Cervical lymphatic malformations amenable to transhairline robotic surgical excision in children: A case series.

ABSTRACT: Lymphatic malformations are rare benign malformations that predominantly occur in the head and neck region. The advent of surgical robots in head and neck surgery may provide beneficial outcomes for pediatric patients. Here, we describe our experiences with transhairline incisions for robot-assisted surgical resection of cervical lymphatic malformations in pediatric patients.In this prospective longitudinal cohort study, we recruited consecutive patients under 18 years of age who were diagnosed with congenital cervical lymphatic malformations and scheduled for transhairline approach robotic surgery at a single medical center. We documented the docking times, console times, surgical results, complications, and postoperative follow-up outcomes.The studied patients included 2 with mixed-type lymphatic malformations and 2 with macrocystic-type lymphatic malformations. In all 4 patients, the incision was hidden in the hairline; the incision length was <5 cm in 3 patients but was extended to 6 cm in 1 patient. Elevating the skin flap and securely positioning it with Yang retractor took <1 hour in all cases. The mean docking time was 5.5 minutes, and the mean console time was 1 hour and 46 minutes. All 4 surgeries were completed endoscopically with the robot. The average total drainage volume in the postoperative period was 21.75 mL. No patients required tracheotomy or nasogastric feeding tubes. Neither were adverse surgery-associated neurovascular sequelae observed. All 4 patients were successfully treated for their lymphatic malformations, primarily with robotic surgical excisions.Cervical lymphatic malformations in pediatric patients could be accessed, properly visualized, and safely resected with transhairline-approach robotic surgery. Transhairline-approach robotic surgery is an innovative method for meeting clinical needs and addressing esthetic concerns.

Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein-losing enteropathy. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined. Methods and Results This was a retrospective, single-center study of patients undergoing first-time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2-weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty-eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high-grade lymphatic abnormalities had 6 times greater odds of developing early complications (P=0.001). Conclusions There is substantial morbidity in the early post-Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher-grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.

Diferenciación entre linfadenopatía cervical benigna y maligna mediante ecografía en niños / Differentiation of benign from malignant cervical lymphadenopathy by ultrasonography in children

Introducción. Las causas más frecuentes de la linfadenopatía cervical son las afecciones inflamatorias y reactivas; solo unos pocos casos representan una patología seria. El objetivo fue evaluar la relación entre los hallazgos ecográficos y el diagnóstico histopatológico. Población y métodos. Este estudio retrospectivo abarcó la linfadenopatía cervical en los menores de 20 años seguidos en nuestro centro, entre enero de 2007 y diciembre de 2016. Según los informes anatomopatológicos, se dividió a los pacientes en dos grupos: benigno y maligno. Se compararon los resultados anatomopatológicos y los hallazgos ecográficos. Resultados. Después del análisis de los resultados histopatológicos y los hallazgos ecográficos, se incluyó a 107 pacientes con linfadenopatía cervical persistente (44 casos malignos; 63, benignos). La media de edad de los grupos maligno y benigno fue de 14 ± 6,1 años y de 11,9 ± 4,8 años, respectivamente. La presencia de vascularidad hiliar fue estadísticamente significativa (p < 0,0001) en la linfadenopatía benigna, mientras que el flujo periférico y la vascularidad mixta lo fueron (p < 0,05) en la linfadenopatía maligna. No se observó una diferencia significativa en el diámetro máximo (27,3 ± 11,1 mm y 29,8 ± 12,3 mm, respectivamente), pero sí en el diámetro mínimo entre los grupos benigno y maligno (13,7 ± 7,3 mm y 18,7 ± 8,8 mm, respectivamente). Conclusiones. Este estudio sugiere que existe una relación entre los hallazgos ecográficos y de la biopsia para la diferenciación entre la linfadenopatía benigna y maligna, en especial, en el patrón vascular intraganglionar y el hilio ganglionar.

