{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:03:13Z","timestamp":1760144593332,"version":"build-2065373602"},"reference-count":43,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2024,5,5]],"date-time":"2024-05-05T00:00:00Z","timestamp":1714867200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health with \u201cCurrent Research funds\u201d"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Holoprosencephaly is a complex human brain malformation resulting from incomplete cleavage of the prosencephalon into both hemispheres. Congenital nasal pyriform aperture stenosis (CNPAS) is sometimes found in patients with mild forms of holoprosencephaly. Surgical treatment is required. Low-invasive surgical approaches involve balloon dilation of the pyriform opening. We present the case of an 8-day-old girl diagnosed with holoprosencephaly, CNPAS, and the presence of a solitary median maxillary central incisor. Once examined by neonatologist, geneticist, pneumologist, otolaryngologist, and pediatric dentist, a combined otolaryngological\u2013orthodontic approach was used. The obstruction of the right nasal cavity was treated by widening the nasal cavities and stabilizing them with a balloon dilation technique. After surgery, the respiratory space was increased by applying a neonatal palatal expander plate (NPEP) considering the palatal deformity: ogival shaped, anterior vertex growth direction, reduction of transverse diameters. The NPEP promoted distraction of the median palatine suture and assisted the nasal dilation. Therefore, after the insertion of NPEP, the physiological sucking\u2013swallowing mechanism was activated. In infants with CNPAS, NPEP can be useful to ensure the safe stability of nasal dilation. A multidisciplinary approach is fundamental. In our experience, the close collaboration between an otolaryngologist and orthodontist is essential for the management of the patient with CNPAS.<\/jats:p>","DOI":"10.3390\/children11050554","type":"journal-article","created":{"date-parts":[[2024,5,6]],"date-time":"2024-05-06T15:05:01Z","timestamp":1715007901000},"page":"554","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Use of an Orthodontic and Otolaryngological Approach in an Infant with Holoprosencephaly"],"prefix":"10.3390","volume":"11","author":[{"given":"Angela","family":"Galeotti","sequence":"first","affiliation":[{"name":"Dentistry Unit, Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni Carlo","family":"De Vincentiis","sequence":"additional","affiliation":[{"name":"Private Practice, 00174 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emanuela","family":"Sitzia","sequence":"additional","affiliation":[{"name":"Otolaryngology Unit, Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9088-7749","authenticated-orcid":false,"given":"Giuseppe","family":"Marzo","sequence":"additional","affiliation":[{"name":"Department of Life, Health, Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wanda","family":"Maldonato","sequence":"additional","affiliation":[{"name":"Dentistry Unit, Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gaia","family":"Bompiani","sequence":"additional","affiliation":[{"name":"Dentistry Unit, Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Beatrice","family":"Chiarini Testa","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology & Respiratory Intermediate Care Unit, Sleep and Long-Term Ventilation Unit, Acdemic Department of Pediatrics (DPUO), Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5278-145X","authenticated-orcid":false,"given":"Alessandra","family":"Putrino","sequence":"additional","affiliation":[{"name":"Dentistry Unit, Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9765-7460","authenticated-orcid":false,"given":"Andrea","family":"Bartuli","sequence":"additional","affiliation":[{"name":"Rare Diseases and Medical Genetics Unit, Academic Department of Pediatrics, Bambino Ges\u00f9 Children\u2019s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), 00165 Rome, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Festa","sequence":"additional","affiliation":[{"name":"Dentistry Unit, AORN Santobono-Pausilipon, 80100 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,5,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Society for Maternal-Fetal Medicine (SMFM), and Monteagudo, A. (2020). Holoprosencephaly. Am. J. Obstet. Gynecol., 223, B13\u2013B16.","DOI":"10.1016\/j.ajog.2020.08.178"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1148\/rg.351140040","article-title":"Holoprosencephaly: A survey of the entity, with embryology and fetal imaging","volume":"35","author":"Winter","year":"2015","journal-title":"Radiographics"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1616","DOI":"10.1002\/pd.5777","article-title":"Are the prevalence of Trisomy 13 and the incidence of severe holoprosencephaly increasing in Africa?","volume":"40","author":"Okoye","year":"2020","journal-title":"Prenat. Diagn."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"e22","DOI":"10.1097\/SCS.0000000000003171","article-title":"Facial Evaluation in Holoprosencephaly","volume":"28","author":"Pucciarelli","year":"2017","journal-title":"J. Craniofac. Surg."