A fatal case of hepatic portal venous gas following percutaneous endoscopic gastrostomy in a patient with systemic sclerosis-myositis overlap syndrome and pneumatosis cystoides intestinalis

We report a fatal case of hepatic portal venous gas (HPVG) following percutaneous endoscopic gastrostomy (PEG) and initiation of enteral nutrition in a 57-year-old Japanese woman with systemic sclerosis–myositis overlap syndrome complicated by pneumatosis cystoides intestinalis (PCI). She had interstitial lung disease with right heart strain, congestive heart failure, and respiratory muscle fatigue, requiring mechanical ventilation. After improvement in ventilatory failure, PEG was performed, and enteral feeding was initiated. Transient abdominal pain and recurrent vomiting developed, and computed tomography (CT) revealed intramural gastric gas and extensive HPVG. Based on the clinical course, absence of peritoneal irritation, and postmortem CT findings, gastrointestinal ischemia or necrosis was considered unlikely. The abdominal pain was attributed to gastric overdistension with intramural dissection from luminal gas entry, while HPVG was thought to result from gas tracking from the PEG site into the portal system. The presumed mechanism involved delayed wound healing at the PEG site due to systemic sclerosis–related gastric involvement, long-term glucocorticoid therapy, and malnutrition, combined with elevated intragastric pressure from routine feeding advancement and peristalsis, enabling luminal gas to dissect into the wall. The marked reduction of PCI findings when HPVG was detected suggested that gas from PCI may have contributed to its formation. Although the exact cause of death could not be determined, gas embolism was suspected. This case underscores the need for extreme caution when initiating enteral nutrition in similar patients.