The patient’s general condition was stable, and antibiotics, analgesics, and promotility agents were started. Despite the absence of other causes and due to the chronic presentation, pneumoperitoneum was considered a manifestation of systemic sclerosis. The patient was discharged on day 7 with symptomatic medication (butylscopolamine, domperidone, and simethicone).

Six months later, abdominopelvic computed tomography was repeated and revealed a smaller pneumoperitoneum and the presence of small air bubbles in the colonic wall; no obstructive lesion was identified. The symptoms improved with oral medication. One year later, magnetic resonance enterocolonography showed mild pneumoperitoneum, normal contrast progression, and distension of the small intestine; there were no stenotic lesions, fistulas, or contrast extravasation into the pneumoperitoneum. At outpatient follow-up 24 months later, the patient had recurrent mild abdominal distension but remained clinically well. In this case, the most suitable cause of the nonsurgical recurrent pneumoperitoneum was systemic sclerosis.

Discussion

Pneumoperitoneum is often caused by visceral perforation, with symptoms of peritonitis, and requires an emergent surgical intervention [1]. Nonsurgical spontaneous pneumoperitoneum is a rare entity not associated with organ perforation and usually treated conservatively [1, 2].

Nonsurgical pneumoperitoneum is associated with postoperatively retained air, ventilatory support, spontaneous rupture of pulmonary blebs, cardiopulmonary resuscitation, endoscopy, diverticulosis, pneumatosis cystoides intestinalis, pneumocholecystitis, collagen vascular diseases, gynecologic causes, iatrogenic, and idiopathic causes [2]. In our case, systemic sclerosis was the apparent etiology [1, 3]. There are few reports of chronic scleroderma-related pneumoperitoneum [1–3]. The excessive fibrosis, inflammation, and vascular dysfunction characteristic of systemic sclerosis can affect any site within the gastrointestinal tract [1]. In the small intestine, it causes hypomotility, pseudo-obstructions, and luminal distension [1]. Nevertheless, the pathogenesis of this condition is uncertain, and treatment is usually supportive (promotility and probiotic agents, laxatives, and rifaximin) [1].

Most patients with nonsurgical pneumoperitoneum have clinical clues or associated conditions that may indicate the possibility of this diagnosis. When signs of peritoneal irritation, fever, and leukocytosis are absent, nonsurgical causes of pneumoperitoneum should be considered, and conservative management with supportive care and careful observation is indicated [1–3]. Thus, recognized, nonsurgical pneumoperitoneum allows avoidance of an unnecessary laparotomy and its associated morbidity [1–3].

References