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Pyloric Atresia
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Pyloric atresia (PA)
              • Epidermolysis bullosa-pyloric atresia (EB-PA)
              • Hereditary multiple intestinal atresia or multiple intestinal atresia with immunodeficiency (HMIA/MIAI)
            • Synonyms

              • EB-PA: Carmi syndrome
            • Definitions

              • Uncommon congenital gastric outlet obstruction

            IMAGING

            • Radiographic Findings

              • Ultrasonographic Findings

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Staging, Grading, & Classification

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Image Interpretation Pearls

                                Selected References

                                1. Hicks TD et al: Carmi syndrome in a preterm neonate: a multidisciplinary approach and ethical challenge. Case Rep Pediatr. 2018:4548194, 2018
                                2. Kansra M et al: Congenital pyloric atresia - nine new cases: single-center experience of the long-term follow-up and the lessons learnt over a decade. J Pediatr Surg. 53(11):2112-6, 2018
                                3. Walker GD et al: Epidermolysis bullosa with pyloric atresia and significant urologic involvement. Pediatr Dermatol. 34(1):e61-4, 2017
                                4. Kajal P et al: Congenital pyloric atresia: early and delayed presentations-a single centre experience of a rare anomaly. Indian J Gastroenterol. 35(3):232-5, 2016
                                5. Al-Salem AH et al: Congenital pyloric atresia, presentation, management, and outcome: a report of 20 cases. J Pediatr Surg. 49(7):1078-82, 2014
                                6. Fischer RT et al: Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency. Pediatr Transplant. 18(2):190-6, 2014
                                7. Merrow AC et al: Pyloric atresia with epidermolysis bullosa: fetal MRI diagnosis with postnatal correlation. Pediatr Radiol. 43(12):1656-61, 2013
                                8. Al-Salem AH: Congenital pyloric atresia and associated anomalies. Pediatr Surg Int. 23(6):559-63, 2007
                                9. Tomá P et al: Pyloric atresia: report of two cases (one associated with epidermolysis bullosa and one associated with multiple intestinal atresias). Pediatr Radiol. 32(8):552-5, 2002
                                10. Pfendner EG et al: Epidermolysis Bullosa with Pyloric Atresia. 2008 Feb 22 [updated 2017 Sep 7]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Pathology

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Pyloric atresia (PA)
                                            • Epidermolysis bullosa-pyloric atresia (EB-PA)
                                            • Hereditary multiple intestinal atresia or multiple intestinal atresia with immunodeficiency (HMIA/MIAI)
                                          • Synonyms

                                            • EB-PA: Carmi syndrome
                                          • Definitions

                                            • Uncommon congenital gastric outlet obstruction

                                          IMAGING

                                          • Radiographic Findings

                                            • Ultrasonographic Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Staging, Grading, & Classification

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Image Interpretation Pearls

                                                              Selected References

                                                              1. Hicks TD et al: Carmi syndrome in a preterm neonate: a multidisciplinary approach and ethical challenge. Case Rep Pediatr. 2018:4548194, 2018
                                                              2. Kansra M et al: Congenital pyloric atresia - nine new cases: single-center experience of the long-term follow-up and the lessons learnt over a decade. J Pediatr Surg. 53(11):2112-6, 2018
                                                              3. Walker GD et al: Epidermolysis bullosa with pyloric atresia and significant urologic involvement. Pediatr Dermatol. 34(1):e61-4, 2017
                                                              4. Kajal P et al: Congenital pyloric atresia: early and delayed presentations-a single centre experience of a rare anomaly. Indian J Gastroenterol. 35(3):232-5, 2016
                                                              5. Al-Salem AH et al: Congenital pyloric atresia, presentation, management, and outcome: a report of 20 cases. J Pediatr Surg. 49(7):1078-82, 2014
                                                              6. Fischer RT et al: Intestinal transplantation in children with multiple intestinal atresias and immunodeficiency. Pediatr Transplant. 18(2):190-6, 2014
                                                              7. Merrow AC et al: Pyloric atresia with epidermolysis bullosa: fetal MRI diagnosis with postnatal correlation. Pediatr Radiol. 43(12):1656-61, 2013
                                                              8. Al-Salem AH: Congenital pyloric atresia and associated anomalies. Pediatr Surg Int. 23(6):559-63, 2007
                                                              9. Tomá P et al: Pyloric atresia: report of two cases (one associated with epidermolysis bullosa and one associated with multiple intestinal atresias). Pediatr Radiol. 32(8):552-5, 2002
                                                              10. Pfendner EG et al: Epidermolysis Bullosa with Pyloric Atresia. 2008 Feb 22 [updated 2017 Sep 7]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.