Pneumatosis cystoides intestinales : A forgotten entity

Oluwole Gbolagunte Ajao; Mohammad Y Al Naami; Tarek S Malatani; Nader Morad; El-Dawi N El-Tayeb; Ayed A Al-Shahrani

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CASE REPORT

Year : 1998 | Volume : 4 | Issue : 2 | Page : 101-104

Pneumatosis cystoides intestinales : A forgotten entity

Oluwole Gbolagunte Ajao, Mohammad Y Al Naami, Tarek S Malatani, Nader Morad, El-Dawi N El-Tayeb, Ayed A Al-Shahrani
Departments of Surgery and Pathology, College of Medicine and Asir Central Hospital, Abha, Saudi Arabia

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Date of Submission	16-Sep-1996
Date of Acceptance	22-Dec-1997

How to cite this article:
Ajao OG, Al Naami MY, Malatani TS, Morad N, El-Tayeb EDN, Al-Shahrani AA. Pneumatosis cystoides intestinales : A forgotten entity. Saudi J Gastroenterol 1998;4:101-4

How to cite this URL:
Ajao OG, Al Naami MY, Malatani TS, Morad N, El-Tayeb EDN, Al-Shahrani AA. Pneumatosis cystoides intestinales : A forgotten entity. Saudi J Gastroenterol [serial online] 1998 [cited 2022 Jun 25];4:101-4. Available from: https://www.saudijgastro.com/text.asp?1998/4/2/101/33898

Pneumatosis cystoides intestinalis is a rare condition, not often seen on surgical services. Because of this it tends to be a forgotten entity and it is not often thought of until laparotomy is performed for a "baffling" case of pneumoperitoneum without much systemic toxicity and with only minimal abdominal findings.

At Asir Central Hospital, Abha, during the period from 1989 to 1996, two cases of pneumatosis cystoides intestinalis were seen. The two cases are hereby presented.

Case # 1

A 75-year-old Saudi woman was seen on 20-6-1416 H (13.11.1995) because of a two-day history of abdominal pain, abdominal distension and vomiting. Previously, upper endoscopy confirmed a gastric ulcer for which she was treated conservatively.

She presented again the second time with a confirmed gallbladder stones and because liver function tests were abnormal endoscopic retrograde cholangio-pancreatography (ERCP) was performed on 28-6-1416 H (21.11.1995) and this was normal.

On 5-7-1416 H (27.11.1995) cholecystectomy was performed. At surgery because the common bile duct was dilated to about 2 cm in diameter, it was explored. Multiple gravel-like stones were found in the common bile duct and one impacted in the ampulla of Vater. Choledocho-duodenostomy was then performed. Postoperative course was uneventful except persistent cough and bilateral basal crepitations on auscultating the chest.

About 17 days after surgery she started complaining of epigastric pain again, and because the biopsy report of a previous upper endoscopy showed gastritis she was treated for Helicobacter pylori with Flagyl, tetracycline, colloidal bismuth subcitrate and omeprazole 20mg daily for two weeks. She was eventually discharged on 22-7-1416 H (14.12.1995). The patient was readmitted on 1511.1416 H (3.4.1996) with epigastric pain and twoday history of abdominal distention.

Examination revealed an elderly ematiated lady with some abdominal discomfort and distention. Temperature was 37°C, pulse was 88 per minute, regular and good volume, blood pressure was 120/80 and respiratory rate 16 per minute and thoraco-abdominal in nature. White blood cell count (WBC) was 5.2x10⁹ /L. Hematocrit (HCt) was 43.2, Hemoglobin (Hb) was 15.7g/dl. However, chest Xray, abdominal X-ray and abdominal ultrasonography showed an alarming pneumoperitoneum. (see Figs. A & B).

The patient was rushed to the operating room on the basis of the diagnosis of a perforated viscus and the abdomen was explored. Numerous multiple cysts were found on the anterior abdominal wall, cecum, transverse colon and terminal ileum. The other organs were normal. About 20 cm of the terminal ileum, about 24 cm from ileocecal junction were riddled with intramural cysts partially occluding the lumen. This was resected (see Figs. C & D) and endto-end anastomosis performed. Postoperatively the patient was put on nasal oxygen at 5 liters per minute and metronidazole. She was discharged in satisfactory condition, 10 days after the laparotomy.

Case # 2

A 45-year-old Saudi male was admitted because of chronic abdominal pain for over five years. He was diagnosed as having peptic ulcer and put on medications only on clinical grounds.

Two days before admission, the pain became severe and more generalised. On examination the patient was found to be lean, but in no severe distress. Abdomen was soft, no rigidity, no guarding, no rebound tenderness.

White blood cell count (WBC) was 7.0x10⁹ /L. Temperature was 37.2°C. Pulse was 84 per minute, regular and good volume, blood pressure was 110/75 and respiratory rate was 18 per minute and thoraco-abdominal in nature. Plain X-ray of the abdomen showed large pneumoperitoneum. The patient was rushed to the operating room with a diagnosis of perforated peptic ulcer. At surgery, numerous multiple cysts were found in the cecal area, anterior abdominal wall, the transverse colon and the mid-ileal segment. The mid-ileal segment was heavily involved with multiple cysts. About 10 cm of the area which was heavily affected was resected and end-to-end anastomosis performed. The postoperative course was uneventful.

