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Purpose: Rex shunt is an optimal surgery for the treatment of extra-hepatic portal venous obstruction (EHPVO) in children. Anticoagulant therapy has been used to keep the patency of the bypass vein in the Rex shunt. This study was to investigate the effectiveness of anticoagulant therapy using heparin combined with Plavix in improving the prognosis and shunt patency of Rex shunt. Methods: From January 2010 to September 2019, 51 children with EHPVO underwent a portal cavernoma- Rex shunt. Based on whether using the anticoagulant therapy after the Rex shunt, all patients were divided into two groups: the anticoagulant group and the non-anticoagulant group. The diameter and flow velocity of the bypass vein were measured by the post-operative ultrasound, which was used to calculate the flow volume of the bypass vein (FV) and standard portal venous flow (SPVF). The bypass venous flow index (BVFI) was used to evaluate the ability of portal blood into the liver through the bypass vein after the Rex shunt, which was a ratio of FV to SPVF. The incidence of post-operative re-bleeding, the postoperative patency rate of the bypass vein, the remission rate of postoperative hypersplenism, the remission rate of postoperative esophagogastric varices and the BVFI were compared between the two groups. Results: Of the 51 patients, 12 patients in the anticoagulant group were treated with heparin combined with Plavix after Rex shunt; 39 patients in the non-anticoagulant group were not treated with any anticoagulant therapy. 8 of 51 patients suffered from postoperative re-bleeding, of whom 6 patients with thrombosis of the bypass vein and 2 patients with anastomotic stenosis of the bypass vein. All 8 patients with re-bleeding belonged to the non-anticoagulant group. The remission rate of hypersplenism was no significant difference between the two groups after surgery (91% vs. 58%, P = 0.100). However, 3 patients without hypersplenism before surgery suffered from hypersplenism after surgery, who belonged to the non-anticoagulant group. There was no significant difference in the remission rate of esophagogastric varices (33% vs. 46%, P = 1.000). The BVFI of the anticoagulant group was significantly higher than that of the non-anticoagulant group (5.71 ± 5.89 vs. 1.1 ± 1.52, P = 0.003). Conclusions: Anticoagulant therapy using heparin combined with Plavix plays an important role in maintaining the patency of the bypass vein, which improved the portal blood flow into the liver through the bypass vein after the Rex shunt.
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Introduction: Portal hypertension is a syndrome characterized by increased pressure in the portal vein system and can be caused by impaired blood flow in the portal vein, hepatic veins, or inferior vena cava. The main complications of this condition are bleeding from varicose veins of the esophagus (in our study in 100% of patients), splenomegaly with hypersplenism (in our study in 98% of patients), ascites (in our study in 1 patient). The main goal of treating portal hypertension is to prevent bleeding from esophageal varices. However, today the goal of surgical treatment of portal hypertension in children is not only to prevent the development of bleeding but also the possible restoration of intrahepatic blood flow. Materials and Methods: A retrospective analysis of the results of treatment of portal hypertension in 75 children (41 boys, 34 girls) operated in our Center for the period from 2019 to 2022 was carried out. The mean age of the patients was 7 ± 1 years. Sixty-nine patients had an extrahepatic form of portal hypertension, and 6 patients had an intrahepatic form (liver fibrosis). In 14 patients (18.6%), the operation was repeated (a vascular shunt was previously applied in another hospital; 4 children were operated on repeatedly). Results: A good result was obtained in all children, and the risk of bleeding from varicose veins of the esophagus was eliminated. Vascular bypass surgery was performed in all cases: mesoportal bypass in 17 (22.7%) patients, splenorenal bypass in 37 (49.3%) patients, mesocaval bypass in 21 (28%) patients. In 10 (13%) cases, repeated bypass surgery was required due to dysfunction or thrombosis of the previously performed bypass. In 14 (18.6%) patients with mesoportal shunts, blood flow in the liver was completely restored. Conclusions: The main method of surgical treatment of portal hypertension today is portosystemic bypass surgery, which effectively prevents bleeding from varicose veins of the esophagus. Mesoportal shunting is a definitive treatment for extrahepatic portal hypertension that restores portal perfusion of the liver.
