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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
- Midduodenum to middistal transverse colon may be affected
- 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
- Atresias from pylorus to rectum, including long segments of occlusion
- Multiple intestinal atresia (MIA)
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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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
- Midduodenum to middistal transverse colon may be affected
- 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
- Atresias from pylorus to rectum, including long segments of occlusion
- Multiple intestinal atresia (MIA)
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
