Postgraduate Medicine
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Pneumatosis Cystoides Intestinalis Hakim Mujahid & Wali M. Aseem To cite this article: Hakim Mujahid & Wali M. Aseem (1975) Pneumatosis Cystoides Intestinalis, Postgraduate Medicine, 57:2, 103-106, DOI: 10.1080/00325481.1975.11713967 To link to this article: http://dx.doi.org/10.1080/00325481.1975.11713967
Published online: 07 Jul 2016.
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Date: 05 August 2017, At: 16:20
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Pneumatosis Cystoides lntestinalis Pneumatosis cystoides intestinalis is an uncommon disease, characterized by an air cyst in the subserosa! or submucosal layer, or both, of the gastrointestinal tract. Du Vernoi 1 first described the disease in 17 30, and Meyer 2 introduced the term "pneumatosis cystoides intestinalis" in 1882. In 1899, Hahn3 reported the first case treated surgically. The disease was first reported in American literature in 1908 4 and was first diagnosed preoperatively by x-ray in 1924.5 Pneumatosis cystoides intestinalis is most common in people aged 25 to 55, but cases involving a 12-day-old infant and an 81-year-old man have been reported. 6 The condition is three times more prevalent in men than in women. The small bowel is most commonly affected, usually in the distal jejunum or proximal ileal region. However, the disease may involve the rectum or colon. 7 Submucosal pneumatosis cystaides intestinalis is more serious than the subserosal type, especially in children.
Various theories have been proposed regarding the etiology of air cysts in the subserosa I or submucosal layer of the gastrointestinal tract. Mechanical intestinal obstruction and possibly a paralytic condition in a segment of small intestine precipitated the signs and symptoms in the two cases described, but whether one or both are cause or effect of pneumatosis cystoides intestinalis remains to be proved. HAKIM MUJAHID, MD WALl M. ASEEM, MD Kabul University Hospital Kabul, Afghanistan
Etiologic Theories The cause of pneumatosis cystoides intestinalis remains unknown, but various theories have been proposed. Mechanical theories-Peptic ulcer or pyloric stenosis is thought to precipitate air leakage from the gastrointestinal tract through the ulcerated mucosa. 8 If this theory is correct, residue or signs of air leakage should be apparent in the wall of the stomach, duodenum, or proximal jejunum. Yet, as we and others9 have noted, most cases of pneumatosis cystoides intestinalis involve the jejunoileal region without involvement of the proximal jejunum, duodenum, or
Vol. 57 • No. 2 • February 1975 • POSTGRADUATE MEDICINE
103
I
Plain x-ray films of the abdomen can • be diagnostic in pneumatosis cystoides • intestinal is, especially if the disease •
Downloaded by [University of Calgary] at 16:20 05 August 2017
is of the subserosa I or mixed type. • stomach. Out of 1,500 cases of peptic ulcer treated surgically at Kabul University Hospital, Afghanistan, over the past 40 years, we have found no cases of pneumatosis cystoides intestinalis. Another hospital reports one case of pneumatosis cystoides intestinalis involving the jejunoileal region in a patient in whom duodenal ulcer was an incidental finding. Mechanical intestinal obstruction increases intraluminal pressure enormously and may force air outward through any abrasion or tear in the mucosa, according to McGregor and McKinnon.10 The concept is sound; but if correct there should always be an abrasion or tear in the mucosa. Intestinal obstruction secondary to volvulus is common in the eastern part of the world, but the association of pneumatosis cystoides intestinalis with volvulus of the large or small intestine is rarely seen. In 30 years, over 10,000 cases of intestinal obstruction have been treated surgically at Kabul University Hospital and other hospitals in the region, and secondary pneumatosis cystoides