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Várices Esofágicas y Gástricas , Hipertensión Portal , Várices , Masculino , Femenino , Niño , Humanos , Derivación Portosistémica Quirúrgica/efectos adversos , Derivación Portosistémica Quirúrgica/métodos , Estudios Retrospectivos , Hipertensión Portal/complicaciones , Hipertensión Portal/cirugía , Vena Porta/cirugía , Várices Esofágicas y Gástricas/complicaciones , Várices Esofágicas y Gástricas/cirugía , Várices/complicacionesRESUMEN
Klippel-Trénaunay syndrome is typically a complex combined capillary-lymphatic-venous malformation in lower limb. Gastrointestinal involvement is not infrequent in Klippel-Trénaunay syndrome. Rectal bleeding is the most common complication. In recent years, this condition has been increasingly reported. However, most authors simply described extreme manifestations or various combinations of clinical observations. The underlying pathophysiology of gastrointestinal involvement in Klippel-Trénaunay syndrome has been underrecognized. Pathophysiologically, some seemingly adequate managements are pitfalls in treatment. Anorectosigmoid vascular malformations in KTS have distinct and more complicated pathophysiologies than anorectal vascular malformation. Once understanding the pathophysiology, some patients can be successfully managed with a staged plan in our practice. Therefore, recognizing the pathophysiologies of gastrointestinal involvement is needed to evaluate, prevent pitfalls, and determine adequate managements for practitioners. Because of the complexity and rarity of this condition, prospective controlled study or a large cohort of patients is impossible. Based on literature review and our practice, we discuss pathophysiologies, evaluation, pitfalls, and treatment strategies for gastrointestinal involvement in Klippel-Trénaunay syndrome.
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Malformaciones Anorrectales , Síndrome de Klippel-Trenaunay-Weber , Vasos Linfáticos , Humanos , Síndrome de Klippel-Trenaunay-Weber/complicaciones , Estudios ProspectivosRESUMEN
Purpose: To review our single-center surgical outcomes of redo operations after failed Rex shunt procedures. Methods: From September 2017 to October 2021, a total of 20 patients (11 males, 9 females; median age: 8.6 years) with Rex shunt occlusions were admitted to our hospital. Two of these patients were previously operated on in our hospital, and the remaining 18 were from other centers. All patients underwent repeat operations after detailed preoperative evaluations. Results: Preoperative wedged hepatic vein portography (WHVP) was conducted for 18 patients. Thirteen patients exhibited well-developed Rex recessus and intrahepatic portal vein during WHPV examination, consistent with the intraoperative exploration results. Fifteen patients (75%, 15/20) underwent redo-Rex shunt, four underwent Warren shunt and one underwent devascularization surgery. During the redo-Rex shunt operations, the left internal jugular veins (IJV) were used as bypass grafts in 11 patients; the intra-abdominal veins were used in 4 patients. The patients were followed up for 12-59 months (mean, 24.8 months). After redo Rex shunts, the grafts were patent in 14 patients (93.3%, 14/15), but 1 graft had thrombosis (6.7%, 1/15). Three patients suffered from postoperative anastomotic stenosis, and all of the stenosis was relieved with balloon dilatations. After re-Rex shunts, esophageal varices and spleen size were substantially reduced, and the platelet count significantly increased. Postoperative graft thrombosis was found in 1 patient after Warren shunt (1/4, 25%), and there was no graft stenosis. Compared with Warren surgery, patients who underwent re-Rex shunt had a significantly higher rate of platelet increase. Conclusions: Redo-rex shunts can be finished in most patients with failed Rex shunts. Re-Rex shunt is a preferred surgical choice after a failed Rex shunt when a good bypass graft is available, and the surgical success rate can reach more than 90%. A suitable bypass graft is essential for a successful redo Rex shunt. Preoperative WHVP is recommended for the design of a redo surgical plan preoperatively.
