PNEUMATOSIS INTESTINALIS IN THE NEONATE*

Transcription

1 VOL. 120, No. 2 PNEUMATOSIS INTESTINALIS IN THE NEONATE* By ARVIN E. ROBINSON, M.D.,t HERMAN GROSSMAN, M.D., and GEORGE W. BRUMLEY, M.D. \ ATHILE recent emphasis has been on V V the association of pneumatosis intestinalis (intramural bowel gas) with necrotizing enterocolitis in the premature infant, other diseases during the neonatal period can display air within the wall of the bowel. In describing necrotizing enterocolitis, Berdon et al.4 stressed that pneumatosis intestinalis was not a disease, but a manifestation of an underlying diseased bowel. Recent reports have regarded pneumatosis intestinalis in the neonate as the roentgenographic manifestation of necrotizing enterocoli tis. 273 However, pneumatosis intestinalis can also be associated with other neonatal disease states presenting with intestinal obstruction or vascular compromise. The clinical presentation, diagnostic studies, treatment and prognosis vary with the underlying pathology and not with the presence or degree of intramural bowel air. The purpose of this report is to emphasize the consideration of mechanisms other than necrotizing enterocolitis that can lead DURHAM, NORTH CAROLINA to the appearance of pneumatosis intestinalis. Although no attempt was made to obtain a complete statistical series related to the incidence of pneumatosis intestinalis among various neonatal conditions, the clinical features and roentgenographic recommendations related to these different diseases will be emphasized. PREDISPOSING CONDITIONS A. INTESTINAL OBSTRUCTION Pneumatosis is occasionally seen with abdominal distention secondary to intestinal obstruction in the newborn period It may develop from the escape of intestinal gas through mucosal tears with subsequent submucosal and/or subserosal dissection, or possibly be related to bowel wall ischemia from marked overdistention. The pneumatosis in nonresected bowel is noted to regress spontaneously when the obstruction is relieved. In cases of intestinal obstruction, pneumatosis can be located in the more dilated proximal bowel anywhere along the gastrointestinal tract. One patient with air in the wall of the stomach was a newborn with duodenal stenosis (Fig. i, 4 and B). In spite of marked distention with small bowel atresia, pneumatosis appears to occur less frequently in small bowel obstructions than in colon obstructing lesions Intramural bowel air has been noted in patients with imperforate anus (Fig. 2), meconium ileus (Fig. 3), I patient with meconium plug syndrome (Fig., 4 and B), and neonatal aganglionic megacolon (Fig., 4-C). When pneumatosis is seen in association with Hirschsprung s disease, the possibility of superimposed inflammation, a serious complication, must also be considered.32 B. VASCULAR COMPROMISE There has been recent evidence relating bowel wall ischemia to the development of pneumatosis by various mechanisms. One popular theory draws an explanation from the normal diving mechanism observed in some aquatic mammals where splanchnic blood flow is shifted to more oxygen dependent regions under stress. 3 This mechanism, when combined with respiratory insufficiency and hypoxia, would shunt blood flow away from the splanchnic bed with resultant bowel wall ischemia. Diminished flow may be accentuated by additional ob- * Presented at the Fifteenth Annual Meeting of the Society for Pediatric Radiology, Washington, D. C., October 1-2, From the Departments of Radiology and Pediatrics, Duke University Medical Center, Durham, North Carolina. t Advanced Fellow in Academic Radiology of the James Picker Foundation as recommended by the NRC/NAS. 333

2 334 A. E. Robinson, H. Grossman and G. W. Brumley FEBRUARY, FIG. I. Newborn infant with Down s syndrome, complicated by marked duodenal stenosis. Gas can be seen in the wall of the distended stomach in the (A) upright and (B) supine projections. The pneumatosis resolved when the obstruction was relieved. struction, such as an umbilical artery catheter impeding blood flow or an umbilical vein catheter partially obstructing portal venous return. This combination of hypoxia and portal obstruction may precipitate bowel ischemia and subsequent pneumatosis intestinalis. Recent emphasis has been placed on the danger of major vessel thrombosis and embolization from indwelling umbilical catheters Pneumatosis intestinalis was observed following long term umbilical artery catheterization for fluid and calorie administration and blood gas monitoring in a premature infant. A transumbilical aortogram revealed thrombus formation in the thoracic and abdominal aorta with occlusion of the inferior mesenteric artery (Fig. 6, 4 and B). This prompted the performance of aortography on II other infants with indwelling arterial catheters. Two additional instances of thrombus formation were detected in infants without clinical evidence of vascular compromise. As indicated in other studies, large vessel thrombosis can be precipitated by monitoring catheters or dehydration with resultant bowel ischemia and pneumatosis. Unfortu- #{149} FIG. 2. This newborn male presented with distention from a high imperforate anus. A catheter was passed through the urethral-rectal fistula. Contrast medium filling of the distal colon demarcates air within the wall of the bowel anteriorly along the contrast medium column as well as along the more proximal distended loops.

