Superior vena cava syndrome in chronic intestinal failure patients: When the going gets tough.

BACKGROUND & AIMS: Catheter-related venous thrombosis is a severe complication of home parenteral nutrition (HPN) with potentially devastating consequences such as superior vena cava syndrome (SVCS). Early recognition and awareness of factors leading to its development are of paramount importance. However, studies are lacking in HPN patients focusing on this topic. In this study, we aimed to determine the incidence of SVCS in HPN patients and describe SVCS-related outcomes. METHODS: This retrospective cohort study comprised all adult HPN patients who developed SVCS between 2000 and 2022 at our national HPN referral center. Primary outcome was the incidence of SVCS. Secondary outcomes include SVCS-related symptoms, tip location of central venous access device (CVAD) post-insertion and at time of SVCS, diagnostics and treatment. RESULTS: SVCS was diagnosed in 38 of 616 patients (6%), with an annual cumulative incidence rate ranging between 0 and 4.2%. Most common presenting symptoms were facial edema (82%) and arm edema (50%). Post-insertion, 17% (6/36) of patients had a correct position of the CVAD tip and 11% (4/36) during SVCS diagnosis. Computed tomography was the most used diagnostic imaging technique (66%). Sixty-three percent of patients started, 11% switched, and 21% continued anticoagulant treatment. CONCLUSIONS: The incidence of SVCS is relatively high in our vulnerable HPN population. It is key to recognize whenever such patients present with vascular obstruction-related symptoms and treat them in an early stage by a multidisciplinary team.

Epidemiology, risk factors and impact of cachexia on patient outcome: Results from the Japanese Lung Cancer Registry Study.

BACKGROUND: Cancer cachexia is a syndrome that does not fully recover with nutritional support and causes appetite loss and body weight loss. It worsens a patient's quality of life and prognosis. In this study, the epidemiology of cachexia in lung cancer, its risk factors and its impact on chemotherapy response rate and prognosis were examined using the national database of the Japan Lung Cancer Society. Understanding these things related to cancer cachexia is important as a starting point in overcoming cancer cachexia in patients with lung cancer. METHODS: In 2012, 12 320 patients from 314 institutions in Japan were registered in a nationwide registry database (Japanese Lung Cancer Registry Study). Of these, data on body weight loss within 6 months were available for 8489 patients. We defined the patients with body weight loss ≥ 5% within 6 months, which is one of the three criteria listed in the 2011 international consensus definition of cancer cachexia, as cachectic in this study. RESULTS: Approximately 20.4% of the 8489 patients had cancer cachexia. Sex, age, smoking history, emphysema, performance status, superior vena cava syndrome, clinical stage, site of metastasis, histology, epidermal growth factor receptor (EGFR) mutation status, primary treatment method and serum albumin levels were significantly different between patients with and without cachexia. Logistic analyses showed that smoking history, emphysema, clinical stage, site of metastasis, histology, EGFR mutation, serum calcium and albumin levels were significantly associated with cancer cachexia. The response to initial therapy, including chemotherapy, chemoradiotherapy or radiotherapy, was significantly poorer in the patients with cachexia than in those without cachexia (response rate: 49.7% vs. 41.5%, P < 0.001). Overall survival was significantly shorter in the patients with cachexia than in those without cachexia in both univariate and multivariable analyses (1-year survival rate: 60.7% vs. 37.6%, Cox proportional hazards model, hazard ratio: 1.369, 95% confidence interval: 1.274-1.470, P < 0.001). CONCLUSIONS: Cancer cachexia was seen in approximately one fifth of the lung cancer patients and was related to some baseline patient characteristics. It was also associated with a poor response to initial treatment, resulting in poor prognosis. The results of our study may be useful for early identification and intervention in patients with cachexia, which may improve their response to treatment and their prognosis.

Predictive risk score of respiratory complications in children with mediastinal tumors: A case-control study.