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Malta, M., AlMutiri, R., Martin, C.S., and Srour, M. (2023). Holoprosencephaly: Review of Embryology, Clinical Phenotypes, Etiology and Management. Children, 10.","DOI":"10.3390\/children10040647"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.ijporl.2004.07.011","article-title":"Congenital nasal pyriform aperture stenosis with semilobar holoprosencephaly","volume":"69","author":"Chan","year":"2005","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"37","DOI":"10.2500\/ar.2016.7.0146","article-title":"Newborn nasal obstruction due to congenital nasal pyriform aperture stenosis","volume":"7","author":"Serrano","year":"2016","journal-title":"Allergy Rhinol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1016\/j.ijporl.2013.08.021","article-title":"Nasal cavity dimensions in congenital pyriform aperture stenosis","volume":"77","author":"Reeves","year":"2013","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1186\/1824-7288-38-28","article-title":"Congenital nasal pyriform aperture stenosis: Diagnosis and management","volume":"38","author":"Sesenna","year":"2012","journal-title":"Ital. J. Pediatr."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"bcr1220092562","DOI":"10.1136\/bcr.12.2009.2562","article-title":"Respiratory distress in neonate due to congenital nasal pyriform aperture stenosis","volume":"2010","author":"Nakwan","year":"2010","journal-title":"BMJ Case Rep."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1097\/00005537-200205000-00025","article-title":"Congenital pyriform aperture stenosis: Surgery and evaluation with three-dimensional computed tomography","volume":"112","author":"Lee","year":"2002","journal-title":"Laryngoscope"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.amjoto.2013.12.016","article-title":"Balloon dilatation for congenital nasal piriform aperture stenosis (CNPAS): A novel conservative technique","volume":"35","author":"Gungor","year":"2014","journal-title":"Am. J. Otolaryngol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1186\/s13052-021-01124-2","article-title":"Balloon dilation and rapid maxillary expansion: A novel combination treatment for congenital nasal pyriform aperture stenosis in an infant","volume":"47","author":"Sitzia","year":"2021","journal-title":"Ital. J. Pediatr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"dev191064","DOI":"10.1242\/dev.191064","article-title":"Gene-environment interactions: Aligning birth defects research with complex etiology","volume":"147","author":"Beames","year":"2020","journal-title":"Development"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1002\/ajmg.c.30236","article-title":"The molecular genetics of holoprosencephaly","volume":"154C","author":"Roessler","year":"2010","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"ref_16","unstructured":"Adam, M.P., Feldman, J., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., Bean, L.J.H., Gripp, K.W., and Amemiya, A. (2024). GeneReviews\u00ae [Internet], University of Washington."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1002\/bdr2.1834","article-title":"Identifying environmental risk factors and gene-environment interactions in holoprosencephaly","volume":"113","author":"Addissie","year":"2021","journal-title":"Birth Defects Res."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1002\/ajmg.c.31618","article-title":"Prenatal diagnosis of holoprosencephaly","volume":"178","author":"Kousa","year":"2018","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1002\/ajmg.c.31627","article-title":"Challenging issues arising in counseling families experiencing holoprosencephaly","volume":"178","author":"Hadley","year":"2018","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"9717","DOI":"10.1080\/14767058.2022.2050900","article-title":"Sonography of fetal holoprosencephaly: A guide to recognize the lesser varieties","volume":"35","author":"Montaguti","year":"2022","journal-title":"J. Matern. Fetal Neonatal Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1007\/s00381-016-3015-4","article-title":"Holoprosencephaly: Antenatal and postnatal diagnosis and outcome","volume":"32","author":"Kaliaperumal","year":"2016","journal-title":"Childs Nerv. Syst."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1016\/S1079-2104(97)90368-1","article-title":"Solitary median maxillary central incisor, short stature, choanal atresia\/midnasal stenosis (SMMCI) syndrome","volume":"84","author":"Hall","year":"1997","journal-title":"Oral. Surg. Oral. Med. Oral. Pathol. Oral. Radiol. Endod."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/S0899-7071(98)00034-5","article-title":"Congenital nasal pyriform aperture stenosis and single central maxillary incisor: CT and MRI findings","volume":"22","author":"Huang","year":"1998","journal-title":"Clin. Imaging"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1007\/s002470050606","article-title":"Single central maxillary incisor with nasal pyriform aperture stenosis--CT diagnosis prior to tooth eruption","volume":"29","author":"Royal","year":"1999","journal-title":"Pediatr. Radiol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1111\/j.1440-1754.2005.00639.x","article-title":"Neonatal nasal obstruction and a single maxillary central incisor","volume":"41","author":"Levison","year":"2005","journal-title":"J. Paediatr. Child. Health"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/S1808-8694(15)31319-7","article-title":"Congenital nasal pyriform aperture stenosis","volume":"71","author":"Tagliarini","year":"2005","journal-title":"Braz. J. Otorhinolaryngol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1597\/07-182.1","article-title":"Congenital nasal pyriform aperture stenosis: Diagnosis and management","volume":"46","author":"Devambez","year":"2009","journal-title":"Cleft Palate Craniofac J."