[Systemic sclerosis with various gastrointestinal problems including pneumoperitoneum, pneumatosis cystoides...

We describe here an experience of successful treatment of systemic sclerosis (SSc) complicated with various gastrointestinal (GI) problems including pneumoperitoneum, pneumatosis cystoides intestinalis and malabsorption syndrome. A 35-year-old female had developed selerodactyly since February, 1990. She had been treated under the diagnosis of SSc at other hospital. She had required several hospitalizations because of nausea, vomitting and abdominal distension, but her GI symptoms had gradually deteriorated. In April 1993, she was referred to our hospital and admitted for the treatment of her GI problems. On admission, she had systemic cutaneous sclerosis and marked abdominal distension without peritoneal signs was recognized. Chest and abdominal roentgenograms demonstrated massive free air under the diaphragm, marked dilation of small and large bowels, and multiple intestinal cysts (pneumatosis cystoides intestinalis ; PCI). We treated her GI problems with various modalities combined with medications, oxygen breathing, intravenous hyperalimentation and hyperbaric oxygen therapy. Pneumoperitoneum and PCI had disappeared after 8 courses of hyperbaric oxygen therapy and her GI symptoms had been well controled by intravenous hyper alimentation. Thereafter, she has been on intermittent parenteral nutrition through subcutaneous port inplantation. During the courses of this treatment, she developed an episode of Wernicke-Kolsakoff (W-K) syndrome which was considered to associate with malabsorption syndrome. The W-K syndrome had recovered by intravenous administration of vitamin B1.