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Megaduodenum

MedGen UID:
75604
Concept ID:
C0266177
Congenital Abnormality
SNOMED CT: Megaduodenum (235699006)
 
HPO: HP:0030996

Definition

Dilation and elongation of the duodenum with hypertrophy of all layers of the duodenum. [from HPO]

Conditions with this feature

Mungan syndrome
MedGen UID:
369554
Concept ID:
C1969653
Disease or Syndrome
Mungan syndrome (MGS) is characterized by chronic intestinal pseudoobstruction (CIPO), megaduodenum, long-segment Barrett esophagus, and cardiac abnormalities of variable severity (summary by Bonora et al., 2015).
Visceral myopathy 1
MedGen UID:
1785391
Concept ID:
C5542197
Disease or Syndrome
ACTG2 visceral myopathy is a disorder of smooth muscle dysfunction of the bladder and gastrointestinal system with phenotypic spectrum that ranges from mild to severe. Bladder involvement can range from neonatal megacystis and megaureter (with its most extreme form of prune belly syndrome) at the more severe end, to recurrent urinary tract infections and bladder dysfunction at the milder end. Intestinal involvement can range from malrotation, neonatal manifestations of microcolon, megacystis microcolon intestinal hypoperistalsis syndrome, and chronic intestinal pseudoobstruction (CIPO) in neonates at the more severe end to intermittent abdominal distention and functional intestinal obstruction at the milder end. Affected infants (with or without evidence of intestinal malrotation) often present with feeding intolerance and findings of non-mechanical bowel obstruction that persist after successful surgical correction of malrotation. Individuals who develop manifestations of CIPO in later childhood or adulthood often experience episodic waxing and waning of bowel motility. They may undergo frequent abdominal surgeries (perhaps related to malrotation or adhesions causing mechanical obstruction) resulting in resection of dilated segments of bowel, often becoming dependent on total parenteral nutrition (TPN).
Visceral myopathy 2
MedGen UID:
1783630
Concept ID:
C5543466
Disease or Syndrome
Visceral myopathy-2 (VSCM2) is characterized by gastrointestinal symptoms resulting from intestinal dysmotility and paresis, including abdominal distention, pain, nausea, and vomiting. Some patients exhibit predominantly esophageal symptoms, with hiatal hernia and severe reflux resulting in esophagitis and stricture, whereas others experience chronic intestinal pseudoobstruction. Bladder involvement resulting in megacystis and megaureter has also been observed and may be evident at birth (Dong et al., 2019; Gilbert et al. (2020)).

Professional guidelines

PubMed

Touloukian RJ
World J Surg 1993 May-Jun;17(3):310-7. doi: 10.1007/BF01658697. PMID: 8337876
Grosfeld JL, Rescorla FJ
World J Surg 1993 May-Jun;17(3):301-9. doi: 10.1007/BF01658696. PMID: 8337875
Spigland N, Yazbeck S
J Pediatr Surg 1990 Nov;25(11):1127-30. doi: 10.1016/0022-3468(90)90746-v. PMID: 2273425

Recent clinical studies

Etiology

Taghavi K, Sharpe C, Stringer MD, Zuccollo J, Marlow J
Aust N Z J Obstet Gynaecol 2017 Dec;57(6):636-642. Epub 2017 Jul 12 doi: 10.1111/ajo.12655. PMID: 28699212
Taghavi K, Sharpe C, Stringer MD
J Pediatr Urol 2017 Feb;13(1):7-15. Epub 2016 Oct 8 doi: 10.1016/j.jpurol.2016.09.003. PMID: 27889224
Papis D, Marangoni G
Acta Chir Belg 2016 Oct;116(5):305-308. Epub 2016 Jul 5 doi: 10.1080/00015458.2016.1147263. PMID: 27410460
Zhang XW, Abudoureyimu A, Zhang TC, Zhao JR, Fu LB, Lin F, Qiu XH, Chen YJ
J Pediatr Surg 2012 May;47(5):1038-42. doi: 10.1016/j.jpedsurg.2012.02.008. PMID: 22595598
Basilisco G
Am J Gastroenterol 1997 Jan;92(1):150-3. PMID: 8995957