intestinalis has been seen only in the two cases reported here. The gases in the intestinal lumen differ greatly in composition and concentration from the gases in air cysts of pneumatosis cystoides intestinalis. Therefore, it appears that some factors other than leakage must be responsible for the formation of air cysts in the wall of the gastrointestinal tract. Acid-base theory11-Acid-base imbalance secondary to chronic obstructive lung disease as a cause of pneuma.tosis cystoides intestinalis is a complicated theory thought to involve elimination of excess carbon dioxide through the intestine. Doub and Shea12 reported a series in which pneumatosis cystoides intestinalis developed in
104
15 of 16 asthmatic patients. However, the mechanism by which air from alveolar sacs dissects through the mediastinum and reaches the intestinal wall via the mesentery without leaving detectable traces in the mesentery is unclear. Mediastinal emphysema secondary to a tracheal, bronchial, or esophageal tear does not clear up within 48 hours. In addition, as shown by Koss, 8 the air cysts are lined by endothelial cells, which rules out dissection of air along the vascular distribution to the intestinal wall. Bacterial theory13-Gas-forming organisms are thought to penetrate and infect the submucosal layer of the gastrointestinal tract. This theory does not explain the absence of inflammatory reaction in the intestinal wall in most cases and the presence of clear fluid 14 and sterile odorless gas in the cysts. Chemical theory 15-Gas is produced in the lymphatics and is absorbed by the mucosa as a result of change in the hydrogen ion concentration in the intestinal lumen. The literature contains no reports of pneumatosis cystoides intestinalis associated with hyperacidity of the stomach or Zollinger-Ellison syndrome.
Pathology Grossly, the air cysts in the subserosa! layer have the appearance of aggregates of clear soap bubbles or, in cross section, of a honeycomb. They range in size from a few millimeters to 2 cm. Occasionally small cysts coalesce to form larger cysts. They are rarely seen in the muscularis mucosae. Microscopic examination is not specific for the disease. Sometimes a few giant cells and inflammatory cells are seen at the lining of the cyst wall. Diagnosis The patient may present with such symptoms as vague abdominal pain, indigestion, diarrhea, melena, or constipation. Mild cases may be asymptomatic. Plain x-ray films of the abdomen can be diagnostic; especially if the disease is of the subserosal or mixed type. Air cysts in the subserosa! surface of the bowel show radiolucent areas/ 2 and sometimes a loop of bowel is seen interposed between the liver and diaphragm. Free air in the peritoneal cavity without sign or symptom
POSTGRADUATE MEDICINE • February 1975 • Vol. 57 • No. 2
Downloaded by [University of Calgary] at 16:20 05 August 2017
Figure 1. Section of bowel removed from 65-year-old man. Lefl Fibrous band between two loops of bowel formed kink that completely blocked bowel transit. Right. Mesenteric lymphadenopathy close to involved area.
of peritonitis is suggestive of pneumatosis cystaides intestinalis.16 There may be no physical finding if the small bowel is involved, but barium study may show radiolucent areas adjacent to the lumen of the bowel; projection of the cyst to the lumen is seen in cases of submucosal involvement. Similarly, a barium enema study will show a cluster of radiolucent areas in the wall of the colonP If the disease involves the rectum or sigmoid colon, a rubbery mass with crepitation may be palpable in the left lower quadrant. Proctoscopy or sigmoidoscopy best cpnfirms diagnosis. Case Reports
Case 1-A 65-year-old man was admitted to Kabul University Hospital complaining of abdominal pain, nausea, and vomiting of three days' duration. He had not expelled flatus or had a bowel movement for two days and appeared acutely ill and restless. Temperature was 38
c.
