False-negative upper extremity ultrasound in the initial evaluation of patients with suspected subclavian vein thrombosis due to thoracic outlet syndrome (Paget-Schroetter syndrome).

OBJECTIVE: To assess the utilization and consequences of upper extremity Duplex ultrasound in the initial diagnostic evaluation of patients with suspected subclavian vein (SCV) thrombosis and venous thoracic outlet syndrome (VTOS). METHODS: A retrospective single-center review was conducted for patients that underwent primary surgical treatment for VTOS between 2008 and 2017, in whom an upper extremity ultrasound had been performed as the initial diagnostic test (n = 214). Clinical and treatment characteristics were compared between patients with positive and false-negative ultrasound studies. RESULTS: There were 122 men (57%) and 92 women (43%) that had presented with spontaneous idiopathic arm swelling, including 28 (13%) with proven pulmonary embolism, at a mean age of 30.7 ± 0.8 years (range 14-69). Upper extremity ultrasound had been performed 23.8 ± 12.2 days after the onset of symptoms, with confirmation of axillary-SCV thrombosis in 169 patients (79%) and negative results in 45 (21%). Of the false-negative ultrasound study reports, only 8 (18%) acknowledged limitations in visualizing the central SCV. Definitive diagnostic imaging (DDI) had been obtained by upper extremity venography in 175 (82%), computed tomography angiography in 24 (11%), and magnetic resonance angiography in 15 (7%), with 142 (66%) undergoing catheter-directed axillary-SCV thrombolysis. The mean interval between initial ultrasound and DDI was 48.9 ± 14.2 days with no significant difference between groups, but patients with a positive ultrasound were more likely to have DDI within 48 hours than those with a false-negative ultrasound (44% vs 24%; P = .02). At the time of surgical treatment, the SCV was widely patent following paraclavicular decompression and external venolysis alone in 74 patients (35%). Patch angioplasty was performed for focal SCV stenosis in 76 (36%) and bypass graft reconstruction for long-segment axillary-SCV occlusion in 63 (29%). Patients with false-negative initial ultrasound studies were significantly more likely to require SCV bypass reconstruction than those with a positive ultrasound (44% vs 25%; P = .02). CONCLUSIONS: Duplex ultrasound has significant limitations in the initial evaluation of patients with suspected SCV thrombosis, with false-negative results in 21% of patients with proven VTOS. This is rarely acknowledged in ultrasound reports, but false-negative ultrasound studies have the potential to delay definitive imaging, thrombolysis, and further treatment for VTOS. Initial false-negative ultrasound results are associated with progressive thrombus extension and a more frequent need for SCV bypass reconstruction at the time of surgical treatment.

Tulip piercing the aorta: a rare case of IVC filter aortic perforation and obstruction.

Prolonged implantation of inferior vena cava (IVC) filters can lead to significant morbidity. We present a 25-year-old man with antiphospholipid syndrome, lower extremity deep vein thrombosis, and subsequent Gunther-Tulip IVC filter placement. More than 10 years following IVC filter placement he developed progressive abdominal and back pains. Cross-sectional angiography revealed that he had a chronic IVC occlusion, and IVC filter limb extensions into the infrarenal aorta, lumbar spine, and right psoas muscle. The IVC filter limb protruding into the aorta had also pierced through the backwall to lead to partial lumen thrombosis and obstruction. The patient underwent a transabdominal exposure of the infrarenal IVC and aorta, filter explantation and aortic patch angioplasty repair. This case highlights the severity of aortic injury from a protruding IVC filter limb that necessitated open aortic repair. Improved selection, monitoring and retrieval stewardship of IVC filters can help reduce the risk of unintended aortic complications.

Effort thrombosis of the subclavian artery as a consequence of a unique anomaly.

