Objective To determine the response rate, toxicity, and rate of complete resection after induction chemoradiotherapy for locally advanced thymic tumors, which were defined by specific radiographic ...criteria. Methods A single-arm, pilot trial was conducted at 4 institutions. Patients with thymoma or thymic carcinoma who met specific criteria on computed tomography were accrued. Induction therapy consisted of 2 cycles of cisplatin and etoposide combined with 45 Gy of thoracic radiotherapy. Patients underwent computed tomography and positron emission tomography before and after induction therapy and then resection was attempted. Postoperative chemoradiotherapy was administered in selected patients. The primary endpoint was the pathologic response to induction therapy. The secondary endpoints were toxicity, surgical complications, radiographic response, and the rate of R0 resection. Results A total of 22 patients were accrued during a 5-year period (1 patient withdrew before starting induction therapy). Of the 22 patients, 21 completed induction therapy, and 9 (41%) experienced grade 3 or 4 toxicity. A total of 10 patients had a partial radiographic response and 11 had stable disease. Of the 21 patients, 17 (77%) underwent an R0 resection, 3 (14%) an R1 resection, and 1 (5%) underwent debulking. Eight patients sustained surgical complications (36%), and two patients (9%) died postoperatively. Of the 21 patients, 13 (62%) had either thymic carcinoma or B3 thymoma and 15 (71%) had either Masaoka stage III or IV disease. No patient had a complete pathologic response, but 5 specimens (24%) had <10% viable tumor. Conclusions The present induction chemoradiotherapy protocol, which used specific computed tomography inclusion criteria to successfully select locally advanced thymic tumors, appeared to be tolerable and resulted in a high rate of complete surgical resection.
Background Liver dysfunction increases death and morbidity after cardiac operations. There are currently no data evaluating liver function in patients undergoing transcatheter aortic valve ...replacement (TAVR). We aimed therefore to evaluate our TAVR results in regard to liver function. Methods A total of 640 consecutive TAVR patients were evaluated. Of those, 11 patients presented with chronic liver disease before TAVR. The Model for End-Stage Liver Disease score was used to measure liver function in these patients. The primary study end point was 30-day mortality in patients presenting with liver dysfunction. Secondary study end point was liver enzymes after TAVR. Results The mean Model for End-Stage Liver Disease score in patients with chronic liver disease was 16.8 ± 6.2 (median, 18; range, 7 to 26). The 30-day mortality was 9.1% (57 of 629) in patients presenting without liver disease and 9.1% (1 of 11) in patients with liver disease ( p = 1.00). Patients with chronic liver disease showed significantly higher preoperative levels of γ-glutamyl transpeptidase ( p < 0.001). After TAVR, we observed a significant increase in alanine aminotransferase on postoperative day 3 compared with preoperative values ( p < 0.001), accompanied by a decrease in albumin ( p < 0.001). Conclusions Liver cirrhosis per se is not considered as a contraindication for cardiac operations. In the present study, we did not observe a higher 30-day mortality rate in liver cirrhotic patients undergoing TAVR, suggesting TAVR as a feasible alternative with acceptable outcomes in patients with chronic liver disease. Moreover, the present study is the first to evaluate liver variables in patients undergoing TAVR.
Ebstein's anomaly is associated with a high incidence of atrial and ventricular arrhythmias. The Cone procedure has become an effective hemodynamic intervention for this malformation. In response to ...two late postoperative sudden deaths in our early institutional Cone experience, a standardized plan for aggressive rhythm evaluation was instituted, including preoperative electrophysiologic studies (EPS) and intraoperative implantable loop recorder (ILR) placement.
The purpose of this study was to measure the yield of this diagnostic protocol for patients with Ebstein's anomaly undergoing surgical repair and to describe its influence on patient management.
All patients at Boston Children's Hospital with Ebstein's anomaly who underwent the Cone procedure from December 2006 to September 2012 were reviewed. Pre- and postoperative arrhythmias and therapies were documented. For patients who underwent preoperative EPS, all arrhythmia substrates and interventions were recorded.
A total of 74 patients were included, 42 of whom underwent preoperative EPS. Significant findings were documented during EPS in 29 of the 42 patients (69%), including eight patients with no prior suspicion of arrhythmias. Seventeen patients had successful catheter ablation during EPS, and EPS data guided one or more intraoperative rhythm interventions for the remainder. During follow-up, diagnostic yield from ILR was low. Since implementing a more aggressive diagnostic strategy, no further sudden deaths have occurred.
Preoperative EPS has a high diagnostic and therapeutic yield in patients with Ebstein's anomaly undergoing the Cone operation. It is reasonable to recommend EPS as a routine preoperative test for this population.
Left ventricular noncompaction (LVNC) cardiomyopathy is variably defined by numerous trabeculations, deep intertrabecular recesses, and noncompacted-to-compacted (NC/C) ratio >2. Limited studies ...exist on the reproducibility of diagnosing LVNC.
Clinical records of patients diagnosed with LVNC by echocardiography were reviewed. Blinded review of the index echocardiogram for all patients and a 1:1 match without LVNC was performed independently by two observers, measuring the number of trabeculations and the NC/C ratio.
A total of 104 patients with LVNC were included in the study, 52 with no congenital heart disease (NCongHD) and 52 with congenital heart disease (CongHD). The duration of follow-up was 7.2 years (range, 0.5-23.1 years) for NCongHD and 8.2 years (range, 0-33.3 years) for CongHD. Agreement between observers in determining zero to three versus more than three trabeculations was 59% (NCongHD) and 73% (CongHD). Agreement in measuring an NC/C ratio ≤ 2 versus > 2 was 79% (NCongHD) and 74% (CongHD). Agreement with the original reader in diagnosing LVNC was 67%. There was no association between the number of trabeculations or the NC/C ratio and the likelihood of a major event. Patients with moderate or severe left ventricular dysfunction at the time of diagnosis were more likely to undergo cardiac transplantation or die compared with those with normal or mild dysfunction (NCongHD, 22% vs 0%, P = .01; CongHD, 39% vs 3%, P = .001).