Physical examination revealed a moderately distended abdomen and slight tenderness in the periumbilical region. Results of chest x-ray, rectal examination, and urinalysis were normal. Radiologic examination of the abdomen showed loops of the small bowel distended, with airfluid level. Laboratory studies gave the following values:
Vol. 57 • No. 2 • February 1975 • POSTGRADUATE MEDICINE
red blood cell count 4.5 million/en mm; white blood cell count 10,000/cu mm, with 70% neutrophils, 26% lymphocytes, 2% monocytes, and 2% eosinophils; bleeding time 1.5 minutes; clotting time 5 minutes; total serum cholesterol 140 mg/100 ml; total serum protein 4.3 gm/ 100 ml; blood urea nitrogen 16.1 mg/100 ml; total bilirubin 0.2 mg/100 ml; and thymol turbidity 3 units. Mechanical intestinal obstruction and possibly a paralytic condition in a segment of small intestine· were diagnosed. At laparotomy, about 300 cc of straw-colored fluid was aspirated from the abdominal cavity. Examination of the abdominal viscera revealed air cyst formation on the subserosa! surface of the small bowel about 4 feet proximal to the ileocecal valve. The air cysts were in sheets and clusters involving the bowel for a distap.ce of about 4 feet. One area of bowel wall was thick and indurated. A fibrous band formed a kink between two loops of bowel, completely blocking bowel transit (figure 1, left). Mesenteric lymphadenopathy was seen close to this area (figure 1, right). The affected bowel was resected and end-toend anastomosis was performed. Recovery was uneventful, and the patient was discharged 12 days after operation. Three weeks after discharge he complained of diarrhea and was treated symptomatically. During six months of
105
Dr. Mujahid is a member of the surgical house staff of Kabul University Hospital, Kabul, Afghanistan. He is currently in England on a surgical fellowship.
WALl M. ASEEM
Downloaded by [University of Calgary] at 16:20 05 August 2017
Dr. Aseem is a member of the surgical department of Kabul University Hospital. He is currently in the United States to obtain certification as a specialist in thoracic surgery.
follow-up the patient had no further gastrointestinal disturbances. Case 2-A 45-year-old man with no history of gastrointestinal disease was admitted to Kabul University Hospital with abdominal pain, nausea, and moderate abdominal distention. Radiologic examination of the abdomen was nonspecific except for a few distended loops of small bowel. Results of laboratory studies were normal except for mild leukocytosis and some shift to the left.
Mechanical intestinal obstruction and a possible paralytic condition in the intestine were diagnosed. Laparotomy revealed pneumatosis cystoides intestinalis in the ileum about 2 feet proximal to the ileocecal valve. The involved segment was resected, and end-to-end anastomosis of the small bowel was performed. Recovery was uneventful and the patient was discharged from the hospital in good condition. Conclusions
In the two cases reported, there was no history or sign of peptic ulcer, involvement of other areas of the gastrointestinal tract, or pulmonary disease. From our experience and review of the literature, it is apparent that the etiology and pathogenesis of pneumatosis cystoides intestinalis remains unknown and that treatment is symptomatic. Whether the disease is eliminated by resection of the involved segment of bowel or whether it will recur in other segments remains to be seen. Further investigation is necessary to determine which theory or combination of theories correctly explains pneumatosis cystoides intestinalis or whether myoneural involvement is the precipitating cause. Address reprint requests to Wali M. Aseem, MD, 24950 Rockside Rd, Bldg 2, Apt 748, Bedford Heights, OH 44146.
