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An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series

INTRODUCTION AND IMPORTANCE: The association of intussusception with intestinal malrotation is a rare phenomenon named Waugh syndrome by Brereton et. It is a rare disease entity with less than 100 cases described so far. A cluster presentation is something unusual for rare diseases. CASE PRESENTATIO...

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Autores principales: Elkeir, Ibrahim S., Balla, Walaa, Jagurru, Helen, Fatih, Moh, Mohammed, Suliman Gabir Abdalla, Abdulkarim, Mohamed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9194577/
https://www.ncbi.nlm.nih.gov/pubmed/35700619
http://dx.doi.org/10.1016/j.ijscr.2022.107269
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author Elkeir, Ibrahim S.
Balla, Walaa
Jagurru, Helen
Fatih, Moh
Mohammed, Suliman Gabir Abdalla
Abdulkarim, Mohamed
author_facet Elkeir, Ibrahim S.
Balla, Walaa
Jagurru, Helen
Fatih, Moh
Mohammed, Suliman Gabir Abdalla
Abdulkarim, Mohamed
author_sort Elkeir, Ibrahim S.
collection PubMed
description INTRODUCTION AND IMPORTANCE: The association of intussusception with intestinal malrotation is a rare phenomenon named Waugh syndrome by Brereton et. It is a rare disease entity with less than 100 cases described so far. A cluster presentation is something unusual for rare diseases. CASE PRESENTATION: All four patients have presented with features suggestive of bowel obstruction as all of them have had colicky abdominal pain. Three patients have vomiting as an additional complaint, so as the diarrhea. Fever has also been reported in one patient. Patients have presented in 6 months period, non-consecutively. Abdominal pain, diarrhea and vomiting are alarming in the pediatric population. When each case presented, an immediate resuscitation was performed including IV lines, nasogastric suctioning, electrolytes correction, and blood and FFP transfusion as necessary. Abdominal ultrasonography was performed revealing target sign. After optimization for surgery, manual reduction of the intussusception was done followed by a Ladd's procedure for the malrotation was performed for all 4 patients with excellent outcomes. CLINICAL DISCUSSION: With the surgery, after case optimization, remains the standard of care; the presence of cluster presentation for Waugh syndrome could suggest that some environmental factors might be implicated and further observations can be carried out to look more into such phenomenon. CONCLUSION: Waugh syndrome, a combined presentation of intestinal malrotation and intussusception, is a rare syndrome that can present in children. Optimization for surgery followed by a 2-step surgical approach is the usual treatment with excellent outcomes described.
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spelling pubmed-91945772022-06-15 An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series Elkeir, Ibrahim S. Balla, Walaa Jagurru, Helen Fatih, Moh Mohammed, Suliman Gabir Abdalla Abdulkarim, Mohamed Int J Surg Case Rep Case Report INTRODUCTION AND IMPORTANCE: The association of intussusception with intestinal malrotation is a rare phenomenon named Waugh syndrome by Brereton et. It is a rare disease entity with less than 100 cases described so far. A cluster presentation is something unusual for rare diseases. CASE PRESENTATION: All four patients have presented with features suggestive of bowel obstruction as all of them have had colicky abdominal pain. Three patients have vomiting as an additional complaint, so as the diarrhea. Fever has also been reported in one patient. Patients have presented in 6 months period, non-consecutively. Abdominal pain, diarrhea and vomiting are alarming in the pediatric population. When each case presented, an immediate resuscitation was performed including IV lines, nasogastric suctioning, electrolytes correction, and blood and FFP transfusion as necessary. Abdominal ultrasonography was performed revealing target sign. After optimization for surgery, manual reduction of the intussusception was done followed by a Ladd's procedure for the malrotation was performed for all 4 patients with excellent outcomes. CLINICAL DISCUSSION: With the surgery, after case optimization, remains the standard of care; the presence of cluster presentation for Waugh syndrome could suggest that some environmental factors might be implicated and further observations can be carried out to look more into such phenomenon. CONCLUSION: Waugh syndrome, a combined presentation of intestinal malrotation and intussusception, is a rare syndrome that can present in children. Optimization for surgery followed by a 2-step surgical approach is the usual treatment with excellent outcomes described. Elsevier 2022-05-31 /pmc/articles/PMC9194577/ /pubmed/35700619 http://dx.doi.org/10.1016/j.ijscr.2022.107269 Text en © 2022 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Elkeir, Ibrahim S.
Balla, Walaa
Jagurru, Helen
Fatih, Moh
Mohammed, Suliman Gabir Abdalla
Abdulkarim, Mohamed
An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series
title An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series
title_full An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series
title_fullStr An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series
title_full_unstemmed An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series
title_short An unusual cluster of Waugh syndrome as a cause of intestinal obstruction in children - A case series
title_sort unusual cluster of waugh syndrome as a cause of intestinal obstruction in children - a case series
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9194577/
https://www.ncbi.nlm.nih.gov/pubmed/35700619
http://dx.doi.org/10.1016/j.ijscr.2022.107269
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