Treatment of Pneumatosis Cystoides Intestinalis with Hyperbaric Oxygen JOHN S. T. MASTERSON, M.D.,* LEONARD B. FRATKIN, M.D., THOMAS R. OSLER, M.D., WILLIAM G. TRAPP, M.D.
From the Divisions of General and Cardio-Thoracic Surgery, University of British Columbia at Vancouver General Hospital, Vancouver, B.C., Canada
The use of hyperbaric oxygen for the treatment of pneumatosis cystoides intestinalis is reported. The pathophysiology, etiology and previous treatment reports are discussed, as are the advantages of hyperbaric oxygen therapy over normobaric oxygen therapy or surgery. The use of hyperbaric oxygen appears to represent a significant advance in the treatment of pneumatosis cystoides intestinalis.
creasing frequency in the serosa and subserosa, the submucosa, and the muscularis layers. The cysts are lined by endothelial cells with eosinophilic protoplasm and small, dark round nuclei. Large multinucleated giant cells are seen in close proximity to the lining.7'8'10 The course of the condition is obscure with cases having been described of appearance and disappearance over the period of several months proven by repeat laparotomy. The disease may be complicated by intestinal obstruction, pneumoperitoneum, tension pneumoperitoneum and intussusception. Many theories exist to explain the etiology and pathogenesis of the intramural gas filled cysts. The mechanical theory postulates that intestinal gas dissects into the bowel wall via a breach in the intestinal mucosa. This is supported by the observation of gastric or duodenal ulcer, frequently complicated by pyloric stenosis in 55% of cases.7 It has also been proposed that ruptured alveoli in chronic obstructive lung disease release air that dissects through the mediastinum, along the aorta and mesenteric arteries to the bowel.6 In the bacterial theory, gas forming organisms cause gaseous dissection within the bowel wall. This has been undermined by the absence of inflammatory reaction, and by the failure to culture bacteria from the cysts. It has, however, been reproduced experimentally with the use of clostridial organisms.14
p NEUMATOSIS CYSTOIDES INTESTINALIS is charac-
terized by the presence of multiple, gas filled, sessile or pedunculated cysts involving portions of the gastrointestinal tract.7 It is a relatively rare disease, with an estimated 410 cases having been described in the literature by 1973.10 The cysts are more common in men than women, with an approximate ratio of 3.5:1 .7,10 It has been recorded in all age groups, but is most common in the age group of 25-55 years.8 The literature contains a number of reports of the successful treatment of pneumatosis cystoides. intestinalis with normobaric oxygen. To the best of our knowledge, this is the first report of the use of hyperbaric oxygen to treat this condition. Pathology Macroscopically, sessile or pedunculated, single or multiple gas cysts are visible in the serosal and mucosal surface of the bowel. They may vary in size from a few millimeters to several centimeters. These cysts have also been noted in the submucosa, with the same range of characteristics. Palpation reveals a spongy crepitant cushion-like consistency.7 Several cases of pneumoperitoneum secondary to this condition have been reported. Microscopically, the cysts are found in order of de* Current Address: Department of Surgery, Clinical Sciences Building, University of Alberta, Edmonton, Alberta T6G 2E1. Reprint requests: Hyperbaric Research Unit, Faculty of Medicine, University of British Columbia, 700 West 10th Avenue, Vancouver, B.C.: Canada V5Z lL5. Submitted for publication: June 27, 1977.
0003-4932-78-0300-0245-0060
Case Reports Case 1. An 86-year-old Caucasian woman (V.G.H.: 30-91-86) presented with a 12 day history of vague abdominal discomfort, with steadily increasing nausea. Her bowel movements had been very slow initially, but had been replaced by diarrhea over the latter three days. Her past history was significant in that she suffered from asthma. She also had atherosclerotic heart disease and a history of an hysterectomy and partial thyroidectomy some 30 years earlier. © J. B. Lippincott Company
245
MASTERSON AND OTHERS
246
,
a
....
Ann. Surg. * March 1978
hesions. There was no evidence of pneumatosis cytoides intestinalis at that time. In August 1976 she was admitted for suspect small bowel obstruction, which spontaneously cleared. In February 1977 she was admitted with a one year history of constipation and diarrhea. She gave a history of increasing abdominal pain and of increasing frequency of bowel movements. . ..... tBarium enema examination performed outside V.G.H. revealed the presence of gas filled cysts involving the splenic flexure. She was tentatively booked for a total colectomy and ileoproctoscopy. However, three treatments with hyperbaric oxygen at 2.5 ATA, two hours each, produced a marked decrease in her symptoms. Repeat barium enema performed ten days later revealed gas filled cysts which were much improved from her pretreatment examination. Follow-up is not available at this time.
nz..