Congenital anatomic anomalies and variations are frequent in the thoracic outlet and may be associated with clinical symptoms. Arterial thoracic outlet syndrome (TOS) is characterized by subclavian artery compression and vascular pathology, almost always in the presence of a bony abnormality. We describe here a patient with arterial thromboembolism following a fall on the outstretched arm, who was found to have subclavian artery stenosis and post-stenotic dilatation in the absence of a bony abnormality. Surgical exploration revealed a previously undescribed anomaly in which the subclavian artery passed through the costoclavicular space in front of the anterior scalene muscle, where it was subject to bony compression between the first rib and clavicle. Successful treatment was achieved by scalenectomy, first rib resection and interposition bypass graft reconstruction of the affected subclavian artery. This newly acknowledged anatomical variant adds to our understanding of the diverse factors that may contribute to development of TOS.

Ipilimumab-induced Guillain-Barré Syndrome Presenting as Dysautonomia: An Unusual Presentation of a Rare Complication of Immunotherapy.

Immune-related adverse events are common and well-documented in patients treated with ipilimumab, a cytotoxic T-lymphocyte antigen-4 monoclonal antibody approved for the treatment of metastatic and stage III melanoma. Neurological complications are rare, but widely variable and potentially devastating. Here, we discuss a case of a patient who was treated with a single dose of ipilimumab for resected stage III melanoma. She subsequently developed pandysautonomia that manifested as a tonically dilated pupil, gastrointestinal dysmotility, urinary retention, and profound orthostatic hypotension. Guillain-Barré syndrome was diagnosed on electromyography. She was treated with intravenous immunoglobulin, droxidopa, and supportive care, with prolonged but eventual recovery. Given the broadening use of ipilimumab in the treatment of advanced and metastatic melanoma, awareness and recognition of its profound immune-mediated adverse effects are essential.

A comparison of patency and interventions in thigh versus Hemodialysis Reliable Outflow grafts for chronic hemodialysis vascular access.

OBJECTIVE: With improvements in medical management and survival of patients with end-stage renal disease, maintaining durable vascular access is increasingly challenging. This study compared primary, assisted primary, and secondary patency, and procedure-specific complications, and evaluated whether the number of interventions to maintain or restore patency differed between prosthetic femoral-femoral looped inguinal access (thigh) grafts and Hemodialysis Reliable Outflow (HeRO; Hemosphere Inc, Minneapolis, Minn) grafts. METHODS: A single-center, retrospective, intention-to-treat analysis was conducted of consecutive thigh and HeRO grafts placed between May 2004 and June 2015. Medical history, interventions to maintain or restore patency, and complications were abstracted from the electronic medical record. Data were analyzed using parametric and nonparametric statistical tests, Kaplan-Meier survival methods, and multivariable proportional hazards regression and logistic regression. RESULTS: Seventy-six (43 thigh, 33 HeRO) grafts were placed in 61 patients (54% male; age 53 [standard deviation, 13] years). Median follow-up time in the intention-to-treat analysis was 21.2 months (min, 0.0; max, 85.3 months) for thigh grafts and 6.7 months (min, 0.0; max, 56.3 months) for HeRO grafts (P = .02). The groups were comparable for sex, age, coronary artery disease, diabetes mellitus, peripheral vascular disease, and smoking history (all P ≥ .12). One thigh graft (2%) and five HeRO (15%) grafts failed primarily. In the intention-to-treat analysis, patency durations were significantly longer in the thigh grafts (all log-rank P ≤ .01). Point estimates of primary patency at 6 months, 1 year, and 3 years were 61%, 46%, and 4% for the thigh grafts and 25%, 15%, and 6% for the HeRO grafts. Point estimates of assisted primary patency at 6 months, 1 year, and 3 years were 75%, 66%, and 54% for the thigh grafts and 41%, 30%, and 10% for the HeRO grafts. Point estimates of secondary patency at 6 months, 1 year, and 3 years were 88%, 88%, and 70% for the thigh grafts and 53%, 43%, and 12% for the HeRO grafts. There were no differences in ischemic (P = .63) or infectious (P = .79) complications between the groups. Multivariable logistic regression demonstrated that after adjusting for follow-up time, HeRO grafts were associated with an increased number of interventions (P = .03). CONCLUSIONS: Thigh grafts have significantly better primary, assisted primary, and secondary patency compared with HeRO grafts. There is no significant difference between thigh grafts and HeRO grafts in ischemic or infectious complications. Our logistic regression model demonstrated an association between HeRO grafts and an increased number of interventions to maintain or restore patency. Although HeRO grafts may extend the use of the upper extremity, thigh grafts provide a more durable option for chronic hemodialysis.

