Isolated superior mesenteric venous thrombophlebitis with acute appendicitis

Abstract

INTRODUCTION

Isolated superior mesentericveinous thrmbophlebitis is a rarely recognised condition associated with a high morbidity. It usually develops secondary to infection in the drainage area of the portal venous system, like appendix.

PRESENTATION OF CASE

We report a case of neglected perforated acute appendicitis complicated by superior mesenteric venous pyelephlebitis patiant represented with a vague pain to right of umlicus, which is atypical this why cat scan was done and showed obstructed superiormesentric vein, portal vein was free with acute appendicitis. Appendicectomy and treatment with broad-spectrum antibiotics, anticoagulation, and platelets led to a full recovery. Follow-up imaging after one month revealed complete canalization of superior mesentric vein.

DISCUSSION

Abdominal pain if atypical like our case report need imaging diagnosis. Modern diagnostic imaging techniques help the early diagnosis of acute phase pylephlebitis. CT can detect primary source of infection, extent of pylephlebitis, CT scan is the most reliable initially. Ultrasound scan with color flow Doppler is also a sensitive test for confirming partial patency of the portal vein and portal vein thrombosis accidentally discovered complete obliteration of superior mesenteric vein with thrombosis which remained not propagated by serial Doppler ultrasound of liver.

Appropriate treatment should be initiated as soon as possible. To avoid extension to portal vein. The principal treatment for pylephlebitis is to remove the source of infection as appendicectomy.

Anticoagulants must be used. Regarding the treatment of portal thrombosis, post operative use of heparin has been advocated.

CONCLUSION

Cat scan play an important role in case of atypical abdominal pain.

1. Introduction

Superior mesenteric venous thrombophlebitis is uncommon. It is usually secondary to infection in the drainage area of the portal venous system. Hepatic abscess formation may occur if the thrombus extends through the portal vein. Clinical diagnosis is almost impossible but preoperative diagnostic imaging may increase recognition.

2. Case report

A 28-year-old Indian male presented with a 10 day history of vague, dull aching abdominal pain, mainly periumbilical. He complained of nausea but no vomiting or anorexia. His bowel habit was unremarkable and he had no urinary symptoms. On admission his temperature was 38 degrees, heart rate 90 beats per minute, and blood pressure 116/60 mmHg. His abdomen was lax, only tender to the right of umbilicus near the midline. Investigations revealed an elevated white cell count (17.8 × 10³/μL). His liver function tests were deranged, with mildly elevated AST (65 U/L), ALT (60 U/L) and bilirubin (30 μmol/L). His alkaline phosphatase levels were raised (316 U/L). An abdominal computed tomography scan with contrast demonstrated extensive superior mesenteric venous (SMV) thrombosis with acute appendicitis. Duplex ultrasound examination suggested complete SMV occlusion. A gangrenous appendix was removed the next day. No bowel ischaemia was detected. Post-operatively, he was heparinized and subsequently warfarinized. The warfarin was discontinued after one month. Follow-up duplex ultrasound demonstrated recanalization of the SMV.

3. Discussion

Pylephlebitis is defined as serious septic thrombophlebitis of the portal vein or one of its tributaries, e.g. superior mesenteric vein. It usually develops secondary to infection in the drainage area of the portal venous system,¹ typically appendicitis or diverticulitis. SMV thrombosis is an uncommon cause of bowel ischemia in adults.² It was first recognized more than 100 years ago by Eliot³ who described intestinal gangrene resulting from mesenteric venous occlusion. The exact incidence of SMV thrombophlebitis is unknown. It may not be recognized at laparotomy and may be missed at autopsy.⁴ Greater use of diagnostic radiological imaging may lead to increased recognition.

Before the advent of antibiotics, mesenteric portal vein thrombosis and liver abscess formation complicated up to 50% of cases of appendicitis, diverticulitis, and other inflammatory bowel conditions.⁵ Since the routine administration of antibiotics and early surgical intervention, the incidence of ascending infectious portal thrombophlebitis has dropped to 0.05% for acute appendicitis and 3% for a ruptured appendix.⁶

Clinically, pylephlebitis or at least portal bacteremia should be suspected in any patient with suspected appendicitis who has rigors.⁷ Pylephlebitis secondary to infections (e.g. appendicitis) has been distinguished from thrombosis alone (without infection) due to other causes by its typically non-occlusive nature and absence of portal hypertension. In appendicitis, spread occurs via the ileocolic vasculature to the superior mesenteric vein and eventually to the portal vein.⁸ Pylephlebitis can also give rise to septic emboli within the liver, leading to intrahepatic abscess formation.⁹ Deranged liver enzymes were evident in our case and could have been indicative of early hepatic involvement. In our patient, pylephlebitis was detected at the level of superior mesenteric vein and not propagated to confluent of splenoportal vein and early recognition led to early management.