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PURPOSE: To investigate the feasibility of the re-patent EHPVO (r-EHPVO) as an animal model of Rex shunt and the effectiveness of Rex shunt in improving abnormal portal hemodynamics and portal venous pathology of EHPVO. METHODS: A total of 18 New Zealand white rabbits were randomly divided into three groups: normal control (NC) group, extrahepatic portal venous obstruction (EHPVO) group, and r-EHPVO group. The main portal vein was dissected only in the NC group. The main portal vein was narrowed by a cannula in the EHPVO group. The cannula narrowing the main portal vein was removed to restore the portal blood flow into the liver on day 14 in the r-EHPVO group. The portal pressure, splenic size, blood flow velocity, and diameter of the portal vein were measured on days 14 and 28. The shear stress (SS) and circumferential stress (CS) of the portal vein were calculated. The proximal end of the main portal vein was collected on day 28 for further pathological analysis, and the thickness and area of the intima and media were measured by Image J software. The portal pressure, splenic size, SS, CS, intima and media thickness, the ratio of intimal to medial area (I/M), and the ratio of intimal area to the sum of intimal and medial area (I/I + M) were compared among the three groups. The correlation between SS and intimal thickness and between CS and medial thickness were analyzed. RESULTS: On day 28, the portal pressure of the EHPVO group was significantly higher than that of the NC and r-EHPVO groups, but no significant difference was detected in the portal pressure between r-EHPVO and NC groups. The length and thickness of the spleen in the EHPVO and r-EHPVO groups were significantly higher than those in the NC group (P < 0.01) but were significantly lower in the r-EHPVO group than those in the EHPVO group (P < 0.05). The SS was significantly lower in the EHPVO group than in NC and r-EHPVO groups (P < 0.05) but was significantly higher in the NC group than in the r-EHPVO group (P = 0.003). The CS was significantly higher in the EHPVO and r-EHPVO groups than that in the NC group (P < 0.05) but was significantly lower in the r-EHPVO group than that in the EHPVO group (P < 0.001). The intimal thickness, I/M, and I/I + M of the EHPVO group were significantly higher than those of the NC and r-EHPVO groups (P < 0.05), but no significant difference was observed between the NC and r-EHPVO groups (P > 0.05). The SS is negatively related to intimal thickness (r = - 0.799, P < 0.001). CONCLUSION: The r-EHPVO model is feasible as an animal model of the Rex shunt. The Rex shunt could be beneficial to improving the abnormal portal hemodynamic and portal venous intimal hyperplasia by restoring the portal blood flow into the liver.
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Várices Esofágicas y Gástricas , Hipertensión Portal , Enfermedades Vasculares , Animales , Conejos , Vena Porta/cirugía , Vena Porta/patología , Hipertensión Portal/cirugía , Várices Esofágicas y Gástricas/patología , Modelos Animales , HemodinámicaRESUMEN
OBJECTIVE: The aim: To analyze retrospectively our experience of Rex shunt in children with symptomatic portal hypertension, its effect on hypersplenism regression and varices eradication, assess shunt survival and investigate risk factors, that could lead to shunt dysfunction and thrombosis. PATIENTS AND METHODS: Materials and methods: 24 children (16 males, 8 females) ,with portal hypertension included into the study. All surgeries were performed within single center in a period from January 2010 to March 2022. Follow up period was 6.75±1.19 years. RESULTS: Results: Age at diagnosis was 5.39±0.64 years. 5 (20.8%) had umbilical catheter in anamnesis. 16 (66.7%) manifested bleeding episodes as the first sign of portal hypertension. 9 (37.5%) of children manifested severe hypersplenism. Age at Rex shunting was 7.5±0.7 years. In 7 (31.8%) cases Rex shunt thrombosis occurred. 1 successful thrombectomy and 6 splenorenal shunting were performed. Kaplan-Meyer analysis showed Rex shunt survival 0.670 (95%CI 0.420-0.831). Logistic regression model indicated thrombocytes count (p=0.0423) and cytopenia (p=0.0272) as factors that could influence shunt thrombosis. Follow-up group included 18 patients. Spleen volume regression became significant by 1 p/o year p<0,05, thrombocytes significant increasement reached in 1 p/o months (p<0.01), varices involution was achieved by 1 p/o year (p<0,001). CONCLUSION: Conclusions: Rex shunt effectiveness in study group was 70.9%., shunt survival assessed 0.670 (95%CI 0.420-0.831). Rex shunt was effective in bleeding prophylaxis in all patients of follow up group. Preoperative thrombocytes count (p=0.0423) and cytopenia (p=0.0272) were detected as factors that could influence shunt thrombosis, that is to be considered in RS preoperative period and require following studies.
