Radiol Oncol 1996; .30: 41-5.
Pneumatosis intestini and pneumoperitoneum in acute lymphoblastic leukaemia
Marija Frkovic,1 Ante Mandic,1 Nada Jonic,1 Boris Labar,2 Mirando Mrsic2
! Department of diagnostic and interventional radiology, 2 Division of haematology, Department of medicine, School of Medicine, University of Zagreb, Clinical Hospital center Rebro, Kispatic str no 12, 41000 Zagreb, Croatia
Pneumatosis intestini and pneumoperitoneum can originate after infection or gastrointestinal obstruction. Very rarely this condition develops as a random event in patients with collagen disorders and vasculitis, acute and chronic graft versus host disease and various immunodeficiency with no clinical impact.
A patient with acute lymphoblastic leukaemia who developed pneumatosis intestini and pneumoperitoneum with no clinical evidence is reported on. According to the clinical data we assume that the etiology of this condition in the patient is multifactorial: leukaemia, chemotherapy, corticosteroids, sepsis and/or fungal infection.
Correlation between patient clinical status and radiological evidence of pneumatosis peritonei is crucial to obtaining the right picture of clinical situation.
Key words: leukaemia, pneumoperitoneum; pneumatosis intestinalis
Introduction
Pneumatosis intestini is a common radiological finding in patients with intestinal necrosis. Intestinal perforation is closely accompained with "surgical" pneumoperitoneum and peritonitis. This serious condition has typical clinical finding of acute abdominal pain which leads to urgent abdominal operation.
In contrast to this situation "benign" pneumatosis 1 or "internistical" pneumoperitoneum2
Correspondence to: Marija Frkovic, M.D., Ph.D., Maksimirska street no 12, 41000 Zagreb, Croatia.
UDC 616.155.392-036.11-06:616.381-003.219
usually has no clinical signs and can be diagnosed only by plain abdominal film. This "benign" pneumatosis is caused by other extraintestinal diseases or conditions. Such patients are treated by conservative therapy and very rarely by surgical operation.34
The main purpose of this case report is to point out specific radiologic signs of "benign" pneumatosis and pneumoperitoneum.
Case report
A forty-seven-year-old male was admitted to the hospital because of weakness, high number of WBC, anemia and a low number of platelets. Bone marrow aspiration showed immature cells
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compatible with acute lymphoblastic leukaemia.
Chemotherapy by daunorubicine, cyclophosphamide, vincristine and 6-methylprednisolone was introduced (day + 1). Ten days later, mucositis grade IV was observed. At the same time the patient became febrile and blood culture revealed infection with Streptococcus viri-dans. Antibiotics regimen, consisting of netyl-micin and penicillin, was started. At the same time chest X-ray showed multiple infiltrates in both upper lung fields compatible with fungal infection. Amphotericin B and 5-flucitosine was introduced. Unfortunately, bone marrow aspiration on day + 23 showed 90 % of blast, and salvage chemotherapy of intermediate doses consisting of arabinoside and m-amsacrine over 6 days was started.
During all this period patient did not have signs of abdominal pain or surgical abdomen.
On day + 39 routine chest X-ray was performed because of previously diagnosed multiple lung infiltrates. Examination of this X-ray showed regression of lung infiltrates but with air shaped like a sickle under both sides of diaphragm. Patient was subfebrile, 37.5"C, but in a good condition without any pain or other symptoms. Karnofsky score was 70 % . Clinically we found gentle abdomen with enlargement of liver (3 cm bellow right costal margin). There was an evidence of intestine movement.
Plain-films of abdomen showed evidence of pneumoperitoneum, with air in wall of small and large intestine, especially in transverse colon and mesocolon (Figure 1 and 2). Air collections, were oval or rounded but in some parts irregular shaped. This air mass parallely followed intestinal contour. In mesocolon, it was radially placed but parallely followed the rne-senterium vessels. There was no evidence of significantly distinct intestine meteorisrn.
Patient position had no influence on the above mentioned radiologic finding.
Radiologic evidence of pneumoperitoneum and pneumatosis intestini slowly decreased over 11 days without any clinical impairment.
Figure 1. Pncumoperitoneum: gas shaped like a sicklc show subdiaphragmatic and subhcpatic.
Figure 2. Pneumatosis intestinalis: gas in thc wall of the small and large bowel, cspccially in transverse colon and mesocolon.
