Extrahepatic Portal Hypertension Research Articles

A Unique Case of Noncirrhotic Portal Hypertension Secondary to Extrahepatic Sarcoidosis

Introduction: Sarcoidosis is a multisystem disease of unknown etiology characterized by the presence of non-caseating granulomas. Portal hypertension is a rare manifestation of sarcoidosis, first reported in 1949. Here we report the first case of extrahepatic sarcoidosis with granulomatous lymph node involvement at the porta hepatis, compression of portal vein leading to portal hypertension and recurrent episodes of gastrointestinal bleeding due to jejunal varices. A 57-year-old black female presented with pruritus, jaundice, elevated liver enzymes, and thrombocytopenia. Diagnosed with ophthalmic and pulmonary sarcoidosis in 1998, a mass at the porta hepatis causing intrahepatic dilation was now seen on CT abdomen. ERCP revealed a stricture at the proximal hepatic duct below the bifurcation, relieved by placing a biliary stent. Diagnostic exploratory laparotomy showed unresectable massive lymphadenopathy, extending from porta hepatis to the inferior aspect of liver, encroaching the vessels and bile ducts. Intrahepatic right bile duct choledoco-Roux-en-Y-jejunostomy was completed. Biopsy showed marked fibrosis and noncaseating granulomas of lymph nodes. Liver biopsy was free of sarcoidosis. Repeat episodes of pruritis and jaundice were treated with high-dose steroids and mycophenolate mofetil. Patient was readmitted with melena and severe anemia. Imaging showed slight decrease in size of the mass but effacement of extrahepatic portal vein, non-visualization of the left portal vein, and severe narrowing of right portal vein. Double balloon enteroscopy revealed large, bleeding perianastomotic jejunal varices. Endoscopic therapy failed and varices were surgically oversawn. Mycophenalate mofetil was restarted along with steroids. A year later recurrent episodes of severe bleeding developed. High-dose pulse steroid therapy was initiated and mycophenalte was increased. Mesocaval shunting was completed with temporary control of bleeding before occlusion. Patient was kept on high-dose steroids, mycophenalate, octreotide and nadolol. Later, CT abdomen revealed reduction in size of mass with partial visualization of the right branch of the portal vein. TIPS was completed via cannulation of the right portal vein, leading to resolution of portal hypertension and further episodes of GI bleed. Extrahepatic portal hypertension in the setting of porta hepatis sarcoidosis can be life threatening and very challenging to treat. Here we described how a combination immunosuppressant and radiologically placed shunt can be used successfully to treat recurrent episodes of GI bleed in such a patient.

Aggressive Resection in Patients with Advanced Pancreatic Neuroendocrine Tumors: Case Report and Review of the Literature

There is considerable controversy about the treatment of patients with malignant advanced pancreatic neuroendocrine tumors (PNETs). Aggressive surgery remains a potentially efficacious anti-tumor therapy but is rarely performed because of its possible morbidity and mortality. The purpose of this work is to describe a case of PNETs who underwent aggressive multivisceral pancreatic resection and the literature's review. Case report : A 52- year old man with no medical history, who presented for abdominal pain, recent constipation and intermittent rectal bleeding. Clinically, we found a tender mass of 10x7 cm in the left upper quadrant. Colonoscopy revealed an irregular lesion of 2 cm in the splenic flexure with histological diagnosis at biopsies suggesting a pancreatic neoplastic origin. Computed tomography (CT) of the abdomen demonstrated a large mass in the left pancreas invading the spleen and its vessels, the adjacent left transverse colon with sign of segmental extrahepatic portal hypertension. There were no liver metastases or suspected adenopathies. He underwent laparotomy with extended resection of the left pancreas, the spleen and the left colon. Histological examination confirmed a well differentiated PNET with Ki67 < 2%. The post operative course was uneventful. After 16 months of follow, the patient remains well. There is no sign of recurrence at CT. Conclusion : Many retrospective studies and the European neuroendocrine Tumors’ (ENETs) guidelines claim that debulking and aggressive surgical resection in locally advanced PNETs should be considered in selected patients because it is technically feasible with encouraging disease-specific survival.

RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION

Aim . To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fi brosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension. Description . Patient F., 15 years old, was diagnosed with Congenital Heart Disease – double-chambered right ventricle, ventricular septal defect. Fibromuscular subaortic stenosis. Aortic valve insuffi ciency II. Circulatory failure II A., functional class III. Extrahepatic portal hypertension. Splenomegaly. Thrombocytopenia. The state after the imposition of splenorenal anastomosis in September 1998 and the imposition of mesocaval H-shaped anastomosis in 1998. Non-functioning anastomoses. Patient indicated for surgical correction of CHD, however, given the low level of platelets, expressed splenomegaly, leucopenia, the patient was referred for a preliminary treatment to Rogachev’s Pediatric Hematology, Oncology and Immunology Center. The treatment (stimulating thrombopoiesis using romiplostim) gave no signifi cant effect. Platelet count reached 70–90 A~ 10 9 . Seeing of frequent bleeding from esophageal varices, the patient underwent varix sclerosis. At the time of hospitalization – no esophageal varices. The patient appealed to V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs where he was recommended for surgical treatment. Result . The patient performed surgery: radical correction of CHD: resection of the stenosis of the outfl ow tract of the right ventricle, subaortic stenosis resection, closure of ventricular septal defect, AV plasty under cardiopulmonary bypass. The early postoperative period was uneventful. Leukopenia was observed to 1,2 A~ 10 9 , thrombocytopenia 70–90 A~ 10 9 . Despite the low level of platelets bleeding in pre-and postoperative period was not registered. Antibiotic therapy with tienam. Good postoperative results were obtained. Liver function was not affected. Conclusion . Presented clinical case demonstrates the possibility of surgical intervention of combined heart disease with cardiopulmonary bypass in patient with extrahepatic portal hypertension.

Renal blood flow before and after portosystemic shunt in children with portal hypertension

Variceal haemorrhage in children with extrahepatic portal hypertension is best controlled by an effective decompressive shunt, but long-term follow up of children who have had splenorenal shunt due to extrahepatic portal hypertension (EHPH) gave evidence for assuming the risk of renal venous hypertension (RVH). To study renal hemodynamic before and after portal decompression. The results of 144 portosystemic shunt operations were followed from 2005 to 2013. Seventy-two patients applied central splenorenal shunt (CSS) with splenectomy, ten side-to-side splenorenal shunt without splenectomy (SRSss), ten patients assessed the distal splenorenal shunt (DSS). Forty-three iliacomesenterial anastomosis (IMA) and in nine cases performed mesocaval anastomosis (MCA). Children had a standard pre- and postoperative work up including gastrointestinal endoscopy, Doppler ultrasonography (US), multi-slice computed tomography (MSCT) and renography. In 11 (15.2 %) patients after CSS on duplex, Doppler study revealed signs of impeded venous outflow on the left renal vein (LRV). At long-term follow-up PI and RI of left renal artery remained at high numbers (1.48 ± 0.17 and 0.72 ± 0.19, p ≤ 0.05, respectively) after the CSS. Venous blood flow in the LRV at the hilum showed slower speed performance in groups of CSS and after IMA. After DSS, these signs have not been detected. Four patients after IMA on US Doppler and CT angiography revealed dilated left testicular and ovarian veins, with retrograde blood flow in them, which clinically manifested as left flank pain, macro- and microhematuria, varicocele and ovaricocele. The study shows that CSS and IMA more negatively effect on hemodynamics of left kidney and symptoms of RVH obviously due to shunting the large amounts of blood from a system of high pressure to a low.

Partial splenectomy and use of splenic vein as an autograft for meso-Rex bypass: a clinical observational study.

