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Odontomatosis-aortae esophagus stenosis syndrome

MedGen UID:
320249
•Concept ID:
C1834013
•
Disease or Syndrome
Synonyms: Boder syndrome; Odontoma dysphagia syndrome; Odontomatosis (multiple odontomas) with dysphagia
SNOMED CT: Odontoma dysphagia syndrome (716180009); Boder syndrome (716180009)
 
Monarch Initiative: MONDO:0008118
OMIM®: 164330
Orphanet: ORPHA2724

Definition

Odontoma-dysphagia syndrome is a malformation syndrome, with characteristics of odontomas and severe dysphagia. Less than ten cases have been reported so far. Three of the reported patients manifested multiple odontomas. Occasionally, cardiac, renal and hepatic involvement has been described. In several cases, autosomal dominant inheritance has been suspected. Currently, there are no genes associated with this condition. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOdontomatosis-aortae esophagus stenosis syndrome
Follow this link to review classifications for Odontomatosis-aortae esophagus stenosis syndrome in Orphanet.

Professional guidelines

PubMed

Endoscopic management of enteral tubes in adult patients - Part 2: Peri- and post-procedural management. European Society of Gastrointestinal Endoscopy (ESGE) Guideline.
Gkolfakis P, Arvanitakis M, Despott EJ, Ballarin A, Beyna T, Boeykens K, Elbe P, Gisbertz I, Hoyois A, Mosteanu O, Sanders DS, Schmidt PT, Schneider SM, van Hooft JE
Endoscopy 2021 Feb;53(2):178-195. Epub 2020 Dec 21 doi: 10.1055/a-1331-8080. PMID: 33348410
Eosinophilic esophagitis: Current concepts in diagnosis and treatment.
Gómez-Aldana A, Jaramillo-Santos M, Delgado A, Jaramillo C, Lúquez-Mindiola A
World J Gastroenterol 2019 Aug 28;25(32):4598-4613. doi: 10.3748/wjg.v25.i32.4598. PMID: 31528089Free PMC Article
Treatment of GERD complications (Barrett's, peptic stricture) and extra-oesophageal syndromes.
Bansal A, Kahrilas PJ
Best Pract Res Clin Gastroenterol 2010 Dec;24(6):961-8. doi: 10.1016/j.bpg.2010.09.007. PMID: 21126707Free PMC Article

Recent clinical studies

Etiology

Gastrointestinal Emergency in Neonates and Infants: A Pictorial Essay.
Choi G, Je BK, Kim YJ
Korean J Radiol 2022 Jan;23(1):124-138. doi: 10.3348/kjr.2021.0111. PMID: 34983099Free PMC Article
Endoscopic management of enteral tubes in adult patients - Part 2: Peri- and post-procedural management. European Society of Gastrointestinal Endoscopy (ESGE) Guideline.
Gkolfakis P, Arvanitakis M, Despott EJ, Ballarin A, Beyna T, Boeykens K, Elbe P, Gisbertz I, Hoyois A, Mosteanu O, Sanders DS, Schmidt PT, Schneider SM, van Hooft JE
Endoscopy 2021 Feb;53(2):178-195. Epub 2020 Dec 21 doi: 10.1055/a-1331-8080. PMID: 33348410
Feeding problems and gastrointestinal diseases in Down syndrome.
Ravel A, Mircher C, Rebillat AS, Cieuta-Walti C, Megarbane A
Arch Pediatr 2020 Jan;27(1):53-60. Epub 2019 Nov 26 doi: 10.1016/j.arcped.2019.11.008. PMID: 31784293
Bariatric Surgery and Its Complications in Inflammatory Bowel Disease Patients.
Singh A, Koenen B, Kirby DF
Inflamm Bowel Dis 2020 Jul 17;26(8):1155-1165. doi: 10.1093/ibd/izz246. PMID: 31626698
Clinical and etiological heterogeneity in patients with tracheo-esophageal malformations and associated anomalies.
Brosens E, Ploeg M, van Bever Y, Koopmans AE, IJsselstijn H, Rottier RJ, Wijnen R, Tibboel D, de Klein A
Eur J Med Genet 2014 Aug;57(8):440-52. Epub 2014 Jun 13 doi: 10.1016/j.ejmg.2014.05.009. PMID: 24931924

