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Portal venous gas in the pediatric age group
August, 1971 The Journal of P E D I A T R I C S
255
Portal venous gas in the pediatric age group R e v i e w o[ the literature a n d r e p o r t o f t w e l v e n e w cases Thirty-one reported instances o[ portal venous gas are reviewed, and 12 new cases are added. The presence o[ portal venous gas is usually associated with sepsis, gastroenteritis, and abdominal distention in the first Jew weeks o[ life. The roentgenographic findings are typical and diagnostic. Ths pathogenesis is not completely understood, although in most situations underlying gastrointestinal mucosal destruction is [ound. Since the condition in reported cases has been almost uni[ormly [atal, a vigorous medical and surgical therapeutic approach is suggested.
Rica G. Arnon, M.D., and Judith F. Fishbein, M.D. BROOKLYN,
N . Y.
T r l E O C C U R R E N C E of gas in the portal system, first described in 1955,1 has been considered a rare entity. Nevertheless, within 24 months we have observed 12 new cases, which brings the total number of reported pediatric cases to 43. The prognosis in general is quite grave, and in our experience this finding has been associated with an almost 100 per cent mortality rate. However, a recent report by Stevenson and associates 2 demonstrated a better survival rate. It is our purpose to alert the pediatrician to the existence of this finding and to suggest a possible approach once the diagnosis is made. The presence of portal venous gas is not obvious if not specifically looked for. A roentgenogram of the abdomen and particularly of the right upper quadrant is the only means available to establish the diagnosis. The characteristic radiographic appearance is that of tubular lucencies branching from the From the Department of Pediatrics, Kings County Hospital, and State University o[ New York-Downstate Medical Center. Reprint address: Rica G. Arnon, Department oJ Pediatrics, Kings County Hospital, 451 Ctarkson Ave., Brooklyn, N. Y. 11203.
porta hepatis (Fig. 1) to the edge of the liver. The gas is carried to the periphery of the liver by the centrifugal flow of portal blood where it accumulates and outlines the finer peripheral portal radicals (Figs. 1 to 4). In contrast, intrabiliary gas accumulates in the major biliary radicals due to the centripetal flow of bile. 3 Portal venous gas may be confused with pneumatosis intestinalis and intraluminal gas. Pneumatosis intestinalis (Figs. 1, 3, and 4) and pneumoperitoneum are, nevertheless, frequently associated roentgenographic findings (Table I). The presence of portal venous gas has been described in association with various disease processes such as gastroenteritis, necrotizing enterocolitis, sepsis, and following umbilical vein catheterization (present series) 4-7 and peroxide enema. 's The prognosis in association with umbilical vein catheterization is considerably better (50 per cent survival). The clinical findings of patients with portal venous gas, excluding those following peroxide enema and umbilical vein catheterVol. 79, No. 2, pp. 255-259
256
Arnon and Fishbein
The ]ournaI o[ Pediatrics August 1971
Table I. Summary of findings in 43 patients with portal venous gas
l patients No. of l
Survived
Sepsis
Peroxide enema s
1
--
--
Umbilical vein catheterization ~4-7
6
35, 7
__
Necrotizing enteroeolitis in premature infants e2
10
32
Gastroenteritis and septicemiami, 5, 7, 11-15
23
3~5, 15
18
3
--
--
43
9
26
Associated with
U n c l a s s i f i e d 12
Total
Associated anomalies
T E fistula 4
8
Imperforate anus 1 Duodenal atresia 1 D u o d e n a l stenosis, m o n g o l 5 Malrotation*2, H i r s c h s p r u n g ' s d i s e a s e w i t h colitis~4 E s o p h a g e a l a t r e s i a 11 Esophageal atresia with double TE fistulas ~
GI anomalies
GI = gastrointestinal, TE = tracheoesophageal. ~Present series.
