Peutz-Jeghers syndrome associated with eventration of left hemidiaphragm in a child: A case report

Authors

  • Jiledar Rawat
  • Sudhir Singh

DOI:

https://doi.org/10.46831/jpas.v1i1.22

Keywords:

Intussusception, Eventration of the diaphragm, Intestinal polyps, Peutz-Jeghers syndrome (PJS)

Abstract

Background: Peutz–Jeghers syndrome (PJS) is a rare genetic disorder. PJS with ileo-ileal intussusception and left-sided eventration of the diaphragm is an extremely rare presentation and not been reported so far.

Case Presentation: An 8-year-old child of PJS presented with acute intestinal obstruction. Examination and investigations of the child revealed intussusception with left side elevated hemidiaphragm. Surgical correction of both conditions was done.

Conclusion: PJS with intestinal obstruction due to intussusception needs surgical correction. Sometimes these cases may present with other associated surgical conditions that too need simultaneous surgical correction.

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Author Biographies

Jiledar Rawat

Professor, Department of Pediatric Surgery, King George’s Medical University, Lucknow, UP, India

Sudhir Singh

Associate Professor, Department of Pediatric Surgery, King George’s Medical University, Lucknow, UP, India, Pin-226003

References

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Hernan I, Roig I, Martin B, Gamundi MJ, Martinez-Gimeno M, Carballo M. De novo germline mutation in the serine-threonine kinase STK11/LKB1 gene associated with Peutz-Jeghers syndrome. Clin Genet. 2004; 66:58–62.

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Duan SX, Wang GH, Zhong J, Ou WH, Fu MX, Wang FS, et al Peutz-Jeghers syndrome with intermittent upper intestinal obstruction: A case report and review of the literature. Medicine (Baltimore). 2017; 96:6538.

Heiwegen K, van Heijst AF, Daniels-Scharbatke H, van Peperstraten MC, de Blaauw I, Botden SM. Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study. Eur J Pediatr. 2020; 179:855-63.

Published

2020-07-10

How to Cite

1.
Rawat J, Singh S. Peutz-Jeghers syndrome associated with eventration of left hemidiaphragm in a child: A case report. J Pediatr Adolesc Surg [Internet]. 2020Jul.10 [cited 2020Oct.28];1(1):53-5. Available from: http://jpedas.org/ojs/index.php/jpedas/article/view/22
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