Discussion The use of high concentrations of oxygen in the treatment of gas containing cavities was first proposed in 1935.1 Successful acceleration of absorption of gas from body cavities has been reported for pneumothorax9 and for pneumatosis cystoides intestinalis.24'113 The rationale for this type of treatment depends upon two facts. The first is that the net movement of a dissolved gas follows the direction of the
FIG. la. Anteroposterior radiograph of barium enema study demonstrating intramural gas cysts of the transverse, descending and
sigmoid colon. Abdominal examination revealed a soft distended tympanitic abdomen. Rectal examination revealed numerous 2-3 cm soft, mobile cysts occupying the rectal ampulla with the consistency of the fingers of an inflated surgical glove. Barium enema examination revealed gas filled cysts in close apposition to the barium column within the transverse, descending and sigmoid colon (Figs. Ia and b). This was interpreted as being compatible with intramural gas cysts, or pneumatosis cystoides intestinalis. Treatment with hyperbaric oxygen at 2.5 atmospheres absolute (ATA) for 2.5 hours on two consecutive days was undertaken. This resulted in a remission of the diarrhea, and a considerable improvement in her mental status. Rectal examination revealed complete resolution of the cysts noted above. Repeat barium enema studies performed three days and ten weeks after treatment demonstrated a normal colon without evidence of pneumatosis cystoides intestinalis. Case 2. A 56-year-old Caucasian woman (V.G.H.: 37-02-97) first presented in September 1974 with a one year history of alternating constipation and diarrhea. Prior to admission she had four to seven day episodes of diarrhea with urgency, interspersed with three to four day periods of constipation. Further enquiry was
A'.
noncontributory. Abdominal examination revealed a 2 cm x 2 cm mobile nontender mass on deep palpation in the left upper quadrant. Barium enema revealed pneumatosis cystoides intestinalis of the sigmoid colon. Laparotomy revealed air filled cysts, 1-2.5 cm in diameter involving the submucosa of the sigmoid colon (Fig. 2). A 13 cm loop of sigmoid was resected and a low anterior resection performed. In August 1975 she underwent a laparotomy for lysis of ad-
FIG. lb. Lateral radiograph demonstrating the presence of gas cysts in the rectum.
Vol. 187 * No. 3
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*.
...
.
247
normobaric oxygen,"1 but this recurrence again responded to therapy. Hyperbaric oxygen therapy, at 2.5 ATA, has the advantage of requiring only two to three treatments. It is also noninvasive, and does not cause pulmonary oxygen toxicity. The incidence of clinically significant pulmonary or CNS oxygen toxicity is very low, and has proven reversible on discontinuation of therapy (unpublished clinical observation).
4...........
~~~~~~~~~~~}!
_. ...
References 1.
FIG. 2. Surgical specimen of sigmoid colon demonstrating submucosal cystic structures.
partial pressure gradient between the cavity and the bloodstream. The second involves the fact that the cysts contain gases other than oxygen.2 Detailed analysis of the gases in one case revealed nigrogen 72.5%, hydrogen 10%, nitrous oxide -4.5%, carbon dioxide-1.7%, argon-1.4%, plus traces of Nbutane, iso-butane, propane, methane and ethane.5 The authors postulate that the use of hyperbaric oxygen results in an increased partial pressure of non-oxygen gases within the cyst, and washing out of all nonoxygen gases from the bloodstream. This results in a steeper diffusion gradient which accelerates the reabsorption of the gases within the cyst. The oxygen which equilibrates within the cyst during treatment is absorbed and utilized for cellular metabolism. Surgical resection of involved bowel, with its attendant morbidity and mortality has been complicated by recurrence of the disease. Normobaric oxygen treatment, lasting six to ten days, has the advantage of being noninvasive, but does expose the patient to pulmonary oxygen toxicity.'1 Recurrence following treatment has occurred in one patient treated with
2.
3. 4. 5. 6.
7.
8. 9.
10.
11. 12. 13. 14.
