Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series

Introduction: Segmental absence of intestinal musculature (SAIM) is a pathological diagnosis, denoting focal absence of the muscularis propria in the presence of intact surrounding structures. This condition often presents as spontaneous intestinal perforation (SIP) and can be difficult to distingui...

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Main Authors: Carolina Pinzon-Guzman, Alexander Kevorkov, Karl Grenier, Maeve O'Neill Trudeau, Pramod Puligandla, Etienne St-Louis
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Journal of Pediatric Surgery Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S221357662400143X
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author Carolina Pinzon-Guzman
Alexander Kevorkov
Karl Grenier
Maeve O'Neill Trudeau
Pramod Puligandla
Etienne St-Louis
author_facet Carolina Pinzon-Guzman
Alexander Kevorkov
Karl Grenier
Maeve O'Neill Trudeau
Pramod Puligandla
Etienne St-Louis
author_sort Carolina Pinzon-Guzman
collection DOAJ
description Introduction: Segmental absence of intestinal musculature (SAIM) is a pathological diagnosis, denoting focal absence of the muscularis propria in the presence of intact surrounding structures. This condition often presents as spontaneous intestinal perforation (SIP) and can be difficult to distinguish clinically from necrotizing enterocolitis (NEC). Case presentations: We present 5 cases of premature babies with gestational age ranging from 24 + 3 weeks up to 32 + 1 weeks, who all presented with intestinal perforation before the 15th day of life. Three patients presented with a distended abdomen and discoloration and were subsequently found to have pneumoperitoneum on abdominal x-ray (AXR). The 2 other patients presented with clinical deterioration but without signs of intestinal perforation and were subsequently found to have pneumoperitoneum on AXR. All underwent bowel resection of perforated bowel with either ileostomy with mucous fistula or primary end-to-end anastomosis. Two patients required repeat exploratory laparotomies within a week of their initial surgery due to pneumoperitoneum in subsequent AXR caused by new perforations in other parts of the small bowel. One patient sustained an iatrogenic liver injury intra-operatively and passed away 1 day post-operatively. All patients were found to have SAIM on histopathological examination. Conclusion: SAIM is a pathological diagnosis that can manifest as SIP and may denote a risk for recurrent peritonitis in premature infants who underwent bowel resection for perforated viscus without a clear etiology.
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spelling doaj-art-6b4a2e4a62364be3ad7b1b24f4f8f8682024-11-11T04:25:30ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662024-12-01111102915Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case seriesCarolina Pinzon-Guzman0Alexander Kevorkov1Karl Grenier2Maeve O'Neill Trudeau3Pramod Puligandla4Etienne St-Louis5McGill University Health Centre, Montreal Children's Hospital, Harvey E. Beardmore Division of Pediatric Surgery, Montreal, QC, CanadaMcGill University, Faculty of Medicine and Health Sciences, Montreal, QC, CanadaMcGill University Health Centre, Montreal Children's Hospital, Department of Pathology, Montreal, QC, CanadaMcGill University Health Centre, Montreal Children's Hospital, Harvey E. Beardmore Division of Pediatric Surgery, Montreal, QC, CanadaMcGill University Health Centre, Montreal Children's Hospital, Harvey E. Beardmore Division of Pediatric Surgery, Montreal, QC, CanadaMcGill University Health Centre, Montreal Children's Hospital, Harvey E. Beardmore Division of Pediatric Surgery, Montreal, QC, Canada; Corresponding author.Introduction: Segmental absence of intestinal musculature (SAIM) is a pathological diagnosis, denoting focal absence of the muscularis propria in the presence of intact surrounding structures. This condition often presents as spontaneous intestinal perforation (SIP) and can be difficult to distinguish clinically from necrotizing enterocolitis (NEC). Case presentations: We present 5 cases of premature babies with gestational age ranging from 24 + 3 weeks up to 32 + 1 weeks, who all presented with intestinal perforation before the 15th day of life. Three patients presented with a distended abdomen and discoloration and were subsequently found to have pneumoperitoneum on abdominal x-ray (AXR). The 2 other patients presented with clinical deterioration but without signs of intestinal perforation and were subsequently found to have pneumoperitoneum on AXR. All underwent bowel resection of perforated bowel with either ileostomy with mucous fistula or primary end-to-end anastomosis. Two patients required repeat exploratory laparotomies within a week of their initial surgery due to pneumoperitoneum in subsequent AXR caused by new perforations in other parts of the small bowel. One patient sustained an iatrogenic liver injury intra-operatively and passed away 1 day post-operatively. All patients were found to have SAIM on histopathological examination. Conclusion: SAIM is a pathological diagnosis that can manifest as SIP and may denote a risk for recurrent peritonitis in premature infants who underwent bowel resection for perforated viscus without a clear etiology.http://www.sciencedirect.com/science/article/pii/S221357662400143XSegmental absence of intestinal musculatureIntestinal perforationPremature neonatesCase series
spellingShingle Carolina Pinzon-Guzman
Alexander Kevorkov
Karl Grenier
Maeve O'Neill Trudeau
Pramod Puligandla
Etienne St-Louis
Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series
Journal of Pediatric Surgery Case Reports
Segmental absence of intestinal musculature
Intestinal perforation
Premature neonates
Case series
title Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series
title_full Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series
title_fullStr Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series
title_full_unstemmed Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series
title_short Segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus: A case series
title_sort segmental absence of intestinal musculature increasingly recognized in premature infants with perforated viscus a case series
topic Segmental absence of intestinal musculature
Intestinal perforation
Premature neonates
Case series
url http://www.sciencedirect.com/science/article/pii/S221357662400143X
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