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Published on: April 26, 2019
Inflammatory pseudocyst associated with trisomy 21 and Hirschsprung's disease
J J DuBois1, J Cheng, W J Pokorny
1Baylor College of Medicine, Section of Pediatric Surgery, Houston, TX, USA.
Insights
A rare inflammatory abdominal pseudocyst developed in a child with trisomy 21 and Hirschsprung's disease one month post-Swenson pull-through surgery. This case highlights a novel complication following surgical repair for Hirschsprung's disease.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease is a congenital disorder affecting the large intestine.
- Surgical repair, such as the Swenson pull-through procedure, is standard treatment.
- Abdominal pseudocysts are known complications in various medical contexts.
Observation:
- A 15-month-old male with trisomy 21 and Hirschsprung's disease presented with an inflammatory abdominal pseudocyst.
- The pseudocyst developed one month after undergoing a Swenson pull-through repair.
- This occurred despite no prior history of conditions typically associated with pseudocysts.
Findings:
- This is the first reported instance of an abdominal pseudocyst arising post-Hirschsprung's disease surgical repair.
- The pseudocyst formation was noted one month after the Swenson procedure.
- The patient had concurrent trisomy 21, a factor not previously linked to this complication.
Implications:
- This case expands the differential diagnosis for abdominal masses in pediatric patients post-Hirschsprung's repair.
- It suggests a potential, albeit rare, association between surgical intervention for Hirschsprung's disease and pseudocyst development.
- Further investigation may be warranted to understand the specific mechanisms and risk factors involved.
Abstract:
A 15-month-old black male with trisomy 21 and Hirschsprung's disease developed an inflammatory abdominal pseudocyst 1 month after Swenson pull-through repair. Although abdominal pseudocyst associated with ventriculoperitoneal shunts, peritoneal dialysis catheters, meconium peritonitis, and pancreatitis have been reported, this is the first report of a pseudocyst developing 1 month after a surgical repair of Hirschsprung's disease.
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