Fine, J., Flehling, S., and Starr, A. J.: Experimental Observations on the Effect of 95% Oxygen on the Absorption of Air from the Body Cavities. J. Thorac. Cardiovasc. Surg., 4:635, 1935. Forgacs, P., Wright, P. H. and Wyatt, A. P.: Treatment of Intestinal Gas Cysts by Oxygen Breathing. Lancet, 1:579, 1973. Gruenberg, J. C., Batra, S. K. and Priest, R. J.: Treatment of Pneumatosis Cystoides Intestinalis with Oxygen. Arch. Surg., 112:62, 1977. Hoflin, F. and Van der Linden, W.: Pneumatosis Cystoides Intestinalis Treated by Oxygen Breathing. Scand. J. Gastroenterol., 9:427, 1974. Hughes, D. T. D., Gordon, K. C. D., Swann, J. C. and Bolt, G. L.: Pneumatosis Cystoides Intestinalis. Gut, 7:553, 1966. Keyting, W. S., McCarver, R. R., Kovarik, J. L., et al.: Pneumatosis Intestinalis: a New Concept. Radiology, 76:733, 1961. Koss, L. G.: Abdominal Gas Cysts (Pneumatosis Cystoides Intestinorum Hominis). Arch. Pathol., 53:523-549, 1952. Mujahid, H. and Aseem, W. M.: Pneumatosis Cystoides Intestinalis. Postgrad. Med., 57:103, 1975. Northfield, T. C.: Oxygen Therapy for Spontaneous Pneumothorax. Br. Med. J., 4:86, 1971. Shallal, J. A., Van Heerden, J. A., Bartholomew, L. G. and Cash, J. C.: Pneumatosis Cystoides Intestinalis. Mayo Clin. Proc., 49:180, 1974. Simon, N. M., Nyman, K. E., Divertie, M. B., et al. Pneumatosis Cystoides Intestinalis. JAMA, 231:1354, 1975. Watson, R. D. S.: Successful Treatment of Pneumatosis Coli with Oxygen. Br. Med. J., 1:199, 1976. Wyatt, A. P.: Prolonged Symptomatic and Radiological Remission of Colonic Gas Cysts after Oxygen Therapy. Br. J. Surg., 62:837, 1975. Yale, C. E.: Etiology of Pneumatosis Cystoides Intestinalis. Surg. Clin. North Am., 55:1297, 1975.
From the Divisions of General and Cardio-Thoracic Surgery, University of British Columbia at Vancouver General Hospital, Vancouver, B.C., Canada
The use of hyperbaric oxygen for the treatment of pneumatosis cystoides intestinalis is reported. The pathophysiology, etiology and previous treatment reports are discussed, as are the advantages of hyperbaric oxygen therapy over normobaric oxygen therapy or surgery. The use of hyperbaric oxygen appears to represent a significant advance in the treatment of pneumatosis cystoides intestinalis.
creasing frequency in the serosa and subserosa, the submucosa, and the muscularis layers. The cysts are lined by endothelial cells with eosinophilic protoplasm and small, dark round nuclei. Large multinucleated giant cells are seen in close proximity to the lining.7'8'10 The course of the condition is obscure with cases having been described of appearance and disappearance over the period of several months proven by repeat laparotomy. The disease may be complicated by intestinal obstruction, pneumoperitoneum, tension pneumoperitoneum and intussusception. Many theories exist to explain the etiology and pathogenesis of the intramural gas filled cysts. The mechanical theory postulates that intestinal gas dissects into the bowel wall via a breach in the intestinal mucosa. This is supported by the observation of gastric or duodenal ulcer, frequently complicated by pyloric stenosis in 55% of cases.7 It has also been proposed that ruptured alveoli in chronic obstructive lung disease release air that dissects through the mediastinum, along the aorta and mesenteric arteries to the bowel.6 In the bacterial theory, gas forming organisms cause gaseous dissection within the bowel wall. This has been undermined by the absence of inflammatory reaction, and by the failure to culture bacteria from the cysts. It has, however, been reproduced experimentally with the use of clostridial organisms.14
p NEUMATOSIS CYSTOIDES INTESTINALIS is charac-
terized by the presence of multiple, gas filled, sessile or pedunculated cysts involving portions of the gastrointestinal tract.7 It is a relatively rare disease, with an estimated 410 cases having been described in the literature by 1973.10 The cysts are more common in men than women, with an approximate ratio of 3.5:1 .7,10 It has been recorded in all age groups, but is most common in the age group of 25-55 years.8 The literature contains a number of reports of the successful treatment of pneumatosis cystoides. intestinalis with normobaric oxygen. To the best of our knowledge, this is the first report of the use of hyperbaric oxygen to treat this condition. Pathology Macroscopically, sessile or pedunculated, single or multiple gas cysts are visible in the serosal and mucosal surface of the bowel. They may vary in size from a few millimeters to several centimeters. These cysts have also been noted in the submucosa, with the same range of characteristics. Palpation reveals a spongy crepitant cushion-like consistency.7 Several cases of pneumoperitoneum secondary to this condition have been reported. Microscopically, the cysts are found in order of de* Current Address: Department of Surgery, Clinical Sciences Building, University of Alberta, Edmonton, Alberta T6G 2E1. Reprint requests: Hyperbaric Research Unit, Faculty of Medicine, University of British Columbia, 700 West 10th Avenue, Vancouver, B.C.: Canada V5Z lL5. Submitted for publication: June 27, 1977.
