Primary Wilms tumor of the urinary bladder

ABSTRACT Wilms tumor (WT) can occur at various extrarenal sites; however, the urinary bladder as the primary site is occasional. A 4-year-old-female child presented with difficulty in micturition for the past month. The contrast-enhanced magnetic resonance imaging with magnetic resonance (MR) urography revealed a polypoidal, heterogeneous mass in the urinary bladder with no abnormality in the kidneys. Cystoscopy-guided biopsy was reported as an extrarenal Wilms tumor (ERWT) with triphasic components. Post-chemotherapy, a computed tomography scan revealed a residual tumor for which she underwent partial cystectomy. The diagnosis of ERWT was confirmed. She received adjuvant chemotherapy and remained well at the 9th month post completion of chemotherapy. The primary bladder WT must be considered in the differential of a small blue round cell tumor at an extrarenal site in the pediatric age group. The diagnosis is especially challenging in small biopsy material, although it has immense significance in management and prognosis.

An Infant with Interstitial Lung Disease

Interstitial lung disease in infants, unlike older children and adults, has diverse etiology, including infective, metabolic, autoimmune,genetic, malignant and idiopathic causes. Clinical recognition of the interstitial pattern of lung involvement is important as the etiology andmanagement is entirely different from that of recurrent or chronic lung parenchymal pathologies. We discuss the clinical and pathologicalfindings of an infant with interstitial pneumonia, who succumbed to hospital-acquired sepsis.

Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major

Objective: To evaluate the efficacy of prolonged deferiprone monotherapy in patients with ?-thalassemia major. Methods: This cross-sectional study included 40 patients (age range 9 to38 years) with thalassemia major receiving deferiprone for ?5 years. Serum ferritin, andmyocardial iron concentration (MIC) and liver iron concentration (LIC) assessed by T2*MRIwere recorded. Results: The patients were receiving deferiprone for a mean (SD) duration of12.1 (4.7) years. The median (IQR) dose of deferiprone was 85 (74.3, 95) mg/kg/day. TheMIC was normal or had a mild, moderate or severe elevation in 29 (72.5%), 3 (7.5%), 3(7.5%), and 5 (12.5%) patients. The LIC was normal or had a mild, moderate or severeelevation in 2 (5%), 4 (10%), 11 (27.5%) and 23 (57.5%) patients. Conclusions: The majorityof patients receiving deferiprone had a moderate/severe hepatic but normal cardiac iron load.Prolonged deferiprone monotherapy was suboptimal for hepatic iron load in the majority.

Inflammatory pseudotumor of the omentum.

Inflammatory pseudotumor is an unusual quasineoplastic lesion, of uncertain natural history and etiopathogenesis, which consists of inflammatory cells and myofibroblastic spindle cells; and is usually found in children and young adults. Clinical manifestations and radiological feature of this rare entity, can be indistinguishable from a malignant sarcomatous or lymphoproliferative disorders. Here, a case of surgically proven inflammatory pseudotumor of the omentum in a child and its review of literature is presented. Conservative surgery is curative in most cases of inflammatory pseudotumors. Awareness of this lesion is essential for clinicians, radiologists and pathologists, as pre-operative recognition may spare the child from unnecessary radical surgery or harmful chemoradiotherapy.

Computed tomographic pulmonary angiography: Utility in acute pulmonary embolism in providing additional information and making alternative clinical diagnosis.

Objective: To evaluate the role of computed tomographic (CT) pulmonary angiography (CT-PA) in detecting additional information that may help in making an alternative clinical diagnosis in patients referred to CT for a suspected acute pulmonary embolism (PE). Materials and Methods: 50 patients (34 males, 16 females) in the age group of 18-72 years (mean 42.3 years), having high clinical suspicion of PE, underwent CTPA over a 2 year period. Chest x-ray, arterial blood gas (ABG) analysis, echocardiography were done in all patients. All patients underwent at least one other imaging examination besides CTPA: ventilation perfusion scan, Doppler ultrasound or compression ultrasound (for DVT). All patients were followed for 3 months after completion of the diagnostic work up at baseline. The final diagnosis was achieved by a combination of clinical, imaging, and laboratory analysis, after adequate imaging, laboratory tests, and follow up. Result: CTPA helped correctly identify 29 of 30 patients with PE. In the remaining 20 patients (with no evidence of PE), CT-PA provided additional information (that suggested or confirmed alternate clinical diagnosis) in 15 patients (75%): pleural effusion (n=8), mediastinal or hilar lymphadenopathy (6), pneumonia/airspace consolidation (5), atelectasis/collapse (2), aspergilloma (1), malignancy (1), and others (2). Conclusion: CT-PA is highly specific and sensitive for diagnosis of PE. In addition, in a majority of patients who do not have PE, it also provides important ancillary additional information and helps in making an alternative clinical diagnosis.

CT virtual bronchoscopy: new non invasive tool in pediatric patients with foreign body aspiration.

A 2-year-old girl presented with recurrent cough, wheese and breathing difficulty. Her imaging (CT and virtual bronchoscopy) revealed a foreign body in tracheobronchial tree, that was removed by rigid bronchoscopy.

Clinical and ultrasound assessment of the knee in children with juvenile rheumatoid arthritis.

OBJECTIVE: To correlate clinical features with ultrasound (USG) findings in the detection, quantification and follow up of inflammatory signs of knee in children with mono or pauciarticular juvenile rheumatoid arthritis (JRA). METHODS: Thirty patients (11 girls, 19 boys) with pauciarticular JRA (14 with monoarticular and 16 with bilateral knee involvement) were studied. Mean disease duration was 10 months (range 2 months to 5 yr). All knees were classified into two groups, according to the presence or absence of acute inflammation. Clinical assessment and ultrasound was done in all patients on the same day. All the patients received naproxen (15-20 mg/Kg/day) for a period of six months, after which clinical assessment and ultrasound study was repeated. RESULTS: Synovial proliferation and effusion, was demonstrated in a much higher frequency in those clinically active (Group A) as compared to these in clinical remission (Group B). Statistically significant differences between clinical and USG indices were seen. CONCLUSION: USG of knee is more sensitive than clinical assessment in detection of synovial effusion and thickening and plays a useful role in monitoring evolution of the inflammatory process, its quantification and for follow up.

Congenital intrahepatic porto-systemic venous shunt with galactosemia.

We report a 51-day-old infant with congenital intrahepatic porto-systemic venous shunt associated with galactosemia, who presented with cholestatic jaundice. He was treated with ursodeoxycholic acid, calcium supplements and galactose-free diet. The child was asymptomatic six weeks later.