ization, have been nonspecific. The infants appeared critically ill, were acidotic, and often in shock. Frequently, they presented with gastroenteritis, bloody stools, and a distended abdomen. Autopsy and operative material were available in 35 patients. Ten patients (23 per cent) had undeHying congenital gastrointestinal anomalies; 28 patients had other pathologic findings in the gastrointestinal tract ranging from mild hyperemia of the mucosa to severe ulceration and gangrene of the bowel with perforation; 15 patients had peritonitis and 20 patients had pneumatosis intestinalis (Table I ) . In one of our 12 patients, the gas disappeared radiologically 8 days prior to death, and none could be found at the time of postmortem examination. In the rest of our patients, gas was readily observable in the mesenteric and portal veins and in the bowel wall. One infant also had gas in the inferior vena cava and a crepitant liver containing subcapsular air. The pathogenesis of portal venous gas is not well understood, except in cases in which
air is presumably introduced by an umbilical catheter or a peroxide enema. Wolfe and Evans 1 postulated that luminal gas under increased pressure passes through the bowel wall into the mesenteric veins and collects in the portal system. This has been partially supported by Yenerman, ~ who produced pneumatosis intestinalis experimentally in cadavers by increasing the intraluminal gas pressure in the presence of mucosal ulcerations. Bilger 1~ demonstrated that the composition of the submucosal gas in pneumatosis intestinalis may be compared to that of atmospheric air. Wiot and Felson *~ postulated that gasforming bacteria gives rise to portal venous gas. In their series all patients had antemortem or postmortem blood cultures positive for gas-forming organisms. The organisms were frequent inhabitants of the bowel and following distruption of the intestinal mucosa entered the portal venous system. These poorly substantiated theories are currently the only explanations available to account for the presence of air in the portal venous system. The absence of pathologic
Volume 79 Number 2
No. of patients
Pathology Meconium ileus
Necrosis of the intestine Pneumatosis intestinalis Perforation of intestine Peritonitis
10
Portal venous gas
No. o[ patients
Survivors Analysis
7 10 3 5
Removal of segment of necrotic bowel (4 deaths--involvement of entire intestine found at surgery)
3
Osteomyelitis, sepsis, and diarrhea 15 Malrotation with removal of segmental colitis5 Hirschsprung's disease with colitis and colostorny~
No GI mucosal pathology ~11 Other changes varied from hyperemia to ulceration necrosis and perforation of bowel Pneumatosis intestinalis Perforation Peritonitis
10 5 10
Pneumatosis intestinalis Peritonitis Perforation No GI pathology
20 15 8 3
findings in the gastrointestinal tract in some cases, 11 including the present series, is somewhat puzzling unless gaseous distention alone can force air into the submucosa without evidence of mucosal damage. The presence of portal venous gas often portends a fatal outcome, although the gas in itself is not the direct cause of death. Aside from cases secondary to umbilical catheterization, only 6 patients (14 per cent) have survived (Table I ) . Eight patients died one week or more after the diagnosis was established. However, the majority of patients lived only a few hours. The rapid progression and high mortality rate apparently reflect the natural history of the underlying disease process; therapy should be directed accordingly, both quickly and intensively. Most patients with portal venous gas had cultures positive for gram-negative organisms. Escherichia coli was found in 20. Aerobacter in 4, Proteus in 2, Candida in 2, and Salmonella in one. Vigorous antibiotic therapy aimed at the above organisms, guided later by sensitivity studies, should there-
257
I
No. of patients
1 1 1
fore be instituted at once in all patients with portal venous gas, realizing that gas may be introduced via an umbilical vein catheter in otherwise well babies. Fluid, electrolyte and acid-base balance, and neutral thermal environment must be maintained. Early surgical intervention, regardless of the clinical status of the patient, appears in some instances, 2, 5 including patients in our series, to have been life saving. It is imperative to determine the status of the bowel as soon as possible. Contrast studies of the gastrointestinal tract may be helpful in localizing and determining the nature of the underlying process. In a series of 21 patients with necrotizing enterocolitis, Stevenson and associates 2 described 6 patients with portal venous gas. Three patients survived and are still living following resection of the necrotic bowel. One of our patients with Hirschsprung's disease complicated by necrotizing enterocolitis and portal venous gas survived following a colostomy. In addition, perforation of the bowel and peritonitis were frequently noted during surgery, although there had been no preop-
258
Arnon and Fishbein
Fig. 1. A 4-week-old infant with sepsis, diarrhea, and distended abdomen. Film of right upper quadrant reveals distention of both small and large bowel. Gas is demonstrated within the bowel wall (lower arrow) as linear and circular lucencies outlining the lumen of the distended bowel. A large collection of gas is present in the portal vein (upper arrow), from which linear streaks of gas within the portal radicals are seen radiating to the periphery of the liver.
