3-D technology used to accurately understand equine ileocolonic aganglionosis

Eliane Muniz, Aliny A.B. Lobo Ladd, Fernando V. Lobo Ladd, Andrea A.P. Da Silva, Fernanda V. Kmit, Alexandre S. Borges, Raffaella Teixeira, Lígia S.L.S. Da Mota, Carla B. Belli, André L.V. De Zoppa, Luis C.L.C. Da Silva, Mariana P. De Melo, Antonio A. Coppi

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


Ileocolonic aganglionosis (ICA) is the congenital and hereditary absence of neurons that constitute the enteric nervous system and has been described in various species including humans - Hirschsprung's disease - and horses - overo lethal white syndrome (OLWS). Hirschsprung's disease affects circa 1 in 5,000 live births. At best, this disease means an inability to absorb nutrients from food (humans). At worse, in horses, it always means death. Despite our general understanding of the functional mechanisms underlying ICA, there is a paucity of reliable quantitative information about the structure of myenteric and submucosal neurons in healthy horses and there are no studies on horses with ICA. In light of these uncertainties, we have used design-based stereology to describe the 3-D structure - total number and true size - of myenteric and submucosal neurons in the ileum of ICA horses. Our study has shown that ICA affects all submucosal neurons and 99% of myenteric neurons. The remaining myenteric neurons (0.56%) atrophy immensely, i.e. 63.8%. We believe this study forms the basis for further research, assessing which subpopulation of myenteric neurons are affected by ileocolonic aganglionosis, and we would like to propose a new nomenclature to distinguish between a complete absence of neurons - aganglionosis - and a weaker form of the disease which we suggest naming 'hypoganglionosis'. Our results are a step forward in understanding this disease structurally.

Original languageEnglish (US)
Pages (from-to)160-168
Number of pages9
JournalCells Tissues Organs
Issue number2
StatePublished - Oct 1 2013


  • Horses
  • Ileocolonic aganglionosis
  • Myenteric plexus
  • Overo lethal white syndrome
  • Stereology
  • Submucosal plexus


Dive into the research topics of '3-D technology used to accurately understand equine ileocolonic aganglionosis'. Together they form a unique fingerprint.

Cite this