REFERENCES 1. Du Vernoi JG: Anatomische Beobachtungen der unter der aussern und innern Haut der Gedarme eingeschlossenen Luft. Phys Med Abhandl Akad Wissensch Petersb Riga 2:182-188, 1783 2. Meyer AF: Pneumatosis cystoides intestinorum. J Pract Heilk 61:67, 1925 3. Hahn E: Ueber Pneumatosis cystoides intestinorum hominis und einen durch Laparotomie behandelten Fall. Dtsch Med Wochenschr 25:657, 1899 4. ' Finney JM: Gas cyst of the intestine. JAMA 51: 1291, 1908 5. Reverdin A: Maladie kystique de J'intestin. Rev Med Suisse Romande 44:545, 1924 6. MacKenzie EP: Pneumatosis intestinalis. Review of the literature and report of 13 cases. Pediatrics 7:537-549, 1951 7. Fitzgerald-Finch OP, Smith JC, Galloway JK: Pneumatosis cystoides intestinalis. Postgrad Med J 45 :436-440, 1970 8. Koss LG: Abdominal gas cysts (pneumatosis cystoides intestinorum hominis). Arch Pathol 53: 523-549, 1952 9. Ecker ]A, Williams RG, Clay T
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Pneumatosis Cystoides Intestinalis Hakim Mujahid & Wali M. Aseem To cite this article: Hakim Mujahid & Wali M. Aseem (1975) Pneumatosis Cystoides Intestinalis, Postgraduate Medicine, 57:2, 103-106, DOI: 10.1080/00325481.1975.11713967 To link to this article: http://dx.doi.org/10.1080/00325481.1975.11713967
Published online: 07 Jul 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [University of Calgary]
Date: 05 August 2017, At: 16:20
Downloaded by [University of Calgary] at 16:20 05 August 2017
Pneumatosis Cystoides lntestinalis Pneumatosis cystoides intestinalis is an uncommon disease, characterized by an air cyst in the subserosa! or submucosal layer, or both, of the gastrointestinal tract. Du Vernoi 1 first described the disease in 17 30, and Meyer 2 introduced the term "pneumatosis cystoides intestinalis" in 1882. In 1899, Hahn3 reported the first case treated surgically. The disease was first reported in American literature in 1908 4 and was first diagnosed preoperatively by x-ray in 1924.5 Pneumatosis cystoides intestinalis is most common in people aged 25 to 55, but cases involving a 12-day-old infant and an 81-year-old man have been reported. 6 The condition is three times more prevalent in men than in women. The small bowel is most commonly affected, usually in the distal jejunum or proximal ileal region. However, the disease may involve the rectum or colon. 7 Submucosal pneumatosis cystaides intestinalis is more serious than the subserosal type, especially in children.
Various theories have been proposed regarding the etiology of air cysts in the subserosa I or submucosal layer of the gastrointestinal tract. Mechanical intestinal obstruction and possibly a paralytic condition in a segment of small intestine precipitated the signs and symptoms in the two cases described, but whether one or both are cause or effect of pneumatosis cystoides intestinalis remains to be proved. HAKIM MUJAHID, MD WALl M. ASEEM, MD Kabul University Hospital Kabul, Afghanistan
Etiologic Theories The cause of pneumatosis cystoides intestinalis remains unknown, but various theories have been proposed. Mechanical theories-Peptic ulcer or pyloric stenosis is thought to precipitate air leakage from the gastrointestinal tract through the ulcerated mucosa. 8 If this theory is correct, residue or signs of air leakage should be apparent in the wall of the stomach, duodenum, or proximal jejunum. Yet, as we and others9 have noted, most cases of pneumatosis cystoides intestinalis involve the jejunoileal region without involvement of the proximal jejunum, duodenum, or
Vol. 57 • No. 2 • February 1975 • POSTGRADUATE MEDICINE
103
I
Plain x-ray films of the abdomen can • be diagnostic in pneumatosis cystoides • intestinal is, especially if the disease •
Downloaded by [University of Calgary] at 16:20 05 August 2017
is of the subserosa I or mixed type. • stomach. Out of 1,500 cases of peptic ulcer treated surgically at Kabul University Hospital, Afghanistan, over the past 40 years, we have found no cases of pneumatosis cystoides intestinalis. Another hospital reports one case of pneumatosis cystoides intestinalis involving the jejunoileal region in a patient in whom duodenal ulcer was an incidental finding. Mechanical intestinal obstruction increases intraluminal pressure enormously and may force air outward through any abrasion or tear in the mucosa, according to McGregor and McKinnon.10 The concept is sound; but if correct there should always be an abrasion or tear in the mucosa. Intestinal obstruction secondary to volvulus is common in the eastern part of the world, but the association of pneumatosis cystoides intestinalis