3 VOL. 120, No. 2 Pneumatosis Intestinalis in the Neonate 335 nately, some patients with pneumatosis intestinalis secondary to umbilical artery catheters have been included in series of infants having necrotizing enterocoa disease process not characterized by large vessel thrombosis. These vascular accidents usually present later in the newborn period than primary necrotizing enterocolitis and can involve full term infants as well as prematures. Other causes of vascular compromise, arterial and venous, exist in the newborn infant. Although uncommon, intussusception can occur in the newborn period and will occasionally produce enough ischemia to demonstrate pneumatosis intestinalis and subsequent stricture formation (Fig. 7, 4-C). Similarly, pneumatosis can be seen in malrotation with volvulus24 or a shortened vascular pedicle, and in postoperative abdomens where ischemic bowel has been left behind. Venous obstruction has also been implicated in bowel ischemia. Most of the attention has been placed on large and small bowel perforations following umbilical p FIG. 3. Newborn with meconium ileus obstruction and pneumatosis intestinalis involving more proximal bowel segments, best seen in the left lower quadrant. The presence of pneumatosis was confirmed at autopsy. (Courtesy of Dr. Wisniewski, St. Vincent s Hospital, New York City.) FIG.. (A and B) One day old near term infant with abdominal distention and hyperactive bowel sounds. Small bubbles in the right lower quadrant represent pneumatosis intestinalis. Barium enema examination showed a normal appearing rectum. A 6 inch meconium plug passed on evacuation of the barium. Since cystic fibrosis was excluded by repeated sweat chloride determinations, and there was no evidence of neonatal aganglionic megacolon on subsequent barium enema studies or clinical examination, a diagnosis of meconium plug syndrome was entertained. vein catheterizations for exchange transfusion \Ve observed an infant who developed gastric pneumatosis with bloody gastric aspirate week after an exchange transfusion for hperbilirubinemi a secondary to sepsis. The transfusion catheter was positioned inadvertently in a hepatic portal vein (Fig. 8, 4 and B). Transient gastric pneumatosis can be explained by

4 336 A. E. Robinson, H. Grossman and G. \V. Brumley FEBRUARY, 1974

5 Voi.. 120, No. 2 Pneumatosis Intestinalis in the Neonate 337 altered portal hemodynamics with increased portal pressure and congestion. C. INFLAMMATION Necrotizing enterocolitis should be considered as a distinct clinical entity of premature infants. The onset is usually 3-10 days of age, although occasionally it can be as late as 3 weeks. #{176}The presenting symptoms are abdominal distention, bile stained emesis, and blood in the stool. When the disease progresses, jaundice, sepsis and shock are usually terminal events. Pneumatosis intestinalis is often evident early in FIG. 6. This full term infant suffered perinatal asphyxic brain damage with subsequent aspiration pneumonia He was maintained on hypertonic feedings via variably positioned umbilical artery catheters. (A) Abdominal distention with pneumatosis intestinalis developed 24 days after the initial catheter insertion. (B) An umbilical catheter aortogram revealed mural thrombi within the thoracic and abdominal aorta with occlusion of the inferior mesenteric artery. These findings were confirmed at autopsy 2 days later.

6 338 A. E. Robinson, H. Grossman and G. W. Brumley FEBRUARY, 1974 FIG. 8. This premature infant was meconium stained at birth and developed beta-streptococcal septicemia and jaundice shortly thereafter. (A) An exchange transfusion was performed through an umbilical vein catheter inadvertently placed in an hepatic portal venous radical (catheter retouched). (B) l he bacteremia responded promptly to antibiotic therapy, but subsequent examination revealed pneumatosis localized to the stomach. The air within the gastric wall cleared spontaneously and the baby recovered uneventfully. the clinical course (Fig. 9) In some instances, hepatic portal venous gas, an ominous sign, may be more obvious than the pneumatosis (Fig. 10, 4 and B).8 35 Intestinal perforation requires surgical intervention; the prognosis depends on the extent of diseased bowel and the patient s general condition. Varied opinions have been offered as etiologic mechanisms.4 22 Major vessel thromboses are not seen in these babies, although vascular occlusion of small arteries or capillaries as a secondary phenomenon is likely. A pneumatosis-like picture has been reproduced in animals with the intramural injection of gram-negative gas forming organisms, but not with the injection of air alone.28 D. OTHER CAUSES Pneumomediastinum and hyperaerated pulmonary states have been known to precipitate benign pneumatosis intestinalis in adults.7 The mechanism postulated is dissection of air from the mediastinum along the aorta and its tributaries to the bowel wall. The first report attributing pneumatosis in an infant to pneumothorax and pneumomediastinum also had a history of FIG.. This premature infant presented with bile stained emesis, distention, and blood in the stool characteristic of necrotizing enterocolitis. A lateral roen tgenographic examination revealed pneumatosis intestinalis.

