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Esophageal aperistalsis following fundoplication in a patient with trisomy 21
J Bozinovski1, D Poenaru, W Paterson
1Departments of Surgery and Medicine, Queen's University, Kingston, Ontario, Canada.
Insights
Gastrointestinal issues are common in Down syndrome (DS). In a DS patient, Nissen fundoplication worsened esophageal aperistalsis, leading to long-term tube feeding and highlighting the need for preoperative motility assessment.
Area of Science:
- Pediatric Gastroenterology
- Clinical Genetics
- Surgical Outcomes
Background:
- Gastrointestinal (GI) abnormalities, particularly gastroesophageal reflux (GER), are prevalent in individuals with Down syndrome (DS).
- Surgical interventions like Nissen fundoplication are sometimes employed to manage severe GER in this population.
Observation:
- A 10-year-old boy with DS presented with severe vomiting, diagnosed with GER, and underwent laparoscopic Nissen fundoplication.
- Postoperatively, the patient developed an inability to tolerate solids, tight gastroesophageal junction, and poor esophageal peristalsis.
Findings:
- Despite esophageal dilatation, symptoms persisted, and esophageal manometry revealed complete esophageal aperistalsis.
- The patient required a percutaneous endoscopic gastrostomy for long-term tube feeding due to persistent GI motility issues.
Implications:
- Esophageal aperistalsis is a rare complication in DS, potentially exacerbated by GER and surgical interventions.
- Preoperative esophageal manometry is crucial to identify underlying motility disorders in DS patients undergoing fundoplication.
- This assessment can guide management decisions and potentially prevent adverse outcomes in children with DS and GI dysfunction.
Abstract:
Gastrointestinal abnormalities are frequent in patients with Down syndrome (DS), gastroesophageal reflux (GER) being prominent among them. A 10-year-old boy with DS presented with progressive daily vomiting and an upper gastrointestinal study documenting reflux. A laparoscopic Nissen fundoplication was performed uneventfully. Postoperative inability to take solids was noted and a contrast study showed a tight gastroesophageal junction and poor peristalsis. Persistent symptoms were not alleviated by esophageal dilatation, despite a relaxing lower esophageal sphincter. Esophageal manometry documented complete esophageal aperistalsis. A percutaneous endoscopic gastrostomy was placed and the patient required long-term tube feeds. Esophageal aperistalsis is a rare condition in DS, likely superimposed on GER. Fundoplication may adversely affect the already abnormal esophageal motility in these children. Esophageal manometry preoperatively will identify motility disorders and assist in selecting the best management for these patients.