Fibrosing mediastinitis causing rapidly progressive dyspnea, pulmonary edema and death in a 16 yr old male

Malagari, Katerina; Papiris, Spyros

doi:10.4081/monaldi.2004.711

Cited by 5 publications

(4 citation statements)

References 15 publications

(25 reference statements)

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“…It is thought to occur due to exposure to the Histoplasma capsulatum antigen within the mediastinum, triggering an intense inflammatory host response resulting in proliferation and invasion of fibrous tissue into vital structures [3] , [4] . One complication of the fibrotic reaction is pulmonary vein (PV) stenosis, previously described in case reports and series [1] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] . Diagnosis of PV stenosis is challenging due to its gradual onset of nonspecific symptoms including fatigue and dyspnea, and thus presentation is typically delayed.…”

Section: Introductionmentioning

confidence: 99%

Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

Ponamgi

DeSimone

Lenz

et al. 2015

IJC Heart & Vasculature

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IntroductionFibrosing mediastinitis (FM) is a rare but fatal disease characterized by an excessive fibrotic reaction in the mediastinum, which can lead to life-threatening stenosis of the pulmonary veins (PV). Catheter-based intervention is currently the only viable option for therapy. However, the current literature on how best to manage these difficult cases, especially in regards to sequential interventions and their potential complications is very limited.MethodsWe searched through a database of all patients who have undergone PV interventions at the Earl H. Wood Cardiac Catheterization Laboratory in Mayo Clinic, Rochester. From this collection, we selected patients that underwent PV intervention to relieve stenosis secondary to FM.ResultsEight patients were identified, with a mean age of 41 years (24–59 years). Five were men, and three were women. Three patients underwent balloon angioplasty alone, and five patients had stents placed. The majority of patients had acute hemodynamic and symptomatic improvement. More than one intervention was required in five patients, four patients had at least one episode of restenosis, and four patients died within four weeks of their first PV intervention.ConclusionsWe describe the largest reported case series of catheter-based intervention for PV stenosis in FM. Although catheter-based therapy improved hemodynamics, short-term vascular patency, and patient symptoms, the rate of life-threatening complications, restenosis, and mortality associated with these interventions was found to be high. Despite these associated risks, catheter-based intervention is the only palliative option available to improve quality of life in severely symptomatic patients with PV stenosis and FM. Patients with PV stenosis and FM (especially those with bilateral disease) have an overall poor prognosis in spite of undergoing these interventions due to the progressive and recalcitrant nature of the disease. This underscores the need for further innovative approaches to manage this disease.

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Section: Introductionmentioning

confidence: 99%

Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

Ponamgi

DeSimone

Lenz

et al. 2015

IJC Heart & Vasculature

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“…Pulmoner venöz oklüzyonlu hastalar sıklıkla "psödo-mitral stenoz sendromu" olarak adlandırılan hemoptizi ve progresif egzersiz dispnesi kliniği ile prezente olur (5,11). Uzun süren pulmoner venöz konjesyon, pulmoner arteryel hipertansiyon, kor pulmonale ve olguların çoğunda mortaliteye neden olur.…”

Section: Discussionunclassified

An Adolescent with Pulmonary Hypertension Secondary to Idiopathic Fibrosing Mediastinitis: Case Report

