Figure 1
Chest x-ray
Initial chest X-ray showing air under the diaphragm and basal fibrosis.
Pneumatosis intestinalis in a patient with scleroderma
SectionAbdominal imaging
Case TypeClinical Cases
AuthorsCarr C, Gibson E
Connected authors
71 years, female
Clinical History
A 71-year-old woman presented to the Emergency Department with a two-day history of vomiting and abdominal distension. She had a PEG tube in situ due to recurrent aspiration pneumonias and had a past medical history of dementia and scleroderma.
Inflammatory markers and serum lactate levels were normal.
Inflammatory markers and serum lactate levels were normal.
Imaging Findings
Initial chest and abdominal plain films showed dilated loops of small bowel, pneumoperitoneum and fibrotic change at both lung bases.
IV contrast enhanced CT abdomen and pelvis showed multiple loops of dilated small bowel with extensive intramural air. A small volume of retroperitoneal and intraperitoneal free air was also identified. Of note, there was no portal venous or mesenteric venous gas and the coeliac axis and superior mesenteric artery were well-opacified with contrast.
Chest and abdominal plain films 5 days later showed resolution of the intramural gas and free air.
IV contrast enhanced CT abdomen and pelvis showed multiple loops of dilated small bowel with extensive intramural air. A small volume of retroperitoneal and intraperitoneal free air was also identified. Of note, there was no portal venous or mesenteric venous gas and the coeliac axis and superior mesenteric artery were well-opacified with contrast.
Chest and abdominal plain films 5 days later showed resolution of the intramural gas and free air.
Discussion
Pneumatosis intestinalis is defined as the presence of gas in the bowel wall and can be classified as either primary or secondary. Primary pneumatosis intestinalis radiologically and pathologically consists of cystic collections of air, usually located in the colon. Secondary pneumatosis intestinalis is characterised by linear or circumferential air in any part of the gastrointestinal tract and is associated with many clinical conditions [1].
Secondary pneumatosis accounts for approximately 85% of cases [2].
Underlying causes include mechanical/traumatic, inflammatory/autoimmune, infectious, pulmonary, drug induced and connective tissue disorders such as scleroderma [3].
The wide variety of associated conditions is reflected in the numerous terms used to refer to this phenomenon, including pneumatosis cystoides intestinalis, pneumatosis coli, cystic lymphopneumatosis and emphysematous gastritis [4].
The pathophysiology of pneumatosis intestinalis is not fully understood but two basic mechanical features characterise the majority of cases: mucosal injury, the most important and prevalent feature and increased intraluminal pressure [1]. Rupture of intramural blebs is the hypothesised source of benign pneumoperitoneum in patients with pneumatosis intestinalis, causing free air without true transmural perforation [3].
It is important to understand that pneumatosis intestinalis is a sign, not a disease and it must be interpreted relative to the patient’s overall condition [5].
Sclerodema is characterised by excessive collagen production, autoimmune disease-induced inflammation and microvascular injury [6]. The resultant pathological change consists of smooth muscle atrophy and fibrosis caused by collagen deposition in the tunica muscularis [7].
Nearly 90% of patients with scleroderma have evidence of gastrointestinal involvement, which is a substantial cause of morbidity [8].
Small bowel findings include hypomobility from smooth muscle atrophy and fibrosis, which leads to stasis, dilatation and pseudo-obstruction [6].
The clinical importance of pneumatosis intestinalis is that it may be misdiagnosed or, if identified correctly, its significance may be misinterpreted, leading to unnecessary surgical intervention [9].
In our case, the lack of portal venous gas, normal inflammatory markers and rapid clinical improvement would suggest that a diagnosis of ischaemia is unlikely.
Secondary pneumatosis accounts for approximately 85% of cases [2].
Underlying causes include mechanical/traumatic, inflammatory/autoimmune, infectious, pulmonary, drug induced and connective tissue disorders such as scleroderma [3].
The wide variety of associated conditions is reflected in the numerous terms used to refer to this phenomenon, including pneumatosis cystoides intestinalis, pneumatosis coli, cystic lymphopneumatosis and emphysematous gastritis [4].
The pathophysiology of pneumatosis intestinalis is not fully understood but two basic mechanical features characterise the majority of cases: mucosal injury, the most important and prevalent feature and increased intraluminal pressure [1]. Rupture of intramural blebs is the hypothesised source of benign pneumoperitoneum in patients with pneumatosis intestinalis, causing free air without true transmural perforation [3].
It is important to understand that pneumatosis intestinalis is a sign, not a disease and it must be interpreted relative to the patient’s overall condition [5].
Sclerodema is characterised by excessive collagen production, autoimmune disease-induced inflammation and microvascular injury [6]. The resultant pathological change consists of smooth muscle atrophy and fibrosis caused by collagen deposition in the tunica muscularis [7].