The reproducibility of making measurements to diagnose LVNC by accepted criteria is poor. Heart transplantation and death are associated with significant ventricular dysfunction and not with increased trabeculations or NC/C ratios.
The second edition of the World Health Organization (WHO) classification of thymic tumors (2004) has resumed the previous separation of thymic carcinomas (TCs) from thymomas. This "reseparation" was ...mainly based on new genetic data. Consequently, it is no longer recommended to label TCs as type C thymomas. TCs are very heterogeneous and comprise squamous, basaloid cell, mucoepidermoid, neuroendocrine, and many other subtypes. They resemble morphologic mimics in other organs and are labeled accordingly. However, only thymic squamous cell carcinomas (TSCCs) and lymphoepithelioma-like carcinomas are relatively common. For TSCCs, quite specific immunohistochemical markers (eg, CD5, CD70, CD117, CD205, FOXN1) and chromosomal gains and losses have been defined that help to distinguish TSCCs not only from malignant thymomas but also from pulmonary squamous cell carcinomas. Recognition of these differences is clinically important, because the prognosis of TSCC is better compared with the other TC subtypes and also compared with lung tumors. Considering the need to treat advanced TC more effectively, disparate findings in predictive molecular markers (eg, KIT mutations in TSCC, but not in thymomas) suggest that targeted treatments will have to be different in thymomas and TC. Preliminary data from single case collections and small treatment trials support this prediction.
Background We reviewed reoperations for persistent or recurrent sporadic parathyroid adenoma to evaluate and compare our current results and outcomes to our previous experience. Methods From 1996 to ...2008, 237 patients with persistent or recurrent hyperparathyroidism after failed operation underwent reoperation. Patients were re-explored with the assistance of non-invasive and sometimes invasive imaging. Results A missed adenoma was suspected pre-operatively in 163 patients. Reoperation resulted in long-term resolution of hypercalcemia in 92%. Adenomas were in entopic locations in 32%; the most frequent ectopic location was the thymus (20%). Sestamibi scanning and ultrasonography were the most successful non-invasive imaging studies (96% positive predictive value (PPV) and 84% PPV respectively). Forty-four percent of patients had a reoperation based solely on non-invasive imaging. Of the invasive procedures performed, arteriography resulted in the best localization (92% PPV). Permanent recurrent laryngeal nerve injury occurred in 1.8%. Conclusion Compared to our prior experience (1982–1995), outcomes remained similar (92% resolution of hypercalcemia and 1.8% recurrent nerve injury currently versus 96% and 1.3% previously). Fewer patients received invasive studies for pre-operative localization (56% vs 73%, respectively). The decreased use of invasive imaging is due to technical improvements and greater confidence in the combination of ultrasonography and sestamibi scanning.
Objective Macrophage migration inhibitory factor is a proinflammatory mediator of innate immunity, enhances cell growth, and plays a role in preterm delivery. We speculated that funisitis, reflecting ...fetal systemic inflammation, would be associated with higher concentrations of macrophage migration inhibitory factor in airways of extremely premature infants. Study Design We measured macrophage migration inhibitory factor by enzyme linked immunosorbent assay in tracheobronchial aspirate fluid of 35 ventilated infants less than 30 weeks’ gestational age, throughout the first week of life. Three groups were distinguished histologically: chorioamnionitis, funisitis, and control. Results Unexpectedly, funisitis was associated with significantly decreased macrophage migration inhibitory factor in tracheobronchial aspirate fluid on day 1 ( P < .01) and levels remained lower than in the chorioamnionitis group thereafter. For the 35 patients in total, macrophage migration inhibitory factor steadily declined. Conclusion Decreased macrophage migration inhibitory factor concentrations in airways of extremely premature infants with systemic fetal inflammation early in life might predispose them to pulmonary infection and interfere with maturation of the lung, contributing to adverse pulmonary outcome.
Background Operation for multiple endocrine neoplasia (MEN)1-related hyperparathyroidism (HPT) includes a neck exploration with resection of 3.5 or 4 parathyroid glands and transcervical thymectomy ...(TCT). We reviewed our experience with initial operation for primary HPT to determine the outcome and utility of routine TCT. Methods All patients with MEN1 who underwent initial neck exploration from 1993 to 2007 under an institutional review board-approved protocol were reviewed. Results We identified 66 patients with initial operation for HPT in MEN1. In 34 patients, 4 glands were found; in 32 patients, <4 glands were found. In 2 of the 34 (6%) and 17 of the 32 (53%), intrathymic parathyroid tissue was found on permanent pathology. No thymic carcinoid tissue was found in any specimen. Conclusion These data highlight the importance of performing TCT when <4 entopic parathyroid glands are found at first operation.
Abstract We report on a 5-week-old male infant with recurrent respiratory distress since birth and congenital thymic hyperplasia. Acute life-threatening thymic bleeding apparently from ruptured ...thymic cysts into the pleural spaces complicated the clinical situation. Thoracotomy and complete thymectomy were performed. Histologic examination revealed normal thymic architecture with cysts of different sizes and an increased thymic weight of 30 g. The combination of true thymic hyperplasia and cyst bleeding in a newborn has not been previously reported and will be discussed in relation to the available literature on respiratory distress due to thymic pathology in childhood.
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