Timing of optimal surgical intervention for neonates with necrotizing enterocolitis.

Up to half of all patients with necrotizing enterocolitis require acute surgical treatment. Determining when to operate on these patients can be challenging. Utilizing a combination of clinical and metabolic indicators, we sought to identify the optimal timing of surgical intervention. A retrospective chart review was conducted on patients with necrotizing enterocolitis from 2001 to 2010. Previously validated clinical (abdominal erythema, palpable abdominal mass, hypotension), radiographic (pneumoperitoneum, portal venous gas, fixed bowel loop, severe pneumatosis intestinalis), and laboratory (acidosis, bacteremia, hyponatremia, bandemia, neutropenia, thrombocytopenia) indicators were assessed for the ability to predict the need for acute surgical intervention as a simple indicator score, based on the sum of the indicators listed above. A total of 197 patients were included. One hundred and twenty-four procedures (28 peritoneal drains, 96 laparotomy) were performed on 122 patients (62%). Median indicator score was 4 (range: 0-8). Logistic regression identified abdominal erythema (odds ratio [OR] = 3.3, P = 0.001), acidosis (OR = 2.6, P = 0.004), and hypotension (OR = 1.9, P = 0.05) as independently associated with surgical intervention. A significant increase in surgical intervention was noted for patients with indicator score of 3 or more. In conclusion, if three or more indicators exist, operative intervention is very likely required. In the absence of pneumoperitoneum, abdominal erythema, acidosis, and hypotension are especially important.

Multidisciplinary team approach to end-stage dialysis access patients.

BACKGROUND: The hemodialysis reliable outflow (HeRO) access device is a permanent dialysis graft used in patients with central venous obstruction. Given the complexity of care related to end-stage dialysis access (ESDA) patients, a multidisciplinary approach has been used to achieve operative success of HeRO graft placement. METHODS: The single-center retrospective review included adult patients that were seen in ESDA clinic who underwent a HeRO graft placement from September 2010-September 2014 under the care of a team consisting of a nephrologist, an interventional radiologist, and a surgeon. The effectiveness of the multidisciplinary approach was evaluated using outcome variables including successful HeRO graft placement, operative complications, the rate of obtaining central venous access, and advanced endovascular maneuvers performed by interventional radiology to obtain central venous access. RESULTS: A multidisciplinary approach has been used in 33 ESDA patients. Access to the right atrium was achieved in 100% of cases. Fifty-eight percent of patients required advanced endovascular maneuvers in the interventional radiology suite to obtain central venous access. Successful HeRO graft placement was achieved in 94% (31 of 33) of the study population. No intraoperative complications were encountered. Median primary and secondary patency rates were 83 d (interquartile range: 45-170) and 345 d (interquartile range: 146-579) per HeRO graft placement, respectively. Primary and secondary patency rates at 60 d were 70% (23 of 33) and 79% (26 of 33), respectively. CONCLUSIONS: In this difficult patient population, a multidisciplinary team can provide a unique and collaborative approach to HeRO graft placement in patients with complex central venous outflow obstruction.

Enteral nutrition and total parenteral nutrition components in the course of total parenteral nutrition-associated cholestasis in neonatal necrotizing enterocolitis.

