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Congenital Colonic Stenosis: A Rare Gastrointestinal Malformation in Children
Archika Gupta1, Abhishek Kumar Singh1, Kanoujia Sunil1
1Department of Pediatric Surgery, King George's Medical University, Lucknow, Uttar Pradesh, India.
Insights
Congenital colonic stenosis (CCS) is a rare cause of low-intestinal obstruction in children. Early diagnosis and individualized surgical treatment are crucial for optimal outcomes in these rare cases.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Diagnostics
Background:
- Congenital colonic stenosis (CCS) is an exceptionally rare condition causing low-intestinal obstruction in neonates and children.
- Accurate intraoperative diagnosis is essential as initial presentations can mimic other conditions like Hirschsprung's disease or ileal atresia.
Purpose of the Study:
- To report the clinical experience with seven cases of congenital colonic stenosis.
- To propose a treatment algorithm for congenital colonic stenosis to achieve adequate outcomes.
- To highlight the importance of considering CCS in the differential diagnosis of pediatric intestinal obstruction.
Main Methods:
- A retrospective analysis of seven patients diagnosed with CCS between 2014 and 2019.
- Data collected included age at presentation, clinical and radiological findings, surgical details, and patient outcomes.
- Histopathological examination confirmed the diagnosis and identified the stenotic segment.
Main Results:
- Five patients were initially misdiagnosed (five with Hirschsprung's disease, two with ileal atresia).
- The stenotic segment involved the ascending colon (3), transverse colon (2), and sigmoid colon (2).
- Surgical management varied, including primary anastomosis, secondary anastomosis after stoma, and staged ileocolic anastomosis.
Conclusions:
- Congenital colonic stenosis is a rare but significant cause of large-bowel obstruction in pediatric patients, especially with a history of chronic constipation.
- CCS should be considered in the differential diagnosis of neonatal and pediatric intestinal obstruction.
- Individualized treatment based on clinical status and anomalies is key to minimizing morbidity and maximizing outcomes.
Aims:
Congenital colonic stenosis (CCS) is an extremely rare cause of low-intestinal obstruction in neonates/child. We report our experience with seven cases of CCS presenting with low-intestinal obstruction and diagnosed intraoperatively and also propose an algorithm for its appropriate treatment for the adequate outcome.
Materials And Methods:
It was a retrospective study of seven patients of CCS including two neonates (5-days and 15-days old), four infants (age range - 2-11 months), and one 24-month-old child admitted from 2014 to 2019. Information regarding the age of presentation, clinical presentation, physical findings, radiological and laboratory findings, details of surgery, and outcome was retrieved and analyzed.
Results:
The male-to-female ratio was 5:2. Patients were initially diagnosed as cases of Hirschsprung's disease in five and ileal atresia in two. A final diagnosis of CCS was made during surgery and histopathological examination of resected stenotic segment. The segment involved was ascending colon in three, transverse colon in two, and sigmoid colon and junction of descending and sigmoid colon each in one patient. Resection of stenotic colonic segment and primary end-to-end anastomosis was performed in two, divided stoma after resection of the stenotic segment and secondary anastomosis in three, and proximal loop terminal ileostomy followed by resection of the stenotic colonic segment and ileocolic anastomosis after 10-12 weeks in two.
Conclusions:
CCS is a rare but possible cause of large-bowel obstruction, in neonatal, infant, and children particularly when associated with a history of chronic constipation since birth. It should be kept in mind as a differential diagnosis while managing a case of neonatal and pediatric intestinal obstruction, particularly low-bowel obstruction along with a history of chronic constipation. Treatment should be individualized for each patient based on clinical status and associated anomalies to give the best results with less morbidity.
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