Distal splenorenal shunt in an 8-year-old girl with rupture esophageal varices due to non-cirrhotic portal hypertension: A case report.

INTRODUCTION: Esophageal varices are dilated distal submucosal esophageal veins that connect the portal and systemic circulations. They are formed due to portal hypertension, which commonly resulted from cirrhosis. We presented an 8-year-old girl with esophageal varices without signs of cirrhosis. This case report aims to describe non-cirrhotic portal hypertension causing esophageal varices in a school-age girl. PRESENTATION OF CASE: An 8-year-old girl presented with one month of progressive hematemesis. The patient had been diagnosed with esophageal varices since she was 6 years old. Liver function test was within normal limits, complete blood count revealed bicytopenia. DISCUSSION: Due to well-preserved liver function, the patient underwent distal splenorenal shunt (DSRS). After the surgical procedure, the patient's clinical condition generally improved. CONCLUSION: NCPH (non-cirrhotic portal hypertension) is treated similarly with cirrhotic portal hypertension. DSRS could be the treatment of choice for refractory esophageal varices.

Pancreaticoduodenectomy and Vascular Reconstruction: Indications and Techniques.

Pancreaticoduodenectomy with vascular resection/reconstruction can be safely completed following 6 standard steps plus basic principles of vascular surgery. Particular attention is paid to the location of the tumor relative to the 2 first-order vein branches, portal vein -splenic vein -superior mesenteric vein confluence, inferior mesenteric vein, and the presence of arterial perineural invasion. Successful resection following neoadjuvant therapy can result in median survival 3 times that of historical controls.

An analysis on the use of Warren's distal splenorenal shunt surgery for the treatment of portal hypertension at the University Hospitals Leuven.

INTRODUCTION: Extrahepatic portal vein thrombosis (PVT) is the most common cause of portal hypertension (PH), particularly in children. PH-related manifestations include refractory variceal bleeding, splenomegaly and ascites. Albeit more rarely performed, the distal splenorenal shunt (Warren's shunt) has proven to be effective in selectively decompressing the collateral circulation. The aim of our study was to describe our experience with the distal splenorenal shunt and to determine the long-term effect on PH-related side-effects. METHODS: Distal splenorenal shunt operations performed at our institution between 2000 and 2014 were reviewed for: age, male/female ratio, children/adults ratio, body mass index, indications, grade of PVT (Yerdel classification), maximal shunt-flow velocity, shunt patency and thrombosis, re-intervention for variceal bleeding and survival. Complications of PH (esophageal variceal bleeding and ascites) were compared pre- versus post-operatively (last follow-up). Paired student t-test and fisher's exact were applied for pre- versus post-operative comparison. Results are reported as median [range]. RESULTS: Fourteen patients with PVT and refractory complications of PH underwent distal splenorenal shunt surgery. Age was 15 years [4.5-66]. Male/female ratio was 7/7. PVT -grade was 2 [1-4]. Follow-up was 3 [0.5-14]. All shunts were patent (100%) with no shunt thrombosis (0%) at last follow-up. There was no re-intervention for variceal bleeding (0%) and survival at last follow-up was 100%. Occurrence of esophageal variceal bleeding was higher pre-operatively (57%) than postoperatively (0%) (p = .0032) and also the incidence of ascites was higher pre-operatively (79%) than postoperatively (0%) (p < .0001). CONCLUSIONS: Based on our experience, the distal splenorenal shunt can be considered a valuable surgical technique for PVT-induced PH, with excellent post-operative prevention of complications of PH.

W. Dean Warren, MD, FACS, Father of Selective Shunts for Variceal Hemorrhage: Lessons Learned.

Dr Dean Warren was born in 1924 and died prematurely from cancer in 1989. He was a man of uncommon intelligence, wit, collegiality, integrity, honesty, and a true leader in American surgery. In 1966, he and his colleagues (Drs Zeppa and Fomon) presented a new concept for surgical shunts to control variceal hemorrhage while maintaining portal perfusion or hepatopetal blood flow. He termed this new shunt the distal splenorenal shunt (DSRS), which was the first selective shunt invented. The DSRS selective shunt was a brilliant improvement over the total shunt concept proposed by Nicolai Eck and was practiced worldwide during the 1980s. In a space of 2 decades, Dr Warren's pioneering work would show that the selective DSRS was superior to total shunts for treatment of portal hypertension, but that endoscopic sclerotherapy was a better first-line treatment for variceal hemorrhage than his own creation. His absolute adherence to the principles he espoused in his presidential address to the Society for Surgery of the Alimentary Tract in 1973 were employed in his research and treatment of patients. This paper details Dr Warren's extraordinary research accomplishments and sets a lesson for us that well-designed clinical trials including randomization are essential in the advancement of the care of surgical patients.

Cystic fibrosis and portal hypertension: Distal splenorenal shunt can prevent the need for future liver transplant.

BACKGROUND: The management of portal hypertension (PHT) in children with well compensated cirrhosis and cystic fibrosis (CF) is controversial. We present our experience with distal splenorenal shunting (DSRS) for the treatment of PHT as an alternative to liver transplantation (LT). METHODS: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9) was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). RESULTS: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ±â€¯6 vs 28 ±â€¯4, p < 0.001). All 5 DSRS patients had grade III-IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ±â€¯1.5 cm to 4.4 ±â€¯1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ±â€¯48 to 91.8 ±â€¯35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24-7.79). CONCLUSIONS: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. TYPE OF STUDY: Case series with no comparison group. LEVEL OF EVIDENCE: IV.

