Abstract
Intestinal Dysganglionoses (IDs) represent a heterogeneous group of Enteric Nervous System anomalies including Hirschsprung's disease (HD), Intestinal Neuronal Dysplasia (IND), Internal Anal Sphincter Neurogenic Achalasia (IASNA) and Hypoganglionosis. At present HD is the only recognised clinico-pathological entity, whereas the others are not yet worldwide accepted and diagnosed. This report describes the areas of agreement and disagreement regarding definition, diagnosis, and management of IDs as discussed at the workshop of the fourth International Meeting on "Hirschsprung's disease and related neurochristopathies." The gold standards in the preoperative diagnosis of IDs are described, enlighting the importance of rectal suction biopsy in the diagnostic workup. The most important diagnostic features of HD are the combination of hypertrophic nerve trunks and aganglionosis in adequate specimens. Acetylcholinesterase staining is the best diagnostic technique to demonstrate hypertrophic nerve trunks in lamina propia mucosae, but many pathologist from different centers still use H&E staining effectively. Moreover, the importance of an adequate intraoperative pathological evaluation of the extent of IDs to avoid postoperative complications is stressed. Although it is not clear whether IND is a separate entity or some sort of secondary acquired condition, it is concluded that both IND and IASNA do exist. Other interesting conclusions are provided as well as detailed results of the discussion. Further investigation is needed to resolve the many controversies concerning IDs. The fourth International Conference in Sestri Levante stimulated discussion regarding these entities and led to the International guidelines to serve the best interest of our patients.
| Original language | English |
|---|---|
| Pages (from-to) | 1527-1531 |
| Number of pages | 5 |
| Journal | Journal of Pediatric Surgery |
| Volume | 40 |
| Issue number | 10 |
| DOIs | |
| Publication status | Published - Oct 2005 |
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Keywords
- Agangglionosis
- ENS
- Enteric nervous system
- Hirschsprung's disease
- Intestinal neuronal dysplasia
ASJC Scopus subject areas
- Surgery
Cite this
Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system : A report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies. / Martucciello, Giuseppe; Prato, Alessio Pini; Puri, Prem; Holschneider, Alexander M.; Meier-Ruge, William; Jasonni, Vincenzo; Tovar, Juan A.; Grosfeld, Jay L.
In: Journal of Pediatric Surgery, Vol. 40, No. 10, 10.2005, p. 1527-1531.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system
T2 - A report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies
AU - Martucciello, Giuseppe
AU - Prato, Alessio Pini
AU - Puri, Prem
AU - Holschneider, Alexander M.
AU - Meier-Ruge, William
AU - Jasonni, Vincenzo
AU - Tovar, Juan A.
AU - Grosfeld, Jay L.
PY - 2005/10
Y1 - 2005/10
N2 - Intestinal Dysganglionoses (IDs) represent a heterogeneous group of Enteric Nervous System anomalies including Hirschsprung's disease (HD), Intestinal Neuronal Dysplasia (IND), Internal Anal Sphincter Neurogenic Achalasia (IASNA) and Hypoganglionosis. At present HD is the only recognised clinico-pathological entity, whereas the others are not yet worldwide accepted and diagnosed. This report describes the areas of agreement and disagreement regarding definition, diagnosis, and management of IDs as discussed at the workshop of the fourth International Meeting on "Hirschsprung's disease and related neurochristopathies." The gold standards in the preoperative diagnosis of IDs are described, enlighting the importance of rectal suction biopsy in the diagnostic workup. The most important diagnostic features of HD are the combination of hypertrophic nerve trunks and aganglionosis in adequate specimens. Acetylcholinesterase staining is the best diagnostic technique to demonstrate hypertrophic nerve trunks in lamina propia mucosae, but many pathologist from different centers still use H&E staining effectively. Moreover, the importance of an adequate intraoperative pathological evaluation of the extent of IDs to avoid postoperative complications is stressed. Although it is not clear whether IND is a separate entity or some sort of secondary acquired condition, it is concluded that both IND and IASNA do exist. Other interesting conclusions are provided as well as detailed results of the discussion. Further investigation is needed to resolve the many controversies concerning IDs. The fourth International Conference in Sestri Levante stimulated discussion regarding these entities and led to the International guidelines to serve the best interest of our patients.
AB - Intestinal Dysganglionoses (IDs) represent a heterogeneous group of Enteric Nervous System anomalies including Hirschsprung's disease (HD), Intestinal Neuronal Dysplasia (IND), Internal Anal Sphincter Neurogenic Achalasia (IASNA) and Hypoganglionosis. At present HD is the only recognised clinico-pathological entity, whereas the others are not yet worldwide accepted and diagnosed. This report describes the areas of agreement and disagreement regarding definition, diagnosis, and management of IDs as discussed at the workshop of the fourth International Meeting on "Hirschsprung's disease and related neurochristopathies." The gold standards in the preoperative diagnosis of IDs are described, enlighting the importance of rectal suction biopsy in the diagnostic workup. The most important diagnostic features of HD are the combination of hypertrophic nerve trunks and aganglionosis in adequate specimens. Acetylcholinesterase staining is the best diagnostic technique to demonstrate hypertrophic nerve trunks in lamina propia mucosae, but many pathologist from different centers still use H&E staining effectively. Moreover, the importance of an adequate intraoperative pathological evaluation of the extent of IDs to avoid postoperative complications is stressed. Although it is not clear whether IND is a separate entity or some sort of secondary acquired condition, it is concluded that both IND and IASNA do exist. Other interesting conclusions are provided as well as detailed results of the discussion. Further investigation is needed to resolve the many controversies concerning IDs. The fourth International Conference in Sestri Levante stimulated discussion regarding these entities and led to the International guidelines to serve the best interest of our patients.
KW - Agangglionosis
KW - ENS
KW - Enteric nervous system
KW - Hirschsprung's disease
KW - Intestinal neuronal dysplasia
UR - http://www.scopus.com/inward/record.url?scp=27444439452&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=27444439452&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2005.07.053
DO - 10.1016/j.jpedsurg.2005.07.053
M3 - Article
C2 - 16226977
AN - SCOPUS:27444439452
VL - 40
SP - 1527
EP - 1531
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 10
ER -