Journal of Thrombosis and Thrombolysis

, Volume 38, Issue 2, pp 218–222

Portal vein thrombosis after laparoscopic splenectomy during childhood

Authors

    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
  • Aurélien Scalabre
    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
  • Frédéric Hameury
    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
  • Rémi Dubois
    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
  • Céline Grosos
    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
  • Pierre D. Mouriquand
    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
  • Pierre-Yves Mure
    • Pediatric Surgery DepartmentFemme Mère Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1
Article

DOI: 10.1007/s11239-013-1037-2

Cite this article as:
Gelas, T., Scalabre, A., Hameury, F. et al. J Thromb Thrombolysis (2014) 38: 218. doi:10.1007/s11239-013-1037-2

Abstract

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of laparoscopic splenectomy (LS) and can lead to bowel ischemia or portal hypertension. In childhood, this complication is reported in 5–10 % of the cases whereas it can be up to 50 % in adult population. Our aim was to evaluate PVT incidence after LS and associated risks factors. A retrospective chart review identified 37 children who underwent elective LS from 2005 to 2013. The main indications were spherocytosis or sickle cell disease. Median age and weight were respectively 7.4 years and 25.1 kg. Thromboembolic prophylaxis was not routinely given. Duration of surgery was 129 min and hospital length of stay 4 days. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases. Post-operative course was uneventful in all but one patient. She was a 17 year-old girl previously operated for an ovarian tumor with hyperandrogenism. Histopathology revealed a splenic lymphoma. At day 4, a systematic USS showed a PVT extending in the portal branches. Therapeutic low molecular weight heparin was used and then transitioned to fluindione for 3 months. Follow-up USS performed at 1 and 4 months demonstrated complete resolution of the PVT. PVT after pediatric LS is a rare event in our series. Clinician should be cautious in oncologic cases and if very large spleen or if thrombocythemia >650.109/L is present. If detected early, PVT can be treated efficiently. We therefore recommend a systematic USS during the first postoperative week.

Keywords

PediatricSplenectomyPortal vein thrombosisSplenomegalyThrombocythemia

Introduction

Portal vein thrombosis (PVT) is a rare but potentially life-threatening complication of splenectomy. It can lead to bowel infarction or portal hypertension. In childhood, this complication is reported in 5–10 % of the cases whereas it can be up to 50 % in adult population. Laparoscopic splenectomy (LS) is nowadays a well admitted procedure in children and is the technique of choice when compared to open splenectomy (OS). Our aim was to evaluate PVT incidence after LS in children and associated risks factors.

Patients and methods

A retrospective chart review identified 37 children (20 females, 17 males) who consecutively underwent elective LS during a 7-year period (May 2005–August 2013) at a single teaching hospital. Traumatologic cases were excluded. Information recorded for each patient included age, sex, underlying hematologic disease, platelets count values. The primary study outcome measures included operative time, duration of hospital stay and outcome of surgery.

A pre-operative ultrasound scan was performed in all patients to assess the size of the spleen and potential cholelithiasis. All splenectomies were performed laparoscopically; the patient was placed in a 45° right lateral decubitus position. A kidney rest was raised to create the largest possible distance between the iliac crest and the costal margin. Initial laparoscopic access was through the umbilicus using an open technique. Pneumoperitoneum was established to a pressure of 8–12 mmHg, 2 or 3 additional ports were then placed (epigastric, left iliac fossa and midline). Dissection was performed from a lateral approach. The major vessels were ligated using clips, bipolar electrocautery, electrofusion (ENSEAL®, SurgRxTM, Redwood City, CA) or with ultrasound scissors (ULTRACISION®, Ethicon Endo-Surgery, LLC, Norderstedt, Germany).The specimen was placed in a plastic specimen bag and removed through the umbilical port, in a piecemeal fashion. All specimens were weighted in addition to histopathologic evaluation. Post-operative thromboembolic prophylaxis was not usually given. In recent cases, a post-operative doppler USS was routinely performed to assess the portal vein patency, if possible during the first post-operative week. No statistical analysis was performed due to the number of patients with PVT.

