Total colonic aganglionosis in Hirschsprung disease.

The Appendix and Aganglionosis. A Note of Caution—How the Histology Can Mislead the Surgeon in Total Colonic Hirschsprung Disease

We present the case of a child with presumed total colonic Hirschsprung disease (HD) to highlight the problems the surgeon is likely to encounter if he/she relies on the appendix alone for histopathologic diagnosis. A newborn male infant, who was presumed to have total colonic aganglionosis when the appendix was found to be aganglionic at the time of initial exploratory laparoscopy, was managed...

Zonal colonic aganglionosis, a variant of Hirschsprung's disease.

Rare Association of Extended Total Colonic Aganglionosis and Intestinal Malrotation

Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the paediatric surgeon. We report a case of a neonate that presented with extended total colonic aganglionosis and malrotation, along with a spectrum of central nervous system and renal abnormalities. The clinical and radiological features and potential diagno...

Total Colonic Aganglionosis: a Rare Entity in Children

ABSTACT Total colonic aganglionosis being an unusual form of Hirschsprung’s disease is described in two children. Both the patients were male, one 8-year old and the other 12-year old. They presented with features of sub acute intestinal obstruction preceded by a past history of chronic constipation since birth and operative intervention for similar complaints few years back. A high index of su...

A Retrospective Cohort Study of Total Colonic Aganglionosis: Is the Appendix a Reliable Diagnostic Tool?

We would like the authors to note the recent review on the subject in Lane et al. “The Appendix and Aganglionosis. A Note of Caution-How the Histology Can Mislead the Surgeon in Total Colonic Hirschsprung’s Disease”, [2] This report showed that the appendix is not reliable in determining whether there is Hirschsprung’s disease as many normal appendices have no ganglion cells. When we looked at ...