BackgroundMeso-Rex bypass (MRB) surgery is being increasingly used to treat chronic prehepatic portal hypertension secondary to extrahepatic portal vein thrombosis (EPVT) and cavernous transformation (EPVCT) in children. Rather than using the internal jugular vein (IJV, the traditional venous graft), we used an autogenous splenic vein segment graft for MRB.Material/MethodsWe examined 25 children with extrahepatic portal hypertension and a history of recurrent upper gastrointestinal (GI) variceal bleeding despite previous endoscopic sclerotherapy. All patients had melena, splenomegaly, hypersplenism, or some combination thereof. Left portal vein (LPV) patency was verified in 22 patients using intraoperative direct portography through the umbilical vein. Partial splenectomy was performed to enable the harvest of the splenic vein trunk, which was anastomosed between the superior mesenteric vein (SMV) and the left portal vein (LPV). All patients were followed for 12–48 months (mean=25.6 months) and no patients were lost to follow-up.ResultsPreoperative Doppler ultrasound (US) imaging indicated that 18/25 patients had adequate intrahepatic portal veins for shunting, with no blood flow in the LPVs of 7 patients. LPV patency in 22/25 patients was verified using intraoperative direct portography, with successful MRB. Shunting was converted into a portosystemic shunt in the remaining 3/25 patients with thrombosed LPVs. A Doppler US evaluation of the vein conduit revealed excellent postoperative flow. The patients’ mean hemoglobin, platelet, and white blood cell counts increased significantly, and in all cases the endoscopic status obviously improved after shunting. Occlusion or narrowing occurred in 2/22 patients after discharge. At 12 months (for 1 patient) and 24 months (for 1 patient), the shunt was converted into a portosystemic shunt. The cumulative graft patency rate was 91% (20/22).ConclusionsPartial splenectomy and splenic vein autografting in MRB surgery can successfully resolve prehepatic portal hypertension and hypersplenism in children.

Warren shunt combined with partial splenectomy in children with extra-hepatic portal hypertension, massive splenomegaly and severe hypersplenism

Extra-hepatic portal vein obstruction (EHPVO) is one of the most often causes of portal hypertension in children. Establishing the importance of shunt surgery in combination with partial spleen resection in selected pediatric patients with EHPVO, enormous splenomegaly and severe hypersplenism. Distal splenorenal shunt (DSRS) with partial spleen resection was performed in 22 children age from 2 to 17 years with EHPVO. Indications for surgery were pain and abdominal discomfort caused by spleen enlargement, as well as symptomatic hypersplenism with leucopenia, thrombocytopenia and anemia. The partial spleen resection was performed by ligation of blood vessels to caudal two thirds of the spleen. After ischemic parenchymal demarcation transection with electrocautery LigaSure was performed with preservation of 20-30% of spleen tissue, and then Warren DSRS was created. Platelet and leucocytes counts and liver function tests were obtained before, one month and one year after surgery. Growth was assessed with SD scores (Z scores) for height, weight and body mass index at the time of surgery and one year later. In all patients postoperative period was without significant complications. Platelets and leucocytes counts were normalized. Patency rate of shunts was 100%. Two significant shunts stenosis were observed and successfully treated with percutaneous angioplasty. During the follow-up period (1 to 9 years) all patients were asymptomatic, with improved quality of life and growth. Results of our study indicate that shunt surgery with a partial spleen resection is an effective and safe procedure for patients with enormous splenomegaly and severe hypersplenism caused by EHPVO.

Portal hypertension: A critical appraisal of shunt procedures with emphasis on distal splenorenal shunt in children.

