Spontaneous Portosystemic Shunt with Budd-Chiari Syndrome.

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Refractory and Severe Hepatogenous Diabetes in a Patient with Cirrhosis Improved by Balloon-Occluded Retrograde Transvenous Obliteration of a Large Portosystemic Shunt.

A 54-year-old male with liver cirrhosis (Child-Pugh score 5) presented with severe hepatogenous diabetes (HbA1c 12.6%). Contrast-enhanced CT showed a large portosystemic shunt from the inferior mesenteric vein to the left internal iliac vein. Glucose monitoring showed postprandial hyperglycemia and reactive hypoglycemia. After balloon-occluded retrograde transvenous obliteration (BRTO) and partial splenic transarterial embolization, postprandial hyperglycemia was diminished. Seven months later, HbA1c had improved from 12.6% to 6.7%. In this case, postprandial hyperglycemia occurred by direct delivery of glucose into the systemic circulation via the shunt, and fasting hypoglycemia occurred during treatment with oral antidiabetic agents and insufficient gluconeogenesis. BRTO of the portosystemic shunt resulted in improvement in hepatogenous diabetes.

Embolization of congenital portosystemic shunt presenting after pediatric liver transplantation: Case report and literature review.

This case report describes a 13-year 10-month-old girl who underwent a deceased-donor split LT for primary diagnosis of biliary atresia at the age of 12 months, who presented with a lower GI bleed. Ultrasound and CT revealed a venous vascular anomaly involving the cecum and ascending colon, with communication of the SMV and pelvic veins consistent with a CEPS. Associated varices were noted in the pelvis along the uterus and urinary bladder. These findings were confirmed by trans-hepatic porto-venography, which was diagnostic and therapeutic as a successful embolization of the CEPS was performed using micro-coils. There were no complications following the procedure and no further GI bleeding occurred, illustrating the efficacy of this treatment option for CEPS. We discuss the literature regarding the presenting complaint of GI bleeding post-LT, CEPS as a rare cause of GI bleeding and its association with PV, and the classification and treatment of CEPS.

Surgical Ligation for the Treatment of an Unusual Presentation of Type II Abernethy Malformation.

Abernethy malformation is a very rare congenital vascular malformation consisting of diversion of portal blood away from liver, and it is commonly associated with multiple congenital anomalies. Here, we present a case of a male from China with nonspecific abdominal pain associated with an unusual pattern of type II Abernethy malformation, whose was diagnosed with a portosystemic shunt via a giant portal-inferior vena cava fistula (17.22 mm in diameter). The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of a type II Abernethy malformation presenting as a portosystemic shunt via the giant portal-inferior vena cava fistula.

Abernethy malformation: Single-center experience from India with review of literature.

Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. This is a retrospective analysis of data from nine patients with Abernethy malformation at a single center. This is a referral center for Pediatric Cardiology and for Hepatobiliary and Pancreatic Surgery. The patients presented to the Pulmonary Hypertension Clinic/the Hepatobiliary Surgery Clinic. Out of nine patients, four were male. Type II Abernethy malformation was present in five patients whereas three patients had type I malformation. One of the patients had communication between inferior mesenteric vein and internal iliac vein. Five out of nine patients were erroneously diagnosed as idiopathic primary pulmonary hypertension and were treated with vasodilators. One patient required living donor liver transplant. One patient was managed with surgical shunt closure whereas two patients required transcatheter shunt closure. The rest of the patients were managed conservatively. Abernethy malformation is more common than previously thought and the diagnosis is often missed. There are various management options for Abernethy malformation, which includes surgical or transcatheter shunt closure and liver transplant. Management of Abernethy malformation depends upon type, presentation, and size of shunt.

Antiphospholipid syndrome presenting as acute mesenteric venous thrombosis involving a variant inferior mesenteric vein and successful treatment with rivaroxaban.

Acute mesenteric venous thrombosis (MVT) is the rarest cause of acute mesenteric ischaemia, so thrombosis of a variant inferior mesenteric vein (IMV) is especially uncommon in the setting of antiphospholipid syndrome (APS). Here, we present such a case of seronegative APS initially manifesting as an anomalous IMV thrombosis in a 76-year-old woman. Although guidelines support anticoagulation with vitamin K antagonists in these patients, we anticoagulated with rivaroxaban (a direct oral anticoagulant (DOAC)) due to patient preference, which resulted in complete clinical and endoscopic resolution. IMV thrombosis is a rare form of MVT, only two case reports describe successful anticoagulation with DOACs in the setting of MVT and none report APS as an underlying aetiology. Therefore, this case provides the opportunity to review the pathophysiology of MVT, APS and their medical management including current trends in anticoagulation.

Is color Doppler a reliable method for the diagnosis of malrotation?