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Hiperesplenismo , Hipertensión Portal , Trombosis , Várices , Niño , Masculino , Femenino , Humanos , Preescolar , Derivación Portosistémica Quirúrgica/efectos adversos , Vena Porta/cirugía , Estudios Retrospectivos , Hiperesplenismo/complicaciones , Hiperesplenismo/cirugía , Hipertensión Portal/complicaciones , Hipertensión Portal/cirugía , Trombosis/cirugía , Trombosis/complicaciones , Várices/complicacionesRESUMEN
Rex shunt, which was first put in use in 1992, has been considered as an ideal surgical method for the treatment of extra-hepatic portal venous obstruction (EHPVO) due to its reconstruction of the hepatopetal portal blood flow. However, despite its long tradition, there are only a few reports about the application and advances in Rex shunt for the treatment of EHPVO in children. In this paper, we summarized the literature related to Rex shunt and discussed the new advances of Rex shunt in the following aspects: surgical method of Rex shunt, the indications of Rex shunt, the strengths of Rex shunt, the effectiveness of Rex shunt, factors affecting the efficacy of Rex shunt, methods that improve the prognosis of Rex shunt, and treatment strategy for recurrence after Rex shunt.
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BACKGROUND: Extrahepatic portal vein obstruction (EHPVO) is the most important cause of hematemesis in children. Intrahepatic left portal vein and superior mesenteric vein anastomosis, also known as meso-Rex bypass (MRB), is becoming the gold standard treatment for EHPVO. We analyzed the value of preoperative computed tomography (CT) in determining whether MRB is feasible in children with EHPVO. RESULTS: We retrieved data on 76 children with EHPVO (50 male, 26 female; median age, 5.9 years) who underwent MRB (n = 68) or the Warren procedure (n = 8) from 2013 to 2019 and retrospectively analyzed their clinical and CT characteristics. The Rex recess was categorized into four subtypes (types 1-4) depending on its diameter in CT images. Of all 76 children, 7.9% had a history of umbilical catheterization and 1.3% had leukemia. Sixteen patients (20 lesions) had associated malformations. A total of 72.4% of Rex recesses could be measured by CT, and their mean diameter was 3.5 ± 1.8 mm (range 0.6-10.5 mm). A type 1, 2, 3, and 4 Rex recess was present in 9.2%, 53.9%, 11.8%, and 25.0% of patients, respectively. MRB could be performed in patients with types 1, 2, and 3, but those with type 4 required further evaluation. The sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of CT were 100%, 83.8%, 42.1%, 100%, and 85.5%, respectively. CONCLUSIONS: Among the four types of Rex recesses on CT angiography, types 1-3 allow for the performance of MRB.
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BACKGROUND: The Rex shunt was widely used as the preferred surgical approach for cavernous transformation of the portal vein (CTPV) in children that creates a bypass between the superior mesenteric vein and the intrahepatic left portal vein (LPV). This procedure can relieve portal hypertension and restore physiological hepatopetal flow. However, the modified procedure is technically demanding because it is difficult to make an end-to-end anastomosis of a bypass to a hypoplastic LPV. Many studies reported using a recanalized umbilical vein as a conduit to resolve this problem. However, the feasibility of umbilical vein recanalization for a Rex shunt has not been fully investigated. AIM: To investigate the efficacy of a recanalized umbilical vein as a conduit for a Rex shunt on CTPV in children by ultrasonography. METHODS: A total of 47 children who were diagnosed with CTPV with prehepatic portal hypertension in the Second Hospital, Cheeloo College of Medicine, Shandong University, were enrolled in this study. Fifteen children received a recanalized umbilical vein as a conduit for a Rex shunt surgery and were enrolled in group I. Thirty-two children received the classic Rex shunt surgery and were enrolled in group II. The sonographic features of the two groups related to intraoperative and postoperative variation in terms of bypass vessel and the LPV were compared. RESULTS: The patency rate of group I (60.0%, 9/15) was significantly lower than that of group II (87.5%, 28/32) 7 d after (on the 8th d) operation (P < 0.05). After clinical anticoagulation treatment for 3 mo, there was no significant difference in the patency rate between group I (86.7%, 13/15) and group II (90.6%, 29/32) (P > 0.05). Moreover, 3 mo after (at the beginning of the 4th mo) surgery, the inner diameter significantly widened and flow velocity notably increased for the bypass vessels and the sagittal part of the LPV compared to intraoperative values in both shunt groups (P < 0.05). However, there was no significant difference between the two surgical groups 3 mo after surgery (P > 0.05). CONCLUSION: For children with hypoplastic LPV in the Rex recessus, using a recanalized umbilical vein as a conduit for a Rex shunt may be an effective procedure for CTPV treatment.
