Nonmalignant Adult Thoracic Lymphatic Disorders

Itkin, Maxim; McCormack, Francis X.

doi:10.1016/j.ccm.2016.04.004

Cited by 31 publications

(18 citation statements)

References 82 publications

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“…Chylothorax can be seen in approximately 20-30% of LAM patients (Moua et al, 2012;Ryu et al, 2006). While the exact prevalence of chylous congestion in LAM is not well established, 7% of LAM patients were reported to have chyloptysis in a national LAM Registry (Itkin & McCormack, 2016).…”

Section: Lymphatic Manifestationsmentioning

confidence: 99%

“…In severe cases that do not respond to treatment with mTOR inhibition, further management options include pleurodesis, thoracic duct embolization, and thoracic duct ligation. In these situations, an attempt to study the underlying lymphatic anatomy by using dynamic contrast enhanced magnetic resonance lymphangiogram or intranodal lymphangiogram can be helpful in identifying the source of the chyle leak and direct further interventions such as thoracic duct embolization or ligation (Itkin & McCormack, 2016). …”

Section: Introductionmentioning

confidence: 99%

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Pulmonary manifestations in tuberous sclerosis complex

Gupta

Henske

2018

American J of Med Genetics Pt C

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Tuberous sclerosis complex has manifestations in many organ systems, including brain, heart, kidney, skin, and lung. The primary manifestations in the lung are lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). LAM affects almost exclusively women, and causes cystic lung destruction, pneumothorax, and chylous pleural effusions. LAM can lead to dyspnea, oxygen dependence, and respiratory failure, with more rapid disease progression during the premenopausal years. In contrast, MMPH affects men and women equally, causing small nodular pulmonary deposits of type II pneumocytes that rarely progress to symptomatic disease. Here, we review the clinical features and pathogenesis of LAM and MMPH.

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Section: Lymphatic Manifestationsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pulmonary manifestations in tuberous sclerosis complex

Gupta

Henske

2018

American J of Med Genetics Pt C

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“… 2 Chylous effusions and chylothorax, consequent to the abnormal proliferation of lymphatic vessels may induce respiratory failure with a high mortality risk. 4 5 6 Extrapulmonary alterations may include chylous ascites, lymphopenia, and destructing bone disease for overgrowth of lymphatic vessels. 4 5 6 7 The pathogenesis of the disease is unclear and the prognosis is related to disease localizations.…”

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confidence: 99%

Gorham-Stout Disease Management during Pregnancy

Bargagli

Piccioli

Cavigli

et al. 2017

AJP Rep

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Gorham-Stout Disease (GSD) is a rare lymphatic disorder affecting children or young adults with no predilection of sex. It is generally associated with vanishing bone osteolytic lesions, thoracic and abdominal involvement, and diffuse pulmonary lymphangiomatosis. Chylous effusions and chylothorax, consequent to the abnormal proliferation of lymphatic vessels, may induce respiratory failure with a high mortality risk. Extrapulmonary alterations may include chylous ascites, lymphopenia, and destructing bone disease for overgrowth of lymphatic vessels. Here, we report the case of a young woman who developed a severe and recalcitrant GSD with persistent unilateral chylothorax during pregnancy. The complex management of this patient during and after pregnancy was discussed and compared with literature data to contribute to the definition of a correct diagnostic and therapeutic approach to this rare lymphatic disease.

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“…Accurate histological evaluation is essential for the diagnosis of any vascular anomaly. Sometimes the clinical picture, with a soft‐tissue mass, bone lytic lesions, and effusions, may be more suggestive of a malignant process than an LM . Therefore, it is important to keep CLAs in the differential diagnosis and to use appropriate antibodies for immunostaining to prove or rule out a CLA (Figure ).…”

Section: Recommendationsmentioning

confidence: 99%

Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies—expert opinion consensus

Iacobas

Adams

Pimpalwar

et al. 2019

Pediatric Blood & Cancer

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Objective Complicated lymphatic anomalies (CLAs) are chronic, progressive, and debilitating conditions that share clinical features, yet key elements for optimal evaluation and management have not been established. We aimed to formulate expert opinion consensus‐based guidelines for comprehensive evaluation of CLAs. Study Design Patient support groups dedicated to CLAs organized an international conference for vascular anomaly experts from 16 specialties to address the objective. Participants received a set of questions before the meeting and reviewed the literature. Data extracted from international lymphatic anomaly registries were presented and the group separated for panel discussions during the conference. The recommendations achieving consensus within the panel were presented to the entire audience. Open debate occurred until majority approval was achieved. Results The expert group was composed of 52 physicians who defined the clinical elements required to evaluate and diagnose a CLA. The radiology panel established the preferred anatomical and functional imaging methods for diagnosis and the elements required to be described during interpretation. Two medical panels compiled the metabolic and hematologic tests at diagnosis and also recommended functional studies. The surgical group recommended precautions for biopsy and the pathology panel provided biopsy specimen processing guidelines. Conclusions Patients with CLAs require a comprehensive and targeted diagnostic plan for appropriate management, prevention of complications, and conservation of resources. As this population is managed by diverse medical and surgical specialties, we offer an expert multidisciplinary consensus‐based opinion on the current literature and on data extracted from international lymphatic anomaly registries.

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Nonmalignant Adult Thoracic Lymphatic Disorders

Cited by 31 publications

References 82 publications

Pulmonary manifestations in tuberous sclerosis complex

Pulmonary manifestations in tuberous sclerosis complex

Gorham-Stout Disease Management during Pregnancy

Multidisciplinary guidelines for initial evaluation of complicated lymphatic anomalies—expert opinion consensus

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