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Is pneumatosis cystoides intestinalis a lymphatic pathology?: A case of small bowel lymphangioma and subsequent development of pneumatosis cystoides intestinalis in a 57-year-old female.

ABSTRACT: The etiology of pneumatosis cystoides intestinalis (PCI) is multifactorial and its corresponding treatment is similarly complex. One causation of PCI, is lymphatic disruption. This association is discussed in this case report involving an incident of abdominal lymphangioma and subsequent development of PCI in a 57-year-old female. The purpose of this paper is to further understand the pathology of PCI, specifically as it relates to lymphatic disruption. Furthermore, the purpose of this article is to define a treatment protocol for this disease when it involves lymphatic pathology; i.e. when a surgical vs conservative approach is warranted.

SUBMITTER: Harris E

PROVIDER: S-EPMC6344922 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature

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Is pneumatosis cystoides intestinalis a lymphatic pathology?: A case of small bowel lymphangioma and subsequent development of pneumatosis cystoides intestinalis in a 57-year-old female.

Harris Emily E Giannotti Giovanni G

Journal of surgical case reports 20190124 1

The etiology of pneumatosis cystoides intestinalis (PCI) is multifactorial and its corresponding treatment is similarly complex. One causation of PCI, is lymphatic disruption. This association is discussed in this case report involving an incident of abdominal lymphangioma and subsequent development of PCI in a 57-year-old female. The purpose of this paper is to further understand the pathology of PCI, specifically as it relates to lymphatic disruption. Furthermore, the purpose of this article i ...[more]

PMID: 30697407

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Project description: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.

Dataset Information

Is pneumatosis cystoides intestinalis a lymphatic pathology?: A case of small bowel lymphangioma and subsequent development of pneumatosis cystoides intestinalis in a 57-year-old female.

Publications

Is pneumatosis cystoides intestinalis a lymphatic pathology?: A case of small bowel lymphangioma and subsequent development of pneumatosis cystoides intestinalis in a 57-year-old female.

Similar Datasets

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