Fig. 2. A 20-day-old infant with chronic diarrhea, acidosis, and distended abdomen. Film of right upper q u a d r a n t reveals characteristic peripheral distribution of portal venous gas, in contrast to the central distribution of gas within the biliary system.
The ]ournal o[ Pediatrics August 1971
Fig. 3. A 6-week-old male with sepsis, diarrhea, and distended abdomen. Gas is seen within the wail of the bowel as well as within the perlpheral branches of the portal system. Since the bowel is seen en face, the intramural gas (arrows) assumes a circular configuration.
Fig. 4. A 6-week-old premature infant with diarrhea, dehydration, and distended abdomen. Film of the abdomen reveals marked distention of both small and large bowel. Pneumatosis is present in most of the viscera on the right. A considerable amount of gas is distributed in the portal radicals of the liver.
Volume 79 Number 2
erative radiologic evidence thereof. 2 Early surgical i n t e r v e n t i o n is therefore strongly reco m m e n d e d i n most instances of necrotizing enterocolitis with progressive pneumatosis intestinalis, especially with worsening of the clinical picture a n d / o r evidence of p n e u m o peritoneum. The authors wish to thank Dr. Jonathan T. Lanman for constructive criticism and Dr. Jack G. Rabinowitz for encouragement, guidance, and making available to us the records of the survivor with Hirschsprung's disease. REFERENCES
1. Wolfe, J. N., and Evans, W. A.: Gas in the portal veins of the liver in infants: A roentgenographic demonstration with postmortem anatomical correlation, Amer. J. Roentgen. 74: 486, 1955. 2. Stevenson, J. K., Graham, B. C., Oliver, T. K., and Goldberg, V. E.: Neonatal necrotizing enterocolitis. A report of twenty-one cases with fourteen survivors, Amer. J. Surg. 118: 260, 1969. 3. Susrnan, N., and Senturia, H. R.: Gas embolization of the portal venous system, Amer. J. Roentgen. 83: 847, 1960. 4. Schmidt, A. G.: Portal vein gas due to administration of fluid via the umbilical vein, Radiology 88: 293, 1967.
Portal venous gas
259
5. Miskin, M., and Reilly, B. J.: Gas in the intestinal wall and portal venous system in infants, Canad. ivied. Ass. J. 101: 129, 1969. 6. Frates, R. E.: Incompetence of the sphincter of oddi in the newborn, Radiology 85: 875, 1965. 7. Fred, L. H., Mayhall, C. G., and Harle, T. S.: Hepatic portal venous gas. A review and report on six new cases, Amer. J. Med. 44: 557, 1968. 8. Shaw, A., Cooperman, A., and Fusco, J.: Gas embolism produced by hydrogen peroxide, New Eng. J. Med. 277: 238, 1967. 9. Yenerman, M.: Report to the Fifth Congress of Int. Comp. Anat., Instanbul, 1949. 10. Bilger, M.: Pneumatosis cystoides intestinalis in children, J. PEDIAT. 49: 445, 1956. I1. Wiot, J. F., and Felson, B.: Gas in the portal venous system, Amer. J. Roentgen. 86: 920, 1961. 12. Schoor, S.: Small-intestinal intramural air, Radiology 81: 285, 1963. 13. Pagan-Carlo, J., and DeMony, E. H.: Hepatoportal pneumatosis with mesenterie venous thrombosis in an infant. Report of a case, Amer. J. Roentgen. 91: 699, 1964. I4. Verby, H. D., Castellino, R. A., Frledland, G. W., and Northway, W. H.: Portal vein gas complicating Hirschsprung's disease with enterocolitis, J. PEDIAT. 73: 599, 1968. i5. Goldstein, W. B. Cusmando, J. V., Gallagher, J. J., and Hemley, S.: Portal vein gas. A case report with survival, Amer. J. Roentgen. 97: 220, 1966.