PURPOSE: The gold standard for the diagnosis of malrotation is barium contrast study of the upper gastrointestinal system (UGCS), while color Doppler ultrasonography (CDUS) is another method used in the diagnosis. We investigated the value of CDUS for the diagnosis of malrotation in this study. METHODS: UGCS images, CDUS images, plain abdominal images, demographic data, and symptoms of 82 patients who were investigated for presumed malrotation during a 7-year period were evaluated, retrospectively. RESULTS: All patients underwent CDUS, and 18% of these patients were diagnosed with malrotation as the superior mesenteric vein was seen to be on the left of the superior mesenteric artery. We found that 16% of the 75 patients who underwent UGCS were diagnosed with malrotation. The sensitivity and specificity of CDUS in the diagnosis of malrotation was found to be 93.8 and 100%, respectively. The respective values for UGCS were 91.7 and 98.4%. CONCLUSION: Current data in the literature and our results underline that UGCS may yield false-positive and false-negative results. Although CDUS was found to be a reliable method for the diagnosis of malrotation in our study, the limitations of UGCS are also recognized for CDUS. Prospective studies are needed to determine the more valuable method.

Minimally invasive treatment of mesenteric arterioportal fistulas in two dogs.

CASE DESCRIPTION Two Pembroke Welsh Corgis with gastrointestinal signs including inappetence, diarrhea, lethargy, and hypersalivation were referred for evaluation. CLINICAL FINDINGS Diagnostic testing included abdominal ultrasonography and CT angiography. One patient had a cranial mesenteric artery-to-mesenteric vein fistula with multiple acquired extrahepatic portosystemic shunts. The second patient had both cranial and caudal mesenteric artery-to-mesenteric vein fistulas and multiple acquired extrahepatic portosystemic shunts. TREATMENT AND OUTCOME Both patients underwent minimally invasive coil embolization of the mesenteric arterioportal fistulas, with complete occlusion confirmed by means of angiography at procedure completion. Clinical outcome approximately 1 year after treatment was assessed as fair to good because of recurrence of clinical signs that required medical management in 1 dog and some persistent serum biochemical abnormalities. CLINICAL RELEVANCE Outcome for the 2 patients described suggested that coil embolization may be a feasible and effective minimally invasive technique for the treatment of mesenteric arterioportal fistulas in dogs. However, further investigation of the potential for chronic hepatic disease in patients with a history of acquired portosystemic shunts is warranted.

Percutaneous treatment of a superior mesenteric artery pseudoaneurysm and arteriovenous fistula with coil embolization: a case report.

Visceral artery pseudoaneurysm and arteriovenous fistula following penetrating abdominal injuries is a rarely observed complication. Presently described is the case of a 44-year-old male admitted to the hospital after having previously experienced penetrating abdominal trauma. The patient had developed a pseudoaneurysm in the superior mesenteric artery and an arteriovenous fistula between the superior mesenteric artery and vein following surgery. The patient underwent successful coil embolization procedure and he was discharged 1 day after intervention.

Recurrent Hyperammonemia After Abernethy Malformation Type 2 Closure: a Case Report.

The Abernethy malformation is a rare congenital malformation defined by the presence of an extrahepatic portosystemic shunt. Although most patients are asymptomatic, clinical encephalopathy is present in 15% of cases. We present a patient with type 2 Abernethy malformation, hyperammonemia, and encephalopathy. Shunt closure was performed successfully using interventional angiography; however, hyperammonemia recurred 3 months later. The diagnosis of Abernethy malformation can be made easily, but the ideal patient management strategy has not yet been established. This is the first reported patient with recurrence of hyperammonemia after interventional treatment; we discuss the therapeutic options for Abernethy malformation.

A strange case of superior mesenteric venous collar around the superior mesenteric artery.

During our dissection classes, we saw a venous collar formed around the superior mesenteric artery (SMA) by the jejunal tributaries of superior mesenteric vein in an adult male cadaver. The jejunal tributaries united themselves to form two common jejunal veins. Upper common jejunal vein crossed superficial to SMA and opened into the SMV in front of the uncinate process of pancreas. The inferior common jejunal vein crossed behind the SMA from left to right and opened into the SMV, 4 cm below this level. A communicating vein connected the two common jejunal veins with each other.

Color Doppler-An effective tool for diagnosing midgut volvulus with malrotation.

BACKGROUND: Malrotation with midgut volvulus is a common surgical emergency in children. A diagnostic tool for malrotation with characteristics ideal in emergency settings such as non-invasiveness and rapidity remains a keenly debated issue among surgeons and radiologists alike. METHODS: Fifty-two pediatric patients of suspected malrotation with midgut volvulus were studied between 1998 and 2016. All patients underwent plain abdominal X-ray and Color Doppler; contrast upper gastrointestinal (GI) studywas done in some patients. All cases were operated and diagnosis was confirmed. A subset of 60 pediatric patients with nonspecific GI complaints were also examined to see relative position of superior mesenteric vein (SMV)/superior mesenteric artery (SMA) in control population. RESULT: A total of 52 suspected cases of malrotation were admitted from May 1998 to November 2015, 43 had inversion of SMA/SMV and nine had SMV anterior of SMA in Color Doppler. All 43 cases of inversion of SMA/SMV were cases of malrotation after surgical confirmation; while five out of nine cases of SMV anterior to SMA had malrotation. CONCLUSION: In appropriate clinical settings, Color Doppler documenting the reversal or aberrant SMV/SMA axis is not only predictive but also diagnostic of malrotation of gut.