Discussion

Various theories have been postulated as to the etiology of pneumatosis cystoides intestinales. The condition has been found to be associated with peptic ulcer disease, ^[1],[2] inflammatory bowel disease, chronic pulmonary pathology, steroid therapy ^[3] cryptosporidiosis and HIV infection, ^[4] dermatomyositis, ischemic bowel disease, surgical anastomosis, and surgical procedures such as sigmoidoscopy and colonoscopy^[5],[6],[7] . It has also been reported to be a complication of needle catheter jejunostomy^[8],[9] .

It has been postulated that gas enters the bowel through a mucosal defect and then is distributed distally by peristalsis ^[6] . The two cases presented had histories of peptic ulcer disease. The first case reported here had choledochoduodenostomy five months prior to developing this condition. Also cases associated with gastric outlet obstruction ^[2],[9] have been reported. Others have suggested infection, bowel ischemia and leaking pulmonary emphysematous bullae as etiological factors^[5],[6] . Our area is 2800 to 3000 meters above the sea level. We do not know whether this high altitude bears any relevance to the etiology of this condition.

Our two patients were of poor nutritional status when they presented with this condition. This will suggest a lax submucosal tissue, therefore allowing gas to easily penetrate and dissect distally.

The multiple cysts occupied both the submucosal as well as the subserosal layers ^[10] (see Figs. C & D) of the affected bowel segments and the cysts contain mainly nitrogen gas with a variable amount of hydrogen and carbon dioxide^[11],[12] . Oxygen therapy therefore has been shown to be beneficial in the treatment, because it washes out and replaces the nitrogen in the cysts. Then it is absorbed to cause the collapse of the cysts. Miralbes et al^[12] have suggested the use of 40 percent oxygen through a non-rebreathing mask and to give oxygen at the rate of 5 liters per minute with nasal cathether for seven days.

The use of metronidazole^[11] has also been claimed to have a beneficial effect.

Complications of pneumatosis cystoides intestinalis include intestinal obstruction by the cysts usually at the distal segment of the ileum, pneumoperitoneum, pneumopericardium and malabsorption^[10] .

Conclusion

The presence of an unexplained large pneumoperitoneum without clinical abdominal findings of severe peritonitis and systemic toxicity, especially in a wasted individual is very suggestive of pneumatosis cystoides intestinalis. Despite the diagnosis preoperatively, however, surgery is justified to eliminate a more serious condition such as bowel perforation, and to release the obstruction that may be present.

References

1.	Padwell A, Kopelman C. Pneumatosis cystoides intestinalis asssociated with pyloric stenosis. Postgrad Med J. 1981;57:799-820.
2.	Khan AR, Malatani T, Hussain NK. Pneumatosis intestinalis associated with gastric outlet obstruction and complicated by localized peritoneal inflammation (Letter). Ann Saudi Med. 1990;10:222-3.
3.	Pasquier E, Wattiaux MJ, Peigney N. First case of pneumatosis cystoides intestinalis in adult dermatomyositis. J. Rheum 1993;20:499-503.
4.	Collins CD, Blanshard C, Cramp M, Gazzard B, Gleeson JA. Case Report: Pneumatosis intestinalis occurring in association with cryptosporidiosis and HIV infection. Clinical Radiology 1992;46:410-1. [PUBMED]
5.	Muller CF, Morehead R, Alter J, Michener W. Pneumatosis intestinalis in collagen disorders. Am J Roentgenol 1972;115:300-5.
6.	Galandiuk S, Fazio VW. Pneumatosis cystoides intestinalis. Dis Colon Rectum 1986;29:358-63. [PUBMED]
7.	Heer M, Altorfer J, Pirovino M, Schmid M. Pneumatosis cystoides coli: a rare complication of colonoscopy. Endoscopy 1983;15:119-20. [PUBMED]
8.	Cogbill TH, et al. Massive pneumatosis intestinalis and subcutaneous emphysema: Complication of needle catheter jejunostomy. PEN J Parenter Enteral Nutr 1983;7:171-5.
9.	Swiatczak C, Kowianski Z, Rye K, Kuligowski P. Unusually extensive pneumatosis cystoides intestinalis in a patient with pyloric stenosis and mechanical ileus. Wiad-Lek 1983;36:1885-8.
10.	Chippindale AJ, Desai S. Two unusual cases of pneumatosis coli. Clinical Radiology 1991;43:180-2. [PUBMED] [FULLTEXT]
11.	Bailey H, Love M. Revised by A. J. Harding Rains and Charles V. Mann. Pneumatosis cystoides intestinalis. In: "A short practice of surgery" 20th ed. London: H.K. Lewis & Co. Ltd., 1990;PP1051-2.
12.	Miralbes M, Hinojosa J, Alonso J, Berenguer J. Oxygen therapy in pneumatosis coli: What is the minimum oxygen requirement ? Dis Colon Rectum 1983;26:458-60.