Rosai-Dorfmann Disease: A Rare Disease Presenting as a Unilateral Neck Swelling.

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. A 14-year-old boy presented with painless right sided cervical lymphadenopathy without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed infiltration of sheets of histiocytes showing emperipolesis with areas of fibrosis and hyalinisation. The sinus histiocytes were strongly positive for S-100 protein. RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies.

Erdheim-Chester disease with chorioretinal and orbital involvement: a case report / Doença de Erdheim-Chester com envolvimento coriorretiniano e orbitário: relato de caso

ABSTRACT A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.

RESUMO Uma mulher de 42 anos apresentou proptose bi-lateral, quemose, dor nas pernas e perda de visão. Com base em achados clínicos, radiológicos e patológicos, foi diag-nosticada doença de Erdheim-Chester com acometimento orbitário, coriorretiniano e multiorgânico. Trata-se de uma rara histiocitose não Langerhans negativa para a mutação BRAF. Foi iniciado tratamento com interferon alfa-2a (IFNα-2a) e o quadro clínico melhorou. No entanto, quatro meses depois, a paciente apresentou perda visual após a cessação do IFNα-2a. A mesma terapia foi administrada novamente e sua condição clínica melhorou novamente. A doença de Erdheim-Chester é uma doença proliferativa histiocítica crônica rara que necessita de uma abordagem multidisciplinar e pode ser fatal se não tratada, devido a envolvimentos multissistêmicos.

The essential role of thoracic duct embolization in management of traumatic iatrogenic chylothorax.

This study aims to describe a case series of patients who underwent thoracic duct embolization (TDE) to treat traumatic iatrogenic chylothorax (TIC). Three patients were included: Case #1, a 49-year-old woman with follicular lymphoma developed a TIC following video-assisted thoracoscopic surgery to resect a solid right paravertebral mass and was treated with TDE using microcoils and N-butyl cyanoacrylate (NBCA) glue. Case #2, a 68-year-old man with cardiac amyloidosis developed a TIC following heart transplantation and was treated with TDE using microcoils and ethylene vinyl alcohol copolymer. Case#3: A 6-year-old patient with congenital heart disease developed a TIC following a Fontan procedure and was treated with TDE using NBCA glue. All lesions were identified during lymphangiography and TDE was successfully performed in all cases. TDE is a safe and valuable technique that provides minimally invasive treatment for TCI.

Este estudo objetiva descrever uma série de casos de pacientes submetidos a embolização do ducto torácico (EDT) para tratamento de quilotórax iatrogênico (QI). Três pacientes foram incluídos. Caso 1: um homem de 49 anos com linfoma folicular apresentou QI após ressecção de uma massa paravertebral por toracoscopia vídeo-assistida e foi submetido a EDT com micromolas e n-butil-cianoacrilato (NBCA). Caso 2: um homem de 68 anos com amiloidose cardíaca apresentou QI após ser submetido a transplante cardíaco e foi submetido a EDT com micromolas e copolímero de etileno e álcool vinílico. Caso 3: um paciente de 6 anos com malformação cardíaca congênita apresentou QI após cirurgia de Fontan e foi submetido a EDT com NBCA. Todas as lesões foram identificadas durante a linfangiografia, e a EDT foi realizada com sucesso. A EDT é uma técnica segura e valiosa, que pode oferecer um tratamento minimamente invasivo em casos de QI.

Profound and selective lymphopaenia in primary lymphatic anomaly patients demonstrates the significance of lymphatic-lymphocyte interactions.

