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Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria.
AJP Rep 2019; 9(3):e218-e225AR

Abstract

Background and Aim Congenital segmental intestinal dilatation (CSID) is a neonatal condition with unclear etiology and pathogenesis. Typically, the newborn with CSID presents with a limited (circumscribed) bowel dilatation, an abrupt transition between normal and dilated segments, neither intrinsic nor extrinsic perilesional obstruction, and no aganglionosis or neuronal intestinal dysplasia. We aimed to review this disease and the long-term follow-up at the Children's Hospital of the Medical University of Innsbruck, Tyrol, Austria. Study Design Retrospective 25-year review of medical charts, electronic files, and histopathology of neonates with CSID. Results We identified four infants (three girls and one boy) with CSID. The affected areas included duodenum, ileum, ascending colon, and sigmoid colon. Noteworthy, all patients presented with a cardiovascular defect, of which two required multiple cardiac surgical interventions. Three out of the four patients recovered completely. To date, the three infants are alive. Conclusion This is the first report of patients with CSID and cardiovascular defects. The clinical and surgical intervention for CSID also requires a thorough cardiologic evaluation in these patients. CSID remains an enigmatic entity pointing to the need for joint forces in identifying common loci for genetic investigations.

Authors+Show Affiliations

Department of Orthopedics, Tianyou Hospital, Wuhan University of Science and Technology, Wuhan, Hubei, P.R. China. Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, Alberta, Canada. Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.Institute of Pathology, University Hospital of Essen, University Duisburg-Essen, Essen, Germany. Institute of Pathology, Medical University of Innsbruck, Innsbruck, Austria.Department of Pediatric Surgery, Medical University of Innsbruck, Innsbruck, Austria.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

31304051

Citation

Sergi, Consolato, et al. "Congenital Segmental Intestinal Dilatation: a 25-Year Review With Long-Term Follow-up at the Medical University of Innsbruck, Austria." AJP Reports, vol. 9, no. 3, 2019, pp. e218-e225.
Sergi C, Hager T, Hager J. Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria. AJP Rep. 2019;9(3):e218-e225.
Sergi, C., Hager, T., & Hager, J. (2019). Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria. AJP Reports, 9(3), pp. e218-e225. doi:10.1055/s-0039-1693164.
Sergi C, Hager T, Hager J. Congenital Segmental Intestinal Dilatation: a 25-Year Review With Long-Term Follow-up at the Medical University of Innsbruck, Austria. AJP Rep. 2019;9(3):e218-e225. PubMed PMID: 31304051.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria. AU - Sergi,Consolato, AU - Hager,Thomas, AU - Hager,Josef, Y1 - 2019/07/11/ PY - 2017/12/29/received PY - 2019/04/26/accepted PY - 2019/7/16/entrez PY - 2019/7/16/pubmed PY - 2019/7/16/medline KW - Hirschsprung's disease KW - aganglionosis KW - dilatation KW - heart defect KW - intestine KW - obstruction SP - e218 EP - e225 JF - AJP reports JO - AJP Rep VL - 9 IS - 3 N2 - Background and Aim Congenital segmental intestinal dilatation (CSID) is a neonatal condition with unclear etiology and pathogenesis. Typically, the newborn with CSID presents with a limited (circumscribed) bowel dilatation, an abrupt transition between normal and dilated segments, neither intrinsic nor extrinsic perilesional obstruction, and no aganglionosis or neuronal intestinal dysplasia. We aimed to review this disease and the long-term follow-up at the Children's Hospital of the Medical University of Innsbruck, Tyrol, Austria. Study Design Retrospective 25-year review of medical charts, electronic files, and histopathology of neonates with CSID. Results We identified four infants (three girls and one boy) with CSID. The affected areas included duodenum, ileum, ascending colon, and sigmoid colon. Noteworthy, all patients presented with a cardiovascular defect, of which two required multiple cardiac surgical interventions. Three out of the four patients recovered completely. To date, the three infants are alive. Conclusion This is the first report of patients with CSID and cardiovascular defects. The clinical and surgical intervention for CSID also requires a thorough cardiologic evaluation in these patients. CSID remains an enigmatic entity pointing to the need for joint forces in identifying common loci for genetic investigations. SN - 2157-6998 UR - https://www.unboundmedicine.com/medline/citation/31304051/Congenital_Segmental_Intestinal_Dilatation:_A_25-Year_Review_with_Long-Term_Follow-up_at_the_Medical_University_of_Innsbruck,_Austria L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-0039-1693164 DB - PRIME DP - Unbound Medicine ER -