BACKGROUND: The aim of this study was to examine risk factors of respiratory complications at the diagnosis and establish an algorithm of clinical management in children and adolescents with mediastinal tumors. METHODS: We retrospectively collected clinical information of all children and adolescents who presented with mediastinal tumors at Saitama Children's Medical Center from 1999 to 2019, including age, sex, pathological diagnosis, eight major clinical symptoms (cough, dyspnea, hypoxia, orthopnea, chest pain, wheeze, superior vena cava syndrome, and stridor), chest computed tomography (CT) findings (tumor location, mediastinal mass ratio, pleural fluid, pericardial effusion, and compression of trachea and bronchi), types of diagnostic procedure and anesthesia, respiratory complications (severe hypoxia, difficult ventilation, respiratory failure, and cardiopulmonary arrest), and clinical outcome. Subsequently, we calculated the risk score for predicting respiratory complications by combining clinical and radiological findings. RESULTS: Of the 57 patients, 7 (12%) developed respiratory complications. Cough, dyspnea, hypoxia, and orthopnea were significantly more common in patients with complications (p = 0.02, p = 0.02, p < 0.01, p = 0.03, respectively). The reduction of percentage of tracheal cross-sectional area (%TCA) and compression of the carina in chest CT were also significantly more common in patients with complications (p < 0.01 and <0.01, respectively). We calculated the risk score of respiratory complications by combining cough, wheeze, stridor, orthopnea, dyspnea, hypoxia, %TCA < 0.5, and compression of the carina. A risk score ≥ 7 showed high predictive accuracy for complications (sensitivity: 100%, specificity: 97.7%, positive likelihood ratio: 43.0). CONCLUSION: The risk score combining clinical symptoms with radiological findings is a promising predictive tool for respiratory complications in children with mediastinal tumors.

[Superior vena cava syndrome associated with bilateral angle closure glaucoma]. / A vena cava superior szindrómához társuló kétoldali zárt zugú glaucoma.

Superior vena cava syndrome is a set of symptoms resulting from partial or complete blockage of the superior vena cava. In the majority of cases, it develops secondary to lung tumors and lymphoma. It is characterized by edema of the head, neck, and upper limbs, large veins, and dyspnoea. Ophthalmological changes related to the syndrome rarely occur: eyelid swelling, conjunctival chemosis and suffusion have been described previously, and in one case, elevated episcleral pressure was also reported. Our 57-year-old female patient's superior vena cava syndrome was resolved with stent placement, and her small cell lung tumor was treated with chemotherapy. As part of the syndrome, he complained of bilateral blurred vision with a history of 4 months. During his examination, we found a bilateral closed angle, an intraocular pressure of 60 mmHg on both sides, and severe visual impairment. We performed a lens exchange combined with better semolysis, which achieved a significant reduction in intraocular pressure and managed to preserve the remaining visual acuity. After Nd:YAG laser iridotomy on the left eye, we started antiglaucoma drop treatment to reduce pain. After phacoemulsification, the morphology of the anterior chamber in the right eye improved significantly, which could be quantified by anterior segment optical coherence tomography. The ophthalmic parameters remained stable during the treatment of the underlying disease. The purpose of this publication is to draw attention to the ophthalmic symptoms of vena cava syndrome as well as to present the ophthalmic condition that sometimes accompanies the syndrome causing rapid, severe visual impairment, which, to our knowledge, has not been described before. Orv Hetil. 2022; 163(49): 1967-1971.

Azygos Vein Enlargement Secondary to Superior Vena Cava Syndrome on 18 F-FDG PET/CT.

ABSTRACT: Obstruction of the blood flow from the superior vena cava (SVC) to the right atrium causes the SVC syndrome. The azygos system is the most important way to overcome SVC obstruction. Azygos vein enlargement can be seen secondary to the SVC syndrome. Although a few cases showing 18 F-FDG uptake along the SVC in SVC syndrome were reported before, 18 F-FDG PET/CT findings of azygos vein enlargement have not been documented yet. Herein, we presented an intriguing case of azygos vein enlargement secondary to SVC syndrome on 18 F-FDG PET/CT.

Clinical Implications of a Persistent Left Superior Vena Cava in a Patient With Right Superior Vena Cava Thrombosis Undergoing Emergency Deceased Donor Liver Transplantation: A Case Report.

BACKGROUND: Persistent left superior vena cava (PLSVC) is the most common congenital thoracic venous anomaly. It is usually found incidentally on examination or during invasive procedures. In most cases, the blood flows back to the right atrium through the coronary sinus without hemodynamic abnormalities and it is usually asymptomatic. There is some controversy regarding the clinical use of PLSVC. In a few cases, a PLSVC has been used for hemodialysis or large-bore intravenous access. CASE REPORT: A 62-year-old woman with a previous hepatectomy for hepatocellular carcinoma and liver cirrhosis developed hepatic failure. Owing to her worsening condition, she needed liver transplantation (LT). However, a superior vena cava thrombus was found between the right atrium and proximal superior vena cava on preoperative transesophageal echocardiography. Usually, right-sided central venous catheterization is performed for LT preparation, but the embolic risk was very high in our patient. Fortunately, she had already been diagnosed with PLSVC. Therefore, we decided to perform fluoroscopy-guided catheterization through the PLSVC. For the safe use of a PLSVC catheter during surgery, the rapid infusion system pressure, coronary sinus inflow pressure, and intraoperative transesophageal echocardiography were monitored. The patient successfully underwent LT. CONCLUSIONS: Based on a literature review and this case, PLSVC can be used clinically when accompanied by a detailed history, preoperative imaging examination, and close intraoperative monitoring. We suggest that a PLSVC is a feasible alternative to central venous access for LT.

