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Multiple Intestinal Atresias
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Intestinal atresias multifocal in 6-32% of atresia cases
            • 2 distinct disease processes
              • Multiple intestinal atresia (MIA)
                • Mid-duodenum to mid-distal transverse colon may be affected
                  • Due to vascular insult to superior mesenteric artery territory (midgut)
                • Atresias of types I, II, IIIa, IIIb
              • Hereditary multiple intestinal atresia (HMIA) or multiple intestinal atresia with immunodeficiency (MIAI)
                • 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

                                    1. Fischer RT et al: Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency. Pediatr Transplant. 18(2):190-6, 2014
                                    2. Githu T et al: Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation. Pediatr Radiol. 44(3):349-54, 2014
                                    3. 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
                                    4. 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
                                    5. Cole C et al: Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg. 45(4):E21-4, 2010
                                    6. Baglaj M et al: Multiple atresia of the small intestine: a 20-year review. Eur J Pediatr Surg. 18(1):13-8, 2008
                                    7. Gilroy RK et al: Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. Blood. 103(3):1171-4, 2004
                                    8. 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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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Definitions

                                              • Intestinal atresias multifocal in 6-32% of atresia cases
                                              • 2 distinct disease processes
                                                • Multiple intestinal atresia (MIA)
                                                  • Mid-duodenum to mid-distal transverse colon may be affected
                                                    • Due to vascular insult to superior mesenteric artery territory (midgut)
                                                  • Atresias of types I, II, IIIa, IIIb
                                                • Hereditary multiple intestinal atresia (HMIA) or multiple intestinal atresia with immunodeficiency (MIAI)
                                                  • 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

                                                                      1. Fischer RT et al: Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency. Pediatr Transplant. 18(2):190-6, 2014
                                                                      2. Githu T et al: Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation. Pediatr Radiol. 44(3):349-54, 2014
                                                                      3. 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
                                                                      4. 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
                                                                      5. Cole C et al: Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg. 45(4):E21-4, 2010
                                                                      6. Baglaj M et al: Multiple atresia of the small intestine: a 20-year review. Eur J Pediatr Surg. 18(1):13-8, 2008
                                                                      7. Gilroy RK et al: Donor immune reconstitution after liver-small bowel transplantation for multiple intestinal atresia with immunodeficiency. Blood. 103(3):1171-4, 2004
                                                                      8. McHugh K et al: Multiple gastrointestinal atresias: sonography of associated biliary abnormalities. Pediatr Radiol. 21(5):355-7, 1991