Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases

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 t...

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Main Authors: Manabu Kaneko, Shin Sasaki, Shuzo Teruya, Kosuke Ozaki, Kazuhiro Ishimaru, Emi Terai, Hiroshi Nakayama, Toshiyuki Watanabe
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Gastrointestinal Medicine
Online Access:http://dx.doi.org/10.1155/2016/2474515
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author Manabu Kaneko
Shin Sasaki
Shuzo Teruya
Kosuke Ozaki
Kazuhiro Ishimaru
Emi Terai
Hiroshi Nakayama
Toshiyuki Watanabe
author_facet Manabu Kaneko
Shin Sasaki
Shuzo Teruya
Kosuke Ozaki
Kazuhiro Ishimaru
Emi Terai
Hiroshi Nakayama
Toshiyuki Watanabe
author_sort Manabu Kaneko
collection DOAJ
description 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.
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institution Kabale University
issn 2090-6528
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language English
publishDate 2016-01-01
publisher Wiley
record_format Article
series Case Reports in Gastrointestinal Medicine
spelling doaj-art-de7ecd0346544db4b086d6fc9e2a9d4c2025-02-03T06:00:17ZengWileyCase Reports in Gastrointestinal Medicine2090-65282090-65362016-01-01201610.1155/2016/24745152474515Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese CasesManabu Kaneko0Shin Sasaki1Shuzo Teruya2Kosuke Ozaki3Kazuhiro Ishimaru4Emi Terai5Hiroshi Nakayama6Toshiyuki Watanabe7Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Internal Medicine, Division of Rheumatology, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanDepartment of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, JapanPneumatosis 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.http://dx.doi.org/10.1155/2016/2474515
spellingShingle Manabu Kaneko
Shin Sasaki
Shuzo Teruya
Kosuke Ozaki
Kazuhiro Ishimaru
Emi Terai
Hiroshi Nakayama
Toshiyuki Watanabe
Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
Case Reports in Gastrointestinal Medicine
title Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
title_full Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
title_fullStr Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
title_full_unstemmed Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
title_short Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases
title_sort pneumatosis cystoides intestinalis in patients with systemic sclerosis a case report and review of 39 japanese cases
url http://dx.doi.org/10.1155/2016/2474515
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