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Rett syndrome and gastric perforation
Malay B Shah1, James G Bittner, Michael A Edwards
1Virtual Education and Surgical Simulation Laboratory, Department of Surgery, Medical College of Georgia School of Medicine, Augusta, Georgia 30912, USA.
The American Surgeon
|May 6, 2008
Summary
Rett Syndrome can cause severe gastric dilation, leading to rare but fatal gastric necrosis and perforation. Early recognition and gastric decompression are crucial for improving patient outcomes.
Area of Science:
- Gastroenterology
- Pediatric Neurology
- Surgical Case Reports
Background:
- Rett Syndrome is a rare genetic neurodevelopmental disorder.
- Patients often experience gastrointestinal dysmotility, including decreased esophageal peristalsis and delayed gastric emptying.
- These issues can lead to significant gastric dilation and, rarely, severe complications.
Observation:
- A 31-year-old female with Rett Syndrome presented with severe emesis and abdominal pain.
- Clinical presentation included multiorgan system failure, hypovolemic shock, and metabolic acidosis.
- Radiographic evidence of free air indicated a perforated viscus, necessitating emergency surgery.
Findings:
- Surgical exploration revealed a severely dilated stomach with a necrotic segment and gross perforation.
- The patient underwent resection of necrotic tissue and primary gastric closure.
- Despite intensive care, the patient succumbed to refractory shock and hypoxemia postoperatively.
Implications:
- This case highlights gastric necrosis and perforation as a rare but critical complication of severe gastric dilation in Rett Syndrome.
- Sustained high gastric pressures may compromise gastric perfusion, contributing to necrosis and perforation.
- Prompt gastric decompression, potentially via gastrostomy, should be considered in patients with significant gastric distention to mitigate perforation risk and improve survival.
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