J Thromb Thrombolysis DOI 10.1007/s11239-013-1037-2
Portal vein thrombosis after laparoscopic splenectomy during childhood Thomas Gelas • Aure´lien Scalabre • Fre´de´ric Hameury • Re´mi Dubois • Ce´line Grosos Pierre D. Mouriquand • Pierre-Yves Mure
•
Springer Science+Business Media New York 2013
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.
T. Gelas (&) A. Scalabre F. Hameury R. Dubois C. Grosos P. D. Mouriquand P.-Y. Mure Pediatric Surgery Department, Femme Me`re Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1, 59 Boulevard PINEL, 69500 Bron, France e-mail: [email protected]
Keywords Pediatric Splenectomy Portal vein thrombosis Splenomegaly Thrombocythemia
Introduction Portal vein thrombosis (PVT) is a rare but potentially lifethreatening 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
123
T. Gelas et al.
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 Fig. 1 Doppler USS—complete PVT in the main portal vein (POD 4)
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
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 (9109/L)
Median (range min–max)
Pre-operative
213 (7–457)
Post-operative
458 (188–1,314)
123
the patients operated after December 2010 had a USS at a median time of 3 days). 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 postpubertal 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.
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
Portal vein thrombosis after laparoscopic splenectomy Table 3 Reported portal vein thrombosis incidence after open or laparoscopic splenectomy Authors
Year
Surgery
Patient number
PVT incidence (%)
Study
Soyer T [14] Vargu¨n R [21]
2006
OS
68
5.88
Retrospective
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 Fig. 2 Doppler USS. Partial portal vein thrombosis after 1-week of treatment (arrow)
Fig. 3 Doppler USS. Normal portal vein flow without residual thrombosis, 4 months after surgery
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 b-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 preand 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. 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 [9–11] have emphasis the increased risk of PVT in presence of
123
T. Gelas et al. 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 NA
Rescorla FJ [22]
2007
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
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
123
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 postoperative week.
Conflict of interest of interest.
The authors declare that they have no conflict
References 1. Delatour HB (1895) III. Thrombosis of the mesenteric veins as a cause of death after splenectomy. Ann Surg 21(1):24–28 2. Krauth MT, Lechner K, Neugebauer EA, Pabinger I (2008) The postoperative splenic/portal vein thrombosis after splenectomy and its prevention—an unresolved issue. Haematologica 93(8): 1227–1232 3. Ikeda M, Sekimoto M, Takiguchi S, Kubota M, Ikenaga M, Yamamoto H, Fujiwara Y, Ohue M, Yasuda T, Imamura H, Tatsuta M, Yano M, Furukawa H, Monden M (2005) High incidence of thrombosis of the portal venous system after
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laparoscopic splenectomy: a prospective study with contrastenhanced CT scan. Ann Surg 241(2):208–216 Elalfy MS, Andrawes NG, Sadek AM, Hussein O, Abdou A (2012) D-dimer and portal vein status in splenectomized Egyptian b-thalassemia major patients: a prospective single-thalassemia center experience. Pediatr Hematol Oncol 29(3):247–253 Tran T, Demyttenaere SV, Polyhronopoulos G, Se´guin C, Artho GP, Kaneva P, Fried GM, Feldman LS (2010) Recommended timing for surveillance ultrasonography to diagnose portal splenic vein thrombosis after laparoscopic splenectomy. Surg Endosc 24(7):1670–1678 Vu LT, Nobuhara KK, Lee H, Farmer DL (2008) Determination of risk factors for deep venous thrombosis in hospitalized children. J Pediatr Surg 43(6):1095–1099 Okada T, Sasaki F, Minagawa N, Naito S, Kobayashi R, Kubota M, Todo S (2005) Postoperative portal and splenic vein thrombosis in children: identification of risk factors. Pediatr Surg Int 21(11):918–920 Stamou KM, Toutouzas KG, Kekis PB, Nakos S, Gafou A, Manouras A, Krespis E, Katsaragakis S, Bramis J (2006) Prospective study of the incidence and risk factors of postsplenectomy thrombosis of the portal, mesenteric, and splenic veins. Arch Surg 141(7):663–669 Fujita F, Lyass S, Otsuka K, Giordano L, Rosenbaum DL, Khalili TM, Phillips EH (2003) Portal vein thrombosis following splenectomy: identification of risk factors. Am Surg 69(11):951–956 Targarona EM (2008) Portal vein thrombosis after laparoscopic splenectomy: the size of the risk. Surg Innov 15(4):266–270 Alexakis N, Dardamanis D, Albanopoulos K, Ptohis N, Skalistira M, Karagiorga M, Zografos G, Leandros E (2013) Incidence, risk factors, and outcome of portal vein thrombosis after laparoscopicassisted splenectomy in b-thalassemia patients: a prospective exploratory study. J Laparoendosc Adv Surg Tech A 23(2):123–128 Danno K, Ikeda M, Sekimoto M, Sugimoto T, Takemasa I, Yamamoto H, Doki Y, Monden M, Mori M (2009) Diameter of splenic vein is a risk factor for portal or splenic vein thrombosis after laparoscopic splenectomy. Surgery 145(5):457–464 Discussion 465–466 Brink JS, Brown AK, Palmer BA, Moir C, Rodeberg DR (2003) Portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy. J Pediatr Surg 38(4):644–647 Soyer T, Ciftci AO, Tanyel FC, Senocak ME, Bu¨yu¨kpamukc¸u N (2006) Portal vein thrombosis after splenectomy in pediatric hematologic disease: risk factors, clinical features, and outcome. J Pediatr Surg 41(11):1899–1902