Diagnosis

Phenotypic variability and gastrointestinal manifestations/interventions for growth in NAA10-related neurodevelopmental syndrome.
Sandomirsky K, Marchi E, Gavin M, Amble K, Lyon GJ
Am J Med Genet A 2023 May;191(5):1293-1300. Epub 2023 Feb 21 doi: 10.1002/ajmg.a.63152. PMID: 36810866Free PMC Article
Gastrointestinal Emergency in Neonates and Infants: A Pictorial Essay.
Choi G, Je BK, Kim YJ
Korean J Radiol 2022 Jan;23(1):124-138. doi: 10.3348/kjr.2021.0111. PMID: 34983099Free PMC Article
Feeding problems and gastrointestinal diseases in Down syndrome.
Ravel A, Mircher C, Rebillat AS, Cieuta-Walti C, Megarbane A
Arch Pediatr 2020 Jan;27(1):53-60. Epub 2019 Nov 26 doi: 10.1016/j.arcped.2019.11.008. PMID: 31784293
Eosinophilic esophagitis: Current concepts in diagnosis and treatment.
Gómez-Aldana A, Jaramillo-Santos M, Delgado A, Jaramillo C, Lúquez-Mindiola A
World J Gastroenterol 2019 Aug 28;25(32):4598-4613. doi: 10.3748/wjg.v25.i32.4598. PMID: 31528089Free PMC Article
Vascular rings.
Backer CL, Mongé MC, Popescu AR, Eltayeb OM, Rastatter JC, Rigsby CK
Semin Pediatr Surg 2016 Jun;25(3):165-75. Epub 2016 Feb 22 doi: 10.1053/j.sempedsurg.2016.02.009. PMID: 27301603

Therapy

Exploring the role of lipoprotein(a) in cardiovascular diseases and diabetes in Chinese population.
Hussain Z, Iqbal J, Liu H, Zhou HD
Int J Biol Macromol 2023 Apr 1;233:123586. Epub 2023 Feb 8 doi: 10.1016/j.ijbiomac.2023.123586. PMID: 36758756
Endoscopic management of enteral tubes in adult patients - Part 2: Peri- and post-procedural management. European Society of Gastrointestinal Endoscopy (ESGE) Guideline.
Gkolfakis P, Arvanitakis M, Despott EJ, Ballarin A, Beyna T, Boeykens K, Elbe P, Gisbertz I, Hoyois A, Mosteanu O, Sanders DS, Schmidt PT, Schneider SM, van Hooft JE
Endoscopy 2021 Feb;53(2):178-195. Epub 2020 Dec 21 doi: 10.1055/a-1331-8080. PMID: 33348410
Bariatric Surgery and Its Complications in Inflammatory Bowel Disease Patients.
Singh A, Koenen B, Kirby DF
Inflamm Bowel Dis 2020 Jul 17;26(8):1155-1165. doi: 10.1093/ibd/izz246. PMID: 31626698
Eosinophilic esophagitis: Current concepts in diagnosis and treatment.
Gómez-Aldana A, Jaramillo-Santos M, Delgado A, Jaramillo C, Lúquez-Mindiola A
World J Gastroenterol 2019 Aug 28;25(32):4598-4613. doi: 10.3748/wjg.v25.i32.4598. PMID: 31528089Free PMC Article
Pathophysiology of Eosinophilic Esophagitis.
O'Shea KM, Aceves SS, Dellon ES, Gupta SK, Spergel JM, Furuta GT, Rothenberg ME
Gastroenterology 2018 Jan;154(2):333-345. Epub 2017 Jul 27 doi: 10.1053/j.gastro.2017.06.065. PMID: 28757265Free PMC Article