Introduction: The lymphatic system has a pivotal role in immune homeostasis. To better understand this, we investigated the impact of Primary Lymphatic Anomalies (PLA) on lymphocyte numbers and phenotype. Methods: The study comprised (i) a retrospective cohort: 177 PLA subjects from the National Primary Lymphatic Anomaly Register with clinical and laboratory data, and (ii) a prospective cohort: 28 patients with PLA and 20 healthy controls. Patients were subdivided using established phenotypic diagnostic categories and grouped into simplex (localised tissue involvement only) and systemic (involvement of central lymphatics). Further grouping variables included genital involvement and the likelihood of co-existent intestinal lymphangiectasia. Haematology laboratory parameters were analysed in both cohorts. In the prospective cohort, prospective blood samples were analysed by flow cytometry for markers of proliferation, differentiation, activation, skin-homing, and for regulatory (CD4+Foxp3+) T cells (Treg). Results: In patients with PLA, lymphopaenia was frequent (22% of subjects), affected primarily the CD4+ T cell subset, and was more severe in subjects with systemic versus simplex patterns of disease (36% vs 9% for lymphopaenia; 70% vs 33% for CD4+ cells). B cells, NK cells and monocytes were better conserved (except in GATA2 deficiency characterised by monocytopaenia). Genital oedema and likelihood of concomitant intestinal lymphangiectasia independently predicted CD4+ T cell depletion. Analysing CD4+ and CD8+ T cells by differentiation markers revealed disproportionate depletion of naïve cells, with a skewing towards a more differentiated effector profile. Systemic PLA conditions were associated with: increased expression of Ki67, indicative of recent cell division, in naïve CD4+, but not CD8+ T cells; increased levels of activation in CD4+, but not CD8+ T cells; and an increased proportion of Treg. Skin-homing marker (CCR10, CLA and CCR4) expression was reduced in some patients with simplex phenotypes. Discussion: Patients with PLA who have dysfunctional lymphatics have a selective reduction in circulating lymphocytes which preferentially depletes naïve CD4+ T cells. The presence of systemic disease, genital oedema, and intestinal lymphangiectasia independently predict CD4 lymphopaenia. The association of this depletion with immune activation and increased circulating Tregs suggests lymphatic-lymphocyte interactions and local inflammatory changes are pivotal in driving immunopathology.

[Genetics, diagnostics and clinical presentation of primary lymphoedema]. / Genetik, Diagnostik und Klinik des primären Lymphödems.

Primary lymphoedema is a hereditary genetic disorder of the lymphatic system. These genetic disorders can result in malformation or dysfunction of the lymphatic system, which leads to an accumulation of fluid in the tissue and, thus to the formation of oedema. The most common form is peripheral lymphoedema of the lower limbs, but systemic manifestations such as intestinal lymphangiectasia, ascites, chylothorax or hydrops fetalis may also occur. The clinical presentation and the degree of lymphoedema varies depending on the causative gene and the specific gene alteration. Primary lymphoedema is divided into five categories: (1) disorders with somatic mosaicism and segmental growth abnormality, (2a) syndromal disorders, (2b) disorders with systemic involvement, (2c) congenital lymphoedema and (2d) disorders that occur after the first year of life (late onset lymphoedema). Targeted genetic diagnosis is based on the patient's clinical presentation and classification into one of the five categories. In general, the diagnosis usually starts with basic diagnostics, which include cytogenetic and molecular genetic testing. Subsequently, a molecular genetic diagnosis is made by performing single-gene analyses, gene panel examinations, exome sequencing or whole genome sequencing. This allows the identification of genetic variants or mutations that are considered to be causative for the presenting symptoms. Combined with human genetic counselling, the genetic diagnosis allows for conclusions about inheritance, the risk of recurrence and potential concomitant symptoms. In many cases, only this approach allows the definite form of primary lymphoedema to be described.

Ureteropelvic junction obstruction with primary lymphoedema associated with CELSR1 variants.

BACKGROUND: Primary lymphoedema (PL) is a chronic, debilitating disease caused by developmental and functional defects of the lymphatic system. It is marked by an accumulation of interstitial fluid, fat and tissue fibrosis. There is no cure. More than 50 genes and genetic loci have been linked to PL. We sought to study systematically cell polarity signalling protein Cadherin Epidermal Growth Factor Laminin G Seven-pass G-type Receptor 1 (CELSR1) variants linked to PL. METHODS: We investigated 742 index patients from our PL cohort using exome sequencing. RESULTS: We identified nine variants predicted to cause CELSR1 loss of function. Four of them were tested for nonsense-mediated mRNA decay, but none was observed. Most of the truncated CELSR1 proteins would lack the transmembrane domain, if produced. The affected individuals had puberty/late-onset PL on lower extremities. The variants had a statistically significant difference in penetrance between female patients (87%) and male patients (20%). Eight variant carriers had a kidney anomaly, mostly in the form of ureteropelvic junction obstruction, which has not been associated with CELSR1 before. CELSR1 is located in the 22q13.3 deletion locus of the Phelan-McDermid syndrome. As variable renal defects are often seen in patients with the Phelan-McDermid syndrome, CELSR1 may be the long-sought gene for the renal defects. CONCLUSION: PL associated with a renal anomaly suggests a CELSR1-related cause.