Background:Extrahepatic portal venous obstruction (EHPVO) is the most common cause of pediatric portal hypertension. We analyzed the investigative protocol and results of portosystemic shunts in this group of patients.Materials and Methods:A total of 40 consecutive children aged below 12 years operated with a diagnosis of extra-hepatic portal hypertension formed the study group. Historical data and clinical data were collected. All patients underwent upper gastrointestinal endoscopy, ultrasound Doppler and computed tomographic portogram pre-operatively and post-operatively. Results with respect to shunt patency, hypersplenism and efficacy of different radiological investigations were collected.Results:A total of 40 patients, 28 boys and 12 girls constituted the study group. Lienorenal shunt (LRS) was performed in 14 patients; distal splenorenal shunt in 21 patients and side-to-side lienorenal shunt in 4 patients, inferior mesenteric renal shunt was performed in 1 patient. Follow-up ranged from 36 to 70 months. At a minimum follow-up of 3 years, 32 (80%) patients were found to have patent shunts. Patent shunts could be visualized in 30/32 patients with computer tomographic portogram (CTP) and 28/32 with ultrasound. Varices regressed completely in 26/32 patients and in the rest incomplete regression was seen. Spleen completely regressed in 19/25 patients. Hypersplenism resolved in all patients with patent shunts.Conclusions:Portosystemic shunting in children with EHPVO is a viable option. While long-term cure rates are comparable with sclerotherapy, repeated hospital visits are reduced with one time surgery. Pre-operative and post-operative assessment can be performed with complimentary use of ultrasound, CTP and endoscopy.

Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: Anatomy and clinical relevance

BackgroundChildren with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not. MethodsAll children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography. Venous anatomy was categorized into five subtypes (A to E), depending on the presence of thrombosis in the Rex recessus, or not, and its extension within the intrahepatic portal venous system. ResultsEighty-nine children entered the study. Previous umbilical vein catheterization is usually associated with Rex thrombosis, while the Rex recessus and the intrahepatic portal venous system are patent in idiopathic cases, thus allowing for the performance of a Meso-Rex Bypass with a good outcome. ConclusionsWedged hepatic venous portography is a very effective tool for detailed preoperative assessment and identification of children being considered for Meso-Rex Bypass surgery. An anatomic–radiological classification is useful in selecting patients for Meso-Rex Bypass with anticipation of a high rate of success.

Unshuntable extrahepatic portal hypertension revisited: 43 years' experience with radical esophagogastrectomy treatment of bleeding esophagogastric varices

BackgroundIn 1994, the authors reported their experience with radical esophagogastrectomy for bleeding esophagogastric varices due to unshuntable extra-hepatic portal hypertension. Since then, the series has expanded from 22 to 44 patients. The aim of this study was to assess the validity of the previous observations and conclusions in the largest series with the longest follow-up. MethodsFrom 1968 to 2005, 44 patients with unshuntable extra-hepatic portal hypertension were treated by total gastrectomy and resection of the distal two thirds of the esophagus. Before referral, the patients experienced 4 to 24 episodes of variceal bleeding requiring a mean 130 U of blood transfusion, 15 hospital admissions, and 6 previous unsuccessful operations. ResultsTransient postoperative complications occurred in 50% of patients. The survival rate is 100%, with no recurrence of variceal bleeding during 7 to 43 years of follow-up. Liver function and biopsy results have been normal. Quality of life has been excellent or good in 91%. Eighty-six percent have resumed employment or full-time housekeeping. ConclusionsIn unshuntable extra-hepatic portal hypertension, radical esophagogastrectomy is the only consistently effective treatment of variceal hemorrhage. Prompt use of this lifesaving procedure is warranted.

Unilateral Hypoplastic Kidney in a Case of Holt-Oram Syndrome

Holt-Oram syndrome (HOS) is characterised by skeletal abnormalities of the upper limb &amp; congenital cardiac defects. Here we report a case of one day old male baby with skeletal abnormalities of forearms and hands, atrial septal defect of ostium secundum type and unilateral hypoplastic kidney. Till date, other associated anomalies like extra-hepatic portal hypertension, renal artery malformations etc have been reported with Holt-Oram syndrome, but it is the first case to be reported in association with hypoplastic kidney. DOI: http://dx.doi.org/10.3126/jnps.v33i1.7127 J Nepal Paediatr Soc. 2013;33(1):77-79

PWE-059 Single Dose Endoscopic Thrombin Injection for Acute Variceal Bleeding

IntroductionEndoscopic Human Thrombin injection appears a technically simple yet efficacious alternative to cyanoacrylate for gastric varices with fewer complications from studies to date, but data remains limited. We evaluated the...

