Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax.

We describe the outcome of a 20-month-old female and a 6-year-old male, both of whom had acutely developed severe respiratory distress with tachypnea, cyanosis and, in Patient 2, thoracic pain. Chest X-ray and CT scan showed interstitial pulmonary involvement and a bullous process with bilateral pneumothoraces for both children. Pulmonary biopsy confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Laboratory testing and skeletal radiography did not reveal any other involvement of LCH. The patients received chemotherapy (prednisone, vinblastine, 6-mercaptopurine). They had recurrent episodes of pneumothorax during follow-up and placement of chest tubes was the treatment chosen. They were asymptomatic, with regression of bullae and disappearance of pneumothorax at 58 and 63 months of follow-up, respectively. Pulmonary function tests done during follow-up were normal in both patients. Despite severe pulmonary involvement, conservative surgical treatment and moderate chemotherapy produced good results in these two rare cases.

Outcome in children with pulmonary Langerhans cell Histiocytosis.

BACKGROUND: The aim of this study was to evaluate features and outcome of children with Langerhans cell Histiocytosis (LCH) and pulmonary involvement. PROCEDURE: Retrospective evaluation of LCH patients was performed from 1987 to 2001. Multisystem patients were classified according to the pattern of organ system involvement into Groups: A (no pulmonary, hematologic, or hepatic involvement), B (pulmonary involvement), C (pulmonary and hematologic or hepatic involvement), and D (hematologic or hepatic involvement). All had skin, bone, or lymph node involvement. Chest X-ray was performed in all patients and computed tomography (CT) in 21. Diagnostic lung biopsy was performed in five patients. RESULTS: Pulmonary involvement was found in 36/220 patients studied. Two patients had isolated pulmonary involvement. Multisystem involvement was present in 83 patients, 34 of whom had pulmonary involvement. In 20/36 patients, tachypnea, cough, and thoracic pain occurred. Diffuse interstitial involvement was found in all cases. Pulmonary function tests were performed in nine patients, six of whom revealed mild to moderate restrictive respiratory involvement. The two patients having isolated pulmonary involvement survived 2 and 2.7 years after diagnosis. Median follow-up of all multisystem patients was 2.1 years, with a 5-year survival probability of 0.59. The 5-year survival probability of Groups A-D was 0.94, 0.83, 0.23, and 0.40, respectively. The survival difference between Groups B and C was statistically different (P < 0.0071). CONCLUSIONS: According to our data, pulmonary compromise without other risk organ involvement does not appear to be a negative prognostic factor in our study.

Hemosiderosis pulmonar idiopática en pediatría / Idiopathic pulmonary hemosiderosis: radiological findings in 13 patients

Propósito. Analizar los hallazgos por imágenes de la hemosiderosis pulmonar idiopática (HPI). Material y método. Evaluamos los hallazgos radiológicos y con tomografía computada de alta resolución (TCAR) en 13 pacientes con diagnóstico de HPI en los últimos 15 años. La edad promedio fue de 7 años (6 meses-17,5 años). La mitad de los pacientes fue menor de 7 años (mediana). Predominó el sexo femenino (8/13). El diagnóstico fue confirmado por biopsia pulmonar quirúrgica (6/13), lavado broncoalveolar (5/13), y lavado gástrico en 2/13 casos (92 por ciento). Analizamos los datos clínicos con los hallazgos obtenidos por el diagnóstico por imágenes (Rx y TCAR de tórax con bajo mAs). Resultados. La Rx de tórax reveló la presencia de opacidad pulmonar basal bilateral en 12/13 (92 por ciento) y nódulos algodonosos en ambos campos pulmonares en 2/13 casos (15 por ciento). La TCAR de tórax mostró patrón de vidrio esmerilado...

Tumores pancreáticos en pediatría / Primary pancreatic neoplasms in children

Los tumores pancreáticos son infrecuentes en niños. Comunicamos los hallazgos de diagnóstico por imágenes observados en una serie de 9 niños tratados durante el período 1989-2000 con diferentes tipos tumorales (insulinoma, pancreatoblastoma, sólido-quístico-papilar y adenocarcinoma). Utilizamos ultrasonido, tomografía computada, aangiografía y resonancia magnética, que permitieron mostrar la masa tumoral en todos los casos, excepto en uno de insulinoma. No pudo determinarse la localización pancreática en un pancreatoblastoma. Las imágenes fueron de fundamental importancia para la estadificación y un abordaje quirúrgico adecuado para esta rara patología pediátrica