Results

Age and weight were respectively 7.4 years (range 2–17) and 25.1 kg (range 10–60). The main indication for LS was sickle cell disease and spherocytosis (see Table 1). Splenomegaly was present in 80 % of cases with a spleen longest axis ranging from 8.5 to 23 cm (median = 13 cm). The median operating time was 129 min (range 67–197), conversion to open surgery was needed in two cases and terminal hand assistance was required in three cases through a Pfannensteil incision. A concomitant laparoscopic cholecystectomy was performed in 11 patients. The median weight of spleen was 198 g (range 33–1690 g). Hospital length of stay was 4 days (range 2–13 days). Median pre- and post-operative platelet counts are reported on Table 2. Only 23 % of the patients had a post-operative thrombocytosis >650.109/L. Doppler ultrasound scan (USS) was performed post-operatively in 26 cases (but all the patients operated after December 2010 had a USS at a median time of 3 days).
Table 1

Indication for splenectomy

Indication for splenectomy

Number

Spherocytosis

17

Sickle cell disease

13

Idiopathic thrombocytopenic purpura

5

Pyruvate kinase deficiency

1

Splenic lymphoma

1

Table 2

Blood values

Platelet count (×109/L)

Median (range min–max)

Pre-operative

213 (7–457)

Post-operative

458 (188–1,314)

Post-operative course was uneventful in all but one patient who experienced PVT. She was a 17 year-old girl (60 kg, 167 cm–BMI = 21.5) previously operated for an ovarian Sertoli-Leydig cell tumor with hyperandrogenism. Histopathology revealed a 23 cm spleen, weighing 1,690 g with a low-grade splenic lymphoma. Due to her post-pubertal status and suspicion of oncological case, thromboembolic prophylaxis was used with subcutaneous low molecular weight heparin (LMWH). At POD 4, while she was asymptomatic and apyrexial, a systematic USS showed a PVT extending into the right and left portal branches (see Fig. 1). Therapeutic LMWH was initially used and transitioned to fluindione for 3 months, adjusted to achieve an INR between 2.0 and 3.0. Over our series, she was the only patient with platelet count over >1,000.109/L. Follow-up abdominal imaging studies performed 1 and 4 months (see Figs. 2, 3) post operatively demonstrated complete resolution of the PVT.
https://static-content.springer.com/image/art%3A10.1007%2Fs11239-013-1037-2/MediaObjects/11239_2013_1037_Fig1_HTML.jpg
Fig. 1

Doppler USS—complete PVT in the main portal vein (POD 4)

https://static-content.springer.com/image/art%3A10.1007%2Fs11239-013-1037-2/MediaObjects/11239_2013_1037_Fig2_HTML.jpg
Fig. 2

Doppler USS. Partial portal vein thrombosis after 1-week of treatment (arrow)

https://static-content.springer.com/image/art%3A10.1007%2Fs11239-013-1037-2/MediaObjects/11239_2013_1037_Fig3_HTML.jpg
Fig. 3

Doppler USS. Normal portal vein flow without residual thrombosis, 4 months after surgery

Discussion

The first case of PVT secondary to splenectomy was described in 1894 by H. Delatour [1] in a 31 year-old woman who died of an intestinal ischemia 24 days after the removal of a 2,240 g spleen. Despite its rarity, portal vein thrombosis is a potentially lethal complication. It can lead to bowel infarction if the thrombus extends into the superior mesenteric vein or to portal hypertension and subsequent gastro-intestinal bleeding due to cavernoma formation and esophageal varices. Laparoscopic splenectomy is nowadays a well admitted procedure in children with hematological diseases and is the technique of choice compared with OS because of the advantages of the minimally invasive approach. The incidence of PVT after LS in adult series is reported in 23 % (range from 10 to 51.5 %) of the prospective studies and 2.3 % of the retrospective studies [2]. It can be as high as 51.5 % if a systematic CT scan is performed post-operatively [3]. This incidence in children is not well known but is probably around 5 % (see Table 3). One prospective study [4] reported no PVT in 12 splenectomized major β-thalassemia patients; Doppler USS was performed 1-week post-splenectomy. Most of the reported cases of PVT after LS in the pediatric population are single reports (see Table 4). Regarding our series, we are performing systematic Doppler USS pre- and post-operatively during the first post-operative week since 2011 (n = 19 patients). In this 3-year prospective approach, only one PVT was encountered (incidence = 5.3 %). Regarding the whole period (prospectively and retrospectively), 26 patients had a post-operative Doppler USS, with a reported incidence of 3.8 %. PVT is probably underestimated because symptoms, such as abdominal pain and fever, are nonspecific and PVT can easily develop without any symptoms. Indeed, Krauth et al. [2] have reported that only 46 % of the patients with PVT have developed symptoms that could be related to PVT. In most of the cases, PVT occurs shortly after surgery (1–2 weeks) and some authors recommend [5] an ultrasonographic screening on post-operative day 7. If asymptomatic PVT has not developed at this time, it is unlikely to develop later, and subsequent screening ultrasound at 1 month is not required.
Table 3

Reported portal vein thrombosis incidence after open or laparoscopic splenectomy

Authors

Year

Surgery

Patient number

PVT incidence (%)

Study

Soyer T [14]

2006

OS

68

5.88

Retrospective

Vargün R [21]

2007

LS

17

0

Retrospective

Rescorla FJ [22]

2007

LS

231

0.43

Retrospective

Hafid M [23]