255
Portal venous gas in the pediatric age group R e v i e w o[ the literature a n d r e p o r t o f t w e l v e n e w cases Thirty-one reported instances o[ portal venous gas are reviewed, and 12 new cases are added. The presence o[ portal venous gas is usually associated with sepsis, gastroenteritis, and abdominal distention in the first Jew weeks o[ life. The roentgenographic findings are typical and diagnostic. Ths pathogenesis is not completely understood, although in most situations underlying gastrointestinal mucosal destruction is [ound. Since the condition in reported cases has been almost uni[ormly [atal, a vigorous medical and surgical therapeutic approach is suggested.
Rica G. Arnon, M.D., and Judith F. Fishbein, M.D. BROOKLYN,
N . Y.
T r l E O C C U R R E N C E of gas in the portal system, first described in 1955,1 has been considered a rare entity. Nevertheless, within 24 months we have observed 12 new cases, which brings the total number of reported pediatric cases to 43. The prognosis in general is quite grave, and in our experience this finding has been associated with an almost 100 per cent mortality rate. However, a recent report by Stevenson and associates 2 demonstrated a better survival rate. It is our purpose to alert the pediatrician to the existence of this finding and to suggest a possible approach once the diagnosis is made. The presence of portal venous gas is not obvious if not specifically looked for. A roentgenogram of the abdomen and particularly of the right upper quadrant is the only means available to establish the diagnosis. The characteristic radiographic appearance is that of tubular lucencies branching from the From the Department of Pediatrics, Kings County Hospital, and State University o[ New York-Downstate Medical Center. Reprint address: Rica G. Arnon, Department oJ Pediatrics, Kings County Hospital, 451 Ctarkson Ave., Brooklyn, N. Y. 11203.
porta hepatis (Fig. 1) to the edge of the liver. The gas is carried to the periphery of the liver by the centrifugal flow of portal blood where it accumulates and outlines the finer peripheral portal radicals (Figs. 1 to 4). In contrast, intrabiliary gas accumulates in the major biliary radicals due to the centripetal flow of bile. 3 Portal venous gas may be confused with pneumatosis intestinalis and intraluminal gas. Pneumatosis intestinalis (Figs. 1, 3, and 4) and pneumoperitoneum are, nevertheless, frequently associated roentgenographic findings (Table I). The presence of portal venous gas has been described in association with various disease processes such as gastroenteritis, necrotizing enterocolitis, sepsis, and following umbilical vein catheterization (present series) 4-7 and peroxide enema. 's The prognosis in association with umbilical vein catheterization is considerably better (50 per cent survival). The clinical findings of patients with portal venous gas, excluding those following peroxide enema and umbilical vein catheterVol. 79, No. 2, pp. 255-259
256
Arnon and Fishbein
The ]ournaI o[ Pediatrics August 1971
Table I. Summary of findings in 43 patients with portal venous gas
l patients No. of l
Survived
Sepsis
Peroxide enema s
1
--
--
Umbilical vein catheterization ~4-7
6
35, 7
__
Necrotizing enteroeolitis in premature infants e2
10
32
Gastroenteritis and septicemiami, 5, 7, 11-15
23
3~5, 15
18
3
--
--
43
9
26
Associated with
U n c l a s s i f i e d 12
Total
Associated anomalies
T E fistula 4
8
Imperforate anus 1 Duodenal atresia 1 D u o d e n a l stenosis, m o n g o l 5 Malrotation*2, H i r s c h s p r u n g ' s d i s e a s e w i t h colitis~4 E s o p h a g e a l a t r e s i a 11 Esophageal atresia with double TE fistulas ~
GI anomalies
GI = gastrointestinal, TE = tracheoesophageal. ~Present series.