Introduction. The most common causes of cervical lymphadenopathy (LAP) are inflammatory and reactive conditions; only a small proportion have serious pathology, such as malignancy. The objective of this study was to evaluate the relationship between USG findings and histopathological diagnosis of the cervical LAP. Population and Methods. This retrospective study comprised the cases of cervical LAP in patients aged under 20 years old followed in our center between January 2007 to December 2016. Based on pathology reports, we divided the patients into two groups: benign and malignant. Pathology results and USG findings were compared. Results. After the analyze of the histopathological results and USG findings, 107 patients with persistent cervical LAP (44 malignant; 63 benign) were included in the study. Mean age of malignant and benign group were 14 ± 6.1; 11.9 ± 4.8 years, respectively. Hilar vascularity for benign LAP was highly statistically significant (P < 0.0001) and peripheral flow and mixed vascularity for malignant LAP were also statistically significant (p < 0.05). There was not a significant difference in the maximum diameter (27.3 ± 11.1 mm and 29.8 ± 12.3 mm, respectively), however, there was a significant difference in the minimum diameter between benign and malignant groups (13.7 ± 7.3 mm and 18.7 ± 8.8 mm, respectively).Conclusions. The present study suggests that there is a relationship between US and biopsy findings for the differentiation of benign from malignant LAP, especially in terms of nodal hilus and intranodal vascular pattern.

Decompression of the thoracic duct: A novel transcatheter approach.

In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life-threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.

Review of axillary web syndrome: What the radiologist should know.

Axillary web syndrome is common after axillary surgery, frequently affecting breast cancer patients. In this condition, patients develop one or more linear bands of firm tissue, also known as "cords", in the axilla and arm, associated with pain and limited range of motion of the shoulder and arm. Radiologists may encounter this syndrome in patients referred for axillary or upper extremity ultrasound, and should be aware of the physical examination and ultrasound findings for accurate diagnosis. However, there are currently limited articles about this syndrome published in radiology journals, suggesting that radiologists may be unaware of this entity. In this work, axillary web syndrome will be discussed, including background knowledge, incidence, clinical presentation, possible etiology, and ultrasound appearance.

Can Symptoms Differentiate Between Chronic Adenoiditis and Chronic Rhinosinusitis in Pediatric Patients.

The purpose of this article is to differentiate pediatric patients with chronic adenoiditis from those with chronic rhinosinusitis (CRS) based on presenting symptoms. A chart review from a tertiary care facility with pediatric patients who presented with suspected CRS from 2006 to 2014 was identified. We compared patient characteristics, clinical symptoms, duration of symptoms, and past medial history using univariate and multivariate logistic regression models. Based on recent literature, utilizing the computed tomography (CT) score, we identified those children with CRS versus those with chronic adenoiditis. Of the 99 pediatric patients included, 22 patients had diagnosis of adenoiditis and 77 had diagnosis of CRS. When purulent rhinorrhea was present with facial pain, CRS was statistically more prevalent than chronic adenoiditis (P = .017). Symptoms including cough (P = .022), rhinorrhea (P = .27), and facial pressure (P = .98) were not predictive of one diagnosis over the other. Past medical history of asthma or allergy was similar in both groups. Smoke exposure was associated with CT scores >5 (odds ratio 2.4, 95% confidence interval, 0.799-7.182). We conclude that purulent rhinorrhea in the presence of facial pain is more indicative of CRS versus chronic adenoiditis. For all other children, an adenoidectomy without the need for a CT scan can be entertained.

Lymphatic dysfunction in critical illness.

