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Duodenal aganglionosis

MedGen UID:
868932
•Concept ID:
C4023345
•
Anatomical Abnormality
HPO: HP:0011465

Definition

A lack of intestinal ganglion cells (i.e., an aganglionic section of bowel) affecting the duodenum. [from HPO]

Professional guidelines

PubMed

Diagnostic decision-making tool for imaging term neonatal bowel obstruction.
Tsitsiou Y, Calle-Toro JS, Zouvani A, Andronikou S
Clin Radiol 2021 Mar;76(3):163-171. Epub 2020 Oct 20 doi: 10.1016/j.crad.2020.09.016. PMID: 33097229
Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood.
de Lorijn F, Boeckxstaens GE, Benninga MA
Curr Gastroenterol Rep 2007 Jun;9(3):245-53. doi: 10.1007/s11894-007-0026-z. PMID: 17511924
The effects of Down syndrome and other chromosomal abnormalities on survival and management in oesophageal atresia.
Beasley SW, Allen M, Myers N
Pediatr Surg Int 1997;12(8):550-1. doi: 10.1007/BF01371897. PMID: 9354723

Recent clinical studies

Etiology

Neonatal abdominal X-rays: indications, procedure and interpretation.
Paul SP, Holbrook F, Plowman L, James J
Nurs Child Young People 2021 Nov 4;33(6):12-18. Epub 2021 Jun 1 doi: 10.7748/ncyp.2021.e1410. PMID: 34060265
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
Gastrointestinal disorders in Down syndrome.
Bermudez BEBV, de Oliveira CM, de Lima Cat MN, Magdalena NIR, Celli A
Am J Med Genet A 2019 Aug;179(8):1426-1431. Epub 2019 Jun 10 doi: 10.1002/ajmg.a.61258. PMID: 31183986
Total intestinal aganglionosis.
Caniano DA, Ormsbee HS 3rd, Polito W, Sun CC, Barone FC, Hill JL
J Pediatr Surg 1985 Aug;20(4):456-60. doi: 10.1016/s0022-3468(85)80241-4. PMID: 4045675
The distended neonate.
Carty H, Brereton RJ
Clin Radiol 1983 Jul;34(4):367-80. doi: 10.1016/s0009-9260(83)80217-7. PMID: 6872440

Diagnosis

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
Neonatal abdominal X-rays: indications, procedure and interpretation.
Paul SP, Holbrook F, Plowman L, James J
Nurs Child Young People 2021 Nov 4;33(6):12-18. Epub 2021 Jun 1 doi: 10.7748/ncyp.2021.e1410. PMID: 34060265
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
Gastrointestinal disorders in Down syndrome.
Bermudez BEBV, de Oliveira CM, de Lima Cat MN, Magdalena NIR, Celli A
Am J Med Genet A 2019 Aug;179(8):1426-1431. Epub 2019 Jun 10 doi: 10.1002/ajmg.a.61258. PMID: 31183986
Neonatal intestinal obstruction.
Reyes HM, Meller JL, Loeff D
Clin Perinatol 1989 Mar;16(1):85-96. PMID: 2721100

Therapy

Risk factors for small bowel bacterial overgrowth and diagnostic yield of duodenal aspirates in children with intestinal failure: a retrospective review.
Gutierrez IM, Kang KH, Calvert CE, Johnson VM, Zurakowski D, Kamin D, Jaksic T, Duggan C
J Pediatr Surg 2012 Jun;47(6):1150-4. doi: 10.1016/j.jpedsurg.2012.03.019. PMID: 22703785Free PMC Article
Tetraethylammonium enhances the rectal and colonic motility in rats and human in vitro.
Li Z, Xu ZL, Liang J, Wu JC, Hu CW, Xie H, Ma WC, Jiang HC, Yang BF, Dong DL
Naunyn Schmiedebergs Arch Pharmacol 2011 Aug;384(2):147-55. Epub 2011 Jun 1 doi: 10.1007/s00210-011-0658-2. PMID: 21630039
Concurrent findings of achalasia and duodenal duplication in a Down syndrome patient.
Chen GC, Cheng DW, Enayati PJ, Chen G, Conklin JL, Soffer EE
J Formos Med Assoc 2009 Jan;108(1):78-81. doi: 10.1016/S0929-6646(09)60036-X. PMID: 19181612
The gastrointestinal tract in Down syndrome.
Levy J
Prog Clin Biol Res 1991;373:245-56. PMID: 1838188
Deficient neurogenic innervation of the myenteric plexus with normal submucous plexus involving the entire small and large bowel.
Mishalany H, Olson A, Khan F, Santos A
J Pediatr Surg 1989 Jan;24(1):83-6; discussion 86-7. doi: 10.1016/s0022-3468(89)80307-0. PMID: 2724000

Prognosis

Determinants of mortality in neonatal intestinal obstruction in Ile Ife, Nigeria.
Ademuyiwa AO, Sowande OA, Ijaduola TK, Adejuyigbe O
Afr J Paediatr Surg 2009 Jan-Jun;6(1):11-3. doi: 10.4103/0189-6725.48568. PMID: 19661658
Contemporaneous chronic rejection of multiple allografts with principal pancreatic involvement in modified multivisceral transplantation.
Takahashi H, Delacruz V, Sarwar S, Selvaggi G, Moon J, Nishida S, Weppler D, Levi D, Kato T, Tzakis A, Ruiz P
Pediatr Transplant 2007 Jun;11(4):448-52. doi: 10.1111/j.1399-3046.2007.00703.x. PMID: 17493229
Surgical conditions of the neonatal intestinal tract.
Kays DW
Clin Perinatol 1996 Jun;23(2):353-75. PMID: 8780909
Neonatal intestinal obstruction in a developing tropical country: patterns, problems, and prognosis.
Adeyemi D
J Trop Pediatr 1989 Apr;35(2):66-70. doi: 10.1093/tropej/35.2.66. PMID: 2724399
Neonatal intestinal obstruction.
Reyes HM, Meller JL, Loeff D
Clin Perinatol 1989 Mar;16(1):85-96. PMID: 2721100

Clinical prediction guides

Gastrointestinal manometric findings in a patient with total intestinal aganglionosis.
Takahashi A, Tomomasa T, Suzuki N, Kuroiwa M, Mochiki E, Tsuchida Y, Morikawa A, Kuwano H
J Pediatr Surg 2004 Jul;39(7):1104-6. doi: 10.1016/j.jpedsurg.2004.03.070. PMID: 15213908
Pathophysiological significance of neuronal nitric oxide synthase in the gastrointestinal tract.
Takahashi T
J Gastroenterol 2003;38(5):421-30. doi: 10.1007/s00535-003-1094-y. PMID: 12768383
Surgical conditions of the neonatal intestinal tract.
Kays DW
Clin Perinatol 1996 Jun;23(2):353-75. PMID: 8780909
The gastrointestinal tract in Down syndrome.
Levy J
Prog Clin Biol Res 1991;373:245-56. PMID: 1838188
Perforation of the gastrointestinal tract in infancy and childhood.
Emanuel B, Zlotnik P, Raffensperger JG
Surg Gynecol Obstet 1978 Jun;146(6):926-8. PMID: 653571

Recent systematic reviews

Sex differences in surgically correctable congenital anomalies: A systematic review.
Black AJ, Lu DY, Yefet LS, Baird R
J Pediatr Surg 2020 May;55(5):811-820. Epub 2020 Feb 12 doi: 10.1016/j.jpedsurg.2020.01.016. PMID: 32061363

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