Table 1 Summary of 17 pediatric cases of acute appendicitis with portomesenteric thrombosis since 1990

Scully [3] (1991)

15/M

40-day RUQ pain, fever

Yes

Yes

No

CT scan

Splenic and portal (with cavernous transformation and liver abscess)

Not identified

Antibiotics, right hemicolectomy

No

Resolution of thrombosis by follow-up USG scan at 5 weeks

Spronsen [4] (1996)

11/M

26 days of diarrhea, vomiting, headache; later spiking fever and RLQ pain, also epigastric pain with RUQ tenderness on palpation

Yes

N/A

No

USG followed by doppler study followed by CT

Superior mesenteric, intrahepatic, and extrahepatic vessels of the portal vein

Yersinia enterocolitica (serology)

Antibiotics, interval appendectomy (4 months later)

No

No stated

Eire [5] (1998)

12/F

Vomiting and diarrhea 2 days after appendectomy

No

N/A

Protein S/C, anti-thrombin III deficiency

CT scan (no occlusion of portal vein/SMV), followed by laparotomy

Small thrombi in the venous branches of the peripheral mesenteric arcades and vasa rectae

N/A

Second look laparotomy, anticoagulants

Yes (IV heparin then warfarin/ 1 year)

Asymptomatic. Not mentioned on follow-up imaging

Kader [6] (1998)

15/M

2.5 weeks of periumbilical then right-side abdominal pain, poor appetite, fatigue, fevers, vomiting and watery diarrhea

No

Yes

No

USG followed by ultrasound doppler then CT scan

Superior mesenteric, portal, splenic

Bacteroides fragilis (in blood culture), Clostridium difficile toxin in stool

Antibiotics, anticoagulants, interval appendectomy (10 days later)

Yes (IV heparin then warfarin/6 months)

Resolution of thrombosis by follow-up USG scan at 3 months

Schmutz [7] (1998)

18/M

RLQ pain fever 2 weeks after appendectomy

No

N/A

No

CT scan

SMV

Not identified

No additional treatment

No

Resolution of thrombosis by follow-up CT scan at 6 months

Vanamo [8] (2001)

7/M

2-week recurrent fever, epigastric and RUQ pain, vomiting and diarrhea

Yes

Yes

No

CT scan

SMV, portal

Microaerophilic streptococci, Bacteroides fragilis (blood culture)

Antibiotics, anticoagulants, appendectomy

Yes (warfarin/1 year)

Resolution of thrombosis by follow-up MRI at 6 months

Chang [9] (2001)

8/M

6-week RUQ and back pain, vomiting, diarrhea, chills and fever

Yes

Yes

No

CT scan

SMV, splenic, portal (with liver abscess)

Escherichiae coli, anaerobic GI flora, and Streptococcus viridans (abdominal fluid culture), CD toxin (stool)

Antibiotics, anticoagulants, ileocolic resection

Yes (LMWH then warfarin/3 months)

Asymptomatic. Not mentioned on follow-up imaging

Pitcher [10] (2003)

17/M

3-week epigastric and RUQ pain, constipation, weight loss, fever

Yes

N/A

No

Ultrasound

Portal (with liver abscess)

N/A

Antibiotics, anticoagulants, appendectomy

Yes (not mentioned/not mentioned)

Resolution of thrombosis by follow-up USG at 4 months

Nishimori [11] (2004)

16/M

7-day fever, nausea and diffuse abdominal pain

No

Yes

No

Ultrasound followed by CT scan

SMV, portal (with liver abscess)

Bacteroides fragilis (blood culture)

Antibiotics, anticoagulants, appendectomy, thrombectomy

Yes (IV heparin then warfarin/not mentioned)

Resolution of thrombosis by follow-up CT scan at 3 weeks

Stitzenbeg [12] (2006)

5/F

7-day fever, vomiting, anorexia and 1-day diffuse abdominal pain

No

Yes

No

CT scan

SMV, left portal

N/A

Antibiotics, anticoagulants, interval appendectomy (4 months)

Yes (LMWH then warfarin/3 months)

No further thrombotic events. Not stated if any follow-up imagings

Levin [13] (2008)

13/M

High fever, jaundice, mild RUQ discomfort

Yes

Yes

No

Ultrasound followed by CT scan

SMV, portal, ileocolic, other SMV tributaries

Antibiotics, anticoagulants, appendectomy

Yes (LMWH then warfarin/6 months)

Resolution of thrombosis by follow-up doppler ultrasound at 3 weeks

Patel [14] (2009)

14/M

2-day fever and night sweats, 3-day RUQ and upper back pain

Yes

Yes

No

CT scan followed by MRI scan

Anterior branch of the right portal (with liver abscess)

Not identified

Antibiotics, laparoscopic appendectomy

No

Resolution of thrombosis by follow-up MRI scan at 2 weeks

Gatibelza [15] (2009)

12/F

10-day epigastric pain and fever

Yes

No

No

CT scan

SMV

Bacteroides distasonis (blood culture)

Antibiotics, anticoagulants and open appendectomy

Yes (LMWH then Fluindione/6 months)

Resolution of thrombosis by follow-up ultrasound at 6 months

Gatibelza [15] (2009)

13/M

7-day persistent fever, vomiting and diffuse abdominal pain

No

Yes

No

CT scan

SMV, portal trunk, intrahepatic veins of segment V, VII, VIII

Escherichiae coli (blood culture)

Antibiotics, anticoagulants and appendectomy

Yes (Fluindione/1.5 year)

Resolution of thrombosis by follow-up ultrasound at 1.5 year

Granero-Castro [16] (2010)

17/M

8-day diffuse abdominal pain, fever and diarrhea

No

Yes

No

Ultrasound followed by CT scan

SMV and its branches, progression at 1 week to intrahepatic branches of portal vein at segments II, III

Escherichiae coli, Bacteroides fragilis (blood culture)

Antibiotics, anticoagulants, appendectomy

Yes (LMWH/4 months)

Progression of thrombus at 1 week, collateral through colic vein at 2 months

Harris [2] (2016)

13/M

Abdominal pain (RUQ and suprapubic), diarrhea, vomiting and hallucination

Yes

Yes

No

Ultrasound followed by CT scan

SMV, portal, ileocolic, other SMV tributaries

Not identified

Antibiotics, anticoagulants, interval appendectomy (3 months)

Yes (LMWH/not mentioned)

Resolution of thrombosis by follow-up CT scan at 1 month

Yoon [17] (2019)

15/M

2-day RLQ pain with 1-day fever

No

Yes

No

CT scan

SMV

Not identified

Antibiotics, anticoagulants, appendectomy

Yes (IV heparin then apixaban/3 months)

Resolution of thrombosis by follow-up ultrasound at 1 month