PURPOSE OF REVIEW: The essential role of the lymphatic system in fluid homeostasis, nutrient transport, and immune trafficking is well recognized; however, there is limited understanding of the mechanisms that regulate lymphatic function, particularly in the setting of critical illness. The lymphatics likely affect disease severity and progression in every condition, from severe systemic inflammatory states to respiratory failure. Here, we review structural and functional disorders of the lymphatic system, both congenital and acquired, as they relate to care of the pediatric patient in the intensive care setting, including novel areas of research into medical and procedural therapeutic interventions. RECENT FINDINGS: The mainstay of current therapies for congenital and acquired lymphatic abnormalities has involved nonspecific medical management or surgical procedures to obstruct or divert lymphatic flow. With the development of dynamic contrast-enhanced magnetic resonance lymphangiography, image-directed percutaneous intervention may largely replace surgery. Because of new insights into the mechanisms that regulate lymphatic biology, pharmacologic inhibitors of mTOR and leukotriene B4 signaling are each in Phase II clinical trials to treat abnormal lymphatic structure and function, respectively. SUMMARY: As our understanding of normal lymphatic biology continues to advance, we will be able to develop novel strategies to support and augment lymphatic function during critical illness and through convalescence.

Vascular Alterations in Axillary and Brachial Vessels in Patients with Axillary Web Syndrome After Breast Cancer Surgery.

BACKGROUND: Surgical manipulations of the axilla may cause a condition known as Axillary Web Syndrome (AWS). The systems compromised and the sequence of events leading to this syndrome remains unknown. This study evaluated clinical, surgical, and vascular factors associated with onset and duration of AWS after breast cancer surgery. METHODS AND RESULTS: In this prospective study, 155 women were included. They were submitted to a physical examination that consisted of ultrasound Doppler of axillary and brachial vessels and the evaluation of AWS in 1, 3, and 6 months after breast cancer surgery. Women with advanced disease had a significantly higher incidence of AWS than those with early stage breast cancer (p = 0.02). In addition, women who underwent mastectomy or axillary lymph node dissection (ALND) had a significantly higher incidence of AWS in the 1-month (p < 0.01; p < 0.01) and 3-months (p < 0.01; p = 0.02) assessment rounds, respectively. The cross-sectional area of brachial artery was significantly smaller (p = 0.04) in women with AWS at the 3-months postoperative visit. The peak systolic velocity and the blood flow of the axillary artery was significantly higher in women with AWS 6 months after surgery (p < 0.03 and p = 0.02 respectively). CONCLUSION: Our study confirm the combined changes of lymphatic and vascular systems in woman with AWS, since AWS was associated with more extensive dissection of axillary lymph nodes, compromised lymph nodes, and with abnormalities of the vascular parameters.

Sclerosing thymoma-like thymic amyloidoma with nephrotic syndrome: a case report.

BACKGROUND: Primary localized amyloidosis presenting as an isolated mediastinal mass is extremely rare, especially in the thymus. Sclerosing thymoma is also an extremely rare anterior mediastinal tumor, pathologically characterized by extensive sclerotic lesions with hyalinization and calcification. Only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date. CASE PRESENTATION: A 78-year-old Japanese woman was diagnosed as having sclerosing thymoma (Masaoka stage IVa pericardial dissemination)-like thymic amyloidoma. She was diagnosed as having either lung cancer or mediastinal tumor with pericardial dissemination, and received palliative treatment. Three years later, she was readmitted with a complaint of general malaise. Since minimal change nephrotic syndrome was suspected based on the disease onset and selectivity index of urinary protein, steroid pulse therapy was started. Subsequently, because a marked reduction in tumor size was observed during maintenance treatment with prednisolone, a thoracoscopic needle biopsy was performed for a definitive diagnosis. According to the pathological findings and clinical investigations, a final diagnosis of sclerosing thymoma (Masaoka stage IVa pericardial dissemination)-like thymic amyloidoma was made. CONCLUSIONS: This is a case report of sclerosing thymoma-like thymic amyloidoma. Both sclerosing thymoma and thymic amyloidoma are extremely rare diseases: only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date. In either diagnosis, our case is the first case in which marked reduction in tumor size was observed with steroid therapy. All reported cases of sclerosing thymomas underwent surgical resection, but steroid therapy to sclerosing thymoma has not been reported. It is still unknown whether steroid therapy is effective or not. The hyalinized components of sclerosing thymoma possibly contain amyloid deposits. The marked reduction in tumor size with steroid therapy may result in amyloid deposits. The association between sclerosing thymoma and thymic amyloidoma remains uncertain. Sclerosing thymoma should be stained with Congo red.