7 VOL. 120, No. 2 Pneumatosis Intestinalis in the Neonate 339 an indwelling umbilical venous catheter for 3 days and blood tinged stool. 2 Death, 21 days after the onset, may have revealed pneumatosis as the lone residual of either vascular compromise or necrotizing enterocolitis. Gas can be introduced into the portal venous system accidentally A small amount of air will often enter through a poorly positioned umbilical venous catheter. A large amount can occasionally be seen with no apparent ill effect. Gas can also enter through an intact mucosa, as seen in hydrogen peroxide enemas both clinically and experimentally.25 DIAGNOSTIC AND I CONSIDERATIONS PROGNOSIS Congenital obstructive processes are usually apparent in the first 24 hours after birth and can usually be well delineated early with an appropriate contrast medium examination. Pneumatosis intestinlis associated with large vessel compromise usually has its onset of symptoms and physical findings many days after birth. When an umbilical artery catheter is suspected as the cause of major vessel occlusion, an umbilical artery catheter angiogram may be helpful in identifying an obstructing thrombus.

8 340 A. E. Robinson, H. Grossman and G. \V. Brumley FEBRUARY, 1974 In premature infants with a clinical picture of necrotizing enterocolitis, a contrast medium colon study should be avoided, since it may precipitate a perforation through the friable bowel wall. Barium enema studies are useful, however, following resolution of the acute symptoms if the patient becomes obstipated or obstructed, since stenosis is a complication of bowel ischemia.1#{176} In infants with intestinal obstruction and intramural air associated with overdistention, the pneumatosis spontaneously clears when the obstruction is corrected. In such cases, the prognosis is dependent on the primary obstructive process and any associated anomaly. When large vessel thrombosis occurs, localized areas of infarcted bowel must be removed. Successful thrombolectomy has not been reported in such instances. Infants with necrotizing enterocoli tis often are septic or their general condition is poor. Antibiotics, blood transfusions, and fluid replacement have been the supportive therapy used. While some surgeons do not recommend surgical exploration unless there are signs of perforation, as demonstrated by free intraperitoneal air,31 others believe that improved survival rates can be achieved by early surgical resection of localized areas of necrotizing enterocolitis. 27 If this aggressive approach is taken, surgery would be considered at the first roentgenographic sign of intramural air in association with bile in the stomach and blood in the colon. If ischemic areas of bowel recover without surgery, focal areas of strictured bowel can develop, #{176} #{176} 2#{176} similar to those noted in adults with vascular thromboses. The strictured areas are resected when signs of obstipation or obstruction develop. SUMMARY Pneumatosis intestinalis (intramural bowel air) can be seen in several disease processes during the neonatal period, notably mechanical obstruction, vascular compromise, and necrotizing enterocoli tis. Pneumatosis intestinalis seen with mechanical obstruction is often uneventful and self-limiting when the obstruction is relieved. Necrotizi ng en terocoli tis will usually have a characteristic presentation of early onset in premature infants with distention, bile-stained emesis, and blood in the stool. Vascular thromboses with subsequent bowel ischemia can mimic necrotizing enterocolitis, but will occur later in the newborn period, may be localized to small bowel, and may be associated with indwelling umbilical artery catheters. Recovery is often complicated by indeterminate periods of malabsorption. The diagnosis of the underlying disease leading to pneumatosis intestinalis is necessary for proper management. Arvin E. Robinson, M.D. Department of Radiology Duke University Medical Center Durham, North Carolina REFERENCES i. ARNON, R. C., and lishbein, J. F. Portal venous gas in pediatric age group. 7. Pediat., 1971, 79, BEHRMAN, R. E., LEES, M. H., PETERSON, E. N., DE LAN NOY, C. W., and SEEDS, A. E. Distribution of circulation in normal and asphyxiated fetal primate. Am. 7. Obst. & Gynec., 1970, zo8, BELL, R. S., GRAHAM, C. B., and STEVENSON, J. K. Roentgenologic and clinical manifestations of neonatal necrotizing enterocolitis: experience with 3 cases. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1971, 112, BERDON, W. E., GROSSMAN, H., BAKER, D. H., MIZRAHI, A., BARLOW, 0., and BLANC, W. A. Necrotizing enterocolitis in premature infant. Radiology, 1964, 83, CASTOR, W. R. Spontaneous perforation of bowel in newborn following exchange transfusion. Canad. M. A. 7., 1968, 99, CORKERY, J. J., DUBOwITZ, V., LISTER, J., and MOOSA, A. Colonic perforation after exchange transfusion. Brit. M. 7., 1968, 4, ECKERT, J. F., MARRIOTT, J. D., and SMITH, B. H. Exercise in radiologic-pathologic correlation: case of month from AFIP. Radiology, 1968, 9!,

