Abstract
Pneumatosis cystoides intestinalis (PCI) is characterized by gas-filled cysts within gastrointestinal tract wall from esophagus to rectum, with preferential involvement of large and small intestine. PCI is rare with an estimated incidence of 0.03 to 0–2% in general population. PCI can be distinguished into idiopathic (15%) or secondary (85%) and the clinical picture ranges from completely asymptomatic to life-threatening intraabdominal complications. Although etiology of PCI appears to be multifactorial, the exact pathophysiology is poorly understood and two main theories have been proposed (mechanical and bacterial). Over the last decades, an enormous therapeutic armamentarium was considered in PCI's management, including hyperbaric oxygen therapy (HBOT). Treatment comprises conservative treatment in mild cases to surgery in highly symptomatic and complicated PCI. In the late 70s, HBOT started to be used in selected cases of PCI not responding to conservative measures. Since then, several case reports, case series, and reviews have been published in the literature with variable outcomes. The overall response rate and complete response were 92.1% (n = 82/89) and 65.2% (n = 58/89), respectively, with a median follow-up of 7 months. Furthermore, HBOT is extremely safe, with few reported complications in the literature when used for PCI. Nevertheless, a randomized, controlled, and double-blind clinical trial is unlikely to occur given the rarity of PCI, logistical issues of HBOT, and methodological considerations related to adequate blinding with a sham-controlled group. HBOT in combination with personalized diet and antibiotics may be beneficial for moderate to severe PCI in patients with no indication for emergency exploratory laparotomy. The purpose of this article is to synthesize the existing data, analyse results of previous studies, identify gaps in knowledge, and discuss PCI' management, including the proposal of an algorithm, with a special focus on HBOT.
Highlights
Pneumatosis cystoides intestinalis (PCI) was first described by Johann George Du Vernoi in an autopsy specimen in 1730 (1691–1759)
With the lack of large randomized control studies to guide the use of hyperbaric oxygen therapy (HBOT) in the treatment of PCI, the goal of this review is to synthesize the existing data, analyse results of previous studies, identify gaps in knowledge, and discuss PCI’
Since the majority of PCI case reports were in Japanese, the help of an interpreter was required for a full-text reading
Summary
Pneumatosis cystoides intestinalis (PCI) was first described by Johann George Du Vernoi ( known as Du Vernoy) in an autopsy specimen in 1730 (1691–1759). In 1825, Mayer coined the name of the disease as “pneumatosis cystoides intestinorum,” which was adopted by several authors [2]. In the literature PCI is described under different denominations, including pneumatosis intestinalis, pneumatosis coli, cystic lymphopneumatosis, peritoneal lymphopneumatosis, intestinal emphysema, intestinal bullous emphysema, pseudolipomatosis, gas cysts of the intestines, and intraluminal gas [4, 5]. The overall incidence of PCI is not precisely known, with some studies showing rates of 0.03 (two cases among 6,553 patients in serial autopsy studies) to 0.2% (two cases in 1,900 examinations) [5,6,7,8,9]. In an Asiatic database systematic review, the peak age at onset was 45.3 ± 15.6 (ranged, 2–81) years, male to female ratio of 2.4:1 and the average disease course of 6 months [11]
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