BACKGROUND: Newborns with necrotizing enterocolitis (NEC) are at high risk for the development of total parenteral nutritional-associated cholestasis (TPNAC). Patients with NEC were evaluated to determine risk factors for development of TPNAC and predictors of resolution. We hypothesized that there are additional factors relating to the timing of enteral nutrition or TPN components that effect development and persistence of TPNAC in patients with NEC that may be altered to decrease the chance of progression to liver failure. METHODS: This was a retrospective chart review of NEC patients from 2001 to 2010. TPNAC was defined as direct bilirubin ≥2 mg/dL, the level used for cholestasis in neonatal studies relating to TPNAC. RESULTS: Of 178 patients with NEC, 96 developed TPNAC, and in 27 TPNAC had resolved by discharge. Days of TPN did not affect TPNAC resolution by discharge (P = 1.0). TPNAC was less likely to occur in patients with body weights >1,500 g, enteral nutrition within the first week of life, no infection, fewer TPN days, and lesser hospital stay (P < .01). There were many factors affecting resolution of cholestasis. Enteral nutrition within 6 days of birth decreased development of TPNAC (P < .01), and resumption of enteral nutrition within 3 weeks after NEC diagnosis increased the resolution of cholestasis (P < .01). No component of TPN was important for the development or resolution of cholestasis. CONCLUSION: Of the factors that effect development and resolution of TPNAC in NEC, the ones that we can alter include early enteral feeds and surveillance for infection.

Multi-institutional analysis of long-term symptom resolution after cholecystectomy for biliary dyskinesia in children.

PURPOSE: Current literature for resolution of abdominal pain after cholecystectomy in children with biliary dyskinesia shows variable outcomes. We sought to compare early outcomes with long-term symptom resolution in children. METHODS: Telephone surveys were conducted on children who underwent cholecystectomy for biliary dyskinesia between January 2000 and January 2011 at two centers. Retrospective review was performed to obtain demographics and short-term outcomes. RESULTS: Charts of 105 patients' age 7.9-19 years were reviewed; 80.9 % were female. All were symptomatic with an ejection fraction (EF) <35 % or pain with cholecystokinin administration. At the postoperative visit, 76.1 % had resolution of symptoms. Fifty-six (53.3 %) patients were available for follow-up at median 3.7 (1.1-10.7) years. Of these, 34 (60.7 %) reported no ongoing abdominal pain. Of the 22 patients with persistent symptoms, satisfaction score was 7.3 ± 2.7 (scale of 1-10) and 19 (86.4 %) were glad that they had a cholecystectomy performed. EF, body mass index percentile (BMI %), and pain with cholecystokinin (CCK) were not predictive of ongoing pain at either follow-up periods. CONCLUSION: Short-term symptom resolution in children undergoing cholecystectomy for biliary dyskinesia is not reflective of long-term results. Neither EF, BMI % nor pain with CCK was predictive of symptom resolution. The majority of patients with ongoing complaints do not regret cholecystectomy.

Pathologic changes in biliary dyskinesia.

PURPOSE: For children with upper abdominal pain and evaluation for acalculous biliary disease, laparoscopic cholecystectomy is an accepted treatment with inconsistent outcomes. The purpose of this study was to identify predictors of outcomes. METHODS: One hundred sixty-seven children underwent laparoscopic cholecystectomy at a single children's hospital. Radiographic findings, histopathology, family history, and demographics (sex, age, height, weight, body mass index-for-age percentile) were evaluated as predictors of postoperative symptomatic resolution using a binomial probability model. The data for radiologic studies and pathologic specimens were obtained via re-review in a blinded fashion. RESULTS: Of 167 children, 43 (25.7%) had a preoperative diagnosis of biliary dyskinesia and 41 (95.3%) had documented follow-up. Mean follow-up was 8.4 months. Twenty-eight patients (68.3%) had symptom resolution. Ejection fraction less than or equal to 15%, pain upon cholecystokinin injection, and a family history of biliary disease were not predictors of symptomatic resolution. Nonoverweight patients (body mass index-for-age <85th percentile) were more likely to have symptom resolution than their overweight counterparts (odds ratio, 2.13). Most patients (68.3%) had a pathologic gallbladder on blinded review. However, this did not correlate with outcome. CONCLUSIONS: Most gallbladders removed for biliary dyskinesia are pathologic. Being overweight can be considered a relative contraindication to cholecystectomy for biliary dyskinesia.