Optimal Timing of Surgery of Hydronephrosis Due to Ureteropelvic Junction Obstruction in Neonates and Infants

PURPOSE: We review our experience with pyeloplasty for unilateral ureteropelvic junction obstruction of moderate to severe hydronephrosis observed by prenatal ultrasonography to assess the appropriate timing of operation for recovery of renal function and obstruction. METHODS: We retrospectively reviewed the records of the total 28 patients who underwent pyeloplasty between 1995 and 2001 at Asan Medical Center. We compared pre and postoperative differentials in renal function and diuretic renogram as measured by technetium-99m-mercaptoacetyl- triglycerine scan and the degree of hydronephrosis by renal ultrasonography. RESULTS: In all 28 patients postoperative follow-up renal ultrasonography revealed significant improvement in hydronephrosis. In 10 poorly functioning hydronephrotic kidneys in which relative renal function function was less than 35%, renal function was improved postoperatively in 3 cases, but not improved in 7 cases. In all 28 patients postoperative follow-up diuretic renogram revealed significant improvement. CONCLUSION: We believe that the early pyeloplasty should be considered when ultrasonography and diuretic renography suggest obstruction because renal function does not improve significantly after pyeloplasty over preoperative value.

Clinical Spectrum and Lung Pathology in Children with Interstitial Lung Disease

PURPOSE: Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. METHODS: Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings, lung pathology and clinical responses after steroid therapy and prognosis. Fifteen patients were performed open lung biopsy, but six patients were diagnosed bronchiolitis obliterans by HRCT. RESULTS: The median ages at onset of ILD was 1 year 5 month old. Common clinical findings were tachypnea(90.0%), hypoxemia(90.0%). 14 patients among 15 patients were diagnosed specifically after open lung biopsy. Interstitial pneumonitis was 6 cases, including 3 nonspecific interstitial pneumonitis, 2 cases of desquamative interstitial pneumonitis and one usual interstitial pneumonitis. Other diagnosis included idiopathic pulmonary fibrosis, cytomegalovirus pneumonitis, diffuse aspiration bronchiolitis, pulmonary lymphangiomatosis, BOOP(bronchiolotis obliterans organizing pneumonia) and pulmonary histiocytosis. Six patients showed clinical and HRCT findings consistent with bronchilitis obliterans. Common radiologic findings were mosaic perfusion(12/21), bronchial wall thickening(9/21). 13 patients among 15 patients with methylprednisolone pulse therapy showed improvement of clinical symptoms. The severity-of-illness score was improved significantly after methylprednisolone pulse therapy. CONCLUSION: Pediatric ILD includes heterogeneous disorders. Open lung biopsy was helpful to make early diagnosis. Severity-of-illness score is a noninvasive and useful measure of disease progression or response to therapy. Methylprednisolone pulse therapy may be effective to treat ILD.

A Case of Disseminated Lymphangiomatosis Involving Mediastinum, Bone, Spleen and Retroperitoneum in an Asymptomatic Healthy Child

Lymphangiomatosis, a benign tumor consisting of a cluster of dilated lymphatic channels, is very unusual. Most lymphangiomatoses are found in the neck and head area. Less than 5% are diagnosed intraabdominally and they are very infrequently encountered in the retroperitoneal area. Herein, we report a rare case of asymptomatic lymphangiomatosis of a 13 year-old boy during a routine chest radiologic exam at school who had disseminated lymphangiomatosis involving anterior mediastinum, multiple bone, spleen and retroperitoneum, which presented occasionally.