Diagnostic Criteria of Pediatric Intestinal Myopathies

The authors aim to identify criteria for the diagnosis of Intestinal Visceral Myopathy (IVM), results were compared with ultrastructural studies (US). Six IVM patients and 7 paediatric control cases (without gastrointestinal diseases) were studied. One case was a typical Megacystis-Mycrocolon-Intestinal Hypoperistalsis Syndrome (MMIHS). The diagnostic path included: Rectal Suction Biopsy, One-Trocar Transumbilical Laparoscopic intestinal full-thickness Biopsy technique (OTTLB). 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 circular/longitudinal thickness was evaluated in all samples, in cases this ratio presented a mean value = 2.91; in controls a mean value = 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.