2009

OS

50

8

Retrospective

Vecchio R [24]

2011

LS

18

5.60

Retrospective

Elalfy MS [4]

2012

OS

12

0

Prospective

OS open splenectomy, LS laparoscopic splenectomy

Table 4

Reported portal vein thrombosis after laparoscopic splenectomy

Authors

Year

Age (years)

Underlying disease

POD

Treatment

Outcome

Brink JS [13]

2003

12

Autoimmune hemolytic anemia

16

IV heparine, anagrelide + warfarine

Free of thrombosis

Miniati DN [25]

2005

16

Idiopathic thrombocytopenic purpura

20

IV heparine

Cavernoma transformation

Lederman HM [26]

2006

10

Chronic myeloid leukemia

8

Acetylsalicylic acid + LMWH + anagrelide

Partial thrombosis

Rescorla FJ [22]

2007

NA

NA

NA

NA

NA

Rossi E [27]

2007

8

Congenital hemolytic Anemia

6

IV heparine + Acetylsalicylic acid

Free of thrombosis

Vecchio R [24]

2011

NA

NA

NA

IV heparine

Free of thrombosis

NA information not available, IV intravenous, LMWH low molecular weight heparine

Combinations of factors leading to hypercoagulability seem mandatory in PVT occurrence. This hypercoagulable state can be due to inherited (protein C or protein S deficiency …) or acquired conditions. Vu et al. [6] have reported that patients at highest risk of deep venous thrombosis (lower extremities) were those in the age range of 15–17 years and with the following comorbid conditions: obesity, inflammatory bowel disease, hematologic malignancy and thoraco-abdominal or orthopedic operations. Regarding splenectomy, the underlying disease appears to be the highest risk factor. Patients with large spleens (mostly myeloproliferative disorders or lymphoma) and patients with congenital hemolytic diseases have the highest risk for PVT [2, 7, 8]. Many authors [911] have emphasis the increased risk of PVT in presence of splenomegaly, with a cut off value of 1,450 g in adults [11]. Conversely, it has been demonstrated that a diameter of the splenic vein >8 mm [12] is also a risk factor of PVT. Ligation of the splenic vein leaving a long and large vascular stump may causes blood turbulence and enhance thrombus formation [13]. Furthermore, post-operative thrombocytosis >650.109/L may increase this risk [8]. Soyer et al. [4] have reported a female predominance in their series. This may be explained by the thrombotic effect of endogenous estrogens (reduction of the peripheral vasodilatation capacity and decreased erythrocyte membrane stability). They therefore proposed that post-pubertal girls should be more closely investigated for the development of PVT after splenectomy. As reported previously [6], childhood obesity might also be a risk factor as it is associated with a procoagulant state and endothelial dysfunction [15].

Portal vein thrombosis incidence seems to be higher after LS compared to OS in prospective adult series (23 % vs. 8.3 %) [2]. During open surgery, vessels are ligated conventionally whereas they are most of the time coagulated, clipped or stapled during LS. Furthermore, pneumoperitoneum with increased abdominal pressure may reduce splanchnic veins blood flow as demonstrated in experimental and clinical studies [16, 17]. But, recent surgical guidelines [18] indicate that routine use of thromboprophylaxis is not recommended for laparoscopic procedures. Furthermore, in a recent randomized control trial [19] (probably underpowered due to its small size) investigating the impact of anticoagulation on the incidence of portal vein thrombosis after laparoscopic splenectomy; thromboprophylaxis with 40 mg of enoxaparin once daily failed to prevent PVT. In pediatric population, thromboprophylaxis is not mandatory during the prepubertal period. Recommendations for low risk patients are early mobilization and good hydration; for moderate risk patients having major general surgery to include physical prophylaxis where size permits (elastic stockings, compression devices). Prophylactic low molecular weight heparin is recommended only for high-risk patients undergoing major orthopedic or general surgery [20]. Patients with documented PVT need to be treated with intravenous or subcutaneous heparin followed by oral anticoagulant therapy for 3–6 months. However, despite a well conduced treatment; a persistent thrombus is noticed in 7.7 % and cavernoma or portal hypertension development is documented in 15.5 % of the cases [2].

Conclusions

Portal vein thrombosis after pediatric LS appears to be a rare event in our series (<5 %) but its incidence is probably underestimated. Large or prospective studies are needed to assess the real incidence of PVT in children. Clinicians should be cautious in oncologic cases and if very large spleen or if thrombocytemia >650.109/L is present. When detected early, PVT can be treated efficiently using heparin followed by oral anticoagulant therapy. Due to potential life-threatening complications of PVT, we therefore recommend a systematic Doppler USS during the first post-operative week.

Conflict of interest

The authors declare that they have no conflict of interest.

Copyright information

© Springer Science+Business Media New York 2013