On day + 50 there was no evidence of air in abdominal cavity. During all this time the patient was maitained on fluid replacement, intestinal decontamination, antibiotics, antifungal and sterile diet. Bone marrow aspiration on day + 65 showed presence of blasts. Because of that patient received high doses of cytosine arabinosie over 6 days.
This period was complicated with heart failure and patient died on day + 75 after starting of initial chemotherapy course.
Pneumatosis intestini and pneumoperitoneum in acute lymphoblastic leukaemia
43
Discussion
Intestinal pneumatosis is collection of air in intestinal wall especially in submucosa and sub-serosa.
"Benign intestinal pneumatosis" was first described in 1973.1
It is a common finding in newborns with severe necrotic enterocolitis.4"10 There were reports on the same condition in patients with leukaemia and lymphoma,11-14 with collagen disorders,15 16 with severe respiratory distress syndrome,2, 17' 18 in acquired and hereditary immunodeficiency disorders,7' 12, 19^22 and after immunosupressive therapy.19, 21
Etiologycal explonation of intestinal pneumatosis is multifactorial.5' 23' 24
Conditions such are disease (leukaemia, lymphoma, malignant diseases, immunodeficiency and collagen disorders) chemo-radio therapy, administration of corticosteroides, bacterial, viral or fungal infection could cause mucosal damage and intestine wall necrosis.
Severe anaemia in patients with malignant disease might cause ischaemic intestine wall
■ 5 14
necrosis. ■'
Administrations of corticosteroides, especially in infancy, might develop atrophia of lymphoid tissue in small and large intestine which leads to submucosal or subserous air disection.12'- 21
A patient receiving high doses of chemora-diotherapy, as a condition regimen prior to marrow transplatation, may experience this condition due to the toxic effect on gastrointestinal tract. Although, gastrointestinal tract is a target tissue for acute and chronic graft versus host disease.
According to Keats13 and Yeager20 mucosal damage appeares 2-3 weeks after finishing chemotherapy.
One of the major factor in developing such condition is presence of bacteria, especially anaerobes, in the bowel.5, 6'23
In some patients with intestinal pneumatosis infections with rotaviruses and adenoviruses were documented.25 Viral enteritis in patients receiving marrow
graft is a very rare and there are no available literature data on this condition.
Clinical features of pneumatosis intestini usually consist of abdominal pain.
In some cases, so called "silent" or "indolent" cases pneumatosis intestini and pneumoperitoneum is a random finding on routine chest X-ray.!1, 12. 24
The main site of this air mass is terminal ileum, large colon, especially colon transversum and ascendent,8 l3' 20 rarely in aboral part of colon.26
In our patient chemotherapy was started 39 days before routine chest X-ray were performed.
The patient experienced no abdominal symptomatology. Plain abdominal film showed air shaped like a sickle under both sides of the diaphragm in diameter of 1cm. Pneumatosis was extended to the terminal ileum, mesocolon and large intestine up to the rectosigmoidal border.
Finding with such extension as in our patient, without clinical impairment, is very rare.12, 19 26
Usually, evidence of air in abdominal cavity disappears within two weeks.13
In our patient 11 days after first X-ray there was no evidence of air collection in the wall of small and large intestine and mesocolon.
The characteristics of "benign" asymptomatic pneumatosis are oval or rounded air cystic collections in the serosa and subserosa. This distinguishes "benign" pneumatosis from symptomatic pneumatosis in which air collection is linear and narrowly located in the submuco-sa.3- 27
"Benign" pneumatosis is also easily distinguished from pneumatosis cystoides intestinalis in which air collections accumulate in the large oval cysts like spaces along the intestine wall.
Moreover, in "benign" pneumatosis patient's position (standing vs lying) and tijne (24 hour period between X-rays) have no influence on the position of air in abdominal cavity.s
In our opinion, such differentiation of the intestinal pneumatosis, despite the fact that it emphasises the importance of radiological findings obtained by standard examinations, which
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is the aim of this paper, is not accurate unless clinical symptomatology of a patient is not build in the diagnosis as a whole.
Similar attitudes are also presented by Thomas VI.28
Some recent studies report usefulness of ultrasound and computerized tomography in determining location and progression of intestinal pneumatosis,9' 27-31, but the main diagnostic method is still plain abdominal film.
In conclusion, we agree with studies of Keats13 and Yeager20 who emphasize that a well informed radiologist and carefully examined series of plain abdominal films may give a very useful information on the development of pneumatosis intestini and "benign" pneumope-ritoneum.
Correlation between patient's clinical status and radiological evidence of pneumatosis inte-stini and pneumoperitoneum is crucial in obtaining the right diagnosis.
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