Treatment of Pneumatosis Intestinalis With Oxygen James C.
Gruenberg, MD; Surinder
K.
Batra, MD; Robert J. Priest,
\s=b\ Breathing high concentrations of oxygen at atmospheric pressure has been promptly effective in achieving objective and subjective relief in pneumatosis cystoides intestinalis. Two patients were treated with 70% oxygen supplied through a nonrebreathing mask. During meals, oxygen was given at 5 liters/min by nasal cannula. Under these conditions, the partial pressures of oxygen and nitrogen are altered substantially; however, the total content of oxygen in the blood is changed only slightly, particularly at the venous end of the capillary. Pneumatosis cyst deflation is believed due primarily to the gradient of the partial pressures of
nitrogen. Because of the possibility of oxygen toxicity, further studies important to evaluate the continued benefit of this therapy and to determine the minimum effective oxygen requirement. are
(Arch Surg 112:62-64, 1977)
cystoides Pneumatosi s by multiple involving portion gastrointestinal
intestinalis is a rare condition characterized intramural clusters of gas of the tract. Clinical any presentation varies from nonspecific bowel symptoms, such as trivial hematochezia, mucus stools, and tenesmus, to more alarming features such as palpable abdominal masses, colon polyps, and pneumoperitoneum. Previously, medical management has been symptomatic, as spontaneous regression may occur after months or
Accepted for publication Sept 9, 1976. From the Department of Surgery (Dr Gruenberg) and the Division of Gastroenterology, Department of Internal Medicine (Drs Batra and Priest), Henry Ford Hospital, Detroit. Reprint requests to the Department of Surgery, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202 (Dr Gruenberg).
Cystoides
MD
Recently, a hastier resolution by breathing concentrations of oxygen has been reported.1 years.
high
-
REPORT OF CASES Case l.-A 50-year-old man was seen in July 1974 with a twomonth history of urgency, tenesmus, and increased flatus, with 10 to 15 watery bowel movements a day. On several occasions he had passed a small amount of blood-tinged mucus in the stool. Rheu¬ matic heart disease with mitral and aortic régurgitation led to an aortic valve replacement three years previously, and since this operation, he had been digitalized and remained on anticoagulant drugs. This patient smokes an average of ten cigarettes a day. Results of his abdominal examination were normal, as were findings of his laboratory studies on admission to the hospital, including blood cell count, urinalysis, and multiphasic chemical analyses. A prosthetic aortic valve was seen on chest roentgenogram that was normal otherwise. Pulmonary function studies were normal. Clusters of sessile polypoid lesions covered by intact mucosa were present on proctosigmoidoscopic examination. The pockets of gas were seen to extend to the midsigmoid area in the barium enema (Fig 1) and were confirmed by colonoscopy examination (Fig 2). Numerous, small partially calcified gallstones were seen in the cholecystogram of a normal functioning gallbladder. Roent¬ genograms of the esophagus, upper gastrointestinal tract, and small intestine were normal. The patient was treated with 70% oxygen supplied through a nonrebreathing mask for four days. The arterial Po._, ranged from 242 to 255 mm rig. Oxygen was given at 5 liters/min by nasal cannula during meals. He was constipated for the first day and thereafter had one or two normal bowel movements daily. A colonoscopy examination was repeated three weeks after the oxygen therapy, and hyperemic mucosa but no gas-filled submucosal cysts were seen. The patient has remained asymptomatic for ten months after oxygen therapy and has declined further barium enema or colonoscopic examination.
Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 06/10/2015
Fig 1 .—Arrows indicate sigmoid colon.
radiolucent air clustered
along contour of
2.—Arrows indicate submucosal gas cysts seen through to treatment with oxygen mask. Mosaic appearance is due to individual glass threads of fiberoptic
Fig
colonoscope prior system.