Redefining WILD syndrome: a primary lymphatic dysplasia with congenital multisegmental lymphoedema, cutaneous lymphovascular malformation, CD4 lymphopaenia and warts.

BACKGROUND: Primary lymphoedema (PL) syndromes are increasingly recognised as presentations of complex genetic disease, with at least 20 identified causative genes. Recognition of clinical patterns is key to diagnosis, research and therapeutics. The defining criteria for one such clinical syndrome, 'WILD syndrome' (Warts, Immunodeficiency, Lymphoedema and anogenital Dysplasia), have previously depended on a single case report. METHODS AND RESULTS: We present 21 patients (including the first described case) with similar clinical and immunological phenotypes. All had PL affecting multiple segments, with systemic involvement (intestinal lymphangiectasia/pleural or pericardial effusions) in 70% (n=14/20). Most (n=20, 95%) had a distinctive cutaneous lymphovascular malformation on the upper anterior chest wall. Some (n=10, 48%) also had hyperpigmented lesions resembling epidermal naevi (but probably lymphatic in origin). Warts were common (n=17, 81%) and often refractory. In contrast to the previous case report, anogenital dysplasia was uncommon-only found in two further cases (total n=3, 14%). Low CD4 counts and CD4:CD8 ratios typified the syndrome (17 of 19, 89%), but monocyte counts were universally normal, unlike GATA2 deficiency. CONCLUSION: WILD syndrome is a previously unrecognised, underdiagnosed generalised PL syndrome. Based on this case series, we redefine WILD as 'Warts, Immunodeficiency, andLymphatic Dysplasia' and suggest specific diagnostic criteria. The essential criterion is congenital multisegmental PL in a 'mosaic' distribution. The major diagnostic features are recurrent warts, cutaneous lymphovascular malformations, systemic involvement (lymphatic dysplasia), genital swelling and CD4 lymphopaenia with normal monocyte counts. The absence of family history suggests a sporadic condition, and the random distribution of swelling implicates mosaic postzygotic mutation as the cause.

The essential role of thoracic duct embolization in management of traumatic iatrogenic chylothorax / O papel fundamental da embolização do ducto torácico no manejo de quilotórax traumático iatrogênico

Abstract This study aims to describe a case series of patients who underwent thoracic duct embolization (TDE) to treat traumatic iatrogenic chylothorax (TIC). Three patients were included: Case #1, a 49-year-old woman with follicular lymphoma developed a TIC following video-assisted thoracoscopic surgery to resect a solid right paravertebral mass and was treated with TDE using microcoils and N-butyl cyanoacrylate (NBCA) glue. Case #2, a 68-year-old man with cardiac amyloidosis developed a TIC following heart transplantation and was treated with TDE using microcoils and ethylene vinyl alcohol copolymer. Case#3: A 6-year-old patient with congenital heart disease developed a TIC following a Fontan procedure and was treated with TDE using NBCA glue. All lesions were identified during lymphangiography and TDE was successfully performed in all cases. TDE is a safe and valuable technique that provides minimally invasive treatment for TCI.

Resumo Este estudo objetiva descrever uma série de casos de pacientes submetidos a embolização do ducto torácico (EDT) para tratamento de quilotórax iatrogênico (QI). Três pacientes foram incluídos. Caso 1: um homem de 49 anos com linfoma folicular apresentou QI após ressecção de uma massa paravertebral por toracoscopia vídeo-assistida e foi submetido a EDT com micromolas e n-butil-cianoacrilato (NBCA). Caso 2: um homem de 68 anos com amiloidose cardíaca apresentou QI após ser submetido a transplante cardíaco e foi submetido a EDT com micromolas e copolímero de etileno e álcool vinílico. Caso 3: um paciente de 6 anos com malformação cardíaca congênita apresentou QI após cirurgia de Fontan e foi submetido a EDT com NBCA. Todas as lesões foram identificadas durante a linfangiografia, e a EDT foi realizada com sucesso. A EDT é uma técnica segura e valiosa, que pode oferecer um tratamento minimamente invasivo em casos de QI.