Hepatoblastoma and Abernethy Malformation Type I: Case Report.

A 2-year-old boy presented with pneumonia and an abdominal mass was noted incidentally. A right lobe hepatic mass classified as PRETEXT III and congenital absence of the portal vein with drainage of the superior mesenteric vein to the inferior vena cava (Abernethy malformation type I) were confirmed by computed tomography and angiography. After a clinical diagnosis of hepatoblastoma had been made, he was treated with 4 cycles of doxorubicin and cisplatin and hepatic arterial chemoembolization with doxorubicin, after which the tumor was classified as POSTEXT III. He underwent a right extended hepatic lobectomy with resection of the caudate lobe but died on postoperative day 4 due to hepatic failure. The Abernethy malformation type I is associated with the development of hepatic tumors, and the abnormal blood flow might predispose to hepatic failure after liver resection. Extensive study of the hepatic vasculature is warranted in patients with suspected malformations. Liver transplant could be considered in patients with congenital portosystemic shunt and malignant liver tumors.

Disabling portosystemic encephalopathy in a non-cirrhotic patient: Successful endovascular treatment of a giant inferior mesenteric-caval shunt via the left internal iliac vein.

Hepatic encephalopathy is suspected in non-cirrhotic cases of encephalopathy because the symptoms are accompanied by hyperammonaemia. Some cases have been misdiagnosed as psychiatric diseases and consequently patients hospitalized in psychiatric institutions or geriatric facilities. Therefore, the importance of accurate diagnosis of this disease should be strongly emphasized. A 68-year-old female patient presented to the Emergency Room with confusion, lethargy, nausea and vomiting. Examination disclosed normal vital signs. Neurological examination revealed a minimally responsive woman without apparent focal deficits and normal reflexes. She had no history of hematologic disorders or alcohol abuse. Her brain TC did not demonstrate any intracranial abnormalities and electroencephalography did not reveal any subclinical epileptiform discharges. Her ammonia level was > 400 mg/dL (reference range < 75 mg/dL) while hepatitis viral markers were negative. The patient was started on lactulose, rifaximin and low-protein diet. On the basis of the doppler ultrasound and abdomen computed tomography angiography findings, the decision was made to attempt portal venography which confirmed the presence of a giant portal-systemic venous shunt. Therefore, mechanic obliteration of shunt by interventional radiology was performed. As a consequence, mesenteric venous blood returned to hepatopetally flow into the liver, metabolic detoxification of ammonia increased and hepatic encephalopathy subsided. It is crucial that physicians immediately recognize the presence of non-cirrhotic encephalopathy, in view of the potential therapeutic resolution after accurate diagnosis and appropriate treatments.

Intestinal malrotation in patients with situs anomaly: Implication of the relative positions of the superior mesenteric artery and vein.

PURPOSE: To assess the usefulness of the relative position of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) in diagnosing intestinal malrotation in situs anomaly. MATERIALS AND METHODS: From January 2004 to April 2015, 33 patients with situs anomalies were enrolled in this study who underwent abdominal USG, CT or MRI as well as upper gastrointestinal series (UGIS) or surgery: situs inversus (n=16), left isomerism (n=10), and right isomerism (n=7); age 21.2±23.2years (mean±standard deviation), range 0-72 years. The intestinal malrotation was confirmed with UGIS and/or operation in 16 patients. Relative positions of the SMV to the SMA were classified into four groups by reviewing abdominal USG, CT, or MRI: right sided, left sided, ventral sided, and dorsal sided. The incidence of malrotation was analyzed for each group. RESULTS: In 16 patients with situs inversus, there was reversed SMA-SMV relationship: left sided (n=11) or ventral sided (n=5). One situs inversus patient with ventral sided SMV had intestinal malrotation (6.25%). 17 patients with situs ambiguus showed various SMA-SMV relationships (ventral sided, n=7; left sided, n=5; right sided, n=4; dorsal sided, n=1). Among them, 15 patients (88.2%) had intestinal malrotation. Two patients with normal rotation had either right sided or dorsal sided SMV. CONCLUSION: Situs ambiguus was commonly associated with intestinal malrotation with a variable SMA-SMV relationship. Reversal of the mesenteric vascular relationship was observed in situs inversus with normal rotation, not excluding the possibility of intestinal malrotation.