Clinicopathologic relationship of hypoganglionosis

Background/Purpose: Congenital motor dysfunction of the intestine associate d with a morphologically abnormal myenteric nervous plexus (MP) is known as Hirschsprung's disease allied disorder (HAD). However, the clinicopatholog ic features of HAD are not well understood, partially because a standardize d method of histologic evaluation of MP has not been established. To elucid ate the clinicopathologic relationship of HAD the authors reviewed 6 cases of HAD using a newly devised histologic evaluation method. Methods: Flat-mounted frozen sections of the ileum were stained for S-100 p rotein by fluorescent immunohistochemistry. Quantitative evaluation of MP w as performed by measuring the fluorescence-positive area (MP ratio), and th e results were compared with those of age-matched normal controls. Results: All of 6 patients required laparotomy within 1 month after birth a nd enterostomy between 23 days and 10 months. Three died of intractable ent eritis by the age of 2.2 years and were totally dependent on parenteral nut rition (PN) throughout their lives. The other 3 have survived for 6 to 10 y ears but have required PN occasionally. MP ratio in controls was more than 0.34 at all ages, whereas that in HAD was significantly lower than that in controls according to the clinical severity. Conclusion: MP size measured on 2-dimensional demonstration is suggested to be an indicator of clinical severity of HAD. J Pediatr Surg 36:898-900. Co pyright (C) 2007 by W.B. Saunders Company.