Nearly 90% of patients with scleroderma have evidence of gastrointestinal involvement, which is a substantial cause of morbidity [8].
Small bowel findings include hypomobility from smooth muscle atrophy and fibrosis, which leads to stasis, dilatation and pseudo-obstruction [6].
The clinical importance of pneumatosis intestinalis is that it may be misdiagnosed or, if identified correctly, its significance may be misinterpreted, leading to unnecessary surgical intervention [9].
In our case, the lack of portal venous gas, normal inflammatory markers and rapid clinical improvement would suggest that a diagnosis of ischaemia is unlikely.
Differential Diagnosis List
Pneumatosis intestinalis associated with scleroderma.
Small bowel infarction
Small bowel obstruction
Small bowel perforation
Final Diagnosis
Pneumatosis intestinalis associated with scleroderma.
References
[1] S J Knechtle, A M Davidoff, and R P Rice (1990) Pneumatosis intestinalis. Surgical management and clinical outcome. Ann Surg Aug; 212(2): 160–165 (PMID: 2375647)
[2] Galandiuk S, Fazio VW (1986) Pneumatosis cystoides intestinalis. A review of the literature. Dis Colon Rectum May;29(5):358-63 (PMID: 3516602)
[3] St Peter SD, Abbas M.A., Kelly K.A. (2003) The Spectrum of Pneumatosis Intestinalis. Arch Surg 138 (1):68-75 (PMID: 12511155)
[4] Feczko PJ1, Mezwa DG, Farah MC, White BD (1992) Clinical significance of pneumatosis of the bowel wall. Radiographics Nov;12(6):1069-78 (PMID: 1439012)
[5] Lisa M. Ho, Erik K. Paulson, William M. Thompson (2007) Pneumatosis Intestinalis in the Adult: Benign to Life-Threatening Causes. AJR 188:1604–1613 (PMID: 17515383)
[6] Anil S. Bhavsar, Sadhna Verma, Ramit Lamba, Chandana G. Lall, Vincent Koenigsknecht, Arumugam Rajesh (2013) Abdominal Manifestations of Neurologic Disorders. RadioGraphics 33:135–153 (PMID: 23322834)
[7] Rohrmann CA Jr, Ricci MT, Krishnamurthy S, Schuffler MD (1984) Radiologic and histologic differentiation of neuromuscular disorders of the gastrointestinal tract: visceral myopathies, visceral neuropathies, and progressive systemic sclerosis. AJR Am J Roentgenol Nov;143(5):933-41 (PMID: 6333163)
[8] Akesson A1, Wollheim FA. (1989) Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. J Rheumatol Aug;28(4):281-6 (PMID: 2787182)
[9] Farshid Ejtehadi, Nikolaos A. Chatzizacharias, and Hugh Kennedy (2012) Pneumatosis Intestinalis as the Initial Presentation of Systemic Sclerosis: A Case Report and Review of the Literature. Case Reports in Medicine Article ID 987410, 3 pages (PMID: 23056057)
Case information
| URL: | https://www.eurorad.org/case/12815 |
| DOI: | 10.1594/EURORAD/CASE.12815 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
Abdominal x-ray
Initial abdomen X-ray showing intramural gas.
Figure 3
Axial CT
Axial CT showing free air around the pancreas.
Figure 4
Sagittal reformat
Sagittal reformat showing pneumatosis, free air and basal lung fibrosis.
Figure 5
Axial CT
Axial CT showing pneumatosis intestinalis of the small bowel.
Figure 6
Sagittal reformat
Sagittal reformat showing contrast opacification of the superior mesenteric artery.
Figure 7
Coronal reformat
Coronal reformat showing lack of portal venous gas.
Figure 8
Chest X-ray
Chest X-ray 5 days after presentation showing resolution of free air.
Figure 9
Abdominal X-ray
Abdominal X-ray 5 days after initial presentation showing resolution of pneumatosis.
Chest x-ray
Initial chest X-ray showing air under the diaphragm and basal fibrosis.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Abdominal x-ray
Initial abdomen X-ray showing intramural gas.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Axial CT
Axial CT showing free air around the pancreas.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Sagittal reformat
Sagittal reformat showing pneumatosis, free air and basal lung fibrosis.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Axial CT
Axial CT showing pneumatosis intestinalis of the small bowel.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Sagittal reformat
Sagittal reformat showing contrast opacification of the superior mesenteric artery.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Coronal reformat
Coronal reformat showing lack of portal venous gas.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Chest X-ray
Chest X-ray 5 days after presentation showing resolution of free air.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Abdominal X-ray
Abdominal X-ray 5 days after initial presentation showing resolution of pneumatosis.
Radiology Department, Antrim Area Hospital, Northern Ireland.
Radiology Department, Antrim Area Hospital, Northern Ireland.