The clinical features of pylephlebitis are non-specific. High fever, chills, malaise, right quadrant pain, and tenderness are the initial clinical manifestations. Gollapudi¹⁰ reported that only 30% of patients with pylephlebitis present with localizing clinical signs of a primary source of sepsis. Laboratory findings, such as leucocytosis and mild abnormalities of liver function tests, are usually non-specific, but jaundice is rare except in case of multiple liver abscesses.^11,12 Cultures revealed no microorganisms in our case. Baril et al.¹¹ reported that bacteremia is present in less than one-half of patients, whereas, Balthazar and Gollapudi¹³ reported that up to 80% of patients have positive blood cultures and Escherichia coli, Bacteroides fragilis, Proteus mirabilis, Klebsiella pneumoniae, and Enterobacter spp. are the most common microorganisms isolated.^12,13

Modern diagnostic imaging techniques help the early diagnosis of acute phase pylephlebitis. The sensitivity and specificity of CT scans for pylephlebitis are not known. However, CT scans could simultaneously detect the primary source of infection, extent of pylephlebitis, and intrahepatic abnormalities, such as liver abscess. Thus, CT scan is the most reliable initial diagnostic choice.^13,14 Air bubbles or thrombi of the portal venous system are the critical CT findings of pylephlebitis¹³. Ultrasound scan with color flow Doppler is also a sensitive test for confirming partial patency of the portal vein and portal vein thrombosis.¹⁵

The principal treatment for pylephlebitis is to remove the source of infection and eradicate the toxic microorganisms using appropriate antibiotics. Immediate surgical intervention is necessary in most cases, but Stitzenberg et al.¹⁶ reported that interval laparoscopic appendectomy can be performed 3 months after treatment with antibiotics and anticoagulants. Regarding the treatment of portal thrombosis, post operative use of heparin has been advocated¹ but a benefit of adjunctive heparin therapy has not been clearly demonstrated. Harch et al. recommended anticoagulation on the presumption that the process might extend and lead to enteric ischaemia.¹⁷ Proponents of anticoagulation identified several factors such as documented progression of thrombus while on antibiotics, fever unresponsive to treatment, and the presence of hypercoagulable state to justify therapy.¹⁸ Anticoagulation may also reduce septic embolization to the liver from infected portal thrombi and prevent liver abscess. The optimum duration of anticoagulation is unclear in the literature. If thrombosis is associated with sepsis and not complicated by infarction or embolization and no underlying thrombophilic factors are identified, then a short duration of therapy seems a reasonable approach, however.

Open thrombectomy and venous ligation¹⁹ appear to have been largely abandoned as therapy for pylephlebitis. Operative or radiological interventions in the form of thrombectomy and thrombolysis with direct intravascular infusion of thrombolytics have been advocated²⁰ for mesenteric and portal vein non-suppurative thrombosis with bowel compromise. Nishimori et al.¹⁴ reported a patient with septic thrombophlebitis and liver abscess who had appendicectomy and thrombus removal from the SMV using a Fogarty catheter.

The mortality rate reported of up to 32% in cases with SMV thrombosis will in part be due to the severity of the associated condition¹⁴ rather than the thrombosis itself but it has been suggested that the complications of untreated portal or SMV thrombosis could be catastrophic.²¹ The reported high mortality rate without anticoagulation and a decreased recurrence rate reported after anticoagulation¹⁵ support our decision to use it in our patient.

4. Conclusion

Superior mesenteric venous thrombophlebitis is a rare but potentially fatal complication of acute intra-abdominal suppuration such as appendicitis. A high index of suspicion is required to allow prompt recognition and treatment.

Conflict of interest statement

The authors have no conflict of interests to declare.

Funding

None.

Ethical approval

Consent from patient available.

Author contributions

All authors read, reviewed and wrote the paper.