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1016\/j.ijporl.2010.05.018","article-title":"A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia","volume":"74","author":"Blackmore","year":"2010","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"266","DOI":"10.4103\/0971-3026.73539","article-title":"Congenital nasal pyriform aperture stenosis: A rare cause of nasal airway obstruction in a neonate","volume":"20","author":"Thomas","year":"2010","journal-title":"Indian. J. Radiol. Imaging"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.ijporl.2011.09.016","article-title":"Congenital nasal pyriform aperture stenosis: A report of 10 cases and literature review","volume":"76","author":"Visvanathan","year":"2012","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1007\/s40368-013-0044-5","article-title":"Solitary median maxillary central incisor syndrome (SMMCI) with congenital nasal puriform aperture stenosis: Literature review and case report with comprehensive dental treatment and 14 years follow-up","volume":"14","author":"Lygidakis","year":"2013","journal-title":"Eur. Arch. Paediatr. Dent."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.ijporl.2016.01.011","article-title":"Congenital nasal pyriform aperture stenosis: Elaboration of a management algorithm from 25 years of experience","volume":"83","author":"Moreddu","year":"2016","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_33","first-page":"e130","article-title":"Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: Case report","volume":"116","author":"Ilhan","year":"2018","journal-title":"Arch. Argent. Pediatr."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1002\/ajmg.c.31624","article-title":"Holoprosencephaly from conception to adulthood","volume":"178","author":"Weiss","year":"2018","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1017\/S0022215117000391","article-title":"Pyriform aperture enlargement in all aspects","volume":"131","author":"Esen","year":"2017","journal-title":"J. Laryngol. Otol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"e240149","DOI":"10.1136\/bcr-2020-240149","article-title":"Novel maxillary enlargement technique in congenital nasal pyriform aperture stenosis: A case report and literature review","volume":"14","author":"Hallikainen","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_37","first-page":"556","article-title":"Dental alveolar bone and dental arch remodeling in children: Orthodontic diagnosis and treatments based on individual child arch development","volume":"34","author":"Xiaobing","year":"2016","journal-title":"Hua Xi Kou Qiang Yi Xue Za Zhi"},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Putrino, A., Caputo, M., Galeotti, A., Marinelli, E., and Zaami, S. (2023). Type I Dentin Dysplasia: The Literature Review and Case Report of a Family Affected by Misrecognition and Late Diagnosis. Medicina, 59.","DOI":"10.3390\/medicina59081477"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1177\/10556656211007689","article-title":"Nonsurgical Orthodontic Airway Plate Treatment for Newborns With Robin Sequence","volume":"59","author":"Choo","year":"2022","journal-title":"Cleft Palate Craniofac J."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"196","DOI":"10.22514\/jocpd.2024.048","article-title":"Oral self-inflicted accidental trauma in patients with neurological disorders: A case report of dental management in infants with cerebellar hypoplasia","volume":"48","author":"Galeotti","year":"2024","journal-title":"J. Clin. Pediatr. Dent."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1002\/ddrr.16","article-title":"Development of swallowing and feeding: Prenatal through first year of life","volume":"14","author":"Delaney","year":"2008","journal-title":"Dev. Disabil. Res. Rev."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1111\/dmcn.14918","article-title":"Fetal movements: The origin of human behaviour","volume":"63","author":"Einspieler","year":"2021","journal-title":"Dev. Med. Child. Neurol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.neulet.2016.12.016","article-title":"New insights into sucking, swallowing and breathing central generators: A complexity analysis of rhythmic motor behaviors","volume":"638","author":"Samson","year":"2017","journal-title":"Neurosci. Lett."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/11\/5\/554\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:39:59Z","timestamp":1760107199000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/11\/5\/554"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,5]]},"references-count":43,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2024,5]]}},"alternative-id":["children11050554"],"URL":"https:\/\/doi.org\/10.3390\/children11050554","relation":{},"ISSN":["2227-9067"],"issn-type":[{"type":"electronic","value":"2227-9067"}],"subject":[],"published":{"date-parts":[[2024,5,5]]}}}