Colonic atresia and anorectal malformation in a Haitian patient: a case study of rare diseases.
| Title: | Colonic atresia and anorectal malformation in a Haitian patient: a case study of rare diseases. |
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| Authors: | Derenoncourt MH; Downstate Medical Center, State University of New York, Brooklyn, NY USA.; Baltazar G; Downstate Medical Center, State University of New York, Brooklyn, NY USA.; Lubell T; Morgan Stanley Children's Hospital of New York, Columbia University Medical Center, New York, NY USA.; Ruscica A; Morgan Stanley Children's Hospital of New York, Columbia University Medical Center, New York, NY USA.; Sahyoun C; Morgan Stanley Children's Hospital of New York, Columbia University Medical Center, New York, NY USA.; Velcek F; Downstate Medical Center, State University of New York, Brooklyn, NY USA. |
| Source: | SpringerPlus [Springerplus] 2014 Apr 26; Vol. 3, pp. 203. Date of Electronic Publication: 2014 Apr 26 (Print Publication: 2014). |
| Publication Type: | Journal Article |
| Language: | English |
| Journal Info: | Publisher: SpringerPlus Country of Publication: Switzerland NLM ID: 101597967 Publication Model: eCollection Cited Medium: Print ISSN: 2193-1801 (Print) Linking ISSN: 21931801 NLM ISO Abbreviation: Springerplus Subsets: PubMed not MEDLINE |
| Imprint Name(s): | Original Publication: Switzerland : SpringerPlus |
| Abstract: | Introduction: Colonic atresia and anorectal malformation are rare congenital anomalies individually. Few reports of the conditions combined in a single patient have been published in the literature. Neither colonic atresia, anorectal malformation or a combination of the disorders has previously been reported in the Haitian population.; Case Presentation: A 5-day-old female presented with feculent emesis, failure to pass stool since birth and an imperforate and stenotic anus. Exploratory laparotomy revealed colorectal atresia distal to a malformed cecum and a Wingspread low subtype anorectal malformation without any associated urogenital fistulae. Temporizing percutaneous ileal drainage was followed by second-stage anal perforation and dilation, ileal J-pouch and pull through.; Discussion: This is the first reported case of colonic atresia, anorectal malformation or the combination of the disorders among the Haitian population and one of only a handful of such cases reported worldwide. Although vascular accidents in utero have been implicated as the etiology of colonic atresia, simultaneous presence of anorectal malformation suggests a multifactorial cause. Investigation for multisystem abnormalities is warranted. Two-staged operative correction is considered the best treatment; however, long-term postoperative outcomes are uncertain.; Conclusion: The coexistence of colonic atresia and anorectal malformation is a very rare occurrence and presents unique clinical and operative challenges. Investigation for additional congenital abnormalities is appropriate, and although two-stage operative correction is considered the best treatment, long-term outcomes are uncertain. |
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| Contributed Indexing: | Keywords: Anorectal malformation; Colonic atresia; Intestinal atresia; Wingspread classification |
| Entry Date(s): | Date Created: 20140517 Date Completed: 20140516 Latest Revision: 20211021 |
| Update Code: | 20260130 |
| PubMed Central ID: | PMC4021029 |
| DOI: | 10.1186/2193-1801-3-203 |
| PMID: | 24834374 |
| Database: | MEDLINE |
Journal Article