15. Singh A, Foster GD, Gunawardana J, McCoy TA, Nguyen T, Vander Veur S, Komaroff E, Rao AK (2012) Elevated circulating tissue factor procoagulant activity, factor VII, and plasminogen activator inhibitor-1 in childhood obesity: evidence of a procoagulant state. Br J Haematol 158(4):523–527 16. Bickel A, Weiar A, Eitan A (2008) Evaluation of liver enzymes following elective laparoscopic cholecystectomy: are they really elevated? J Gastrointest Surg 12(8):1418–1421 17. Eryılmaz HB, Memis¸ D, Sezer A, Inal MT (2012) The effects of different insufflation pressures on liver functions assessed with LiMON on patients undergoing laparoscopic cholecystectomy. Sci World J 2012:172575 18. Geerts W, Bergqvist D, Pineo GF et al (2008) Prevention of venous thromboembolism. Chest 133(6 Suppl):381S–453S 19. Wang H, Kopac D, Brisebois R, Sample C, Shapiro AM (2011) Randomized controlled trial to investigate the impact of anticoagulation on the incidence of splenic or portal vein thrombosis after laparoscopic splenectomy. Can J Surg 54(4):227–231 20. Jackson PC, Morgan JM (2008) Perioperative thromboprophylaxis in children: development of a guideline for management. Paediatr Anaesth 18(6):478–487 21. Vargu¨n R, Go¨llu¨ G, Fito¨z S, Yagmurlu A (2007) En-bloc stapling of the splenic hilum in laparoscopic splenectomy. Minim Invasive Ther Allied Technol 16(6):360–362 22. Rescorla FJ, West KW, Engum SA, Grosfeld JL (2007) Laparoscopic splenic procedures in children: experience in 231 children. Ann Surg 246(4):683–687 Discussion 687–688 23. Hafid M, Kaddouri N, Abdelhak M, Benhmamouch MN, Barahioui M (2009) Portal vein thrombosis after splenectomy in childhood: report of 4 cases. Arch Pediatr 16(11):1477–1480 24. Vecchio R, Marchese S, Gelardi V, Vicari S, Cacciola E, Cacciola RR, Intagliata E (2011) Laparoscopic splenectomy in patients under the age of eighteen. Experience in 18 cases. G Chir 32(5):279–285 25. Miniati DN, Padidar AM, Kee ST, Krummel TM, Mallory B (2005) Portal vein thrombosis after laparoscopic splenectomy: an ongoing clinical challenge. JSLS 9(3):335–338 26. Lederman HM, Fieldston E (2006) Splenic and portal vein thrombosis following laparoscopic splenectomy in a pediatric patient with chronic myeloid leukemia. Sao Paulo Med J 124(5):275–277 27. Rossi E, Michelini ME, Pignatti CB, Zanotti F, Franchella A (2007) A case of portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy in a child. J Pediatr Surg 42(8):1449–1451
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Portal vein thrombosis after laparoscopic splenectomy during childhood Thomas Gelas • Aure´lien Scalabre • Fre´de´ric Hameury • Re´mi Dubois • Ce´line Grosos Pierre D. Mouriquand • Pierre-Yves Mure
•
Springer Science+Business Media New York 2013
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.
T. Gelas (&) A. Scalabre F. Hameury R. Dubois C. Grosos P. D. Mouriquand P.-Y. Mure Pediatric Surgery Department, Femme Me`re Enfant Hospital – Hospices Civils de Lyon and Claude Bernard University Lyon 1, 59 Boulevard PINEL, 69500 Bron, France e-mail: [email protected]
Keywords Pediatric Splenectomy Portal vein thrombosis Splenomegaly Thrombocythemia
Introduction Portal vein thrombosis (PVT) is a rare but potentially lifethreatening 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
123
T. Gelas et al.
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 Fig. 1 Doppler USS—complete PVT in the main portal vein (POD 4)
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
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 (9109/L)
Median (range min–max)
Pre-operative
213 (7–457)
Post-operative
458 (188–1,314)
123
the patients operated after December 2010 had a USS at a median time of 3 days). 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 postpubertal 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.
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
Portal vein thrombosis after laparoscopic splenectomy Table 3 Reported portal vein thrombosis incidence after open or laparoscopic splenectomy Authors
Year
Surgery
Patient number
PVT incidence (%)
Study
Soyer T [14] Vargu¨n R [21]
2006
OS
68
5.88
Retrospective
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 Fig. 2 Doppler USS. Partial portal vein thrombosis after 1-week of treatment (arrow)
Fig. 3 Doppler USS. Normal portal vein flow without residual thrombosis, 4 months after surgery
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 b-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 preand 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. 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 [9–11] have emphasis the increased risk of PVT in presence of
123
T. Gelas et al. 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 NA
Rescorla FJ [22]
2007
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
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
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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 postoperative week.
Conflict of interest of interest.
The authors declare that they have no conflict
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