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Clinicopathologic relationship of hypoganglionosis
A Kubota1, K Yamauchi, T Yonekura
1Department of Surgery II, Kinki University School of Medicine, Osaka, Japan.
Background/Purpose:
Congenital motor dysfunction of the intestine associated with a morphologically abnormal myenteric nervous plexus (MP) is known as Hirschsprung's disease allied disorder (HAD). However, the clinicopathologic features of HAD are not well understood, partially because a standardized method of histologic evaluation of MP has not been established. To elucidate 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 protein by fluorescent immunohistochemistry. Quantitative evaluation of MP was performed by measuring the fluorescence-positive area (MP ratio), and the results were compared with those of age-matched normal controls.
Results:
All of 6 patients required laparotomy within 1 month after birth and enterostomy between 23 days and 10 months. Three died of intractable enteritis by the age of 2.2 years and were totally dependent on parenteral nutrition (PN) throughout their lives. The other 3 have survived for 6 to 10 years 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.
Insights
Hirschsprung's disease allied disorder (HAD) involves abnormal intestinal motor function due to a malformed myenteric plexus (MP). A new method shows MP size correlates with HAD clinical severity, aiding diagnosis.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Developmental Biology
Background:
- Hirschsprung's disease allied disorder (HAD) is a congenital motor dysfunction of the intestine.
- It is characterized by a morphologically abnormal myenteric plexus (MP).
- Clinicopathologic features of HAD are poorly understood due to a lack of standardized MP histologic evaluation.
Purpose of the Study:
- To elucidate the clinicopathologic relationship of HAD.
- To evaluate the utility of a newly devised histologic evaluation method for MP.
Main Methods:
- Reviewed 6 cases of HAD.
- Used flat-mounted frozen ileum sections stained for S-100 protein via fluorescent immunohistochemistry.
- Quantitatively evaluated MP by measuring the fluorescence-positive area (MP ratio) and compared with controls.
Main Results:
- All 6 HAD patients required surgery within 1 month of birth; 3 died by 2.2 years from enteritis, requiring lifelong parenteral nutrition (PN).
- The remaining 3 patients survived 6-10 years with occasional PN dependence.
- The MP ratio was significantly lower in HAD patients than in controls, correlating with clinical severity.
Conclusions:
- MP size, measured via 2D demonstration, is a potential indicator of HAD clinical severity.
- This quantitative method aids in understanding and managing HAD.