Liver lymphatic anatomy and role in systemic lymphatic disease.

OBJECTIVES: To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. METHODS: In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed. RESULTS: During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics. CONCLUSION: We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal. KEY POINTS: • We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity.

Malformaciones linfáticas abdominales en una población pediátrica: experiencia en un centro de referencia de Medellín, Colombia / Abdominal lymphatic malformations in a pediatric population: Experience in a referral center in Medellín, Colombia

Introducción. Los linfangiomas son anormalidades benignas del sistema linfático, que corresponden a dilataciones quísticas de estos vasos y se localizan especialmente en el cuello. Solo el 10 % de todas estas malformaciones se encuentran en el abdomen y presentan síntomas variables de acuerdo al tamaño y su ubicación especifica, siendo el dolor abdominal el principal síntoma. Métodos. Se presentan cinco pacientes pediátricos con malformaciones linfáticas abdominales. Se describen su cuadro clínico, localización, tratamiento y la experiencia en el manejo de dicha patología en un hospital de referencia. Resultados. Los métodos más apropiados para hacer una aproximación diagnóstica son la ecografía, la tomografía computarizada y la resonancia nuclear magnética. Dentro de las opciones descritas para el tratamiento están la farmacológica, la escleroterapia y la resección quirúrgica, tanto por vía abierta como por laparoscopia. Conclusión. Existe una variedad de métodos para realizar la resección de los linfangiomas abdominales, pero la cirugía sigue siendo la más efectiva, especialmente cuando se cuenta con la laparoscopia como una herramienta terapéutica.

Introduction. Lymphangiomas are benign abnormalities of the lymphatic system, which correspond to cystic dilations of these vessels and are located especially in the neck. Only 10% of all these malformations are found in the abdomen and present variable symptoms according to size and their specific location, with abdominal pain being the main symptom. Methods. Five pediatric patients with abdominal lymphatic malformations are presented. Their clinical presentation, location, treatment and experience in the management of this pathology in a referral hospital are described. Results. The most appropriate methods to make a diagnostic approach are ultrasound, computed tomography and magnetic resonance imaging. Among the options described for treatment are pharmacological, sclerotherapy and surgical resection, both open and laparoscopic. Conclusion. There are a variety of methods for resecting abdominal lymphangiomas, but surgery remains the most effective, especially when laparoscopy is used as a therapeutic tool.

Manejo de neoplasias oncohematológicas en el contexto de la pandemia de COVID-19: recomendación de expertos / Management of oncohematological neoplasms in the COVID-19 pandemic: experts recommendation

RESUMEN La pandemia por COVID-19 originado por el Coronavirus 2 causante de síndrome respiratorio agudo severo (SARS-CoV-2) es causante de una crisis de salud pública a nivel global. Muchos reportes indican resultados desalentadores en pacientes con cáncer respecto a la población general. Por ello, los expertos en el manejo de neoplasias oncohematológicas del Instituto Nacional de Enfermedades Neoplásicas, hospitales nacionales y una clínica privada de Lima Metropolitana han desarrollado recomendaciones obtenidas por consenso para continuar con el manejo de pacientes con neoplasias oncohematológicas en forma segura ante la coyuntura de pandemia.

ABSTRACT The ongoing COVID-19 pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a global public health crisis. Many reports indicate disappointing results in cancer patients compared to the general population. Therefore, experts in the management of oncohematological malignancies from the National Institute of Neoplastic Diseases, national hospitals and a private clinic in Metropolitan Lima have developed recommendations obtained by consensus to continue with the management of patients with oncohematological neoplasms safely in the face of the pandemic.

Progressive Transformation of Germinal Centers in Axillary Lymph Nodes Mimicking Metastatic Lymphadenopathy after Breast Cancer Surgery: A Case Report.

