Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2000  |  Volume : 6  |  Issue : 1  |  Page : 47-50
Pneumatosis intestinalis beyond the newborn period


1 Department of Pediatric, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
2 Department of Radiology, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia

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Date of Submission24-Feb-1998
Date of Acceptance31-May-1999
 

How to cite this article:
Shabib SM, Al Mane K, McDonald P. Pneumatosis intestinalis beyond the newborn period. Saudi J Gastroenterol 2000;6:47-50

How to cite this URL:
Shabib SM, Al Mane K, McDonald P. Pneumatosis intestinalis beyond the newborn period. Saudi J Gastroenterol [serial online] 2000 [cited 2022 Jan 27];6:47-50. Available from: https://www.saudijgastro.com/text.asp?2000/6/1/47/33505


Beyond the newborn period, the demonstration of Pneumatosis intestinalis (PI) is rare and information about its pathogenesis and management in older children is limited. The condition is characterized by the presence of gas in the subserosa or submucosa and may occur in any part of gastrointestinal tract, though seen most commonly in the colon. The gas within the bowel wall is seen in radiographs as linear or circular radiolucencies depending on whether the affected loops are seen in profile, or en face[1]. Sometimes a bubbly appearance is produced by submucosal accumulation of the gas. In older patients this form is characterized as pneumatosis cystoides intestinalis and in adults may be associated with chronic pulmonary disease or pyloric obstruction. The diagnosis of PI is essentially a radiologic one, since there are no specific clinical findings associated with it. In the newborn the detection of gas within the wall of the intestine is an important sign of necrotizing enterocolitis, but it is seen in only a minority of patients in whom the clinical diagnosis is made and it is often a transient radiologic appearance. On occasion it may be recognized indirectly by the presence of gas bubbles within the portal vein and its branches[2]during an ultrasound examination. In infants and children the condition is reported mainly in immunocompromised patients[3]. Most reports have advised conservative management of PI[3],[4] without the need for surgical intervention[4]. We report four patients with a radiologic finding of PI.


   Report of cases Top


The charts of all children between the ages of six months and twelve years in whom a diagnosis of pneumatosis intestinalis had been made at the King Faisal Specialist Hospital and Research Centre between January 1985 and February 1995 were reviewed. This excluded infants with necrotizing enterocolitis. Four patients were identified. The underlying condition, symptoms at presentation, physical findings, laboratory and radiographic findings, management, and outcome were reviewed and are summarized in the Table. All were girls, three infants and one older child. Three were immunocompromised with primary diagnoses of Bare Lymphocyte Syndrome, lymphoma with Hyper IgE Syndrome and autoimmune enteropathy respectively and the remaining infant had congenital heart disease. The three immunocompromised patients presented with symptoms suggesting gastroenteritis The radiologic appearance consisted of linear or circular radiolucencies [Figure - 1] , [Figure - 2], and bubbly pattern [Figure - 3]. Pneumatosis intestinalis responded appropriately to conservative management consisting of bowel rest, parenteral nutrition and antibiotics and the intramural gas resolved.


   Discussion Top


It is frequently difficult to diagnose pneumatosis intestinalis because this condition is usually asymptomatic, except for occasional mild gastrointestinal nonspecific complaints, such as abdominal cramps which are associated with constipation, diarrhea or small stools. Diagnosis obtained incidentally on x-ray examination of the abdomen.

The etiology of this condition is not entirely clear and a single mechanism cannot account for all cases. There have been many theories on the cause of PI, both for benign and fulminant forms. These include mechanical, bacterial, nutritional and chemical causes. The mechanical theory suggests that the gas is forced into the bowel by one or more of the following routes: pulmonary, trauma, mucosal break, ischemic mucosal changes, anastomosis obstruction increase pressure and increase peristalsis[3]. The mechanical theory is divided into three groups. The first group consists of extra abdominal conditions, such as chronic obstructive pulmonary disease in which pneumomediastinum may dissect through the retrperitonium into the mesentery , and form air containing cysts in the intestinal wall. This condition has been described in adults and in premature infants with bronchopulmonary dysplasia[5]. In the second group, the presence of air within the bowel wall is a sign of intestinal distress. A typical example is necrotizing enterocolitis. Infection and bowel ischemia allow the intramural migration of gas­forming bacteria. This has been described in patients with ischemic colitis, mesenteric infarction, ischemia secondary to mid gut volvulus[6], severe ganulomatous enteritis[7] , leukemia and lymphoma[4]. In this form, the appearance of pneumoperitoneum is an ominous sign of advanced intestinal necrosis with perforation and may require immediate intervention[5],[7] . A third group consists of intra abdominal conditions, not related to ischemia or ulceration of the gut. Chronic intestinal obstruction may force intra luminal air into the bowel wall through minuscule mucosal defects. The mucosa heals and the integrity of the bowel wall is not affected. These gas cysts may later rupture into the peritoneal cavity causing a benign pneumoperitoneum. If the diagnosis can be established, surgical intervention is not necessary[8]. The bacterial theory of PI remains most attractive. Several analysis of cyst gas have been performed. Hydrogen contents of up to 50% have been reported[9]. Normal intestinal gas contains 14% H,[10]. The high cysts hydrogen level suggest a bacterial or mechanical cause[9]. The nutritional and chemical theories are similar and postulate that an acidic medium related to increased lactic acid levels due to a particular diet or disturbed carbohydrate metabolism leads to decreased carbon dioxide and oxygen resorption with cyst formation[4]