Case 2.-A 59-year-old woman was admitted to Henry Ford Hospital in June 1975 for progressive abdominal discomfort, nausea, vomiting, and massive abdominal distention. During the three years prior to her admission she had been observed for disseminated scleroderma involving her esophagus and small bowel with secondary malabsorption. In March 1975, she had been hospitalized for a brief episode of abdominal pain and spontaneous pneumoperitoneum that resolved with nonoperative management. Eating difficulties and resulting weakness were incapacitating. The patient was in mild acute distress with normal vital signs. A pectus excavatum and kyphorotoscoliosis were present. Her abdomen was seriously distended, with peristaltic loops of bowel easily visible through the abdominal wall. Bowel sounds v^ere normal, and there was mild diffuse tenderness throughout the abdomen. The skin changes of scleroderma were present in her
hands and face.
Hydropneumoperitoneum and pneumatosis cystoides intesti¬ involving the small intestine were present on abdominal roentgenograms. A barium enema showed no pneumatosis involv¬ ing the colon. Pulmonary function studies were compatible with restrictive impairment without obstructive pulmonary disease. Seventy percent oxygen was administered via a nonrebreathing mask for ten days during which representative blood gas values were as follows: Po.,, 238 mm Hg, Pcoa, 34 mm Hg, pH, 7.45, and bicarbonate, 23 mg/dl. As with the previous patient, oxygen was administered at 5 liters/min by nasal cannula during meals, and during the use of the partial rebreathing mask, the forced inspiratory oxygen (FIO,) was measured with a paramagnetic oxygen analyzer four times a day. No change in vital capacity was noted during this time. Marked clinical improvement was noted nalis
within 24 hours and, thereafter, her distended abdomen became progressively softer and she was able to eat more food. A major
improvement of the pneumatosis was seen in the abdominal x-ray film at discharge. She continued to gain weight and to regain her strength. No free intraperitoneal air, pneumatosis cystoides intestinalis, or active lung disease was present on the roentgenograms in November 1975. However, there was a persistent abnormal smallbowel pattern due to scleroderma. She died suddenly at home three months later, having been examined earlier in the month without known recrudescence of the pneumatosis. COMMENT
The etiology of pneumatosis cystoides intestinalis has remained obscure. A mechanical theory postulates that a primary intestinal condition, such as peptic ulcer,1' pyloric stenosis,15 intestinal obstruction," and scleroderma,7 results in increased intraluminal pressure that forces gas intramurally. However, mucosal integrity is rarely violated, and experimental submucosal air injections result in a more linear distribution of intramural air than is present in adults. A second theory emphasizes the association with chronic obstructive pulmonary diseases such as asthma, bullous emphysema, and cystic fibrosis.8 According to this pulmo¬ nary theory of etiology, a painless pulmonary alveolar rupture produces a pneumomediastinum that dissects along the great vessels, retroperitoneally, and then along the mesenteric vessels to the bowel wall. The vascular segmental distribution seen in adult patients has been reproduced experimentally in the dog, pig, and unembalmed human.1' This theory of etiology is favored by
Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 06/10/2015
the absence of clinical, pathologic, or laboratory evidence of infection in most adult cases of pneumatosis. A third theory, perhaps more important in infants with severe enterocolitis, speculates that bacteria are respon¬ sible for gas cysts. Pneumatosis has been produced in gnotobiotic rats by intramural or intraperitoneal monocon¬ tamination with Clostridium perfring ens.10 The gaseous composition of pneumatosis cysts differs slightly from air in that the concentrations of nitrogen and carbon dioxide are slightly elevated whereas the concentra¬ tion of oxygen is lower.11 In a normal man breathing an increased partial pressure of oxygen at atmospheric pres¬ sure, the Pco2 value is decreased only slightly whereas the partial pressures of oxygen and nitrogen vary inversely according to the inspired Po2 value.1- " At the same time, the total oxygen content of the blood is increased only slightly at an increased Po, value, particularly at the venous end of the capillary. Thus, it is believed that the primary mechanism of pneumatosis cyst deflation concerns the gradient of the partial pressures of nitrogen between the cysts and the venous capillary blood. The biochemistry of oxygen toxicity is complex."