Non-cirrhotic portal hypertension - diagnosis and management.

NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.

Colectomy for Porto-Systemic Encephalopathy: Is it Still Topical?

Hepatic encephalopathy (HE) is a common long term complication of porto-systemic shunt. We report herein the case of a 59-year-old man with Child-Pugh A cirrhosis treated successfully 9 years earlier with distal splenorenal shunt for uncontrolled variceal bleeding. In the last year, he developed a severe and persistent hepatic encephalopathy secondary to the shunt, which was resistant to medical therapy. As liver transplantation was not available and obliteration of the shunt was hazardous, we performed subtotal colectomy in order to reduce ammonia production. This therapeutic option proved successful, as the grade of encephalopathy decreased and the patient improved. Our experience indicates that colonic exclusion should be considered as an option in the management of HE refractory to medical treatment in highly selected patients when liver transplantation is not available or even as a bridge given the long waiting time on lists.

Noncirrhotic portal hypertension.

Portal hypertension is characterized by an increase in portal pressure (> 10 mmHg) and could be a result of cirrhosis of the liver or of noncirrhotic diseases. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained by endothelial cell lesions, intimal thickening, thrombotic obliterations, or scarring of the intrahepatic portal or hepatic venous circulation. Many different conditions can determine NCPH through the association of these various lesions in various degrees. Many clinical manifestations of NCPH result from the secondary effects of portal hypertension. Patients with NCPH present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation, and jaundice due to portal hypertensive biliopathy. Other sequelae include hyperdynamic circulation, pulmonary complications, and other effects of portosystemic collateral circulation like portosystemic encephalopathy. At present, pharmacologic and endoscopic treatments are the treatments of choice for portal hypertension. The therapy of all disorders causing NCPH involves the reduction of portal pressure by pharmacotherapy or portosystemic shunting, apart from prevention and treatment of complications of portal hypertension.

Selective decongestive devascularization shunt of gastrosplenic region for treatment of portal hypertension / 中华肝胆外科杂志

Objective To evaluate the curative effect of selective decongestive devascularization shunt of gastrosplenic region(SDDS-GSR) for the treatment of portal hypertension. Methods From September 2000 to June 2008, 44 patients with portal hypertension had received SDDS-GSR in our hospital. Twenty-nine of them had been followed up for 12-85 months (mean=44months). Results Operative mortality was 0 %. Mesenteric area pressure(33.82±5.12 cm H_2O) was higher than splenic area pressure(24.57±4.63 cm H_2O)soon after the operation finished(P<0.01). No re-bleeding ca-ses were found, and the encephalopathy occurred in 2.27% of the patients in the early stage of post-operation. However, the rates of 3.45% for re-bleeding and 3.45% for encephalopathy were noticed in long-term follow-up. The 1-, 3- and 5-year survival were 100%, 95% and 95%, respectively. Dur-ing the long-term follow-up, the platelet counts markedly increased from (49.2±21.8 × 10~9/L) of preoperative value to (77.2±29.5×10~9/L) (P<0.01), while spleen size was significantly reduced.Conclusion SDDS-GSR is a reliable and reasonable surgical procedure for the management of portal hypertension.

Analysis of a Distal Splenorenal Shunt for Treatment of Variceal Bleeding

BACKGROUND: Portal hypertension is a pathologic phenomenon caused by increased pressure in the portal vein. It's clinical importance lies in the development of secondary complications, such as variceal bleeding, hypersplenism, ascites, and hepatic encephalopathy. Especially, bleeding from esophagogastric varices is the single most life-threatening complication of portal hypertension. Nevertherless, these days, non-invasive techniques, such as endoscopic sclerotherapy, endoscopic band ligation, and a transjugular intrahepatic portosystemic shunt (TIPS) are available for treatment of variceal bleeding. However, a surgical procedure like a distal splenorenal shunt (DSRS) is still indicated in selective patients when a non-invasive technique has failed. Thus we reviewed and analyzed our experi ence with a distal splenorenal shunt for treatment of variceal bleeding in portal hypertension. METHODS: We retrospectively analyzed the medical records of 22 cases who had undergone a distal splenorenal shunt from 1980 to 1988 for treatment of portal hypertension with it's secondary com plications according to the patients age, sex, cause of disease, presence of secondary complications of portal hypertension, preoperative treatment, improvement of symptoms, mortality, survival rate, etc. All cases were treated preoperatively with non-invasive technique such as endoscopic sclerotherapy, endoscopic band ligation, a TIPS. RESULTS: The patients' average age was 45.5 years old. The most common cause of cirrhosis was of a viral origin. The duration for the symptoms of portal hypertension was 3.6 years. All cases had a history of recurrent variceal bleeding, and one case had hepatic encephalopathy. Preoperatively endoscopic sclerotherapy was done in 19 cases, and endoscopic band ligation was done in 3 cases. Of these 22 cases, only one case required a TIPS for decompression of the portal vein. According to the Child-Pugh classification, 12 cases were in class A and 10 cases were in class B. The estimated blood loss during the operation was about 800 to 1,900 cc. After operation, no recurrent variceal bleeding was found. The one case with hepatic encephalopathy was also controlled. A liver transplantation was Performed in onepatient, 4 years after DSRS. The operative mortality was 0%, and the survival rate for 1-year was 95%; that for 5-years was 50%. CONCLUSIONS: A distal splenorenal shunt is still a good modality for treating of portal hypertension with it's secondary complications, especially with variceal bleeding, and it could also serve as an excellent long-term bridge to liver tranplantation.