0.4 [0.2\u20130.7] years) to 3.6 (1.3\u20136.1) events per hour (age 2.2 [1.7\u20132.6] years; P\u2009=\u2009.03). BIG scores also improved in this cohort from 2.0 (1.0\u20132.0) at 0.8 (0.4\u20131.5) years of age to 1.0 (1.0\u20132.0) at 2.6 (2.2\u20133.7) years of age (P\u2009=\u2009.02).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSION<\/jats:title>\n                    <jats:p>Nonsurgical management in the first 3\u00a0years of life for mild and moderate macroglossia in BWS is associated with favorable outcomes. Obstructive sleep apnea and macroglossia often improve as the facial skeleton grows to accommodate the tongue.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1542\/peds.2024-068618","type":"journal-article","created":{"date-parts":[[2025,3,11]],"date-time":"2025-03-11T20:02:26Z","timestamp":1741723346000},"source":"Crossref","is-referenced-by-count":3,"title":["Conservative Management of Macroglossia in Beckwith-Wiedemann Syndrome"],"prefix":"10.1542","volume":"155","author":[{"given":"Dominic J.","family":"Romeo","sequence":"first","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Connor S.","family":"Wagner","sequence":"additional","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manisha","family":"Banala","sequence":"additional","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M.","family":"George","sequence":"additional","affiliation":[{"name":"2Division of Human Genetics, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin B.","family":"Massenburg","sequence":"additional","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meagan","family":"Wu","sequence":"additional","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinggang J.","family":"Ng","sequence":"additional","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher M.","family":"Cielo","sequence":"additional","affiliation":[{"name":"3Division of Pulmonology, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer M.","family":"Kalish","sequence":"additional","affiliation":[{"name":"4Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania"},{"name":"5Department of Genetics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jesse A.","family":"Taylor","sequence":"additional","affiliation":[{"name":"1Division of Plastic, Reconstructive, and Oral Surgery, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2025,3,12]]},"reference":[{"issue":"2","key":"2026100510430353900_r1","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1038\/ejhg.2015.88","article-title":"(Epi)genotype-phenotype correlations in Beckwith-Wiedemann syndrome","volume":"24","author":"Mussa","year":"2016","journal-title":"Eur J Hum Genet."},{"key":"2026100510430353900_r2","doi-asserted-by":"crossref","first-page":"562","DOI":"10.3389\/fped.2019.00562","article-title":"Diagnosis and management of Beckwith-Wiedemann syndrome","volume":"7","author":"Wang","year":"2020","journal-title":"Front Pediatr."},{"issue":"suppl 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Genet."},{"issue":"4","key":"2026100510430353900_r6","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1038\/nrendo.2017.166","article-title":"Expert consensus document: clinical and molecular diagnosis, screening and management of Beckwith-Wiedemann syndrome: an international consensus statement","volume":"14","author":"Brioude","year":"2018","journal-title":"Nat Rev Endocrinol."},{"issue":"2","key":"2026100510430353900_r7","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1159\/000329569","article-title":"Impact of tongue reduction on overall speech intelligibility, articulation and oromyofunctional behavior in 4 children with Beckwith-Wiedemann syndrome","volume":"64","author":"Van Lierde","year":"2012","journal-title":"Folia Phoniatr Logop."},{"issue":"4","key":"2026100510430353900_r8","doi-asserted-by":"crossref","first-page":"e25","DOI":"10.1597\/10-232","article-title":"Psychosocial, feeding, and drooling outcomes in children with Beckwith Wiedemann syndrome following tongue reduction surgery","volume":"49","author":"Shipster","year":"2012","journal-title":"Cleft Palate Craniofac J."},{"issue":"12","key":"2026100510430353900_r9","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1016\/j.jcms.2020.09.004","article-title":"Long-term longitudinal evalutation of mandibular growth in patients with Beckwith-Wiedemann Syndrome treated and not treated with glossectomy","volume":"48","author":"Meazzini","year":"2020","journal-title":"J Craniomaxillofac Surg."