with volvulus of the large or small intestine is rarely seen. In 30 years, over 10,000 cases of intestinal obstruction have been treated surgically at Kabul University Hospital and other hospitals in the region, and secondary pneumatosis cystoides intestinalis has been seen only in the two cases reported here. The gases in the intestinal lumen differ greatly in composition and concentration from the gases in air cysts of pneumatosis cystoides intestinalis. Therefore, it appears that some factors other than leakage must be responsible for the formation of air cysts in the wall of the gastrointestinal tract. Acid-base theory11-Acid-base imbalance secondary to chronic obstructive lung disease as a cause of pneuma.tosis cystoides intestinalis is a complicated theory thought to involve elimination of excess carbon dioxide through the intestine. Doub and Shea12 reported a series in which pneumatosis cystoides intestinalis developed in
104
15 of 16 asthmatic patients. However, the mechanism by which air from alveolar sacs dissects through the mediastinum and reaches the intestinal wall via the mesentery without leaving detectable traces in the mesentery is unclear. Mediastinal emphysema secondary to a tracheal, bronchial, or esophageal tear does not clear up within 48 hours. In addition, as shown by Koss, 8 the air cysts are lined by endothelial cells, which rules out dissection of air along the vascular distribution to the intestinal wall. Bacterial theory13-Gas-forming organisms are thought to penetrate and infect the submucosal layer of the gastrointestinal tract. This theory does not explain the absence of inflammatory reaction in the intestinal wall in most cases and the presence of clear fluid 14 and sterile odorless gas in the cysts. Chemical theory 15-Gas is produced in the lymphatics and is absorbed by the mucosa as a result of change in the hydrogen ion concentration in the intestinal lumen. The literature contains no reports of pneumatosis cystoides intestinalis associated with hyperacidity of the stomach or Zollinger-Ellison syndrome.
Pathology Grossly, the air cysts in the subserosa! layer have the appearance of aggregates of clear soap bubbles or, in cross section, of a honeycomb. They range in size from a few millimeters to 2 cm. Occasionally small cysts coalesce to form larger cysts. They are rarely seen in the muscularis mucosae. Microscopic examination is not specific for the disease. Sometimes a few giant cells and inflammatory cells are seen at the lining of the cyst wall. Diagnosis The patient may present with such symptoms as vague abdominal pain, indigestion, diarrhea, melena, or constipation. Mild cases may be asymptomatic. Plain x-ray films of the abdomen can be diagnostic; especially if the disease is of the subserosal or mixed type. Air cysts in the subserosa! surface of the bowel show radiolucent areas/ 2 and sometimes a loop of bowel is seen interposed between the liver and diaphragm. Free air in the peritoneal cavity without sign or symptom
POSTGRADUATE MEDICINE • February 1975 • Vol. 57 • No. 2
Downloaded by [University of Calgary] at 16:20 05 August 2017
Figure 1. Section of bowel removed from 65-year-old man. Lefl Fibrous band between two loops of bowel formed kink that completely blocked bowel transit. Right. Mesenteric lymphadenopathy close to involved area.
of peritonitis is suggestive of pneumatosis cystaides intestinalis.16 There may be no physical finding if the small bowel is involved, but barium study may show radiolucent areas adjacent to the lumen of the bowel; projection of the cyst to the lumen is seen in cases of submucosal involvement. Similarly, a barium enema study will show a cluster of radiolucent areas in the wall of the colonP If the disease involves the rectum or sigmoid colon, a rubbery mass with crepitation may be palpable in the left lower quadrant. Proctoscopy or sigmoidoscopy best cpnfirms diagnosis. Case Reports
Case 1-A 65-year-old man was admitted to Kabul University Hospital complaining of abdominal pain, nausea, and vomiting of three days' duration. He had not expelled flatus or had a bowel movement for two days and appeared acutely ill and restless. Temperature was 38
c.