Intraoperative Collateral Drainage Evaluation Before Superior Caval Interruption in Partial Anomalous Pulmonary Venous Connection Repair.

A superior sinus venosus atrial septal defect and partial anomalous pulmonary venous connection was corrected by a minimally invasive approach by permanently ligating the superior vena cava and a single pericardial patch for rerouting the flow through the enlarged interatrial communication. The patient had persistency of the left superior vena cava draining in the coronary sinus but no innominate vein. This anatomy poses a risk of developing right-sided superior vena cava syndrome. In this article, we describe our intraoperative strategy to assess the safety of this approach in such cases, which facilitates minimally invasive repair and avoids the most important complications of conventional repair.

Forgotten Chemo-Port Leading to Chylothorax: A Rare Presentation.

Chylothorax is an uncommon cause of pleural effusion in routine clinical practice. Thoracic surgery, trauma and malignancy are the leading causes of chylothorax accounting for more than 90% of cases.1,2 We report this rare case of a middle aged lady with treated carcinoma breast who presented with left-sided chylothorax secondary to subclavian vein and superior vena cava thrombosis caused by a longstanding indwelling chemo-port in the right internal jugular vein. Patient was managed on total parenteral nutrition (TPN) leading to complete resolution of chylothorax.

Jejunocolic fistula presenting as chronic diarrhoea from advanced lymphoma.

Jejunocolic fistula, a late complication of intestinal lymphoma, is a rare entity with only five reported cases in the literature. We report a young male presenting with a left lateral neck mass 5 years ago which then progressed to superior vena cava syndrome. Despite receiving radiotherapy and two cycles of chemotherapy, there was still tumour progression. He presented with a 2-week history of diarrhoea, haematochezia and weight loss for which antibiotics provided no relief. Esophagogastroduodenoscopy and colonoscopy showed a jejunocolic fistula. After discussion with the multidisciplinary team, nutritional upbuilding was initiated followed by exploratory laparotomy with segmental resection and anastomosis. On histopathology, diffuse large B-cell lymphoma involving the jejunum and colon was noted. Despite receiving palliative chemotherapy, he still succumbed to disease progression. This case highlights the importance of early recognition of jejunocolic fistulas among patients with intestinal lymphomas to facilitate early multidisciplinary intervention.

Incidence of Deep Vein Thrombosis in Cerebral Palsy Following an Orthopaedic Surgical Event.

PURPOSE: The purpose of this study was to identify the incidence of venous thromboembolism (VTE) and characterize the demographics, comorbidities, and risk factors for patients with cerebral palsy (CP) having orthopaedic surgery. METHODS: All patients diagnosed with CP who underwent an orthopaedic surgical procedure at one institution between 2008 and 2017 were identified. Diagnosis codes and associated patient events were recovered from the electronic medical record. Each VTE event was reviewed to ascertain an actual VTE episode related to a surgical event. RESULTS: The review included 2583 orthopaedic surgical events in 1371 patients. Of the initial 88 cases identified, 28 cases had a deep thrombosis documented. Six cases of VTE occurred within 3 months following the surgical event. Three of these cases had thigh thrombosis, and 2 patients had upper arm thrombosis, and 1 patient had a superior vena cava thrombosis. On further workup, 5 of these 6 patients were identified as having a congenital hypercoagulable condition. CONCLUSIONS: VTE is a relatively rare occurrence after orthopaedic surgery in pediatric patients with CP, but when it occurs, a full hematologic workup for a congenital hypercoagulable condition is indicated. Based on the low incidence of thigh thrombosis, routine pharmacological or intermittent mechanical calf compression is not recommended. A careful clinical and family history should be performed to identify patients with possible genetic hypercoagulable conditions who would merit prophylaxis. LEVEL OF EVIDENCE: Level IV.

Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome.

Immunoglobulin G4 (IgG4)-related disease was first identified as a systemic condition in 2003 when extrapancreatic manifestations were identified in patients with autoimmune pancreatitis. Its peak incidence occurs in the fifth or sixth decades of life. Isolated extraaortic mediastinal involvement is extremely rare. This report describes a case of isolated extraaortic mediastinal IgG4-related disease encasing the superior vena cava (SVC) and manifesting as SVC syndrome in a 25-year-old man with no personal or family history of autoimmune disease. Resection with SVC reconstruction was performed.