Wedel T, Böttner M, Krammer H J
Anatomisches Institut, Christian-Albrechts-Universität zu Kiel, Otto-Hahn-Platz 8, 24118 Kiel, Germany.
Pathologe. 2007 Mar;28(2):143-8. doi: 10.1007/s00292-007-0900-3.
Intestinal innervation disorders are part of the broad etiological spectrum of chronic constipation and need to be specifically addressed in differential diagnosis. The enteric nervous system constitutes the largest peripheral nervous system of its own ("brain in the gut"), and is involved in the mediation of intestinal motility. Morphologically different nerve cell types aggregate into intramural plexus layers and release a multitude of neurotransmitters. Malformations or lesions of the enteric nervous system may lead to a severely prolonged intestinal transit time resulting in chronic constipation resistant to conservative treatment. In contrast to the early manifestation of aganglionosis, non-aganglionic or acquired alterations to the intramural nerve plexus often remain unrecognised up to adulthood. Histopathological diagnosis is carried out by enzyme or immunohistochemical staining, either on sections or whole mount preparations, allowing an optimal visualization of the nerve plexus architecture. To diagnose hypoganglionosis, enteric ganglionitis or alterations in interstitial cells of Cajal, full-thickness biopsies are required. Interstitial cells of Cajal contribute significantly to the mediation of intestinal motility by generating "slow wave" activity. In adult patients with slow-transit constipation and megacolon, the intramuscular networks of the interstitial cells of Cajal show a significantly reduced density.
肠道神经支配紊乱是慢性便秘广泛病因谱的一部分，在鉴别诊断中需要特别关注。肠神经系统构成了其自身最大的外周神经系统（“肠中之脑”），并参与肠道运动的调节。形态各异的神经细胞类型聚集形成壁内神经丛层，并释放多种神经递质。肠神经系统的畸形或病变可能导致肠道运输时间严重延长，从而导致对保守治疗耐药的慢性便秘。与神经节细胞缺乏症的早期表现不同，壁内神经丛的非神经节细胞性或后天性改变在成年前往往未被识别。组织病理学诊断通过酶或免疫组织化学染色进行，可在切片或整装标本上进行，从而最佳地显示神经丛结构。为了诊断神经节细胞减少症、肠神经节炎或 Cajal 间质细胞的改变，需要进行全层活检。Cajal 间质细胞通过产生“慢波”活动，对肠道运动的调节有重要作用。在患有慢传输型便秘和巨结肠的成年患者中，Cajal 间质细胞的肌内网络密度显著降低。