Hepatic Artery Doppler Indices in Children with Extra Hepatic Portal Vein Obstruction

Doppler measurement provides information on the hemodynamics in the hepatic artery and the portal venous system. To study the hepatic artery hemodynamics in children with extra hepatic portal vein obstruction. Hepatic artery indices were studied using Doppler indices in 15 children (<12 years) with extra hepatic portal hypertension (EHPVO) and obliterated esophageal varices. The hepatic artery resistive index, the arterial acceleration time and the acceleration index were used to determine the flow pattern within the hepatic artery. Controls were 15 healthy age-sex matched children, belonging to the same socioeconomic strata in absence of liver disease. The mean age of the children was 8.43±3.2 years and male female ratio was 4:1. All the children had obliterated esophageal varices. The hepatic artery resistive index in the children with EHPVO was normal and similar to controls. The hepatic arterial early systolic acceleration index was significantly higher in cases compared to controls (436±290 vs 214±100; P value <0.004). The hepatic arterial acceleration time though low in the cases (86±35cm/s) was not statistically different from the controls (128±14cm/s). There was a significant increase in hepatic arterial early systolic acceleration in children with chronic EHPVO. The latter may be responsible for an increase in hepatic arterial in flow velocity in a slow flow system despite a normal resistive index.

Erratum to: Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism

Erratum to: Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism

Varices of the Descending Duodenum Explored During Emergency Gastro-Duodenal Resection for Upper Gastrointestinal Haemorrhage. Case Report

Upper gastrointestinal haemorrhage is a major medical emergency and accounts for approximately 7,000 admissions to hospitals in Scotland each year. Over the last 10 years there has been a number of improvements in diagnosis and conservative management of the condition, which significantly reduced the ratio of life-threatening cases requiring an emergency surgery. Despite these achievements surgical intervention or, if accessible, endovascular procedures must be undertaken as emergency actions, should conservative management fail. Vascular malformations of the duodenum are less frequent causes of upper GI bleeding. Duodenal varices found endoscopically occur in 0.4% of patients with portal hypertension (PHT) and are believed to be caused mainly by liver cirrhosis, idiopathic PHT, extrahepatic PHT, or previous surgical trauma. The duodenal bulb is their most common site, followed by the second portion of the duodenum. Forty per cent of patients with PHT have duodenal varices at angiography; however, their penetration unusually affects submucosa, hence no symptoms develop. Isolated bleeding duodenal varices are scarcely reported in literature, although present a significant surgical problem: massive haemorrhage combined with failure to identify them as a source has led to catastrophic outcomes with mortality rate of 40%. The case hereby presented is unique in several aspects. Duodenal varices were explored on emergency laparotomy rather than on prior endoscopies, which, performed by the same well-established endoscopists, were twice negative. This corresponds to the study by Cottam et al. stating that duodenal varices may not penetrate the submucosa, hence haemorrhages of their origin may even be more difficult to diagnose on endocsopy. Secondly, the haemorrhage here reported was undoubtedly a life-threatening condition that required a multidisciplinary team to be managed successfully. Along with Shirashi et al. we confirm that surgical ligation followed by the excision of duodenal / small intestinal varices may be an effective method of their management--both cases have been free of recurrence at 15 months postoperatively. In contrast to the study by Hashizume et al. the duodenal varices here presented were not associated with portal hypertension (PTH). Finally, duodenal varices located in the posterolateral aspect of the descending duodenum are less common as the majority of cases reported so far were of duodenal bulb location.

