The Journal of Pediatrics
Volume 156, Issue 6 , Pages 1031-1031.e2, June 2010

Renal, Pancreatic, Splenic, Mesenteric, and Portal Venous Gas as a Sign of Intestinal Necrosis

Department of Pediatric Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

Department of General Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

Department of Medical Imaging, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

published online 27 January 2010.

Article Outline

 

A 14-year-old boy presented with acute abdominal distention causing a severe restrictive respiratory distress that necessitated endotracheal intubation, diffuse peritoneal irritation, and profound metabolic acidosis. The boy had Waardenburg syndrome, a rare autosomal dominant inherited disorder, with severe hypopigmentation, hearing loss, limb abnormalities, and mental retardation, but without previously identified gastrointestinal involvement.1

With an abdominal radiograph, diffuse gastrointestinal distention, portal venous gas, and gas in the splenic and left renal venous systems was shown (Figure 1). With a computed tomography scan, extensive pneumatosis intestinalis, air in the portal, mesenteric, splenic, and left renal veins, within both kidneys, and in the pancreas was revealed (Figure 2; available at www.jpeds.com). Emphysematous gastritis and pan-intestinal necrosis was revealed with a laparotomy (Figure 3; available at www.jpeds.com). The patient died a few hours after surgery.

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  • Figure 1 

    A, Supine abdominal radiograph demonstrates marked distension of the stomach and small bowel and pneumatosis intestinalis (white arrows). Air is seen in the branches of the portal veins (black arrows) in the B, liver (L), C, spleen (Sp), and along the course of the splenic vein (spl v) and the portal vein (pv).

Portal venous gas can be a result of intestinal ischemia, indicating urgent surgical intervention, or of non-intestinal etiologies, in which case it may respond to conservative treatment. Theories about the pathogenesis of portal air range from mechanical disruption of gut barrier to vascular spread of gas-forming bacteria.2, 3 In patients who are malnourished, there is a high mortality rate.4

Gastrointestinal involvement is rare in patients with Waardenburg syndrome,5 and our patient had no previously identified gastrointestinal tract involvement. However, in retrospect, gastrointestinal distension was seen on earlier thoraco-lumbar spine radiographs. It appears that a prolonged state of malnutrition and extensive gastrointestinal distension with a presumed vascular compromise caused by mechanical compression of the mesenteric vessels all contributed to our patient's severe condition.

The presence of air in the renal vein might indicate an intrahepatic or an extrahepatic connection between the portomesenteric and the systemic venous systems.6, 7 The delineation of the portomesenteric venous system with air showed normal anatomy, with no evidence of air in the hepatic venous system or in the inferior vena cava and without signs of portal hypertension. The possibilities of portosystemic shunts are, therefore, unlikely.

Air in the renal vein has been reported after nephrectomy8 or emphysematous pyelonephritis.9 However, this is the first report of pancreatic and renal venous gas spread associated with extensive pneumatosis intestinalis and portomesenteric and splenic venous gas. These findings emphasize the ominous features associated with portal venous gas, especially when there is such an extensive spread of gas to other vessels.

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Figure 2 

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  • A-C, Non-enhanced axial computed tomography scan with D, coronal reformats, showing marked gastric dilatation (St) and pneumatosis (white arrows) in the A, stomach wall and B, small intestinal wall (SB). A, B, D, Extensive portal venous gas (black arrows) is seen in the liver (L), B and D, and venous air (black arrows) within the spleen (Sp), the kidneys (K), and the pancreas (P), B, along the splenic vein (spl v) and the pancreatic veins, and C, in the renal veins (arrowheads).

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Figure 3 

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  • Intraoperative photograph shows marked distension of the stomach (St) and necrotic small bowel loops (SB), with air bubbles (white arrows) on the small bowel outer wall.

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References 

  1. Zlotogora J, Lerer I, Bar-David S, Ergaz Z, Abeliovich D. Homozygosity for Waardenburg syndrome. Am J Hum Genet. 1995;56:1173–1178
  2. Abboud B, El Hachem J, Yazbeck T, Doumit C. Hepatic portal venous gas: physiopathology, etiology, prognosis and treatment. World J Gastroenterol. 2009;15:3585–3590
  3. Nelson AL, Millington TM, Sahani D, Chung RT, Bauer C, Hertl M, et al. Hepatic portal venous gas: the ABCs of management. Arch Surg. 2009;144:575–581discussion 81
  4. Dagan R, Ben-Yacov O, Mares AJ, Moses SW, Bar-Ziv J. Necrotizing enterocolitis beyond the neonatal period. Eur J Pediatr. 1984;142:56–58
  5. Read AP, Newton VE. Waardenburg syndrome. J Med Genet. 1997;34:656–665
  6. Gallego C, Miralles M, Marin C, Muyor P, Gonzalez G, Garcia-Hidalgo E. Congenital hepatic shunts. Radiographics. 2004;24:755–772
  7. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat. 2008;21:147–157
  8. Martay K, Dembo G, Vater Y, Charpentier K, Levy A, Bakthavatsalam R, et al. Unexpected surgical difficulties leading to hemorrhage and gas embolus during laparoscopic donor nephrectomy: a case report. Can J Anaesth. 2003;50:891–894
  9. Joris L, van Daele G, Timmermans U, Rutsaert RJ. Emphysematous pyelonephritis. Intensive Care Med. 1989;15:206–208

PII: S0022-3476(09)01086-5

doi:10.1016/j.jpeds.2009.11.001

The Journal of Pediatrics
Volume 156, Issue 6 , Pages 1031-1031.e2, June 2010

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