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OBJECTIVE: To assess the clinical application of preoperative retrograde portal venography for children with cavernous transformation of the portal vein (CTPV). METHODS: The clinical data of 8 cases of CTPV admitted in the Children's Hospital of Zhejiang University from January 2018 to September 2019 were retrospectively analyzed. Preoperative retrograde portography was performed to determine the corresponding vascular morphology and size of portal vein system. If the retrograde portography showed that the left branch of the shadow portal vein was unobstructed and its diameter was greater than 3 mm, Rex shunt would be performed after anatomic exploration of Rex recess; if retrograde portography showed that the diameter of left portal vein was less than 3 mm, but the diameter of left renal vein dissected during shunt operation was greater than 5 mm, Warren operation was selected. The patients were followed up for 1, 3 and 6 months after discharge, and then were followed up every 6 months. RESULTS: Retrograde portal venography was successfully performed in 8 child patients.The anatomical position and size of main portal vein and its left and right branches, left renal vein and other important vessels were determined. Among them, there was the well-developed left and right branches of portal vein in 4 child patients, in which the left and right branches of portal vein converged together, but did not communicate with the main portal vein. In addition, the left branch diameter of the portal vein was greater than 3 mm, and the anatomical exploration results during shunt were consistent with it, so Rex shunt was performed. In the other 4 cases, the left branch diameter of the portal vein was small (less than 3 mm) in 3 cases, and the right branch was not clearly developed. Moreover, the left branch of the portal vein was poorly developed and almost occluded in 1 case. However, the left renal vein in these 4 child patients was well developed, the blood flow was unobstructed and the diameter was greater than 5 mm, so Warren operation was performed. Seven patients recovered well after the operation, and the other one had digestive tract rudimentary one year after operation, and the condition was stable after conservative treatment. CONCLUSIONS: The preoperative retrograde portal venography can be used to evaluate the portal vein system in children with CTPV, which provides important clinical basis for making appropriate treatment plan before surgery.
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Vena Porta , Portografía , Niño , Humanos , Vena Porta/diagnóstico por imagen , Vena Porta/cirugía , Estudios RetrospectivosRESUMEN
Extrahepatic portal vein obstruction (EHPVO) is the most common cause of pediatric portal hypertension and can cause life-threatening variceal bleeding. Meso- Rex shunt (MRS) is a surgical procedure that restores physiological portal venous blood flow to the liver by using a graft to connect the superior mesenteric vein and the left portal vein within the Rex recess, and can relieve variceal bleeding and other complications associated with EHPVO. Although the MRS is regarded as an optimal and potentially curative treatment with good long-term patency, graft thrombosis or failure due to unknown causes is not rare, prompting the need for further research on the risk factors of graft failure or poor patency. Herein, we report two cases of EHPVO in patients with recurrent or uncontrolled variceal bleeding, one treated with the classic Rex shunt and the other with the modified Rex shunt, which resulted in a failure and success, respectively.
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PURPOSE: To report our initial experience with splenectomy and proximal spleno-left portal shunt as an alternative to the standard Rex shunt, when not applicable, in children with Extrahepatic Portal Vein Obstruction (EHPVO). METHODS: Patients from March 2015 till September 2018, with EHPVO not suitable for Rex shunt or whose caregivers refused to consent for Internal Jugular Vein (IJV) dissection were assessed and prepared for splenectomy with proximal spleno-left portal shunt. The operative technique includes splenectomy, freeing of the splenic vein from the pancreatic bed till its junction with the inferior mesenteric vein, and then anastomosis with the intrahepatic left portal vein at the Rex recess. A distal lieno-renal shunt was performed in one patient and was excluded from the study. RESULTS: A total of 14 patients (mean age: 4.6â¯years) underwent splenectomy with proximal spleno-left portal shunt during the study period. The mean operative time was 246â¯min, while the mean postoperative hospital stay was 4.1â¯days. The patients' follow up period ranged from 6 to 42â¯months (median: 19.6â¯months). Only two patients had a single attack of variceal bleeding, 2 and 2.5â¯months postoperative respectively, and required endoscopic management with no further bleeding episodes. While the rest of patients showed an improvement of their variceal grades after the surgery. CONCLUSION: Splenectomy with proximal spleno-left portal shunt seems to be a valuable alternative to the standard Rex shunt in treatment of children with EHPVO unsuitable for or following unsuccessful Rex shunt. LEVEL OF EVIDENCE: IV.
