Hemoptysis: rare pulmonary manifestation of tuberous sclerosis in childhood
H. Pinto, M. J. Baptista, R. Anjos, J. Moreira, I. Ruivo, I. Azevedo (Porto, Lisboa, Portugal)
Source: Annual Congress 2006 - Sarcoidosis
Session: Sarcoidosis
Session type: Electronic Poster Discussion
Number: 3144
Disease area: Interstitial lung diseases
Abstract Pulmonary involvement in Tuberous Sclerosis is rare in children, with an estimated incidence of 1-2.3%. A review of the literature revealed only nine other case-reports of pulmonary manifestations in children, none with hemoptysis. The most frequent lesion is lymphangioleiomyoma which can present most commonly with pneumothorax or dyspnoea. A 2 y.o. girl was admitted to our hospital with gross hemoptysis for the last 24 hours. She had been diagnosed with tuberous sclerosis shortly after birth, mother and two siblings being also affected. Diagnosis was based on typical hypopigmented skin lesions, cardiac rhabdomyomas, epilepsy and psychomotor delay. Laboratory analysis yielded decreased hemoglobin and hematocrite levels requiring red blood cell transfusions. Posteroanterior chest radiograph exhibited hyperinflation of left lower lobe. Computed tomography of the chest demonstrated cystic lesions and bullous emphysema in the left lower lobe and bilateral peribronchovascular and interlobular thickening; a vascular conglomerate in the left lower lobe was also seen. Cardiac catheterization evidenced proeminent abnormal systemic-to-pulmonary collateral vessels which were treated during a second intervention by transcatheter coil embolization. She has been asymptomatic ever since. Hemoptysis are exceptional in tuberous sclerosis. There are few reported cases in the literature, all in women older than 25 y.o. The occurrence of hemoptysis in a child has not, to our knowledge, been reported before. Provided computed tomography suspicion of lymphangioleiomyoma is confirmed by lung biopsy, prognosis is poor, due to its progressive evolution, possible complications and precocious age of the child.
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H. Pinto, M. J. Baptista, R. Anjos, J. Moreira, I. Ruivo, I. Azevedo (Porto, Lisboa, Portugal). Hemoptysis: rare pulmonary manifestation of tuberous sclerosis in childhood. Eur Respir J 2006; 28: Suppl. 50, 3144
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