ization, have been nonspecific. The infants appeared critically ill, were acidotic, and often in shock. Frequently, they presented with gastroenteritis, bloody stools, and a distended abdomen. Autopsy and operative material were available in 35 patients. Ten patients (23 per cent) had undeHying congenital gastrointestinal anomalies; 28 patients had other pathologic findings in the gastrointestinal tract ranging from mild hyperemia of the mucosa to severe ulceration and gangrene of the bowel with perforation; 15 patients had peritonitis and 20 patients had pneumatosis intestinalis (Table I ) . In one of our 12 patients, the gas disappeared radiologically 8 days prior to death, and none could be found at the time of postmortem examination. In the rest of our patients, gas was readily observable in the mesenteric and portal veins and in the bowel wall. One infant also had gas in the inferior vena cava and a crepitant liver containing subcapsular air. The pathogenesis of portal venous gas is not well understood, except in cases in which
air is presumably introduced by an umbilical catheter or a peroxide enema. Wolfe and Evans 1 postulated that luminal gas under increased pressure passes through the bowel wall into the mesenteric veins and collects in the portal system. This has been partially supported by Yenerman, ~ who produced pneumatosis intestinalis experimentally in cadavers by increasing the intraluminal gas pressure in the presence of mucosal ulcerations. Bilger 1~ demonstrated that the composition of the submucosal gas in pneumatosis intestinalis may be compared to that of atmospheric air. Wiot and Felson *~ postulated that gasforming bacteria gives rise to portal venous gas. In their series all patients had antemortem or postmortem blood cultures positive for gas-forming organisms. The organisms were frequent inhabitants of the bowel and following distruption of the intestinal mucosa entered the portal venous system. These poorly substantiated theories are currently the only explanations available to account for the presence of air in the portal venous system. The absence of pathologic
Volume 79 Number 2
No. of patients
Pathology Meconium ileus
Necrosis of the intestine Pneumatosis intestinalis Perforation of intestine Peritonitis
10
Portal venous gas
No. o[ patients
Survivors Analysis
7 10 3 5
Removal of segment of necrotic bowel (4 deaths--involvement of entire intestine found at surgery)
3
Osteomyelitis, sepsis, and diarrhea 15 Malrotation with removal of segmental colitis5 Hirschsprung's disease with colitis and colostorny~
No GI mucosal pathology ~11 Other changes varied from hyperemia to ulceration necrosis and perforation of bowel Pneumatosis intestinalis Perforation Peritonitis
10 5 10
Pneumatosis intestinalis Peritonitis Perforation No GI pathology
20 15 8 3
findings in the gastrointestinal tract in some cases, 11 including the present series, is somewhat puzzling unless gaseous distention alone can force air into the submucosa without evidence of mucosal damage. The presence of portal venous gas often portends a fatal outcome, although the gas in itself is not the direct cause of death. Aside from cases secondary to umbilical catheterization, only 6 patients (14 per cent) have survived (Table I ) . Eight patients died one week or more after the diagnosis was established. However, the majority of patients lived only a few hours. The rapid progression and high mortality rate apparently reflect the natural history of the underlying disease process; therapy should be directed accordingly, both quickly and intensively. Most patients with portal venous gas had cultures positive for gram-negative organisms. Escherichia coli was found in 20. Aerobacter in 4, Proteus in 2, Candida in 2, and Salmonella in one. Vigorous antibiotic therapy aimed at the above organisms, guided later by sensitivity studies, should there-
257
I
No. of patients
1 1 1
fore be instituted at once in all patients with portal venous gas, realizing that gas may be introduced via an umbilical vein catheter in otherwise well babies. Fluid, electrolyte and acid-base balance, and neutral thermal environment must be maintained. Early surgical intervention, regardless of the clinical status of the patient, appears in some instances, 2, 5 including patients in our series, to have been life saving. It is imperative to determine the status of the bowel as soon as possible. Contrast studies of the gastrointestinal tract may be helpful in localizing and determining the nature of the underlying process. In a series of 21 patients with necrotizing enterocolitis, Stevenson and associates 2 described 6 patients with portal venous gas. Three patients survived and are still living following resection of the necrotic bowel. One of our patients with Hirschsprung's disease complicated by necrotizing enterocolitis and portal venous gas survived following a colostomy. In addition, perforation of the bowel and peritonitis were frequently noted during surgery, although there had been no preop-
258
Arnon and Fishbein
Fig. 1. A 4-week-old infant with sepsis, diarrhea, and distended abdomen. Film of right upper quadrant reveals distention of both small and large bowel. Gas is demonstrated within the bowel wall (lower arrow) as linear and circular lucencies outlining the lumen of the distended bowel. A large collection of gas is present in the portal vein (upper arrow), from which linear streaks of gas within the portal radicals are seen radiating to the periphery of the liver.