breathing high concentrations of oxygen at one atmosphere of pressure has resulted in permanent pulmo¬ nary damage in man and other mammals with normal lungs. In addition, within species, an individual suscepti¬ bility to pulmonary injury exists in response to various concentrations and duration of oxygen therapy."-1" A progressive decrease in pulmonary vital capacity may be an early and reliable measure of oxygen toxicity in man17 and should be measured along with blood gases. The dramatic responses of our patients indicate that the use of 70% oxygen administered by a nonrebreathing mask will achieve subjective as well as objective relief in pneu¬ matosis cystoides intestinalis. While the patient is eating, the concentration of oxygen is reduced temporarily by giving S liters of oxygen per minute by nasal cannula. Pneumatosis cystoides intestinalis has been considered a disease of uncertain etiology and empiric treatment. Breathing high concentrations of oxygen has been promptly effective, but a long-term follow-up is necessary to evaluate the continued benefit of this therapy and the minimum oxygen requirement compatible with effica¬ ciousness and minimum risk of oxygen toxicity. Ho\ „ver,
References 1. Forgacs P, Wright PH, Wyatt AP: Treatment of intestinal gas cysts by oxygen breathing. Lancet 1:579-582, 1973. 2. Simon NM, Nyman KE, Divertie MB: Pneumatosis cystoides intestinalis: Treatment with oxygen via close-fitting mask. JAMA 231:1354-1356, 1975. 3. Bowen R Jr, Priestley JT: Pneumatosis cystoides intestinalis and large gastric ulcer: Report of a case. Proc Staff Meet Mayo Clinic 34:229-234, 1959. 4. Dhall DP, Mahaffy RG, Matheson NA: Intestinal pneumatosis treated by pyloroplasty. J R Coll Surg Edinb 13:226-229, 1968. 5. Hoffman RV Jr, Gyorgy TRM, Bradley RL: Pneumatosis intestinalis: Report of a case associated with pyloric obstruction. Am Surg 34:300-302, 1968. 6. Block MA, Zikria EA: Preduodenal portal vein causing duodenal obstruction associated with pneumatosis cystoides intestinalis. Ann Surg 153:407-408, 1961. 7. Meihoff WE, Hirschfield JS, Kern F Jr: Small intestinal scleroderma with malabsorption and pneumatosis cystoides intestinalis: Report of three cases. JAMA 204:854-858, 1968. 8. Doub HP, Shea JJ: Pneumatosis cystoides intestinalis. JAMA 172:1238-1242, 1960. 9. Keyting WS, McCarver RR, Kovarik JL, et al: Pneumatosis intesti-
nalis: A new concept. Radiology 76:733-741, 1961. 10. Yale CE, Balish E, Wu JP: The bacterial etiology of pneumatosis cystoides intestinalis. Arch Surg 109:89-94, 1974. 11. Mujahed Z, Evans JA: Gas cysts of the intestine (pneumatosis intestinalis). Surg Gynecol Obstet 107:151-159, 1958. 12. Larson CP Jr, Severinghaus JW: Postural variations in dead space and Co2 gradients breathing air and O2. Appl Physiol 17:417-420, 1962. 13. Van De Water JM, Kagey KS, Miller IT, et al: Response of the lung to six to twelve hours of 100 per cent oxygen inhalation in normal man. N Engl J Med 283:621-626, 1970. 14. Clark JM, Lambertson CJ: Pulmonary oxygen toxicity: A review. Pharmacol Rev 23:37-133, 1971. 15. Kaplan HP, Robinson FR, Kapanci Y, et al: Pathogenesis and reversibility of the pulmonary lesions of oxygen toxicity in monkeys: I. Clinical and light microscopic studies. Lab Invest 20:94-100, 1969. 16. Kapanci Y, Weibel ER, Kaplan HP, et al: Pathogenesis and reversibility of pulmonary lesions of oxygen toxicity on monkeys. Lab Invest 20:101-118, 1969. 17. Clark JM, Lambertsen CJ: Rate of development of pulmonary O2 toxicity in man during O2 breathing at 2.0 Ata. J Appl Physiol 30:739-752, 1971.
Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 06/10/2015
J
Gruenberg, MD; Surinder
K.
Batra, MD; Robert J. Priest,
\s=b\ Breathing high concentrations of oxygen at atmospheric pressure has been promptly effective in achieving objective and subjective relief in pneumatosis cystoides intestinalis. Two patients were treated with 70% oxygen supplied through a nonrebreathing mask. During meals, oxygen was given at 5 liters/min by nasal cannula. Under these conditions, the partial pressures of oxygen and nitrogen are altered substantially; however, the total content of oxygen in the blood is changed only slightly, particularly at the venous end of the capillary. Pneumatosis cyst deflation is believed due primarily to the gradient of the partial pressures of
nitrogen. Because of the possibility of oxygen toxicity, further studies important to evaluate the continued benefit of this therapy and to determine the minimum effective oxygen requirement. are
(Arch Surg 112:62-64, 1977)
cystoides Pneumatosi s by multiple involving portion gastrointestinal
intestinalis is a rare condition characterized intramural clusters of gas of the tract. Clinical any presentation varies from nonspecific bowel symptoms, such as trivial hematochezia, mucus stools, and tenesmus, to more alarming features such as palpable abdominal masses, colon polyps, and pneumoperitoneum. Previously, medical management has been symptomatic, as spontaneous regression may occur after months or
Accepted for publication Sept 9, 1976. From the Department of Surgery (Dr Gruenberg) and the Division of Gastroenterology, Department of Internal Medicine (Drs Batra and Priest), Henry Ford Hospital, Detroit. Reprint requests to the Department of Surgery, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202 (Dr Gruenberg).
Cystoides
MD
Recently, a hastier resolution by breathing concentrations of oxygen has been reported.1 years.
high
-
REPORT OF CASES Case l.-A 50-year-old man was seen in July 1974 with a twomonth history of urgency, tenesmus, and increased flatus, with 10 to 15 watery bowel movements a day. On several occasions he had passed a small amount of blood-tinged mucus in the stool. Rheu¬ matic heart disease with mitral and aortic régurgitation led to an aortic valve replacement three years previously, and since this operation, he had been digitalized and remained on anticoagulant drugs. This patient smokes an average of ten cigarettes a day. Results of his abdominal examination were normal, as were findings of his laboratory studies on admission to the hospital, including blood cell count, urinalysis, and multiphasic chemical analyses. A prosthetic aortic valve was seen on chest roentgenogram that was normal otherwise. Pulmonary function studies were normal. Clusters of sessile polypoid lesions covered by intact mucosa were present on proctosigmoidoscopic examination. The pockets of gas were seen to extend to the midsigmoid area in the barium enema (Fig 1) and were confirmed by colonoscopy examination (Fig 2). Numerous, small partially calcified gallstones were seen in the cholecystogram of a normal functioning gallbladder. Roent¬ genograms of the esophagus, upper gastrointestinal tract, and small intestine were normal. The patient was treated with 70% oxygen supplied through a nonrebreathing mask for four days. The arterial Po._, ranged from 242 to 255 mm rig. Oxygen was given at 5 liters/min by nasal cannula during meals. He was constipated for the first day and thereafter had one or two normal bowel movements daily. A colonoscopy examination was repeated three weeks after the oxygen therapy, and hyperemic mucosa but no gas-filled submucosal cysts were seen. The patient has remained asymptomatic for ten months after oxygen therapy and has declined further barium enema or colonoscopic examination.
Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 06/10/2015
Fig 1 .—Arrows indicate sigmoid colon.
radiolucent air clustered
along contour of
2.—Arrows indicate submucosal gas cysts seen through to treatment with oxygen mask. Mosaic appearance is due to individual glass threads of fiberoptic
Fig
colonoscope prior system.