The Lymphatic System: The Achilles Heel of the Fontan-Kreutzer Circulation.

In spite of excellent long term survival the Fontan Kreutzer procedure commonly presents late failure due to end-organ damage. Several advances have been described to refine single ventricle management and surgical techniques. However, very little research has been dedicated to the lymphatic circulation in the precarious Fontan hemodynamic state. The lymphatic circulation is clearly affected since there is increased lymph production, which requires to be drained at a similar or higher pressure than it is produced, commonly resulting in chronic lymphedema. Chronic lymphedema induces fibrosis and end-organ failure even in normal circulation. Diverting lymph drainage to the low-pressured systemic atrium in Fontan may represent a valid alternative for the treatment of devastating complications as protein-losing enteropathy and plastic bronchitis and may prevent or decrease the development of end-organ fibrosis or failure.

Late Effects of Exposure to Ionizing Radiation and Age on Human Thymus Morphology and Function.

The thymus is essential for proper development and maintenance of a T-cell repertoire that can respond to newly encountered antigens, but its function can be adversely affected by internal factors such as pregnancy and normal aging or by external stimuli such as stress, infection, chemotherapy and ionizing radiation. We have utilized a unique archive of thymus tissues, obtained from 165 individuals, exposed to the 1945 atomic bomb blast in Hiroshima, to study the long-term effects of receiving up to ∼3 Gy dose of ionizing radiation on human thymus function. A detailed morphometric analysis of thymus activity and architecture in these subjects at the time of their natural deaths was performed using bright-field immunohistochemistry and dual-color immunofluorescence and compared to a separate cohort of nonexposed control subjects. After adjusting for age-related effects, increased hallmarks of thymic involution were observed histologically in individuals exposed to either low (5-200 mGy) or moderate-to-high (>200 mGy) doses of ionizing radiation compared to unirradiated individuals (<5 mGy). Sex-related differences were seen when the analysis was restricted to individuals under 60 years of attained age at sample collection, but were not observed when comparing across the entire age range. This indicates that while females undergo slower involution than males, they ultimately attain similar phenotypes. These findings suggest that even low-dose-radiation exposure can accelerate thymic aging, with decreased thymopoiesis relative to nonexposed controls evident years after exposure. These data were used to develop a model that can predict thymic function during normal aging or in individuals therapeutically or accidentally exposed to radiation.

Novel Lymphatic Imaging Techniques.

The lymphatic system plays an important role in immune regulation, transport of metabolites, and fluid balance. The key circulatory role of the lymphatic system is to transport fluid from tissue back into the venous system via lymphovenous connections. Despite the centuries-old recognition of this key role, there has been poor understanding of lymphatic flow pathophysiology because of a lack of a simple reliable noninvasive clinical lymphatic imaging method. This lack of clinical imaging has limited the treatment options for patients with lymphatic flow disorders. Recent development of noncontrast magnetic resonance (MR) lymphangiogram and dynamic contrast MR lymphangiography make it possible to visualize central lymphatic anatomy and flow dynamics with high spatial and temporal resolution. Dynamic contrast MR lymphangiography has provided insight into understanding the pathophysiology of several pulmonary lymphatic flow disorders and provides guidance for interventional procedures. Another important development has been intranodal lymphangiogram, which has now replaced pedal lymphangiogram as the main lymphatic interventional modality, and which provides quick and reliable access to the central lymphatic ducts for interventional procedures. These new techniques have led to a resurgence in interest in the lymphatic system and the development of new treatments for patients with lymphatic flow disorders.

Lymphoscintigraphy for Imaging of the Lymphatic Flow Disorders.