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Hemorragia Gastrointestinal/etiología , Vena Porta/cirugía , Derivación Portosistémica Quirúrgica/métodos , Esplenectomía , Vena Esplénica/cirugía , Enfermedades Vasculares/cirugía , Anastomosis Quirúrgica/métodos , Niño , Preescolar , Várices Esofágicas y Gástricas/etiología , Femenino , Humanos , Hipertensión Portal/etiología , Hipertensión Portal/cirugía , Tiempo de Internación , Masculino , Tempo Operativo , Enfermedades Vasculares/complicacionesRESUMEN
OBJECTIVE: This study investigated the morphologic changes of portal cavernoma in children with extrahepatic portal venous obstruction and explored the relationship with prognosis. METHODS: From February 2008 to October 2017, there were 107 patients with cavernous transformation of the portal vein admitted to our hospital. Rex shunts were performed in 99 cases, Warren shunts in 7 cases, and laparoscopic splenic vessel ligation in 1 case. Intraoperative superior mesenteric venography was used to determine the structure of the portal venous system. According to the morphologic features of the portal vein shown by portal venography, groups were assigned as follows: patients with the cotton form of portal cavernoma; patients with visible collateral veins of portal cavernoma; patients with and without a visible left gastric vein; and patients with and without a clearly visible intrahepatic portal vein. The preoperative and postoperative portal pressure, preoperative incidence of esophageal varices, time at onset, incidence of postoperative rebleeding, preoperative and postoperative size of the spleen, and age at time of operation were compared between these groups. RESULTS: The preoperative incidence of esophageal varices, time at onset, postoperative size of spleen, and age at time of operation were significantly lower in the group with the cotton form than in those with visible collateral veins (P < .05). There was a significant correlation between the visible left gastric vein and esophageal varices (P = .002). The time at onset, preoperative and postoperative size of the spleen, and age at time of operation were markedly lower in the group with a good visible intrahepatic portal vein than in those without a clearly visible intrahepatic portal vein (P < .05). The visible left gastric vein was notably associated with the performance of a gastroportal shunt (P = .000), and the group with a visible left gastric vein had a higher ratio of children undergoing a gastroportal shunt. CONCLUSIONS: The cotton form, an early-stage manifestation of cavernous transformation of the portal vein, typically occurs in younger children with a shorter time to onset. Children with the cotton form of portal cavernoma typically have a better prognosis after Rex shunt.
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Vena Porta/diagnóstico por imagen , Portografía , Enfermedades Vasculares/diagnóstico por imagen , Adolescente , Niño , Preescolar , Toma de Decisiones Clínicas , Circulación Colateral , Constricción Patológica , Várices Esofágicas y Gástricas/diagnóstico por imagen , Várices Esofágicas y Gástricas/etiología , Várices Esofágicas y Gástricas/cirugía , Femenino , Hemorragia Gastrointestinal/diagnóstico por imagen , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/cirugía , Humanos , Hiperesplenismo/diagnóstico por imagen , Hiperesplenismo/etiología , Hiperesplenismo/cirugía , Hipertensión Portal/diagnóstico por imagen , Hipertensión Portal/etiología , Hipertensión Portal/cirugía , Lactante , Circulación Hepática , Masculino , Presión Portal , Vena Porta/fisiopatología , Vena Porta/cirugía , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Enfermedades Vasculares/complicaciones , Enfermedades Vasculares/fisiopatología , Enfermedades Vasculares/cirugíaRESUMEN
OBJECTIVE@#To assess the clinical application of preoperative retrograde portal venography for children with cavernous transformation of the portal vein (CTPV).@*METHODS@#The clinical data of 8 cases of CTPV admitted in the Children's Hospital of Zhejiang University from January 2018 to September 2019 were retrospectively analyzed. Preoperative retrograde portography was performed to determine the corresponding vascular morphology and size of portal vein system. If the retrograde portography showed that the left branch of the shadow portal vein was unobstructed and its diameter was greater than 3 mm, Rex shunt would be performed after anatomic exploration of Rex recess; if retrograde portography showed that the diameter of left portal vein was less than 3 mm, but the diameter of left renal vein dissected during shunt operation was greater than 5 mm, Warren operation was selected. The patients were followed up for 1, 3 and 6 months after discharge, and then were followed up every 6 months.@*RESULTS@#Retrograde portal venography was successfully performed in 8 child patients.The anatomical position and size of main portal vein and its left and right branches, left renal vein and other important vessels were determined. Among them, there was the well-developed left and right branches of portal vein in 4 child patients, in which the left and right branches of portal vein converged together, but did not communicate with the main portal vein. In addition, the left branch diameter of the portal vein was greater than 3 mm, and the anatomical exploration results during shunt were consistent with it, so Rex shunt was performed. In the other 4 cases, the left branch diameter of the portal vein was small (less than 3 mm) in 3 cases, and the right branch was not clearly developed. Moreover, the left branch of the portal vein was poorly developed and almost occluded in 1 case. However, the left renal vein in these 4 child patients was well developed, the blood flow was unobstructed and the diameter was greater than 5 mm, so Warren operation was performed. Seven patients recovered well after the operation, and the other one had digestive tract rudimentary one year after operation, and the condition was stable after conservative treatment.@*CONCLUSIONS@#The preoperative retrograde portal venography can be used to evaluate the portal vein system in children with CTPV, which provides important clinical basis for making appropriate treatment plan before surgery.
