Pediatr Gastroenterol Hepatol Nutr.  2024 Jul;27(4):197-205. 10.5223/pghn.2024.27.4.197.

Time is Gut. Approaching Intestinal Leiomyositis: Case Presentation and Literature Review

Affiliations
  • 1Department of Pediatric Surgery, Altona Children’s Hospital, Hamburg, Germany
  • 2Department of Pediatric Surgery, University Medical Center Hamburg Eppendorf, Hamburg, Germany
  • 3Department of Pediatric Gastroenterology, Altona Children’s Hospital, Hamburg, Germany
  • 4Department of Pathology, University Medical Center Hamburg Eppendorf, Hamburg, Germany
  • 5Department of Pathology, University Hospital Basel, Basel, Switzerland

Abstract

T-lymphocytic intestinal leiomyositis is a rare cause of “pediatric intestinal pseudoobstructions.” Diagnosis may be difficult and requires full-thickness bowel biopsies during laparotomy or laparoscopy with possible enterostomy. Currently, immunosuppressive therapy is the only available treatment. A delay in diagnosis and therapy may negatively affect the prognosis because of ongoing fibrotic alterations; therefore, early diagnosis and consequent treatment are crucial. This review summarizes the available information on the nosology, diagnostic steps, and treatment modalities. Here, we report the youngest case of enteric leiomyositis reported in the last two decades and analyze its management by reviewing previous cases.

Keyword

Pediatric intestinal pseudo-obstruction; Chronic intestinal pseudo-obstruction; Leiomyositis; Intestines; Functional bowel disorder; Intestinal motility disorder
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