9 VOL. 120, No. 2 Pneumatosis Intestinalis in the Neonate FENTON, J. L., REYNOLDS, W. A., and HARRIS, C. H. Intramural intestinal gas in infants: report of two cases. 7. Canad. A. Radiologists, 1969, 20, FRIEDMAN, A. B., ABELLERA, R. M., LID5KY, I., and LUBERT, M. Perforation of colon after exchange transfusion in newborn. New England 7. Med., 1970, 282, GROSSMAN, H., WINCHESTER, P. H., and BRILL, P. Advances in pediatric radiology. Advances Pediat., 1971, I, II. HOPKINs, G. B., GOULD, W. E., STEVENSON, J. K., and OLIVER, T. K. Necrotizing enterocolitis in premature infants. A.M.A. Am. 7. Dis. Child., 1970, 120, LEE, S. B., and KUHN, J. P. Pneumatosis intestinalis following pneumomediastinum in newborn infant. 7. Pediat., 1971, 79, LLOYD, J. R. Etiology of gastrointestinal perforation in newborn. 7. Pediat. Surg., 1969, 4, MI5H KIN, M., and REILLY, B. J. Gas in intestinal wall and portal venous system in infants. Canad. M. A. 7., 1969, 10!, MoRisoN, J. F. Thrombosis of aorta in newborn. 7. Pathology, 1945, 57, i6. NEAL, W. A., REYNOLDS, J. W., JARVIS, C. W., and WILLIAMS, H. J. Umbilical artery catheterization: demonstration of arterial thrombosis by aortography. Pediatrics, 1972, 50, NELSON, S. W. Extraluminal gas collections due to diseases of gastrointestinal tract. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR. MED., 1972, 115, i8. ORME, R. L., and EADES, S. M. Perforation of bowel in newborn as complication of exchange transfusion. Brit. M. 7., 1968, 4, !. 19. POCHACZEVSKY, R., and KASSNER, E. G. Necrotizing enterocolitis of infancy. AM. J. ROENT- GENOL., RAD. THERAPY & NUCLEAR MED., 197!, 113, RABINOWITZ, J. G., WOLF, B. S., FELLER, M. R., and KRASNA, I. Colonic changes following necrotizing enterocolitis in newborn. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1968, 103, !. RIGLER, L. G., and POGUE, W. L. Roentgen signs of intestinal necrosis. AM. J. ROENT- GENOL., RAD. THERAPY & NUCLEAR MED., 1965, 94, SEAMAN, W. B., FLEMING, R. J., and BAKER, D. H. Pneumatosis intestinalis of small bowel. Seminars Roentgenol., 1966, i, SCHMIDT, A. G. Portal vein gas due to administration of fluids via umbilical vein. Radiology, 1967, 88, SCHORR, S. Small intestinal intramural air. Radiology, 1963, 8z, SHAW, A., COOPERMAN, A., and FUSCO, J. Gas embolism produced by hydrogen peroxide. New England 7. Med., 1967, 277, SHAW, D. G. Intrahepatic gas shadows in neonatal duodenal obstruction. Arch. Dis. Childhood, 1972, 47, STEVENSON, J. K., GRAHAM, C. B., OLIVER, F. K., and GOLDENBERG, V. E. Neonatal necrotizing enterocolitis. Am. 7. Surg., 1969, zz8, STONE, H. H., ALLEN, W. B., SMITH, R. B., and HAYNES, C. D. Infantile pneumatosis intestinalis. 7. Surg. Research, 1968, 8, SWAIN, T. J., and GERALD, B. Hepatic portal venous gas in infants without subsequent death. Radiology, 1970, 94, TOOLEY, W. H. What is risk of umbilical artery catheter? Pediatrics, 1972,50, !. TOULOUKIAN, R. J., BERDON, W. E., AMOURY, R. A., and SANTULLI, T. V. Surgical experience with necrotizing enterocolitis in infant. 7. Pediat. Surg., 1967, 2, VERBY, H. D., CASTELLINO, R. A., FRIEDLAND, G. W., and NORTHWAY, W. H. Portal vein gas complicating Hirschsprung s disease with enterocolitis. 7. Pediat., 3968, 73, WALDHAUSEN, J. A., HERENDEEN, T., and KING, H. Necrotizing colitis of newborn: common cause of perforation of colon. Surgery, 1963, 54, WIGGER, H. J., BRANSILVER, B. R., and BLANC, W. A. Thromboses due to catheterization in infants and children. 7. Pediat., 1970,76, 1-I I. 35. WILLIAMS, H. J., JARVIS, C. W., NEAL, W. A., and REYNOLDS, J. W. Vascular thromboembolism complicating umbilical artery catheterization. AM. J. ROENTGENOL., RAD. THERAPY & NUCLEAR MED., 1972, ii#{243}, WOLFE, J.., and EVANS, W. A. Gas in portal veins of liver in infants: roentgenographic demonstration with postmortem anatomical correlation. AM. J. ROENTGENOL., RAD. THER- APY & NUCLEAR MED., 1955, 74,

