Adolescent ; Cyanosis ; complications ; Heart Septal Defects, Atrial ; complications ; Humans ; Male ; Pulmonary Wedge Pressure

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.
Male ; Humans ; Child ; Adolescent ; Budd-Chiari Syndrome/therapy* ; Hepatopulmonary Syndrome/therapy* ; Retrospective Studies ; Hypoxia/complications* ; Oxygen ; Dyspnea/complications* ; Cyanosis/complications* ; Bilirubin

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.
Adolescent ; Anoxia ; Arteriovenous Fistula/etiology ; Biliary Atresia/*diagnosis/etiology ; Cyanosis/complications ; Dyspnea/complications ; Echocardiography, Transesophageal ; End Stage Liver Disease/complications/*surgery ; Female ; Hepatic Artery/abnormalities ; Hepatopulmonary Syndrome/*diagnosis/ultrasonography ; Humans ; *Liver Transplantation ; Osteoarthropathy, Secondary Hypertrophic/complications

Creation of an axillary arteriovenous fistula (AVF) was performed in two patients with high risks of Fontan operation after a cavopulmonary shunt. The patients complained of progressive cyanosis and decrease in exercise tolerance, and they showed pulmonary arteriovenous fistula on the pulmonary angiography. They were uneventfully transferred to the general ward on the first postoperative day and discharged 3 and 4 days after the operation respectively. There was no evidence of fistula-related volume loading and the other postoperative complications. An 8-month follow-up revealed improved symptoms such as, cyanosis and exercise intolerance, but not sufficient regression of pulmonary arteriovenous fistula on a lung perfusion scan and contrast echocardiography, which should be carefully checked hereafter.
Angiography ; Arteriovenous Fistula* ; Axilla ; Cyanosis ; Echocardiography ; Exercise Tolerance ; Follow-Up Studies ; Fontan Procedure* ; Heart Bypass, Right ; Humans ; Lung ; Patients' Rooms ; Perfusion ; Postoperative Complications

This is a report of a successful, rapid two-stage arterial switch operation in an infant with dextrotransposition of great arteries with an intact ventricular septum beyond the appropriate time for arterial switch operation. A 4-month-old female infant was admitted due to severe cyanosis, respiratory insufficiency, and frequent diarrhea secondary to ischemic colitis. Echocardiographic examination presented marked leftward deviation of the interventricular septum, and left and right ventricular pressures as measured by cardiac catheterization were 40/4 mm Hg and 85/2 mm Hg, respectively. Fifteen days after a preparatory operation (pulmonary artery band with modified right Blalock-Taussig shunt), left ventricular-right ventricular systolic pressure ratio increased to 105/90. Arterial switch operation associated with the division of the right Blalock-Taussig shunt and the removal of pulmonary artery band was performed. After the operation, the infant showed normal cardiac function with no postoperative complication.
Arteries* ; Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Colitis, Ischemic ; Cyanosis ; Diarrhea ; Echocardiography ; Female ; Humans ; Infant ; Postoperative Complications ; Pulmonary Artery ; Respiratory Insufficiency ; Ventricular Pressure ; Ventricular Septum*

PURPOSE: Blue toe syndrome consists of blue or purplish toes in the absence of a history of obvious trauma, serious cold exposure, or disorders producing generalized cyanosis. It is a life-threatening and still underrecognized disease. It can be commonly occurred by vascular surgery, invasive cutaneous procedures or anticoagulant therapy. Our case is presented of blue toe syndrome related to atheromatous embolization that was presumably triggered by angio CT. METHODS: A 69-year-old man presented with the suddenly developed pain, cyanosis and livedo reticularis of the toes in right foot. Dorsalis pedis pulses were palpable. He had been performed a diagnostic angio CT 1 month earlier. Angio CT revealed diffuse aortic atheromatous plaque in lower abdominal aorta and both common iliac artery. One month after angio CT, he visited our clinic. There was no visible distal first dorsal metatarsal artery and digital artery of right first toe in lower extremity arteriography. A diagnosis was established of blue toe syndrome. Because his symptom was aggravated, we performed the exploration of the right foot. After exposure of first dorsal metatarsal artery, microsurgical atheroembolectomy was done. RESULTS: There were no postoperative complications. After three months the patient had no clinically demonstrable problems. CONCLUSION: Patient with blue toe syndrome is at high risk of limb loss and mortality despite treatment. Blue toe syndrome produces painful, cyanosed toes with preserved pedal pulses. It needs to be aware of blue toe syndrome. Careful history should reveal the diagnosis. Treatment is controversial, however, most believe that anticoagulation therapy should be avoided.
Aged ; Angiography ; Aorta, Abdominal ; Arteries ; Blue Toe Syndrome ; Cold Temperature ; Cyanosis ; Extremities ; Foot ; Humans ; Iliac Artery ; Livedo Reticularis ; Lower Extremity ; Metatarsal Bones ; Postoperative Complications ; Toes

No abstract available.
Asian Continental Ancestry Group/*genetics ; Chromosome Deletion ; Chromosome Disorders/complications/*diagnosis ; Chromosomes, Human, Pair 18 ; Cyanosis/*etiology ; Female ; Heart Septal Defects, Atrial/complications/*diagnosis ; Humans ; Infant ; Karyotyping ; Republic of Korea

We report here a case with hypereosinophilia and peripheral artery occlusion. A 32-yr-old Korean woman presented to us with lower extremity swelling and pain. Angiography revealed that multiple lower extremity arteries were occlusive. The biopsy specimen showed perivascular and periadnexal dense eosinophilic infiltration in dermis and subcutaneous adipose tissue. Laboratory investigations revealed a persistent hypereosinophilia. She was prescribed prednisolone 60 mg daily. Her skin lesion and pain were improved and the eosinophil count was dramatically decreased. After discharge, eosinophil count gradually increased again. Cyanosis and pain of her fingers recurred. She had been treated with cyclophosphamide pulse therapy. Her eosinophilia was decreased, but the cyanosis and tingling sense were progressive. The extremity arterial stenoses were slightly progressed. Skin biopsy showed perivascular eosinophilic infiltration in the dermis and CD40 ligand (CD40L) positive eosinophilic infiltration. The serum TNF-alpha was markedly increased. These results suggest that CD40L (a member of TNF-alpha superfamily) could play a role in the inflammatory processes when eosinophil infiltration and activation are observed. We prescribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and nifedipine, and she was discharged.
Adult ; Arterial Occlusive Diseases/*diagnosis/etiology ; CD40 Ligand/analysis ; Cyanosis/etiology ; Diagnosis, Differential ; Eosinophilia/*diagnosis/etiology ; Female ; Gangrene/etiology ; Humans ; Hypereosinophilic Syndrome/blood/complications/*diagnosis ; Immunohistochemistry ; Peripheral Vascular Diseases/*diagnosis/etiology ; Skin/chemistry/pathology ; Tumor Necrosis Factor-alpha/metabolism ; Vasculitis/*diagnosis/etiology