Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal complication of systemic sclerosis (SSc) characterized by intramural accumulation of gas within thin-walled cysts. We report the case of an 82-year-old female patient with pneumoperitoneum due to PCI associated with SSc and review the features of the 39 Japanese cases. The median patient age was 57 years (range 24–83 years) and the male/female ratio was 1 : 12. In the recent decade, 14 out of 15 cases (93.3%) evaluated with CT scans were diagnosed with PCI. The results suggest that CT scan may be a useful diagnostic tool for detecting PCI. PCI in patients with SSc is usually benign and requires only conservative therapy. However, two patients (5.1%) with signs of peritoneal irritation required surgery. When peritoneal irritation secondary to additional pathology is observed, surgical treatment may be warranted; a precise diagnosis for this condition is therefore essential.
Open Access Biomedical Image Search Engine
Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
Case Reports in Gastrointestinal Medicine
fig1:A chest radiograph demonstrating pneumoperitoneum in the subdiaphragmatic regions bilaterally (arrows). A plain abdominal radiograph showing several dilated loops of small intestine (arrowheads).
View Article:PubMed Central - PubMed
Additional Figures:
Article
Collection
Results
Abstract
Mentions
An 82-year-old woman was referred to our department for incidental finding of pneumoperitoneum on a chest radiograph during a medical checkup at another hospital. Initial history taking revealed a prior surgical history of only a laparotomy secondary to acute appendicitis approximately 60 years before. During physical examination, the patient was afebrile with a pulse rate of 82 beats per minute and blood pressure of 136/86 mmHg. Abdominal examination revealed a slightly distended but soft, tympanic, and nontender abdomen, without signs of peritoneal irritation. There were no palpable masses. Urgent laboratory investigations showed a slightly low hemoglobin level with normal white blood cell count and C-reactive protein level (Table 1). A plain abdominal radiograph showed free air under the diaphragm bilaterally and small bowel dilatation (Figure 1). Abdominal CT scan revealed massive free air and dilated small intestine with gas inside the bowel wall; however, there was no radiological evidence of a localized point of bowel obstruction and no apparent life-threatening acute causes of PCI (Figure 2). Although autoimmune serological testing showed negative results for antinuclear, anti-Scl-70, and anticentromere antibodies, further detailed history taking revealed that the patient had suffered from Raynaud's phenomenon and was previously diagnosed with SSc approximately 30 years before. She recalled that she was treated and followed up for several years, albeit without detailed information regarding her treatment. A rheumatologist later confirmed that she indeed had limited cutaneous SSc, in view of the presence of sclerodactyly of the fingers, telangiectasia on her face, chest, and hands, and Raynaud's phenomenon, all of which met the criteria for SSc [3]. Her pneumoperitoneum was considered to be due to PCI. As the patient was stable with no life-threatening signs and symptoms, she was treated conservatively with bowel rest and maintenance of fluid levels and subsequently discharged home without surgical intervention. The patient remained well eighteen months after discharge, without any abdominal symptoms.
MeSH