10 This article has been cited by: 1. Cw Wu, Cm Chen, St Chia, Cm Wang, Hj Huang, Mj Tsai X-Ray Quiz: A Full-Term Newborn with Bloody Stool. Hong Kong Journal of Emergency Medicine 23:1, [Crossref] 2. Courtney A. Coursey, Caroline L. Hollingsworth, Cooper Wriston, Craig Beam, Henry Rice, George Bisset, III Radiographic Predictors of Disease Severity in Neonates and Infants With Necrotizing Enterocolitis. American Journal of Roentgenology 193:5, [Abstract] [Full Text] [PDF] [PDF Plus] 3. Monica Epelman, Alan Daneman, Oscar M. Navarro, Iris Morag, Aideen M. Moore, Jae Hong Kim, Ricardo Faingold, Glenn Taylor, J. Ted Gerstle Necrotizing Enterocolitis: Review of State-of-the-Art Imaging Findings with Pathologic Correlation. RadioGraphics 27:2, [Crossref] 4. A. Abhyankar, J.J. Corkery, A.D. Lander Postoperative pneumatosis intestinalis in infants does not automatically preclude enteral feeding. Journal of Pediatric Surgery 36:12, [Crossref] 5. Juliette C. Stefanski, Avinash K. Shetty Abdominal Pain in a Girl with Juvenile Dermatomyositis. Clinical Pediatrics 37:9, [Crossref] 6. M.L.K. Tang, Larry W. Williams Pneumatosis intestinale in children with primary combined immunodeficiency. The Journal of Pediatrics 132:3, [Crossref] 7. K.W. West, F.J. Rescorla, J.L. Grosfeld, D.W. Vane Pneumatosis intestinalis in children beyond the neonatal period. Journal of Pediatric Surgery 24:8, [Crossref] 8. A. Daneman, S. Woodward, M. de Silva The radiology of neonatal necrotizing enterocolitis (NEC) A review of 47 cases and the literature. Pediatric Radiology 7:2, [Crossref] 9. Morton A. Meyers, Gary G. Ghahremani, James L. Clements, Kenneth Goodman Pneumatosis intestinalis. Gastrointestinal Radiology 2:1, [Crossref] 10. S.B. Feinberg, M.Z. Schwartz, S. Clifford, H. Buchwald, R.L. Varco Significance of pneumatosis cystoides intestinalis after jejunoileal bypass. The American Journal of Surgery 133:2, [Crossref] 11. William W. Olmsted, John E. Madewell Pneumatosis cystoides intestinalis: A pathophysiologic explanation of the roentgenographic signs. Gastrointestinal Radiology 1:1, [Crossref] 12. John C. Leonidas, Robert T. Hall Neonatal pneumatosis coli: A mild form of neonatal necrotizing enterocolitis. The Journal of Pediatrics 89:3, [Crossref] 13. John H. Vollman, Wilbur L. Smith, Reginald C. Tsang Necrotizing enterocolitis with recurrent hepatic portal venous gas. The Journal of Pediatrics 88:3, [Crossref]