Progressive transformation of germinal centers (PTGC) is a rarely diagnosed, benign disease of the lymph nodes that commonly manifests as chronic lymphadenopathy. PTGC may be characterized by single or multiple non-tender lymph nodes, and it commonly involves the cervical, axillary, and inguinal areas. Although PTGC is identified with concurrent lymphoma in some patients, it is not considered as a premalignant entity. Histopathologic diagnosis of PTGC is rarely made, and imaging findings have been reported in very few studies. We present a case of PTGC that occurred at the contralateral axillary lymph nodes and mimicked metastatic lymphadenopathy after breast cancer surgery. We also discuss its imaging findings.

Treatment and Impact of Cervical-Cancer-Related Lymphatic Disorders on Quality of Life and Sexuality Compared with Controls.

Background: Lymphatic disorders are frequent complications related to treatment for cervical cancer (CC). The aims of the study are to evaluate the impact of lymphatic disorders on quality of life (QOL) and sexuality in CC survivors after the completion of oncological treatment and to compare them with controls. Methods and Results: An ambispective cohort study was performed by using the Functional Assessment Cancer Therapy (FACT)-Cervix (Cx) fourth version, the World Health Organization Quality of Life-Brief Version (WHOQOL-BREF), and the Female Sexual Function Index (FSFI). Twelve patients affected by lymphatic disorders comprised the study group, 251 comprised the CC control group, and 185 comprised the non-CC control group. Regarding QOL, there were no statistically significant differences between the lymphatic disorder-unaffected and non-CC control groups, except in the WHOQOL-BREF environment domain. A weak positive correlation between lymphatic disorder and FACT-Cx additional concerns (σ = 0.135) was observed. Regarding sexuality, a weak negative correlation was detected between lymphatic disorders and FSFI sexual satisfaction (σ = -0.200) and a weak positive correlation was observed between lymphatic disorders and FSFI dyspareunia (σ = 0.148). We did not observe statistically significant differences in QOL satisfaction between the lymphatic disorder-affected and non-CC control groups. Symptomatic controls reported significantly higher physical health scores than the lymphatic disorder-affected group (p < 0.05). Regarding the psychological domain, the asymptomatic controls obtained significantly higher scores than the lymphatic disorder-affected group (p = 0.003). Conclusions: Lymphatic disorders notably influenced the QOL of CC survivors compared with the non-CC control groups. Lymphatic disorders had a significant negative impact on physical and psychological health. Sexuality was scarcely affected by lymphatic disorders.

Noncontrast MR Lymphography in Secondary Lower Limb Lymphedema.

BACKGROUND: Invasive imaging techniques have been applied for lymphedema (LE) assessment; noncontrast MR lymphography (NCMLR) has potential as an alternative, but its performance is not known in secondary lower limb LE. PURPOSE: To assess the role of NCMRL for the classification and characterization of secondary lower limb LE. STUDY TYPE: Retrospective. POPULATION: Fifty adults with clinically diagnosed secondary LE. FIELD STRENGTH/SEQUENCE: 1.5T, 3D T2 -weighted turbo spin-echo, 3D T2 -weighted turbo spin-echo short tau inversion recovery. ASSESSMENT: Three radiologists assessed the following characteristics on NCMRL: honeycomb pattern, dermal thickening, muscular abnormalities, distal dilated lymphatics, inguinal lymph node number, appearance of iliac lymphatic trunks. An LE grading based on the MR images was assigned. The relationship between imaging findings and clinical staging was evaluated, as well as between dermal backflow at lymphoscintigraphy and MR staging, and between the limb swelling duration and peripheral lymphatics dilatation. STATISTICAL TESTS: Pearson's correlation test and Cramer's V coefficient were computed to measure the strength of association. The Mann-Whitney test was used to compare the limb swelling duration between patients with and without dilated distal vessels. Agreement among raters was assessed through Kendall's W coefficient of correlation. RESULTS: Clinical stage and the MR grading were correlated, with Cramer's V coefficient of 1 for reader 1 (P < 0.05), 0.846 for reader 2 (P < 0.05), and 0.912 (P < 0.05) for reader 3; agreement between interraters was very good (W = 0.0.75; P = 0.05). A honeycomb pattern (P < 0.05), dermal thickening (P < 0.001), muscular abnormalities (P < 0.05), iliac lymphatic trunks appearance (P < 0.05), distal dilated vessels (P < 0.05), and lymph nodes number (P < 0.05) were significantly correlated with LE clinical stage. Dermal backflow at lymphoscintigraphy was described in 10 (20%) patients and showed a significant correlation with the MR grading (P < 0.05). DATA CONCLUSION: These preliminary results suggest that NCMRL may provide information useful for the staging and management of patients affected by secondary lower limb LE. Level of Evidence 4 Technical Efficacy Stage 2 J. MAGN. RESON. IMAGING 2021;53:458-466.

Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings.

Lymphangiectasias are lymphatic malformations characterized by the abnormal dilation and morphology of the lymphatic channels. The classification and treatment of these disorders can be challenging given the limited amount of literature available in children. Various imaging modalities are used to confirm suspected diagnosis, plan the most appropriate treatment, and estimate a prognosis. Prenatal evaluation is performed using both prenatal US imaging and fetal MRI. These modalities are paramount for appropriate parental counseling and planning of perinatal care. During the neonatal period, chest US imaging is a useful modality to evaluate pulmonary lymphangiectasia because other modalities such as conventional radiography and CT display nonspecific findings. Finally, the recent breakthroughs in lymphatic imaging with MRI have allowed us to better classify lymphatic disorders. Dynamic contrast-enhanced lymphangiography, conventional lymphangiography and percutaneous lymphatic procedures offer static and dynamic evaluation of the central conducting lymphatics in children, with excellent spatial resolution and the possibility to provide treatment. The purpose of this review is to discuss the normal and abnormal development of the fetal lymphatic system and how to best depict it by imaging during the prenatal and postnatal life.

Clinical features, microbiological epidemiology and recommendations for management of cellulitis in extremity lymphedema.

BACKGROUND: This high volume, single center study investigated the prevalence, bacterial epidemiology, and responsiveness to antibiotic therapy of cellulitis in extremity lymphedema. METHODS: From 2003 to 2018, cellulitis events from a cohort of 420 patients with extremity lymphedema were reviewed. Demographics, lymphedema grading, symptoms, inflammatory markers, cultures and antibiotic therapy regimens were compiled from cellulitis episodes data. Univariate and multivariate analyses were performed for detailed analysis. RESULTS: A total of 131 separate episodes of cellulitis were recorded from 43 (81.1%) lower limb and 10 (19.9%) upper limb lymphedema patients. The prevalence and recurrence rates for cellulitis in lymphedema patients were 12.6% (53 of 420) and 56.6% (30 of 53), respectively. The most common findings were increased limb circumference (127 of 131; 96.9%) and abnormal C-reactive protein (CRP) level (86 of 113; 76.1%). Blood cultures were obtained in 79 (60.3%) incidents, with 9 (11.4%) returning positive. Streptococcus agalactiae was the most isolated bacterium (5 of 9; 55.5%). CONCLUSIONS: The cellulitis prevalence and recurrence rate in extremity lymphedema were 12.6%, and 56.6%, respectively. Strongest indicators of cellulitis were increased affected limb circumference and elevated CRP level. Empiric antibiotic therapy began with coverage for Steptococcus species before broadening to anti-Methicillin-resistant Staphylococcus aureus and anti-Gram negatives if needed for effective treatment of extremity lymphedema cellulitis.

Investigation of a Peculiar Case of Childhood Lymphadenopathy.

Introduction: Lymphadenopathy is a common physical finding often associated with an infectious etiology. We present a case of a patient with generalized cervical and supraclavicular lymphadenopathy diagnosed with a rare malignancy. Health care providers should be aware of some uncommon historical or physical examination findings that warrant further investigation. Clinical Findings: A 16-year-old female presented to the emergency department with 3 days of a fever, congestion and sore throat with swelling around the jaw. Her physical examination findings included bilateral mandibular swelling and generalized cervical lymphadenopathy with palpable supraclavicular lymph nodes. Outcomes: Complete blood count showed pancytopenia with a white blood cell count of 4.4 × 10e3/uL, hemoglobin of 10.8 g/dL and platelets of 87 × 10e3/uL. CT scan of the neck with IV contrast revealed extensive cervical and mediastinal lymphadenopathy with suspicion of lymphoma. Biopsy results diagnosed a rare form of a hematologic malignancy called blastic plasmacytoid dendritic cell neoplasm. Conclusions: The presence of supraclavicular lymphadenopathy should raise concern for non-infectious etiologies, such as malignant processes.