A 52-year-old woman was presented with 2-week history of increasing dyspnea and dry cough. The chest radiograph revealed bilateral reticular infiltrates. Radiographic infiltrates were rapidly progressed and symptoms from hypoxemia were aggravated. The patient was intubated and bronchoscopy with transbronchial lung biopsies was performed. Biopsies revealed lymphatic vessels plugged by nests of metastatic adenocarcinoma. She died 11 days after admission despite of intensive ventilatory support. We had difficulties in the diagnosis of lymphangitic lung carcinomatosis at initial presentation of her illness because the progression was unusually rapid. Lymphangitic lung carcinomatosis should be included in the differential diagnosis of patients showing rapidly progressive interstitial radiographic findings. Also, transbronchial lung biopsy may be a useful tool to confirm the diagnosis.
Adenocarcinoma ; Anoxia ; Biopsy ; Bronchoscopy ; Carcinoma* ; Cough ; Diagnosis ; Diagnosis, Differential ; Dyspnea ; Female ; Humans ; Lung* ; Lymphatic Vessels ; Middle Aged ; Radiography, Thoracic

Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.
Female ; Gestational Age ; Humans ; Infant, Newborn ; Lung/pathology ; Lung Diseases/*congenital/diagnosis/pathology/radiography ; Lymphangiectasis/*congenital/diagnosis/pathology/radiography ; Lymphatic Vessels/pathology ; Tomography, X-Ray Computed

OBJECTIVE: The aim of this prospective study was to determine whether the additional use of the single photon emission computed tomography/CT (SPECT/CT) technique improves the diagnostic value of planar lymphoscintigraphy in patients presenting with primary lymph edema of the lower limb. MATERIALS AND METHODS: For a defined period of three years (April 2011-April 2014) a total of 34 consecutive patients (28 females; age range, 27-83 years) presenting with swelling of the leg(s) suspicious of (uni- or bilateral, proximal or distal) primary lymphedema were prospectively examined by planar lymphoscintigraphy (lower limbs, n = 67) and the tomographic SPECT/CT technique (anatomical sides, n = 65). RESULTS: In comparison to pathological planar scintigraphic findings, the addition of SPECT/CT provided relevant additional information regarding the presence of dermal backflow (86%), the anatomical extent of lymphatic disorders (64%), the presence or absence of lymph nodes (46%), and the visualization of lymph vessels (4%). CONCLUSION: As an adjunct to planar lymphoscintigraphy, SPECT/CT specifies the anatomical correlation of lymphatic disorders and thus improves assessment of the extent of pathology due to the particular advantages of tomographic separation of overlapping sources. The interpretation of scintigraphic data benefits not only in baseline diagnosis, but also in physiotherapeutical and microsurgical treatments of primary lymphedema.
Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Lower Extremity/anatomy & histology/*radiography ; Lymphatic Vessels/radiography ; Lymphedema/*diagnosis/radiography ; Lymphoscintigraphy ; Middle Aged ; Radiopharmaceuticals/diagnostic use ; Retrospective Studies ; Tomography, Emission-Computed, Single-Photon ; Tomography, X-Ray Computed

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. We experienced a 32-year-old female who was admitted because of worsening exertional dyspnea after hormonal therapy with Clomifen for five months and intermittent hemoptysis. Chest PA showed diffuse ground glass appearance with some reticular infiltrations. High resolution CT scan showed multiple small thin walled cysts distributed homogeneously throughout the entire lung. Pulmonary function test showed characteristic obstructive pattern despite the restrictive interstitial pattern of chest radiography. Thoracoscopic lung biopsy specimen showed abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and pulmonary vessels typical of pulmonary lymphangioleiomyomatosis. Hormonal therapy with medroxyprogesterone was initiated.
Adult ; Biopsy ; Bronchioles ; Clomiphene* ; Dyspnea* ; Female ; Glass ; Hemoptysis ; Humans ; Lung ; Lymphangioleiomyomatosis ; Lymphatic Vessels ; Medroxyprogesterone ; Myocytes, Smooth Muscle ; Radiography ; Rare Diseases ; Respiratory Function Tests ; Thorax ; Tomography, X-Ray Computed