Fig. 2. A 20-day-old infant with chronic diarrhea, acidosis, and distended abdomen. Film of right upper q u a d r a n t reveals characteristic peripheral distribution of portal venous gas, in contrast to the central distribution of gas within the biliary system.
The ]ournal o[ Pediatrics August 1971
Fig. 3. A 6-week-old male with sepsis, diarrhea, and distended abdomen. Gas is seen within the wail of the bowel as well as within the perlpheral branches of the portal system. Since the bowel is seen en face, the intramural gas (arrows) assumes a circular configuration.
Fig. 4. A 6-week-old premature infant with diarrhea, dehydration, and distended abdomen. Film of the abdomen reveals marked distention of both small and large bowel. Pneumatosis is present in most of the viscera on the right. A considerable amount of gas is distributed in the portal radicals of the liver.
Volume 79 Number 2
erative radiologic evidence thereof. 2 Early surgical i n t e r v e n t i o n is therefore strongly reco m m e n d e d i n most instances of necrotizing enterocolitis with progressive pneumatosis intestinalis, especially with worsening of the clinical picture a n d / o r evidence of p n e u m o peritoneum. The authors wish to thank Dr. Jonathan T. Lanman for constructive criticism and Dr. Jack G. Rabinowitz for encouragement, guidance, and making available to us the records of the survivor with Hirschsprung's disease. REFERENCES
1. Wolfe, J. N., and Evans, W. A.: Gas in the portal veins of the liver in infants: A roentgenographic demonstration with postmortem anatomical correlation, Amer. J. Roentgen. 74: 486, 1955. 2. Stevenson, J. K., Graham, B. C., Oliver, T. K., and Goldberg, V. E.: Neonatal necrotizing enterocolitis. A report of twenty-one cases with fourteen survivors, Amer. J. Surg. 118: 260, 1969. 3. Susrnan, N., and Senturia, H. R.: Gas embolization of the portal venous system, Amer. J. Roentgen. 83: 847, 1960. 4. Schmidt, A. G.: Portal vein gas due to administration of fluid via the umbilical vein, Radiology 88: 293, 1967.
Portal venous gas
259
5. Miskin, M., and Reilly, B. J.: Gas in the intestinal wall and portal venous system in infants, Canad. ivied. Ass. J. 101: 129, 1969. 6. Frates, R. E.: Incompetence of the sphincter of oddi in the newborn, Radiology 85: 875, 1965. 7. Fred, L. H., Mayhall, C. G., and Harle, T. S.: Hepatic portal venous gas. A review and report on six new cases, Amer. J. Med. 44: 557, 1968. 8. Shaw, A., Cooperman, A., and Fusco, J.: Gas embolism produced by hydrogen peroxide, New Eng. J. Med. 277: 238, 1967. 9. Yenerman, M.: Report to the Fifth Congress of Int. Comp. Anat., Instanbul, 1949. 10. Bilger, M.: Pneumatosis cystoides intestinalis in children, J. PEDIAT. 49: 445, 1956. I1. Wiot, J. F., and Felson, B.: Gas in the portal venous system, Amer. J. Roentgen. 86: 920, 1961. 12. Schoor, S.: Small-intestinal intramural air, Radiology 81: 285, 1963. 13. Pagan-Carlo, J., and DeMony, E. H.: Hepatoportal pneumatosis with mesenterie venous thrombosis in an infant. Report of a case, Amer. J. Roentgen. 91: 699, 1964. I4. Verby, H. D., Castellino, R. A., Frledland, G. W., and Northway, W. H.: Portal vein gas complicating Hirschsprung's disease with enterocolitis, J. PEDIAT. 73: 599, 1968. i5. Goldstein, W. B. Cusmando, J. V., Gallagher, J. J., and Hemley, S.: Portal vein gas. A case report with survival, Amer. J. Roentgen. 97: 220, 1966.