Prognosis

Exploring the role of lipoprotein(a) in cardiovascular diseases and diabetes in Chinese population.
Hussain Z, Iqbal J, Liu H, Zhou HD
Int J Biol Macromol 2023 Apr 1;233:123586. Epub 2023 Feb 8 doi: 10.1016/j.ijbiomac.2023.123586. PMID: 36758756
Ortner's Syndrome Due to Giant Thoracic Aortic Aneurysm.
Shrimanth YS, Barwad P, Maralakunte M, Sharma A, Sihag BK
J Invasive Cardiol 2022 Apr;34(4):E346. PMID: 35366231
Clinical and etiological heterogeneity in patients with tracheo-esophageal malformations and associated anomalies.
Brosens E, Ploeg M, van Bever Y, Koopmans AE, IJsselstijn H, Rottier RJ, Wijnen R, Tibboel D, de Klein A
Eur J Med Genet 2014 Aug;57(8):440-52. Epub 2014 Jun 13 doi: 10.1016/j.ejmg.2014.05.009. PMID: 24931924
Black esophagus: acute esophageal necrosis syndrome.
Gurvits GE
World J Gastroenterol 2010 Jul 14;16(26):3219-25. doi: 10.3748/wjg.v16.i26.3219. PMID: 20614476Free PMC Article
Surgical treatment of pulmonary artery sling and tracheal stenosis.
Fiore AC, Brown JW, Weber TR, Turrentine MW
Ann Thorac Surg 2005 Jan;79(1):38-46; discussion 38-46. doi: 10.1016/j.athoracsur.2004.06.005. PMID: 15620911

Clinical prediction guides

Exploring the role of lipoprotein(a) in cardiovascular diseases and diabetes in Chinese population.
Hussain Z, Iqbal J, Liu H, Zhou HD
Int J Biol Macromol 2023 Apr 1;233:123586. Epub 2023 Feb 8 doi: 10.1016/j.ijbiomac.2023.123586. PMID: 36758756
In a Setting of Esophageal Replacement, Total Gastric Pull-Up has Fewer Complications than Partial Gastric Pull-Up.
Rubio M, Boglione M, Rührnschopf CG, Gammino LG, Alessandro P, Fraire C, Takeda S, Paz E, Weyersberg C, Barrenechea M
J Pediatr Surg 2023 Sep;58(9):1625-1630. Epub 2022 Nov 19 doi: 10.1016/j.jpedsurg.2022.11.002. PMID: 36581550
Perioperative complications following major vascular surgery. Correlations with preoperative clinical, electrocardiographic and echocardiographic features.
Roberto Bolognesi, Dimitri Tsialtas, Maria Giulia Bolognesi, Stephania Assimopoulos, Matteo Azzarone, Riccardo Volpi
Acta Biomed 2022 Jul 1;93(3):e2022255. doi: 10.23750/abm.v93i3.12961. PMID: 35775755Free PMC Article
Clinical and etiological heterogeneity in patients with tracheo-esophageal malformations and associated anomalies.
Brosens E, Ploeg M, van Bever Y, Koopmans AE, IJsselstijn H, Rottier RJ, Wijnen R, Tibboel D, de Klein A
Eur J Med Genet 2014 Aug;57(8):440-52. Epub 2014 Jun 13 doi: 10.1016/j.ejmg.2014.05.009. PMID: 24931924
Vascular ring abnormalities: a retrospective study of 62 cases.
Bonnard A, Auber F, Fourcade L, Marchac V, Emond S, Révillon Y
J Pediatr Surg 2003 Apr;38(4):539-43. doi: 10.1053/jpsu.2003.50117. PMID: 12677561

Recent systematic reviews

Long-term functional outcomes after replacement of the esophagus in pediatric patients: A systematic literature review.
Garritano S, Irino T, Scandavini CM, Tsekrekos A, Lundell L, Rouvelas I
J Pediatr Surg 2017 Sep;52(9):1398-1408. Epub 2017 Jun 8 doi: 10.1016/j.jpedsurg.2017.05.034. PMID: 28625693
Gastric-tube versus whole-stomach esophagectomy for esophageal cancer: A systematic review and meta-analysis.
Zhang W, Yu D, Peng J, Xu J, Wei Y
PLoS One 2017;12(3):e0173416. Epub 2017 Mar 7 doi: 10.1371/journal.pone.0173416. PMID: 28267808Free PMC Article

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