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Niño , Humanos , Vena Porta/cirugía , Portografía , Estudios RetrospectivosRESUMEN
Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are prototype noncirrhotic causes of portal hypertension (PHT), characterized by normal hepatic venous pressure gradient, variceal bleeds, and moderate to massive splenomegaly with preserved liver synthetic functions. Infections, toxins, and immunologic, prothrombotic and genetic disorders are possible causes in IPH, whereas prothrombotic and local factors around the portal vein lead to EHPVO. Growth failure, portal biliopathy, and minimal hepatic encephalopathy are long-term concerns in EHPVO. Surgical shunts and transjugular intrahepatic portosystemic shunt resolve the complications secondary to PHT. Meso-Rex shunt is now the standard-of-care surgery in children with EHPVO.
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Hipertensión Portal/fisiopatología , Cirrosis Hepática/fisiopatología , Pancitopenia/fisiopatología , Vena Porta , Esplenomegalia/fisiopatología , Trombosis de la Vena/fisiopatología , Animales , Enfermedades de las Vías Biliares/etiología , Enfermedades de las Vías Biliares/terapia , Manejo de la Enfermedad , Modelos Animales de Enfermedad , Progresión de la Enfermedad , Várices Esofágicas y Gástricas , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/prevención & control , Hemorragia Gastrointestinal/terapia , Trastornos del Crecimiento/etiología , Encefalopatía Hepática/etiología , Encefalopatía Hepática/terapia , Humanos , Hipertensión Portal/complicaciones , Hipertensión Portal/diagnóstico , Hipertensión Portal/etiología , Hipertensión Portal/metabolismo , Hipertensión Portal/terapia , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/metabolismo , Trasplante de Hígado , Metabolómica , Pancitopenia/complicaciones , Pancitopenia/diagnóstico , Pancitopenia/metabolismo , Derivación Portosistémica Quirúrgica , Derivación Portosistémica Intrahepática Transyugular , Calidad de Vida , Esplenomegalia/complicaciones , Esplenomegalia/diagnóstico , Esplenomegalia/metabolismo , Transcriptoma , Trombosis de la Vena/complicaciones , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/metabolismo , Hipertensión Portal Idiopática no CirróticaRESUMEN
Interventional radiology's role in the management of portal hypertension in the pediatric population differs from the management of adult portal hypertension. In the pediatric population, portal hypertension is frequently secondary to thrombosis and cavernous transformation of the extrahepatic portion of the portal vein. Transjugular intrahepatic portosystemic shunt can be utilized to manage portal hypertension in children with intrinsic liver disease that results in cirrhosis and portal hypertension, and is often used as a bridge to transplant. While technically feasible in extrahepatic portal vein occlusion, the sequelae of portosystemic shunting are less desirable in a child. The Meso-Rex bypass procedure, which represents the mainstay of management for pediatric portal hypertension, provides surgical relief of extrahepatic portal vein obstruction and restores mesenteric venous blood flow to the liver. This article aims to review management of portal hypertension in children as it pertains to the interventional radiologist, including preoperative assessment, postoperative evaluation, and the management of complications of the Meso-Rex bypass.