},{"issue":"1","key":"2026100510430353900_r10","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.ijom.2018.07.008","article-title":"Tongue reduction in Beckwith-Wiedemann syndrome: outcome and treatment algorithm","volume":"48","author":"Naujokat","year":"2019","journal-title":"Int J Oral Maxillofac Implants."},{"issue":"4","key":"2026100510430353900_r11","doi-asserted-by":"crossref","first-page":"803e","DOI":"10.1097\/PRS.0000000000006673","article-title":"The utility of early tongue reduction surgery for macroglossia in Beckwith-Wiedemann syndrome","volume":"145","author":"Cohen","year":"2020","journal-title":"Plast Reconstr Surg."},{"issue":"1","key":"2026100510430353900_r12","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1097\/SCS.0000000000004823","article-title":"Improved quality of life for children with Beckwith-Wiedemann syndrome following tongue reduction surgery","volume":"30","author":"Harada","year":"2019","journal-title":"J Craniofac Surg."},{"issue":"6","key":"2026100510430353900_r13","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1097\/PRS.0000000000011112","article-title":"(Epi)genotype and timing of tongue reduction predict safety and long-term outcomes in Beckwith-Wiedemann syndrome","volume":"154","author":"Wagner","year":"2024","journal-title":"Plast Reconstr Surg."},{"issue":"9","key":"2026100510430353900_r14","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1016\/j.jcms.2023.07.006","article-title":"Associations between the timing of tongue reduction surgery, (epi)genotype, and dentoskeletal development in patients with Beckwith-Wiedemann syndrome","volume":"51","author":"Wagner","year":"2023","journal-title":"J Craniomaxillofac Surg."},{"issue":"3","key":"2026100510430353900_r15","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1016\/j.ijom.2011.10.021","article-title":"Surgical treatment of macroglossia in patients with Beckwith-Wiedemann syndrome: a 20-year experience and review of the literature","volume":"41","author":"Kadouch","year":"2012","journal-title":"Int J Oral Maxillofac Implants."},{"issue":"2","key":"2026100510430353900_r16","first-page":"e225","article-title":"Beckwith-Wiedemann Syndrome: open bite evolution after tongue reduction","volume":"23","author":"Alonso-Rodriguez","year":"2018","journal-title":"Med Oral Patol Oral Cir Bucal."},{"key":"2026100510430353900_r17","article-title":"A novel macroglossia severity index for Beckwith-Wiedemann Syndrome","volume-title":"Ann Surg.","author":"Romeo","year":"2024"},{"issue":"2","key":"2026100510430353900_r18","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/j.ijom.2012.09.003","article-title":"Tongue reduction for macroglossia in Beckwith Wiedemann syndrome: review and application of new technique","volume":"42","author":"Heggie","year":"2013","journal-title":"Int J Oral Maxillofac Implants."},{"issue":"6","key":"2026100510430353900_r19","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/j.jcms.2016.02.010","article-title":"Taste and speech following surgical tongue reduction in children with Beckwith-Wiedemann syndrome","volume":"44","author":"Maas","year":"2016","journal-title":"J Craniomaxillofac 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Genet."},{"issue":"8","key":"2026100510430353900_r26","doi-asserted-by":"crossref","first-page":"4271","DOI":"10.1007\/s00784-023-05043-w","article-title":"Dentoskeletal features and growth pattern in Beckwith-Wiedemann spectrum: is surgical tongue reduction always necessary?","volume":"27","author":"Defabianis","year":"2023","journal-title":"Clin Oral Investig."},{"key":"2026100510430353900_r27","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.sleep.2022.07.010","article-title":"Respiratory indices during sleep in healthy infants: a prospective longitudinal study and meta-analysis","volume":"99","author":"Stefanovski","year":"2022","journal-title":"Sleep Med."}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2024-068618\/1788729\/pediatrics.2024068618.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2024-068618\/1788729\/pediatrics.2024068618.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T14:43:10Z","timestamp":1791211390000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/155\/4\/e2024068618\/201261\/Conservative-Management-of-Macroglossia-in"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,12]]},"references-count":27,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2025,3,12]]},"published-print":{"date-parts":[[2025,4,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2024-068618","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,12]]},"article-number":"e2024068618"}}