• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.98-101
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• 2004

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures

• Journal of Yeungnam Medical Science
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• v.28 no.1
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• pp.99-104
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• 2011

Essential thrombocythemia (ET), a subcategory of chronic myeloproliferative disorder, is characterized by absolute thrombocytosis due to excessive clonal proliferation of platelets, hyperaggregability of platelets, and increased incidence of thrombosis and hemorrhage. We consider a diagnosis of ET when an unexplained and persistent thrombocytosis is observed. It is difficult to consider ET first when we meet a patient with esophageal varix bleeding or unusual multiple thromboses like mesenteric vein, splenic vein, and portal vein. This article reports a patient who presented initially with esophageal varix bleeding and unusual multiple thromboses, thereafter, she was diagnosed with ET after testing positive for the Janus Tyrosine Kinase 2 (JAK2) V617F mutation. In conclusion, in patients with varix bleeding and unusual multiple thromboses, myeloproliferative disorders like essential thrombocythemia should be considered as a potential cause and testing for the JAK2 mutation is warranted.

• Clinical and Experimental Pediatrics
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• v.56 no.11
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• pp.500-504
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• 2013

Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

• Journal of Chest Surgery
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• v.36 no.1
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• pp.51-54
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• 2003

This patient was an 53-year-old man who had undergone Sengstaken-Blackmore tube insertion for esophageal varix bleeding. Two days after Sengstaken-Blackmore tube insertion, he developed severe left hemothorax and was transferred to our hospital. The esophagoscopic findings revealed a large perforation lengthening 8-cm in the intrathoracic esophagus. A left thoracotomy was performed 33 days after the injury due to repeated varix bleedings and poor conditions. An 8-cm longitudinal perforation of the intrathoracic esophagus with gross suppurative empyema was found. Primary repair and esophageal exclusion was performed 2cm proximal and distal to the perforation, using rows of nonabsorbable staplers(TA stapler 60 $\times$ 4.8) and large bore thoracostomy tubes were placed for local drainage. Six days after intrathoracic esophageal exclusion, an esophagogram revealed a leakage at just above the proximal stapling site. A cervical esophageal exclusion was performed using the same method. One hundred thirty seven days after exclusion operation for the intra-thoracic esophageal perforation, the patient was able to eat per orally without any secondary esophageal reconstructive surgery.

• Journal of Chest Surgery
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• v.12 no.4
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• pp.371-378
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• 1979

The authors have performed operations on vascular system of 108 cases from 1972 through 1979, and analyzed the diseases, surgical procedures and results. They were 1. Arterial system; 45 cases P.D.A. : 20 Occlusive diseases : 13 Coarctation of aorta : 4 Aneurysm : 4 A-V fistula : 2 Trauma : 2 2. Venous system; 6 cases Esophageal varix : 4 S.V.C. syndrome : 1 Varicose vein : 1 3. Arteriovenous shunt for hemodialysis; 57 cases Of the arterial diseases, the worst results came from Burger`s disease. For the bleeding esophageal varices, we have performed ligations of varices or collateral circulations rather than emergency splenorenal shunt with good results. External A-V shunt for hemodialysis had much more complications than the A-V fistula.

• Tuberculosis and Respiratory Diseases
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• v.44 no.3
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• pp.639-648
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• 1997

Background : Arterial hypoxemia has been noted in patients with liver cirrhosis because of bronchial vessel dilatation. Cabenes et al. reported that bronchial hyperresponsiveness to the metacholine inhalation was observed in patients of left side heart failure, he suggested that one of the mechanism was bronchial vessel dilatation. We hypothesized that patients of liver cirrhosis might have bronchial hyperresponsiveness to metacholine inhalation due to portal hypertension. We evaluate the relationship between bronchial responsiveness and severity of liver cirrhosis, severity of portal hypertension. Methods : In the 22 patients of the liver cirrhosis with clinical portal hypertension, metacholine provocation test was done and determined $PC_{20}FEV1$. We classified liver cirrhosis according to Pugh-Child classification. Esophagogastroscopies were performed for the evaluation of the relationship between bronchial hyperresponsiveness and severity of esophageal varix. Results : In the 22 cases of the liver cirrhosis with clinical portal hypertension. The causes of liver cirrhosis, alcoholic hepatitis was 9 cases, hepatitis B virus was 12 cases, hepatitis C virus was 1 case, and 151 cases (68.18%) of total 22 cases were positive in metacholine provocation test. In positive cases. There was no significant relationship between $PC_{20}FEV1$ and severity of liver cirrhosis which were classified by Pugh-Child classification or severity of esophageal varix(p<0.05). Conclusion : we observed that bronchial responsiveness to metacholine increased in the patients of liver cirrhosis and there was no significant relationship between the severity of liver cirrhosis and the severity of esophageal varix.