P-068 Gyneco-obstetrical bleeding in a cohort of female patients with factor XI deficiency. A report from French Canada

terms of pregnancy with threatened abortion. 1st pregnancy (2010) was complicated with repeated bleedings from esophagal and gastric varicose veins. At 20 weeks the patient was undergone urgent surgery (gastrotomy, phleborrhaphy of esophagal and gastric varicose veins). The diagnosis that was made was extrahepatic portal hypertension of thrombotic origin. Esophagal and gastric varicose veins bleeding Splenomegaly. Operation was complicated (subdiaphragmatic abscess with burst in the abdominal cavity, peritonitis, sepsis). At 24 weeks spontaneous abortion happened. The patient was urgently transferred to one of the Moscow clinics where she was reoperated and discharged in a satisfactory condition. On laboratory examination we detected HCV antibodies, Factor V Leiden mutation (+−) and polymorphisms of PAI-1 4G/5G, pro-inflammatory cytokines (IL-6, IL-1β, C-reactive protein). Homocystein level was normal.The antiphospholipid syndrome was excluded. At the moment of the address the patient received Sulodeksid, synthetic progesterone, folic acid. We took into account the medical history of the patient and the confirmed thrombophilic condition and corrected the treatment. We prescribed LMWH, natural micronized progesterone, vitamins, compression stockings. The pregnancy course was uncomplicated.At 39 weeks the patient had normal vaginal delivery, uncomplicated. We recommended to check the child for factor V Leiden mutation. In the postnatal period the treatment with LMWH was continued. Conclusion: The splanchnic vein thromboses are challenging. The clinical picture is various, and if the thrombophilic condition is undiagnosed the patient would remain in the risk group for postnatal portal hypertension.

Isolated inferior mesenteric portal hypertension with giant inferior mesenteric vein and anomalous inferior mesenteric vein insertion

Extrahepatic portal hypertension is not an uncommon disease in childhood, but isolated inferior mesenteric portal varices and lower gastrointestinal (GI) bleed have not been reported till date. A 4-year-old girl presented with lower GI bleed. Surgical exploration revealed extrahepatic portal vein obstruction with giant inferior mesenteric vein and colonic varices. Inferior mesenteric vein was joining the superior mesenteric vein. The child was treated successfully with inferior mesenteric – inferior vena caval anastomosis. The child was relieved of GI bleed during the follow-up.

Endoscopic variceal ligation as a safe and effective alternative to endoscopic sclerotherapy in bleeding ectopic duodenal varix—report of a rare case

Sir, Upper gastrointestinal bleeding (UGIB) following devascularization procedures for portal hypertension is rare. Ectopic varices (EVs), outside the esophagogastric region, pose significant diagnostic and therapeutic challenges and bleeding can bemassive and life-threatening. Endoscopic sclerotherapy (EST) has been used to eradicate EV. Endoscopic variceal ligation (EVL) is often not practised because of higher chances of recurrence and increased chances of rebleeding as reported in the literature. A 25-year-old lady presented with massive UGIB. She was known to be cirrhotic with portal hypertension (PHT) who underwent emergency devascularization with splenectomy, after failed endoscopic sclerotherapy 2 years earlier. She was on regular beta-blocker prophylaxis but had episodes of upper gastrointestinal bleeding after devascularization. The residual esophageal varices were obliterated with endoscopic sclerotherapy. She was conscious, oriented and hemodynamically stable. Esophagogastroduodenoscopy revealed varices in the first part of the duodenum. There were no residual esophageal or gastric varices or portal hypertensive gastropathy. A single band was applied over the duodenal varix (Fig. 1). She had no further episodes of UGIB and was discharged after a week of observation. She is on regular follow up for the past 18 months with no recurrence of varices. We report this case to highlight the successful use of EVL for duodenal EV. EVs account for 1 % to 5 % of all variceal bleeding [1–3] and need a high index of suspicion for diagnosis. They occur predominantly in the duodenum, jejunum, ileum, colon, rectum and enterostomy sites. Bleeding from these EVs is rare in patients with portal hypertension. The duodenum is a common site of EV hemorrhage in patients with PHT. Duodenal variceal bleeding is generally massive and life-threatening with mortality as high as 40 % from the initial bleeding [3–5]. The most common cause of duodenal variceal bleeding is extrahepatic PHT, less commonly intrahepatic PHT [6, 7]. Duodenal varices develop from collateral veins originating from the portal vein trunk or superior mesenteric vein, which empty into the inferior vena cava [1]. Diagnosis of ruptured duodenal varices and control of bleeding are difficult [1]. EST has been reported to be successful in the control of duodenal variceal bleeding [8, 9], but there are reports of rebleeding after EST [5]. Literature discussing the role of banding in patients with ectopic varices is limited. EVL for bleeding duodenal varices is challenging because of the difficulty in maintaining the field of vision. EVL has been tried for EV, but those patients received EST also. Hence, it was not clear as to which modality controlled the bleeding. EVL can be risky if the band applied does not completely encircle a characteristically large ectopic varix which can slough off causing a S. C. Sistla :D. Ram : S. M. Ali :N. Rajkumar Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry 605 006, India

Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism

To establish the importance of shunt surgery combined with partial resection of the spleen for selected pediatric patients with extra-hepatic portal vein obstruction (EHPVO), enormous splenomegaly and severe hypersplenism. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy or band ligation and shunt surgery; however, to our knowledge, this is the first such study to be published. Distal splenorenal shunt with partial resection of the spleen was performed in 16 of 60 children treated for EHPVO in the Gastroenterology Department of our hospital. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade in all patients and band ligation or endoscopic sclerotherapy had been done for children with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen (5-15 cm below the costal margin) and symptomatic hypersplenism with leucopenia, thrombocytopenia, and anemia. Partial resection of the spleen was performed, starting with ligation of the branches and tributaries of the caudal two-thirds. When an ischemic line demarcated the splenic parenchyma, it was transected using electrocautery or LigaSure, leaving 20-30 % of splenic tissue. After the spleen resection, a Warren shunt was performed. Platelet and white blood cell counts and liver function tests were performed before and after the operation. Growth was assessed using SD scores (z scores) for height, weight, and body mass index at the time of surgery and 1 year later. Postoperative recovery was uneventful and the leukocyte and platelet counts normalized. The shunt patency rate was 100 %. Two cases of shunt stenosis were treated successfully with percutaneous angioplasty. There was no postoperative mortality. During the follow-up period, from 1 to 7 years, all 16 children were asymptomatic, with improved quality of life, growth, and nutrition. No episodes of variceal bleeding, sepsis or encephalopathy occurred. Our results demonstrate that shunt surgery with partial resection of the spleen is effective and safe for pediatric patients with massive splenomegaly and severe hypersplenism secondary to EHPVO.

Ectopic varices.

Ectopic varices.

Recurrence of esophageal varices after endoscopic band ligation: single centre experience

Background and objective. Endoscopic band ligation is the main endoscopic treatment for esophageal varices, but the main problem after endoscopic treatment is variceal recurrence. The aim of this study was to evaluate and determine the esophageal varices recurrence rate and the time interval after endoscopic band ligation and to investigate possible risk factors affecting recurrence. Material and methods. The retrospective analysis of endoscopic band ligation procedures, performed in Vilnius University Hospital Santariskiu Clinics during the period 2006–2010, was made. 133 endoscopic band ligation procedures were included in the study. Results. After endoscopic band ligation, esophageal varices recurred in 45% of cases. The early recurrence of esophageal varices occurred in 46.7% of cases. Extrahepatic portal hypertension and a greater size of varices lead to the statistically significant early recurrence of esophageal varices after endoscopic band ligation. Conclusions. Endoscopic band ligation is associated with a high recurrence rate of esophageal varices and half of these cases were indentified as early variceal recurrence.