Portal venous gas in intestinal malrotation with mild midgut volvulus
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Portal venous gas has been considered as a radiological sign requiring urgent operative intervention; however, the reports concerning portal venous gas associated with favorable outcome are recently increasing.
We describe a 9-month-old boy with acute onset high fever and vomiting. The ultrasonography demonstrated micro-gas bubbles continuously floating in the intrahepatic portal vein. Contrast-enhanced CT, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Detorsion and Ladd’s procedure were completed laparoscopically.
Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Ultrasonography can be a sensitive detector to visualize such small amounts of gas.
KeywordsPortal venous gas Malrotation Midgut volvulus Laparoscopic operation
Portal venous gas
Portal venous gas (PVG) has been a well-known radiological finding, suggesting a potentially severe underlying abdominal disease requiring urgent operative intervention that occurs both in pediatric and adult populations . Recently, spread of advanced imaging techniques has resulted in increased reports concerning PVG associated with not only severe or lethal conditions but also benign ones or diagnosed at much earlier stages . The most reported cases concerning PVG with intestinal malrotation are associated with bowel necrosis induced by midgut volvulus [1, 3, 4, 5, 6, 7]. We describe a rare case of intestinal malrotation with mild volvulus who was diagnosed with air bubbles floating in the intrahepatic portal vein detected by bedside emergency ultrasonography.
A 9-month-old male infant weighing 8450 g presented to the primary care pediatrician with acute onset high fever, non-bilious vomiting, and continuous crying in a glum mood. He showed no bloody stool. On clinical examination, his abdomen was almost flat and it seemed that there is no apparent tenderness but he is crying constantly.
Blood investigations revealed no remarkable inflammation, and the white cell count was 7700/mm3 and CRP was 0.35 mg/dl (< 0.14) with normal coagulation parameters. Liver function tests showed mild transaminase elevations with ALT 57 U/L (10–42), AST 71 U/L (13–30), and normal level of total bilirubin 0.7 mg/dl (0.4–1.5). Blood urea was 3.5 mg/dl (< 20), and creatinine 0.26 mg/dl (< 1.07). Serum CK level was in the normal range with 237 U/L (59–248).
Additional file 1: Video S1. Abdominal Ultrasound. The micro-bubbles of gas were detected as highly echogenic particles flowing within the intrahepatic portal vein. (MPG 7968 kb)
The postoperative course was uneventful, and the patient was discharged at the sixth postoperative day.
In the pediatric population, the presence of hepatic portal venous gas (PVG) has been described in association with various disease processes such as necrotizing enterocolitis, gastroenteritis, following umbilical catheter, bowel obstruction, Hirschsprung’s disease, duodenal stenosis, SMA syndrome, and hypertrophic pyloric stenosis [1, 8].
There are four main theories that explain the pathophysiology of PVG: (1) bacterial—intramural gas-forming bacterial proliferation, (2) mechanical—increased intraluminal pressure during gastric or intestinal obstruction, (3) mucosal damage—air enters through disrupted mucosa, and (4) pulmonary disease—alveolar air dissects down through the mediastinum to the gastric wall . In some cases, these factors appear to contribute in combination .
Intramural gas bubbles detected as pneumatosis intestinalis (PI) may be absorbed into the intestinal venous system, may travel into the portal vein, and can be localized as PVG by real-time ultrasound as flowing echogenic dots. Finally, PVG is trapped in the small branches of the portal vein inside the liver inducing dense granular echogenicities in hepatic parenchyma .
Reports of PVG as well as PI detected in the case of intestinal malrotation are rare, and the most reported cases showed intestinal necrosis that necessitated bowel resection and showed poor prognosis [1, 4, 5, 6, 7].
In the present case, the micro-bubbles of gas were detected as highly echogenic particles flowing within the intrahepatic portal vein, and this become an opportunity for further evaluation and the intestinal malrotation was diagnosed. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Raised intraluminal pressure or direct stimulation of the bowel wall induced by the volvulus might allow gas to infiltrate the bowel wall and mesentery portal venous flow. Therefore, PVG in this case appeared to be in a transient process that resolves within a short interval after spontaneous winding down or decompression of twisted bowels.
Recent literatures have stated that midgut volvulus in malrotation can be managed well in infants without deteriorating condition. The laparoscopic approach is feasible and effective for the case that even shows PVG, and should have the advantage of minimally invasive surgery with small incisional scar.
We reported a case of intestinal malrotation detected with air bubbles floating in the intrahepatic portal vein by ultrasonography. Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Sonography is very sensitive for PVG detection even with very small amounts of gas, and this sign could be one of the useful markers for detecting the early stage of volvulus.
RH, HK, TI, and KI determined the therapeutic strategy. NM, MI, and RM determined the diagnostic US. RH, HK, and KI performed the surgery, and contributed to the perioperative care. RH wrote the draft of the manuscript. AI revised the article. All authors read and approved the final manuscript.
None of the authors have anything to disclose.
Ethics approval and consent to participate
Informed consent was obtained from the parents of the patient.
Consent for publication
Written informed consent was obtained from the parents of the patient for the publication of this case report and any accompanying images.
The authors declare that they have no competing interests.
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