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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
[Clinical study of multiple zonal aganglionosis in long segment Hirschsprung's disease]
He-ying Yang1, Qiu-liang Liu, Jia-xiang Wang
1Department of Pediatric Surgery, First Affiliated Hospital, Henan Clinical Medical Key Laborator, Zheng Zhou University, Zhengzhou 450052, China.
Insights
Multiple zonal aganglionosis in Hirschsprung's disease (HD) requires careful intraoperative assessment of the entire colon and ileum. Diagnosis and management may involve unexpected findings beyond typical presentations.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Developmental Biology
Context:
- Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Multiple zonal aganglionosis presents a diagnostic and surgical challenge, particularly in long-segment HD.
- Retrospective review of three pediatric cases highlights the complexities of this rare HD variant.
Purpose:
- To elucidate the pathogeny, diagnosis, and management strategies for multiple zonal aganglionosis in Hirschsprung's disease.
- To emphasize the importance of thorough intraoperative evaluation in long-segment HD cases.
- To discuss potential etiological factors beyond the traditional neurocristopathy theory.
Summary:
- Three pediatric patients with long-segment HD exhibited unexpected distal ileal and proximal ascending colon stenosis due to aganglionosis, confirmed pathologically.
- Surgical intervention included total colectomy and Soave's procedures.
- The study underscores the need for comprehensive surgical inspection of the entire gastrointestinal tract in suspected long-segment HD.
Impact:
- Highlights the variability in Hirschsprung's disease presentation, necessitating expanded diagnostic considerations.
- Informs surgical decision-making for complex pediatric gastrointestinal cases.
- Contributes to understanding the potential multifactorial etiology of neurocristopathy in HD.
Objective:
To discuss pathogeny and diagnosis and management of multiple zonal aganglionosis in Hirschsprung's disease.
Methods:
Records of 3 children, aging 5 days, 29 days and 18 months, 3 boys, with multiple zonal aganglionosis in long segment Hirschsprung's disease between 1987-2005 were reviewed retrospectively. Total colectomy and Soave's operations were performed.
Results:
3 children were diagnosed HD before surgery, but the convulsive stenosis in distal ileum and proximal ascending colon were detected during surgery. The aganglionic cells in the stenosis gut were confirmed by pathologic diagnosis.
Conclusions:
The total colon and ileum should be detected carefully during surgery in children with long segment Hirschsprung's disease. It is believed not sound that the neuroblastic cells stop moving from neuroectoderm to gut in early gestation in HD, but it is also believed that some other causes in the course of gestation might interfere the normal growth of the ganglionic cells.