Onan¹,

Sezer²,

Baykan³

et al. 2012

Erciyes Med J

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ÖZETPulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Fibrosing mediastinitis is a rare disorder caused by proliferation of acellular collagen. In fibrosing mediastinitis, clinical presentation is determined by compression to the vital mediastinal organs. Fibrosing mediastinitis may rarely lead to pulmonary veno-occlusive disease. In this case report, a 16 year old boy with pulmonary arterial hypertension was presented. His right pulmonary artery was completely occluded secondary to idiopathic fibrosing mediastinitis. The patient died after mediastinal biopsy. Herein, we discussed pulmonary arterial hypertension secondary to idiopathic fibrosing mediastinitis in children.Key words: Child, mediastinitis, pulmonary artery, pulmonary hypertension GirişPulmoner hipertansiyon (PH); yeni tetkik ve tedavi seçeneklerine rağmen, halen tanı ve tedavisinde zorluklar olan ciddi bir hastalıktır. Çocuklarda en sık pulmoner hipertansiyon nedeni soldan sağa şantlı konjenital kalp hastalık-larıdır. Sekonder PH nedenleri arasında sıklıkla pulmoner hastalıklar yer alır. Pulmoner veno-okluziv hastalıklara bağlı PH çocuklarda oldukça nadirdir (1-3).Fibröz mediastinit (FM), mediastende asellüler kollajen ve fibröz doku proliferasyonuna neden olan, nadir görülen benin bir hastalıktır (4, 5). FM, enfeksiyonlara ve konnektif doku hastalıklarına sekonder mediastende aşırı fibrotik reaksiyon şeklinde gelişebileceği gibi, idiyopatik olarak da görülebilir (5, 6). İlk FM olgusu 1903'de Osler tarafın-dan yayınlanan olgudur (7). Tanımlanan bu ilk olguda da olduğu gibi FM'e eşlik eden vena cava superior sendromu (VCSS) tablosu sıklıkla bildirilmiştir (6, 8-10).Fibröz mediastinitin nadiren pulmoner arter obstruksiyonu yaptığı da rapor edilmiştir (4-6). Bununla birlikte, idiyopatik FM'e sekonder olarak pulmoner arterin tümüyle okluzyonu, bilebildiğimiz kadarı ile bildirilmemiştir Bu yazıda; çocukluk çağında FM nedeniyle sağ pulmoner arteri tümüyle oklüde olan ve mediastinal biyopsi sonrası kaybedilen olgu, PH etyolojisinde pulmoner arteryel stenoz veya oklüzyonunun nadiren yer alması nedeni ile sunulmuştur. Olgu SunumuOn üç yaşında iken akut göğüs ağrısı ve üst gastrointestinal sistem kanaması öyküsü veren; bir yıl sonra senkop nedeniyle hastanede yatan, on altı yaşındaki erkek, altı aydır devam eden kilo kaybı, efor sırasında yorulma, öksürme, nefes darlığı, halsizlik ve dudaklarda uyuşma şikayetleriyle başvurdu. İlk muayenesinde sistolik ve diyastolik arteriyal basınçları 110 ve 70 mmHg, nabız 100/dk olarak ölçüldü. Kalp atımı ritmik, S2 çift ve sert, triküspid odakta 2/6'lık pansistolik üfürüm saptandı.Arka servikal bölgede 2x1 cm boyutlarında mobil, ağrısız lenfadenopatiler mevcuttu. Toraks muayenesi doğaldı. Arteryel oksijen satürasyonu %95, hemoglobin 15,4 g/dL, trombosit 94000/mm 3 , kreatinin 1,3 mg/dL, total protein 5g/dL, albümin 3,1g/dL idi. Eritrosit sedimantasyon hızı, C-reaktif protein, karaciğer enzimleri, tiroid fonks...

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“…Although FM is strictly a benign entity, the progressive infiltration of major vascular, respiratory, and nervous structures within the mediastinum can cause substantial morbidity and potentially lethal complications [3,4]. FM has both focal (usually granulomatous) and diffuse (usually nongranulomatous) forms, the former form being the more common in the United States and the more published in the scientific literature [5].…”

Section: Introductionmentioning

confidence: 99%

Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease

et al. 2015

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Fibrosing mediastinitis causing rapidly progressive dyspnea, pulmonary edema and death in a 16 yr old male

Cited by 5 publications

References 15 publications

Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

An Adolescent with Pulmonary Hypertension Secondary to Idiopathic Fibrosing Mediastinitis: Case Report

Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease

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