Intestinal atresias are multifocal in 3-32% of atresia cases
2 distinct disease processes
Multiple intestinal atresia (MIA)
Midduodenum to middistal transverse colon may be affected
Due to vascular insult to superior mesenteric artery territory (midgut)
Atresias of types I, II, IIIa, IIIb
Hereditary MIA (HMIA), MIA with immunodeficiency (MIAI), or MIA with combined immunodeficiency (MIA-CID)
Atresias from pylorus to rectum, including long segments of occlusion
Multiple etiologies proposed
Atresias of types I & II only
Develop severe infections from immunodeficiency
IMAGING
Radiographic Findings
Fluoroscopic Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Vinit N et al: How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review. Arch Pediatr. 28(3):226-33, 2021
Jardine S et al: TTC7A: Steward of Intestinal Health. Cell Mol Gastroenterol Hepatol. 7(3):555-70, 2019
Miscia ME et al: Duodenal atresia and associated intestinal atresia: a cohort study and review of the literature. Pediatr Surg Int. 35(1):151-7, 2019
Fayard J et al: TTC7A mutation must be considered in patients with repeated intestinal atresia associated with early inflammatory bowel disease: two new case reports and a literature review. Arch Pediatr. S0929-693X(18)30112-X, 2018
Fischer RT et al: Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency. Pediatr Transplant. 18(2):190-6, 2014
Githu T et al: Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation. Pediatr Radiol. 44(3):349-54, 2014
Samuels ME et al: Exome sequencing identifies mutations in the gene TTC7A in French-Canadian cases with hereditary multiple intestinal atresia. J Med Genet. 50(5):324-9, 2013
Ali YA et al: Hereditary multiple intestinal atresia (HMIA) with severe combined immunodeficiency (SCID): a case report of two siblings and review of the literature on MIA, HMIA and HMIA with immunodeficiency over the last 50 years. BMJ Case Rep. 2011, 2011
Cole C et al: Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg. 45(4):E21-4, 2010
Baglaj M et al: Multiple atresia of the small intestine: a 20-year review. Eur J Pediatr Surg. 18(1):13-8, 2008
Gilroy RK et al: Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. Blood. 103(3):1171-4, 2004
McHugh K et al: Multiple gastrointestinal atresias: sonography of associated biliary abnormalities. Pediatr Radiol. 21(5):355-7, 1991
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Definitions
Intestinal atresias are multifocal in 3-32% of atresia cases
2 distinct disease processes
Multiple intestinal atresia (MIA)
Midduodenum to middistal transverse colon may be affected
Due to vascular insult to superior mesenteric artery territory (midgut)
Atresias of types I, II, IIIa, IIIb
Hereditary MIA (HMIA), MIA with immunodeficiency (MIAI), or MIA with combined immunodeficiency (MIA-CID)
Atresias from pylorus to rectum, including long segments of occlusion
Multiple etiologies proposed
Atresias of types I & II only
Develop severe infections from immunodeficiency
IMAGING
Radiographic Findings
Fluoroscopic Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Vinit N et al: How can we improve perinatal care in isolated multiple intestinal atresia? A retrospective study with a 30-year literature review. Arch Pediatr. 28(3):226-33, 2021
Jardine S et al: TTC7A: Steward of Intestinal Health. Cell Mol Gastroenterol Hepatol. 7(3):555-70, 2019
Miscia ME et al: Duodenal atresia and associated intestinal atresia: a cohort study and review of the literature. Pediatr Surg Int. 35(1):151-7, 2019
Fayard J et al: TTC7A mutation must be considered in patients with repeated intestinal atresia associated with early inflammatory bowel disease: two new case reports and a literature review. Arch Pediatr. S0929-693X(18)30112-X, 2018
Fischer RT et al: Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency. Pediatr Transplant. 18(2):190-6, 2014
Githu T et al: Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation. Pediatr Radiol. 44(3):349-54, 2014
Samuels ME et al: Exome sequencing identifies mutations in the gene TTC7A in French-Canadian cases with hereditary multiple intestinal atresia. J Med Genet. 50(5):324-9, 2013
Ali YA et al: Hereditary multiple intestinal atresia (HMIA) with severe combined immunodeficiency (SCID): a case report of two siblings and review of the literature on MIA, HMIA and HMIA with immunodeficiency over the last 50 years. BMJ Case Rep. 2011, 2011
Cole C et al: Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg. 45(4):E21-4, 2010
Baglaj M et al: Multiple atresia of the small intestine: a 20-year review. Eur J Pediatr Surg. 18(1):13-8, 2008
Gilroy RK et al: Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. Blood. 103(3):1171-4, 2004
McHugh K et al: Multiple gastrointestinal atresias: sonography of associated biliary abnormalities. Pediatr Radiol. 21(5):355-7, 1991
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