0003-4932-78-0300-0245-0060
Case Reports Case 1. An 86-year-old Caucasian woman (V.G.H.: 30-91-86) presented with a 12 day history of vague abdominal discomfort, with steadily increasing nausea. Her bowel movements had been very slow initially, but had been replaced by diarrhea over the latter three days. Her past history was significant in that she suffered from asthma. She also had atherosclerotic heart disease and a history of an hysterectomy and partial thyroidectomy some 30 years earlier. © J. B. Lippincott Company
245
MASTERSON AND OTHERS
246
,
a
....
Ann. Surg. * March 1978
hesions. There was no evidence of pneumatosis cytoides intestinalis at that time. In August 1976 she was admitted for suspect small bowel obstruction, which spontaneously cleared. In February 1977 she was admitted with a one year history of constipation and diarrhea. She gave a history of increasing abdominal pain and of increasing frequency of bowel movements. . ..... tBarium enema examination performed outside V.G.H. revealed the presence of gas filled cysts involving the splenic flexure. She was tentatively booked for a total colectomy and ileoproctoscopy. However, three treatments with hyperbaric oxygen at 2.5 ATA, two hours each, produced a marked decrease in her symptoms. Repeat barium enema performed ten days later revealed gas filled cysts which were much improved from her pretreatment examination. Follow-up is not available at this time.
nz..
Discussion The use of high concentrations of oxygen in the treatment of gas containing cavities was first proposed in 1935.1 Successful acceleration of absorption of gas from body cavities has been reported for pneumothorax9 and for pneumatosis cystoides intestinalis.24'113 The rationale for this type of treatment depends upon two facts. The first is that the net movement of a dissolved gas follows the direction of the
FIG. la. Anteroposterior radiograph of barium enema study demonstrating intramural gas cysts of the transverse, descending and
sigmoid colon. Abdominal examination revealed a soft distended tympanitic abdomen. Rectal examination revealed numerous 2-3 cm soft, mobile cysts occupying the rectal ampulla with the consistency of the fingers of an inflated surgical glove. Barium enema examination revealed gas filled cysts in close apposition to the barium column within the transverse, descending and sigmoid colon (Figs. Ia and b). This was interpreted as being compatible with intramural gas cysts, or pneumatosis cystoides intestinalis. Treatment with hyperbaric oxygen at 2.5 atmospheres absolute (ATA) for 2.5 hours on two consecutive days was undertaken. This resulted in a remission of the diarrhea, and a considerable improvement in her mental status. Rectal examination revealed complete resolution of the cysts noted above. Repeat barium enema studies performed three days and ten weeks after treatment demonstrated a normal colon without evidence of pneumatosis cystoides intestinalis. Case 2. A 56-year-old Caucasian woman (V.G.H.: 37-02-97) first presented in September 1974 with a one year history of alternating constipation and diarrhea. Prior to admission she had four to seven day episodes of diarrhea with urgency, interspersed with three to four day periods of constipation. Further enquiry was
A'.
noncontributory. Abdominal examination revealed a 2 cm x 2 cm mobile nontender mass on deep palpation in the left upper quadrant. Barium enema revealed pneumatosis cystoides intestinalis of the sigmoid colon. Laparotomy revealed air filled cysts, 1-2.5 cm in diameter involving the submucosa of the sigmoid colon (Fig. 2). A 13 cm loop of sigmoid was resected and a low anterior resection performed. In August 1975 she underwent a laparotomy for lysis of ad-
FIG. lb. Lateral radiograph demonstrating the presence of gas cysts in the rectum.