Lymphoscintigraphy has introduced with the great advantage for diagnostic imaging of the lymphatic flow disorders. Lymphoscintigraphy can be performed in patients of any age, including neonates, and even in patient in critical conditions. The procedure is quite simple, and it needs only subcutaneous injection of small amounts of radiotracers. From subcutaneous tissue the radiotracer is taken by the lymphatic vessels and gives off energy in the form of gamma radiation detected by a gamma camera. Radiotracers rarely cause the allergic reaction and can be administered to the patients with allergy to iodine contrast media. Comparing with the Lipiodol, radiotracers cannot cause pulmonary embolism; therefore, it is safe for the patients with respiratory dysfunction. The objective of this article is to describe the indication, technique, equipment, pitfalls, safety, and effectiveness of lymphoscintigraphy for imaging of the lymphatic flow disorders.

A multicenter study on the utility and safety of EBUS-TBNA and EUS-B-FNA in children.

BACKGROUND AND AIM: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and endoscopic ultrasound with an echobronchoscope-guided fine needle aspiration (EUS-B-FNA) are useful modalities in the evaluation of mediastinal lymphadenopathy in adults; however, there is sparse data in children. The aim of this multicenter study is to describe the efficacy and safety of EBUS-TBNA and EUS-B-FNA in children with mediastinal lymphadenopathy of undefined etiology. METHODS: Retrospective analysis of consecutive pediatric (<18 years) subjects who underwent EBUS-TBNA or EUS-B-FNA for the evaluation of mediastinal lymphadenopathy. The demographic characteristics, indications, procedural details, pathological, cytological and microbiological diagnosis, diagnostic yield, and complications are presented. RESULTS: Of the 3,424 EBUS/EUS-B-FNA procedures, 67 (1.9%) were performed in the pediatric (3-17 years) population. Of these, 19 (28.4%) were performed in children ≤12 years of age. Overall, EBUS-TBNA and EUS-B-FNA were performed in 53 and 12 subjects, respectively. In two subjects, no significant lymph node was seen on EBUS. The procedure was performed under moderate sedation in spontaneously breathing subjects in 54 (80.6%) instances. An adequate sample was obtained in 60 (92.3%) subjects while a diagnostic sample was obtained in 37 (56.9%) of the 65 subjects. The diagnostic yield was not significantly different (P = 0.59) between EBUS-TBNA (58.5%) and EUS-B-FNA (50%). The sensitivity of EBUS-TBNA/EUS-B-FNA was 79.1% and led to a change in diagnosis in 28 (41.8%) subjects. Complications, all minor were encountered in six (8.9%) subjects. CONCLUSIONS: EBUS-TBNA and EUS-B-FNA are safe techniques with a good diagnostic yield in the evaluation of children with mediastinal lymphadenopathy. Pediatr Pulmonol. 2016;51:1031-1039. © 2016 Wiley Periodicals, Inc.

Virtual Touch Tissue Imaging Quantification Shear Wave Elastography: Prospective Assessment of Cervical Lymph Nodes.

The goal of this study was to prospectively evaluate the diagnostic performance of Virtual Touch tissue imaging quantification (VTIQ) shear wave elastography in the discrimination of benign and malignant cervical lymph nodes in routine clinical practice. Shear wave velocity was analyzed using VTIQ in 100 patients with 100 histologically proven cervical lymph nodes. Diagnostic performance was evaluated using receiver operating characteristic curve analysis and leave-one-out cross-validation. Agreement between measurements was assessed with intra-class correlation coefficients. The mean shear wave velocity was significantly higher in metastatic lymphadenopathy (4.46 ± 1.46 m/s) than in benign lymphadenopathy (2.71 ± 0.85 m/s) (p < 0.001) at a cutoff level of 3.34 m/s. The cross-validated accuracy, sensitivity and specificity were 77%, 78.9% and 74.4%, respectively. Agreement of measurements with VTIQ was excellent (intra-class correlation coefficient = 0.961). VTIQ shear wave elastography may be a feasible quantitative imaging method for differentiating benign and malignant cervical lymph nodes.