Diagnosis

Chen CP
Taiwan J Obstet Gynecol 2024 Jan;63(1):17-18. doi: 10.1016/j.tjog.2023.11.006. PMID: 38216262
Zeng K, Li D, Zhang Y, Cao C, Bai R, Yang Z, Chen L
BMC Pregnancy Childbirth 2021 May 11;21(1):370. doi: 10.1186/s12884-021-03843-0. PMID: 33975544Free PMC Article
Taghavi K, Sharpe C, Stringer MD, Zuccollo J, Marlow J
Aust N Z J Obstet Gynaecol 2017 Dec;57(6):636-642. Epub 2017 Jul 12 doi: 10.1111/ajo.12655. PMID: 28699212
Papis D, Marangoni G
Acta Chir Belg 2016 Oct;116(5):305-308. Epub 2016 Jul 5 doi: 10.1080/00015458.2016.1147263. PMID: 27410460
Basilisco G
Am J Gastroenterol 1997 Jan;92(1):150-3. PMID: 8995957

Therapy

Milunsky A, Baldwin C, Zhang X, Primack D, Curnow A, Milunsky J
J Pediatr Gastroenterol Nutr 2017 Oct;65(4):384-387. doi: 10.1097/MPG.0000000000001608. PMID: 28422808Free PMC Article
Zaraa I, Belghith I, El Euch D, Karoui S, Mokni M, Fillali A, Ben Osman A
Nutr Clin Pract 2013 Apr;28(2):218-22. Epub 2012 Dec 11 doi: 10.1177/0884533612464783. PMID: 23232750
Radaelli F, Paggi S, Terreni N, Toldi A, Terruzzi V
Gastrointest Endosc 2010 Jun;71(7):1326-7. Epub 2010 Mar 21 doi: 10.1016/j.gie.2009.10.034. PMID: 20304403
Touloukian RJ
World J Surg 1993 May-Jun;17(3):310-7. doi: 10.1007/BF01658697. PMID: 8337876
Hines C Jr, Davis WD Jr
Am J Med 1973 Apr;54(4):539-43. doi: 10.1016/0002-9343(73)90051-x. PMID: 4572175

Prognosis

Taghavi K, Sharpe C, Stringer MD, Zuccollo J, Marlow J
Aust N Z J Obstet Gynaecol 2017 Dec;57(6):636-642. Epub 2017 Jul 12 doi: 10.1111/ajo.12655. PMID: 28699212
Papis D, Marangoni G
Acta Chir Belg 2016 Oct;116(5):305-308. Epub 2016 Jul 5 doi: 10.1080/00015458.2016.1147263. PMID: 27410460
Zhang XW, Abudoureyimu A, Zhang TC, Zhao JR, Fu LB, Lin F, Qiu XH, Chen YJ
J Pediatr Surg 2012 May;47(5):1038-42. doi: 10.1016/j.jpedsurg.2012.02.008. PMID: 22595598
Elli L, Falconieri G, Bardella MT, Caldato M, Pizzolitto S, Bonura A, Zilli M, Maieron R
Acta Gastroenterol Belg 2010 Apr-Jun;73(2):287-91. PMID: 20690573
Basilisco G
Am J Gastroenterol 1997 Jan;92(1):150-3. PMID: 8995957

Clinical prediction guides

Zeng K, Li D, Zhang Y, Cao C, Bai R, Yang Z, Chen L
BMC Pregnancy Childbirth 2021 May 11;21(1):370. doi: 10.1186/s12884-021-03843-0. PMID: 33975544Free PMC Article
Tschannen R, Gobet R, Wisser J
Ultraschall Med 2018 Aug;39(4):407-412. Epub 2017 Nov 21 doi: 10.1055/s-0043-105263. PMID: 29161752
Taghavi K, Sharpe C, Stringer MD, Zuccollo J, Marlow J
Aust N Z J Obstet Gynaecol 2017 Dec;57(6):636-642. Epub 2017 Jul 12 doi: 10.1111/ajo.12655. PMID: 28699212
Weston S, Thumshirn M, Wiste J, Camilleri M
Am J Gastroenterol 1998 Jul;93(7):1085-9. doi: 10.1111/j.1572-0241.1998.00334.x. PMID: 9672335
Basilisco G
Am J Gastroenterol 1997 Jan;92(1):150-3. PMID: 8995957

Recent systematic reviews

Chen L, Guan J, Gu H, Zhang M
Eur J Obstet Gynecol Reprod Biol 2019 Feb;233:120-126. Epub 2018 Dec 13 doi: 10.1016/j.ejogrb.2018.12.007. PMID: 30594021
Taghavi K, Sharpe C, Stringer MD
J Pediatr Urol 2017 Feb;13(1):7-15. Epub 2016 Oct 8 doi: 10.1016/j.jpurol.2016.09.003. PMID: 27889224

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