Case 2.-A 59-year-old woman was admitted to Henry Ford Hospital in June 1975 for progressive abdominal discomfort, nausea, vomiting, and massive abdominal distention. During the three years prior to her admission she had been observed for disseminated scleroderma involving her esophagus and small bowel with secondary malabsorption. In March 1975, she had been hospitalized for a brief episode of abdominal pain and spontaneous pneumoperitoneum that resolved with nonoperative management. Eating difficulties and resulting weakness were incapacitating. The patient was in mild acute distress with normal vital signs. A pectus excavatum and kyphorotoscoliosis were present. Her abdomen was seriously distended, with peristaltic loops of bowel easily visible through the abdominal wall. Bowel sounds v^ere normal, and there was mild diffuse tenderness throughout the abdomen. The skin changes of scleroderma were present in her
hands and face.
Hydropneumoperitoneum and pneumatosis cystoides intesti¬ involving the small intestine were present on abdominal roentgenograms. A barium enema showed no pneumatosis involv¬ ing the colon. Pulmonary function studies were compatible with restrictive impairment without obstructive pulmonary disease. Seventy percent oxygen was administered via a nonrebreathing mask for ten days during which representative blood gas values were as follows: Po.,, 238 mm Hg, Pcoa, 34 mm Hg, pH, 7.45, and bicarbonate, 23 mg/dl. As with the previous patient, oxygen was administered at 5 liters/min by nasal cannula during meals, and during the use of the partial rebreathing mask, the forced inspiratory oxygen (FIO,) was measured with a paramagnetic oxygen analyzer four times a day. No change in vital capacity was noted during this time. Marked clinical improvement was noted nalis
within 24 hours and, thereafter, her distended abdomen became progressively softer and she was able to eat more food. A major
improvement of the pneumatosis was seen in the abdominal x-ray film at discharge. She continued to gain weight and to regain her strength. No free intraperitoneal air, pneumatosis cystoides intestinalis, or active lung disease was present on the roentgenograms in November 1975. However, there was a persistent abnormal smallbowel pattern due to scleroderma. She died suddenly at home three months later, having been examined earlier in the month without known recrudescence of the pneumatosis. COMMENT
The etiology of pneumatosis cystoides intestinalis has remained obscure. A mechanical theory postulates that a primary intestinal condition, such as peptic ulcer,1' pyloric stenosis,15 intestinal obstruction," and scleroderma,7 results in increased intraluminal pressure that forces gas intramurally. However, mucosal integrity is rarely violated, and experimental submucosal air injections result in a more linear distribution of intramural air than is present in adults. A second theory emphasizes the association with chronic obstructive pulmonary diseases such as asthma, bullous emphysema, and cystic fibrosis.8 According to this pulmo¬ nary theory of etiology, a painless pulmonary alveolar rupture produces a pneumomediastinum that dissects along the great vessels, retroperitoneally, and then along the mesenteric vessels to the bowel wall. The vascular segmental distribution seen in adult patients has been reproduced experimentally in the dog, pig, and unembalmed human.1' This theory of etiology is favored by
Downloaded From: http://archsurg.jamanetwork.com/ by a University of Iowa User on 06/10/2015
the absence of clinical, pathologic, or laboratory evidence of infection in most adult cases of pneumatosis. A third theory, perhaps more important in infants with severe enterocolitis, speculates that bacteria are respon¬ sible for gas cysts. Pneumatosis has been produced in gnotobiotic rats by intramural or intraperitoneal monocon¬ tamination with Clostridium perfring ens.10 The gaseous composition of pneumatosis cysts differs slightly from air in that the concentrations of nitrogen and carbon dioxide are slightly elevated whereas the concentra¬ tion of oxygen is lower.11 In a normal man breathing an increased partial pressure of oxygen at atmospheric pres¬ sure, the Pco2 value is decreased only slightly whereas the partial pressures of oxygen and nitrogen vary inversely according to the inspired Po2 value.1- " At the same time, the total oxygen content of the blood is increased only slightly at an increased Po, value, particularly at the venous end of the capillary. Thus, it is believed that the primary mechanism of pneumatosis cyst deflation concerns the gradient of the partial pressures of nitrogen between the cysts and the venous capillary blood. The biochemistry of oxygen toxicity is complex."