Physical examination revealed a moderately distended abdomen and slight tenderness in the periumbilical region. Results of chest x-ray, rectal examination, and urinalysis were normal. Radiologic examination of the abdomen showed loops of the small bowel distended, with airfluid level. Laboratory studies gave the following values:
Vol. 57 • No. 2 • February 1975 • POSTGRADUATE MEDICINE
red blood cell count 4.5 million/en mm; white blood cell count 10,000/cu mm, with 70% neutrophils, 26% lymphocytes, 2% monocytes, and 2% eosinophils; bleeding time 1.5 minutes; clotting time 5 minutes; total serum cholesterol 140 mg/100 ml; total serum protein 4.3 gm/ 100 ml; blood urea nitrogen 16.1 mg/100 ml; total bilirubin 0.2 mg/100 ml; and thymol turbidity 3 units. Mechanical intestinal obstruction and possibly a paralytic condition in a segment of small intestine· were diagnosed. At laparotomy, about 300 cc of straw-colored fluid was aspirated from the abdominal cavity. Examination of the abdominal viscera revealed air cyst formation on the subserosa! surface of the small bowel about 4 feet proximal to the ileocecal valve. The air cysts were in sheets and clusters involving the bowel for a distap.ce of about 4 feet. One area of bowel wall was thick and indurated. A fibrous band formed a kink between two loops of bowel, completely blocking bowel transit (figure 1, left). Mesenteric lymphadenopathy was seen close to this area (figure 1, right). The affected bowel was resected and end-toend anastomosis was performed. Recovery was uneventful, and the patient was discharged 12 days after operation. Three weeks after discharge he complained of diarrhea and was treated symptomatically. During six months of
105
Dr. Mujahid is a member of the surgical house staff of Kabul University Hospital, Kabul, Afghanistan. He is currently in England on a surgical fellowship.
WALl M. ASEEM
Downloaded by [University of Calgary] at 16:20 05 August 2017
Dr. Aseem is a member of the surgical department of Kabul University Hospital. He is currently in the United States to obtain certification as a specialist in thoracic surgery.
follow-up the patient had no further gastrointestinal disturbances. Case 2-A 45-year-old man with no history of gastrointestinal disease was admitted to Kabul University Hospital with abdominal pain, nausea, and moderate abdominal distention. Radiologic examination of the abdomen was nonspecific except for a few distended loops of small bowel. Results of laboratory studies were normal except for mild leukocytosis and some shift to the left.
Mechanical intestinal obstruction and a possible paralytic condition in the intestine were diagnosed. Laparotomy revealed pneumatosis cystoides intestinalis in the ileum about 2 feet proximal to the ileocecal valve. The involved segment was resected, and end-to-end anastomosis of the small bowel was performed. Recovery was uneventful and the patient was discharged from the hospital in good condition. Conclusions
In the two cases reported, there was no history or sign of peptic ulcer, involvement of other areas of the gastrointestinal tract, or pulmonary disease. From our experience and review of the literature, it is apparent that the etiology and pathogenesis of pneumatosis cystoides intestinalis remains unknown and that treatment is symptomatic. Whether the disease is eliminated by resection of the involved segment of bowel or whether it will recur in other segments remains to be seen. Further investigation is necessary to determine which theory or combination of theories correctly explains pneumatosis cystoides intestinalis or whether myoneural involvement is the precipitating cause. Address reprint requests to Wali M. Aseem, MD, 24950 Rockside Rd, Bldg 2, Apt 748, Bedford Heights, OH 44146.
REFERENCES 1. Du Vernoi JG: Anatomische Beobachtungen der unter der aussern und innern Haut der Gedarme eingeschlossenen Luft. Phys Med Abhandl Akad Wissensch Petersb Riga 2:182-188, 1783 2. Meyer AF: Pneumatosis cystoides intestinorum. J Pract Heilk 61:67, 1925 3. Hahn E: Ueber Pneumatosis cystoides intestinorum hominis und einen durch Laparotomie behandelten Fall. Dtsch Med Wochenschr 25:657, 1899 4. ' Finney JM: Gas cyst of the intestine. JAMA 51: 1291, 1908 5. Reverdin A: Maladie kystique de J'intestin. Rev Med Suisse Romande 44:545, 1924 6. MacKenzie EP: Pneumatosis intestinalis. Review of the literature and report of 13 cases. Pediatrics 7:537-549, 1951 7. Fitzgerald-Finch OP, Smith JC, Galloway JK: Pneumatosis cystoides intestinalis. Postgrad Med J 45 :436-440, 1970 8. Koss LG: Abdominal gas cysts (pneumatosis cystoides intestinorum hominis). Arch Pathol 53: 523-549, 1952 9. Ecker ]A, Williams RG, Clay T