Vol. 187 * No. 3
PNEUMATOSIS CYSTOIDES INTESTINALIS
*.
...
.
247
normobaric oxygen,"1 but this recurrence again responded to therapy. Hyperbaric oxygen therapy, at 2.5 ATA, has the advantage of requiring only two to three treatments. It is also noninvasive, and does not cause pulmonary oxygen toxicity. The incidence of clinically significant pulmonary or CNS oxygen toxicity is very low, and has proven reversible on discontinuation of therapy (unpublished clinical observation).
4...........
~~~~~~~~~~~}!
_. ...
References 1.
FIG. 2. Surgical specimen of sigmoid colon demonstrating submucosal cystic structures.
partial pressure gradient between the cavity and the bloodstream. The second involves the fact that the cysts contain gases other than oxygen.2 Detailed analysis of the gases in one case revealed nigrogen 72.5%, hydrogen 10%, nitrous oxide -4.5%, carbon dioxide-1.7%, argon-1.4%, plus traces of Nbutane, iso-butane, propane, methane and ethane.5 The authors postulate that the use of hyperbaric oxygen results in an increased partial pressure of non-oxygen gases within the cyst, and washing out of all nonoxygen gases from the bloodstream. This results in a steeper diffusion gradient which accelerates the reabsorption of the gases within the cyst. The oxygen which equilibrates within the cyst during treatment is absorbed and utilized for cellular metabolism. Surgical resection of involved bowel, with its attendant morbidity and mortality has been complicated by recurrence of the disease. Normobaric oxygen treatment, lasting six to ten days, has the advantage of being noninvasive, but does expose the patient to pulmonary oxygen toxicity.'1 Recurrence following treatment has occurred in one patient treated with
2.
3. 4. 5. 6.
7.
8. 9.
10.
11. 12. 13. 14.
Fine, J., Flehling, S., and Starr, A. J.: Experimental Observations on the Effect of 95% Oxygen on the Absorption of Air from the Body Cavities. J. Thorac. Cardiovasc. Surg., 4:635, 1935. Forgacs, P., Wright, P. H. and Wyatt, A. P.: Treatment of Intestinal Gas Cysts by Oxygen Breathing. Lancet, 1:579, 1973. Gruenberg, J. C., Batra, S. K. and Priest, R. J.: Treatment of Pneumatosis Cystoides Intestinalis with Oxygen. Arch. Surg., 112:62, 1977. Hoflin, F. and Van der Linden, W.: Pneumatosis Cystoides Intestinalis Treated by Oxygen Breathing. Scand. J. Gastroenterol., 9:427, 1974. Hughes, D. T. D., Gordon, K. C. D., Swann, J. C. and Bolt, G. L.: Pneumatosis Cystoides Intestinalis. Gut, 7:553, 1966. Keyting, W. S., McCarver, R. R., Kovarik, J. L., et al.: Pneumatosis Intestinalis: a New Concept. Radiology, 76:733, 1961. Koss, L. G.: Abdominal Gas Cysts (Pneumatosis Cystoides Intestinorum Hominis). Arch. Pathol., 53:523-549, 1952. Mujahid, H. and Aseem, W. M.: Pneumatosis Cystoides Intestinalis. Postgrad. Med., 57:103, 1975. Northfield, T. C.: Oxygen Therapy for Spontaneous Pneumothorax. Br. Med. J., 4:86, 1971. Shallal, J. A., Van Heerden, J. A., Bartholomew, L. G. and Cash, J. C.: Pneumatosis Cystoides Intestinalis. Mayo Clin. Proc., 49:180, 1974. Simon, N. M., Nyman, K. E., Divertie, M. B., et al. Pneumatosis Cystoides Intestinalis. JAMA, 231:1354, 1975. Watson, R. D. S.: Successful Treatment of Pneumatosis Coli with Oxygen. Br. Med. J., 1:199, 1976. Wyatt, A. P.: Prolonged Symptomatic and Radiological Remission of Colonic Gas Cysts after Oxygen Therapy. Br. J. Surg., 62:837, 1975. Yale, C. E.: Etiology of Pneumatosis Cystoides Intestinalis. Surg. Clin. North Am., 55:1297, 1975.