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OBJECTIVE: Patency of the bypass vein after a Rex shunt is an important indicator of prognosis. However, there is no report about the change of caliber of the bypass vein after a Rex shunt. The aim of this study was to identify postoperative changes of the bypass vein and to assess the relationship with prognosis. METHODS: Between October 2008 and October 2016 in our center, 114 children were diagnosed with extrahepatic portal venous obstruction. The portal cavernoma-portal bypass with interposition of grafted portal vessels was performed in 31 children, the gastroportal shunt was performed in 54 children, and other Rex shunts were performed in another 29 children. At follow-up, the patency and diameter of the bypass vein were assessed with ultrasound and computed tomography. The intraoperative and postoperative diameters of the bypass vein were compared to identify postoperative changes of the bypass vein. Prognosis was compared between children with and children without an enlarged bypass vein. RESULTS: The caliber of the bypass vein was enlarged in 50% of children (40/80) at 6 months postoperatively. The postoperative incidences of rebleeding and esophageal varices were significantly lower in children with an enlarged bypass vein than in those without (P < .05). Postoperatively, the reduced splenic size was significantly higher in children with an enlarged bypass vein than in those without (P < .05). The postoperative increase in platelet count in children with an enlarged bypass vein was significantly higher than in those without (P = .006). There was no significant difference in the flow velocity of the bypass vein between children with and children without an enlarged bypass vein (P = .133). The portal pressure was significantly reduced after surgery in children with an enlarged bypass vein than in children without an enlarged bypass vein (P = .017). CONCLUSIONS: The caliber of the bypass vein increases in 50% of children after a Rex shunt using a grafted portal vessel, which is related to a better prognosis.
Asunto(s)
Hipertensión Portal/cirugía , Vena Porta/cirugía , Derivación Portosistémica Quirúrgica/métodos , Adolescente , Factores de Edad , Niño , Preescolar , Angiografía por Tomografía Computarizada , Femenino , Humanos , Hipertensión Portal/diagnóstico por imagen , Hipertensión Portal/fisiopatología , Lactante , Circulación Hepática , Masculino , Flebografía/métodos , Presión Portal , Vena Porta/diagnóstico por imagen , Vena Porta/fisiopatología , Derivación Portosistémica Quirúrgica/efectos adversos , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Ultrasonografía Doppler , Grado de Desobstrucción VascularRESUMEN
PURPOSE: Although Rex shunt is an effective treatment for extrahepatic portal venous obstruction (EHPVO), 4-20% children develop rebleeding postoperatively. This study was used to evaluate the surgical treatment of rebleeding after Rex shunt in our center. METHOD: From June 2008 to Jan 2017, 12 of 122 children with EHPVO underwent a second operation due to graft stenosis and occlusion after Rex shunt in our center. The abdominal ultrasound and computed tomography (CT) showed the occlusion of bypass vein in nine children, stenosis of bypass vein in two children, and the patency of bypass vein in one child with dysplasia of intrahepatic portal vein. A re-Rex shunt was performed in eight children, of which one child required conversion to Warren shunt due to postoperative rebleeding. Five children underwent Warren shunt due to a narrowed left portal vein shown by intraoperative portal angiography and surgical exploration. All patients were followed up in this study. RESULTS: The postoperative incidences of re-bleeding and esophageal varices in children with Warren shunt were significantly lower than those in children undergoing re-Rex shunt (P = 0.027 and 0.015). After a second operation, the rate of bypass vein patency in children with re-Rex shunt was lower than that in children with Warren shunt (50 vs. 100%). The postoperative reduced size of spleen in children undergoing Warren shunt was significantly higher than that of children undergoing re-Rex shunt (P < 0.05). CONCLUSIONS: Comparing to re-Rex shunt, Warren shunt was a better treatment for rebleeding caused by bypass failure after Rex shunt.
Asunto(s)
Oclusión de Injerto Vascular/cirugía , Hemostasis Quirúrgica/métodos , Hipertensión Portal/cirugía , Vena Porta/cirugía , Derivación Portosistémica Quirúrgica/efectos adversos , Hemorragia Posoperatoria/cirugía , Procedimientos Quirúrgicos Vasculares/métodos , Niño , Preescolar , Femenino , Oclusión de Injerto Vascular/etiología , Humanos , Masculino , Vena Porta/diagnóstico por imagen , Hemorragia Posoperatoria/diagnóstico , Hemorragia Posoperatoria/etiología , Reoperación , Estudios Retrospectivos , Resultado del Tratamiento , UltrasonografíaRESUMEN
BACKGROUND: Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM: The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO. CONCLUSION: This review gives a detailed summary of these two vascular conditions of liver-IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.