• Clinical Endoscopy
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• v.55 no.3
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• pp.381-389
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• 2022

Background/Aims: The treatment of superficial esophageal neoplasms (SENs) in cirrhotic patients is challenging and rarely investigated. We evaluated the outcomes of endoscopic submucosal dissection (ESD) to determine the efficacy and safety of treating SENs in patients with liver cirrhosis. Methods: The baseline characteristics and treatment outcomes of patients who underwent ESD for SENs between November 2005 and December 2017 were retrospectively reviewed. Results: ESD was performed in 437 patients with 481 SENs, including 15 cirrhotic patients with 17 SENs. En bloc resection (88.2% vs. 97.0%) and curative resection (64.7% vs. 78.9%) rates were not different between the cirrhosis and non-cirrhosis groups (p=0.105 and p=0.224, respectively). Bleeding was more common in cirrhotic patients (p=0.054), and all cases were successfully controlled endoscopically. The median procedure and hospitalization duration did not differ between the groups. Overall survival was lower in cirrhotic patients (p=0.003), while disease-specific survival did not differ between the groups (p=0.85). Conclusions: ESD could be a safe and effective treatment option for SENs in patients with cirrhosis. Detailed preprocedural assessments are needed, including determination of liver function, esophageal varix status, and remaining life expectancy, to identify patients who will obtain the greatest benefit.

• Journal of Chest Surgery
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• v.17 no.2
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• pp.269-274
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• 1984

Since tuberculosis was a common pulmonary disease in Korea, Aspergillosis was easily misdiagnosed as tuberculosis and an acute form of Aspergillosis was misinterpreted as pneumonia because of their similarities in the X-ray findings. This investigation is designed to illustrate the clinical features and preoperative diagnosis and surgical role in the management of this disease. In a retrospective review of operative cases from Jan. 1963 through Dec. 1983, 36 cases were analyzed. Peak age incidence lies in the 3rd decade [41.7%]. All cases had a history of treatment with antituberculous drugs under diagnosis of pulmonary tuberculosis and the most common chief complaint was hemoptysis [69.5%]. Only nine cases [25%] showed cavitary lesions with mycetoma and preoperative sputum study for fungus showed low positive valve [42.3%]. Anatomical location of lesion was located mainly upper lobe [66.7%] and most of cases were managed by lobectomy. We experienced 7 cases of complication; they were postoperative empyema, hepatic failure, esophageal varix bleeding. Postoperative pathologic findings showed that 29 cases [80.5%] were combined with tuberculosis 3 cases were combined with bronchiectasis and 4 cases were not combined with other disease. In conclusion, when the patient has a longstanding history of pulmonary tuberculosis and has a hemoptysis, he must be suspected fungus super infection. Resectional surgery is the treatment of choice for symptomatic localized disease and needed resection in asymptomatic patient to prevent possible fatal sequelae in the future.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.221-225
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• 2007

A previously healthy 3-year-old girl was admitted to the Department of Pediatrics in Severance Hospital with sudden symptoms of melena. The vital signs were stable, and splenomegaly was found in a physical examination. The patient had moderate thrombocytopenia. There was no evidence of autoimmune disease. A upper gastrointestinal endoscopy and esophagogram showed a varix on the lower esophagus. Coarse liver parenchymal echoes and increased periportal echogenicity were seen on a Doppler sonogram. The velocity of the portal vein mildly increased. Magnetic-resonance-cholangiopancreatogram (MRCP) demonstrated normal portal structures. A sono-guided liver biopsy was performed, but the pathological findings were unremarkable. Based on these findings, we diagnosed the patient with idiopathic portal hypertension. The patient was discharged and was treated with oral beta blocker. We report a case of idiopathic portal hypertension with a brief review of the literature.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.