breathing high concentrations of oxygen at one atmosphere of pressure has resulted in permanent pulmo¬ nary damage in man and other mammals with normal lungs. In addition, within species, an individual suscepti¬ bility to pulmonary injury exists in response to various concentrations and duration of oxygen therapy."-1" A progressive decrease in pulmonary vital capacity may be an early and reliable measure of oxygen toxicity in man17 and should be measured along with blood gases. The dramatic responses of our patients indicate that the use of 70% oxygen administered by a nonrebreathing mask will achieve subjective as well as objective relief in pneu¬ matosis cystoides intestinalis. While the patient is eating, the concentration of oxygen is reduced temporarily by giving S liters of oxygen per minute by nasal cannula. Pneumatosis cystoides intestinalis has been considered a disease of uncertain etiology and empiric treatment. Breathing high concentrations of oxygen has been promptly effective, but a long-term follow-up is necessary to evaluate the continued benefit of this therapy and the minimum oxygen requirement compatible with effica¬ ciousness and minimum risk of oxygen toxicity. Ho\ „ver,
References 1. Forgacs P, Wright PH, Wyatt AP: Treatment of intestinal gas cysts by oxygen breathing. Lancet 1:579-582, 1973. 2. Simon NM, Nyman KE, Divertie MB: Pneumatosis cystoides intestinalis: Treatment with oxygen via close-fitting mask. JAMA 231:1354-1356, 1975. 3. Bowen R Jr, Priestley JT: Pneumatosis cystoides intestinalis and large gastric ulcer: Report of a case. Proc Staff Meet Mayo Clinic 34:229-234, 1959. 4. Dhall DP, Mahaffy RG, Matheson NA: Intestinal pneumatosis treated by pyloroplasty. J R Coll Surg Edinb 13:226-229, 1968. 5. Hoffman RV Jr, Gyorgy TRM, Bradley RL: Pneumatosis intestinalis: Report of a case associated with pyloric obstruction. Am Surg 34:300-302, 1968. 6. Block MA, Zikria EA: Preduodenal portal vein causing duodenal obstruction associated with pneumatosis cystoides intestinalis. Ann Surg 153:407-408, 1961. 7. Meihoff WE, Hirschfield JS, Kern F Jr: Small intestinal scleroderma with malabsorption and pneumatosis cystoides intestinalis: Report of three cases. JAMA 204:854-858, 1968. 8. Doub HP, Shea JJ: Pneumatosis cystoides intestinalis. JAMA 172:1238-1242, 1960. 9. Keyting WS, McCarver RR, Kovarik JL, et al: Pneumatosis intesti-
nalis: A new concept. Radiology 76:733-741, 1961. 10. Yale CE, Balish E, Wu JP: The bacterial etiology of pneumatosis cystoides intestinalis. Arch Surg 109:89-94, 1974. 11. Mujahed Z, Evans JA: Gas cysts of the intestine (pneumatosis intestinalis). Surg Gynecol Obstet 107:151-159, 1958. 12. Larson CP Jr, Severinghaus JW: Postural variations in dead space and Co2 gradients breathing air and O2. Appl Physiol 17:417-420, 1962. 13. Van De Water JM, Kagey KS, Miller IT, et al: Response of the lung to six to twelve hours of 100 per cent oxygen inhalation in normal man. N Engl J Med 283:621-626, 1970. 14. Clark JM, Lambertson CJ: Pulmonary oxygen toxicity: A review. Pharmacol Rev 23:37-133, 1971. 15. Kaplan HP, Robinson FR, Kapanci Y, et al: Pathogenesis and reversibility of the pulmonary lesions of oxygen toxicity in monkeys: I. Clinical and light microscopic studies. Lab Invest 20:94-100, 1969. 16. Kapanci Y, Weibel ER, Kaplan HP, et al: Pathogenesis and reversibility of pulmonary lesions of oxygen toxicity on monkeys. Lab Invest 20:101-118, 1969. 17. Clark JM, Lambertsen CJ: Rate of development of pulmonary O2 toxicity in man during O2 breathing at 2.0 Ata. J Appl Physiol 30:739-752, 1971.
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J
