Diagnostic Criteria of Pediatric Intestinal Myopathies

Laura Lombardi, Elisabeth Bruder, Luca Pio, Paolo Nozza, Elena Thai, Margherita Lerone, Carmine Del Rossi, Girolamo Mattioli, Enrico Maria Silini, Irene Paraboschi, Giuseppe Martucciello

Research output: Contribution to journalArticlepeer-review

Abstract

The authors aim to identify criteria for the diagnosis of intestinal visceral myopathy (IVM); results were compared with ultrastructural studies. Six IVM patients and 7 pediatric control cases (without gastrointestinal diseases) were studied. One case was a typical megacystis-microcolon-intestinal hypoperistalsis syndrome. The diagnostic path included: rectal suction biopsy, one-trocar transumbilical laparoscopic intestinal full-thickness biopsy technique. Pathological analysis included anti-alpha smooth muscle actin staining, and US study of intestinal biopsies. IVM histological examination demonstrated thinning of longitudinal muscle layer. The ratio of circular/longitudinal thickness was evaluated in all samples; in cases, this ratio presented as a mean value of 2.91, and in controls, a mean value of 1.472 (P = 0.0002). Ultrastructural diagnosis revealed variable myofibrils density in smooth muscle cells, irregularity of sarcolemma membranes, interstitial fibrosis, and myofiber disarray. The authors concluded that in IVM, circular/longitudinal thickness ratio and alpha smooth muscle actin staining can be used as significant tools to address the diagnosis.

Original languageEnglish
Pages (from-to)383-386
Number of pages4
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume66
Issue number3
DOIs
Publication statusPublished - Mar 1 2018

Keywords

  • Hirschsprung
  • laparoscopy
  • megacolon
  • microcolon
  • myopathy
  • pseudo-obstruction

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology

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