Multiple factors probably play a role in the development of PI, including immunodeficiency, the administration of cytotoxic drugs and of steroids. Steroids have been shown to cause shrinkage of lymphoid tissue in the intestine and to alter the structural integrity of the mucosa which may allow gas to dissect within the wall[10]. PI has been described in association with acute leukemia[4],[11] following liver transplantation[12],[7] and after bone marrow transplantation[13],[14]. In collagen diseases such as scleroderma and dermatomyositis[15]. Local ischemia due to vasculitis may be a factor and infectious processes either bacterial or viral may also play a role[3]. Rotavirus was detected in two of our patients and the association has been observed previously[10],[12]. In older infants and children the incidental detection of PI as in case four suggests a benign process. Three of our four patients had gastrointestinal symptoms, though in none was there evidence of peritonitis or any systemic disturbance. Intensive medical treatment with bowel rest, intravenous nutrition and the administration of broad-spectrum antibiotics was followed by resolution of PI. This benign outcome has been demonstrated previously[4],[7],[15]. West et al reported 16 patients with PI, 8 patients (age range 2 months-8 years) developed abdominal distension and increased frequency of stools that were grossly bloody on hematest positive. Abdominal radiographs documented PI and subsequent stool cultures for rotavirus were positive. All were treated with fluid support and triple antibiotic coverage, and bowel rest. Radiographic evidence of PI resolved in 2 to 5 days. None required laparotomyl[5] Though surgery has occasionally been said to play a role in the management of this condition[11], others have suggested that even with the development of pneumoperitoneum surgical intervention is unnecessary[14]. Treatment should be directed to the underlying condition when possible. The prognosis remains dependent on the underlying disease and is not fundamentally changed by development of PI. It is concluded that pneumatosis intestinalis, occuring in children and infants beyond the neonatal period should be managed conservatively. This condition usually without clinical evidence of peritonitis or of systemic disturbance has a benign outcome.[16]

 
   References Top

1.Olmsted WW, Madewell JE. Pneumotosis cystoides intestinalis. A pathologic explanation of the roentgenographic signs. Gastro Intest Radiol 1976;1:177-81.  Back to cited text no. 1    
2.King S, Shuckett B. Sonographic diagnosis of portal venous gas in two pediatric liver transplant patients with benign pneumatosis intestinalis. Case reports and literature review. Pediatr Radiol 1992;22:577-8.  Back to cited text no. 2    
3.Day DL, Ramsay NKC, Letoumeau JG. Pneumatosis Intestinalis after Bone Marrow Transplantation. AJR 1988,151:85-7.  Back to cited text no. 3    
4.Jaffe N, Carlson DH, Vawter GF. Pneumatosis Cystoides Intestinalis in Acute Leukemia. Cancer 1972;239-43.  Back to cited text no. 4    
5.West KW, Rescorla FJ, Grosfeld JL, Vane DW. Pneumatosis Intestinalis in Children Beyond the Neonatal Period. J Pediatr Surg 1989;24:818-22.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Eggli, KD, Loyer E, Anderson K: Neonatal pneumatosis cystoides intestinalis caused by volvulus of mid intestine. Arch Dis Child 1989;64:1189-90.  Back to cited text no. 6    
7.Cohen NP, Booth IW, Parashark et al: Successful management of idiopathic intestinal pneumo-obstruction with cisapride. J Pediatr Surg 1988;23:229-30.  Back to cited text no. 7    
8.Simon NM, Nyman KE, Divertic MB;Pneumatosis cysloides intestinatis: treatment with oxygen via close fitting mask. JAMA 1975;231:1354-6.  Back to cited text no. 8    
9.Yale GE. Etiology of pneumatosis cystoides intestinalis. Surg Clin North Am 1975;55:1297-1302.  Back to cited text no. 9    
10.Reynolds Jr. HL, Gauderer MWL, Hrabovsky EE, Shurin SB. Pneumatosis Cystoides Intestinalis in Children Beyond the First Year of Life: Manifestations and Management. J Pediatr Surg 1991;26:1376-80.  Back to cited text no. 10    
11.Katz JA, Wagner ML, Gresik MD, Mahoney Jr. DH, Fembach DJ. Typhlitis. Cancer 1990;65:1041-7.  Back to cited text no. 11    
12.Koep LJ, Peters TG, Starzl . TE. Major Colonic Complications of Hepatic Transplantation. American Society of Colon and Rectal Surgeons 1979;218-20.  Back to cited text no. 12    
13.Kleinman PK, Brill PW, Winchester P. Pneumatosis Intestinalis: Its Occurrence in the Immunologically Compromised Child. Am J Dis Child 1980;134:1149-51.  Back to cited text no. 13  [PUBMED]  
14.Yeager AM, Kanof ME, Kramer SS, Jones B, Saral R, Lake AM, Santos GW. Pneumatosis Intestinalis in Children after Allogeneic Bone Marrow Transplantation. Pediatr Radiol 1987;17:18-22.  Back to cited text no. 14  [PUBMED]  
15.Oliveros MA, Herbst JJ, Lester PD, Ziter FA. Pneumatosis Intestinalis in Childhood Dermatomyositis. Pediatrics 1973;52:711-2.  Back to cited text no. 15  [PUBMED]  
16.Silliman CC, Haase GM, Strain JD, Luckey DW, Blake MA, Caldwell SA, Wilson HL, Odom LF, Ater JL, Greffe BS. Indications for Surgical Intervention for Gastrointestinal Emergencies in Children Receiving Chemotherapy. Cancer 1994;74:203-16.  Back to cited text no. 16  [PUBMED]  

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Correspondence Address:
Souheil M Shabib
Department of Pediatrics, King Faisal Specialist Hospital & RC, P.O. Box 3354, Riyadh 11461
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


PMID: 19864729

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