[Congenital pulmonary malformations: clinical, radiological and treatment features]

Congenital broncho-pulmonary malformations [CBM] are rare, essentially presented by congenital lobar emphysema, bronchogenic cysts, pulmonary sequestrations and cystic adenomatoid malformations. The diagnosis can be in prenatal. In postnatal, symptoms are variable. Radiological investigations lead to diagnosis in all cases. To study the principal clinic, radiologic and therapeutic of the congenital broncho-pulmonary malformations through ten cases. Retrospective study of 10 cases of congenital bronchopulmonary malformations diagnosed between 2003 and 2010 in our institution. The mean ages at the time of diagnosis is 2months [4 days to 16months]. The sex ratio is 1. The symptoms consisted of recurrent pneumonia in 4cases, respiratory distress in 2cases, bronchiolite in 2 cases and 2cases of antenatal diagnosis. All patients have a chest X-ray, night patients have a chest computerized tomography and one patient has a bronchial endoscopy. Ten cases of BPM have been investigated: five congenitals lobar emphysema, tow pulmonary sequestrations, tow cystic ad‚nomatoid malformation and one bronchogenic cyst. Eight patients required surgical treatment involving pneumonectomy [1case], lobectomy [5 cases], segmentectomy [1 case] and in 1 case the pulmonary sequestration was treated by ligature of the anomalous artery with pulmonary resection. The histopathological examination confirmed the diagnosis in all cases. The postoperative period was uneventful in 8 cases with a mean of follow-up of 2 years [5 months to 5years]. Tow patient died after surgical treatment. The diagnosis of BPM malformations can be clinical, confirmed by radiological investigations. The improvement in prenatal ultrasound diagnosis modified the management strategy. The treatment varies frome attitude conservatrice to pneumonectomy

[Primary psoas abscesses of the psoas muscle in children]

Primary psoas abscesses of the psoas muscle are relatively rare in childhood and can determine problems of diagnosis and therapeutic assumption. To demonstrate that ultrasonography is an excellent means in diagnosis and treatment of psoas abscess in children limiting the use of CT more costly and radiant and the surgery that is decaying. Over a 15 years period [January 1995-december 2009], 16 children with psoas abscess were studied retrospectively. The diagnosis gait was based on questioning, clinical examination and imaging techniques and, in first sight, ultrasonography. Median age of our patients was 6.4 years [extremes 18 months-14 years] and mean delay of evolution was 12 days. Fever and lumbar pain were both constants, associated with another signs like painful boitery, psoitis, mictional burns and lumbar mass. The ultrasonography made in 16 patients allowed to the diagnosis in all cases. The tomodensitometry, made in 3 patients, confirmed the ultrasonography. Echo guided percutaneous drainage, made in 14 cases, was successful in 12. The examination of pus showed S Aureus in 11 cases and E coli in the other. Ultrasonography is a very important means in the primary psoas abscess in children. In addition to give diagnosis, this technique allows the drainage and the cure of the collection, limiting the open surgery to some particular cases

[Posterior uretral valves, about a series of 44 cases]

To explore the physical symptoms and radiological findings for the diagnosis of posterior urethral valves [PUV], to point of the therapeutic snags depending on gravity and delay of lesions. We reviewed the records of 44 patients with PUV. Mean age: 2 years 1/2 [range 1 day-13 years]. More than 75% of patients were less than 2 years old. In utero diagnosis was made in 8 patients [14%]. After birth, the diagnosis was based on urological signs [as frequent as diagnosis is made belatedly] and extra urological signs. Cystourethrography [CUG] showed posterior urethral dilation in all cases. Ultrasonography [US] showed abnormalities in 30 cases and intravenous urography [IVU], made in 21 cases, was normal in appearance in 7 of them. At diagnosis, it was a renal failure in 36 patients and 8 of them had a terminal renal deficiency. Urethroscopy made in 40 patients, showed PUV in all cases. There were 29 types I valves, 3 type II valves, 5 type III and 3 unclassifiables cases. Treatment was endoscopic in 41 cases and by lamination in the 3 others. Mean follow up is 9 years [range16months-19 years] and 19 patients have terminal renal failure from which 3 are deceased. PUV are dangerous obstructive uropathies in boy whom end at terminal renal failure in more than 33% of cases. Precocious diagnosis and early ablation of PUV are able to limit the complications inherent to this pathology

[Uropathies and nephropathies in the Bardet-Biedl in child]

The Bardet-Biedl syndrome is an autosomal recessive disease, characterized by obesity, retinal degeneration, hypegenitalism in men, polydactylism and on often moderate mental retardation. With these cardinal features, others clinical findings [secondary features] including diabetes, congenital heart defects, hypertension or syndactyly can be seen. Renal involvement is almost constant, but varies from a moderate impairment of the tubular functions to chronic renal failure caused by malformative uropathy or glomerulopathy. Report a new cases. We report 6 patients with Bardet-Biedel syndrome who had renal involvement. Three patients had cystic dysplasia, one patient an increased fractional sodium excretion, one other a vesico-ureteral reflux and the last patient developed end-stage renal failure following acute post streptococcal glomerulonephritis. We insist on precocious diagnosis and multidisciplinary treatment of these renal lesions, to avoid or, at least, to slow down the evolution to the terminal renal failure, essential prognosis factor. Renal involvement, is considered as a major criteria predicting high morbidity and mortality during Bardet-Diedl disease

[Urinary tract infections in children following renal transplantation: a single-center experience]

Urinary tract infection [UTI] is the most common complication after kidney transplantation and represents a potential life-threatening risk for the immuocompromised child. The aim of this report is to determinate incidence, risk factors, microbilogic features and evaluate the impact of this complication on graft outcome and patient mortality. We performed a retrospective cohort study reviewing the medical records of 17 children from 38 who received a renal transplant in our center between January 1992 and June 2008 and who present an urinary tract infection. All patients received Lich-Gregoire implantation and insertion of double-J stunt. Antibioprophylaxis was not systematic. After a mean period of 6 years, 9 children [5 boys+4 girl] developed early UTI [during the first month after transplantation] and 5 [3boys + 2 girl] had late UTI. Three patients [2boys+1girl] with an indeterminate nephropathy developed early and late UTI. Causal agents are: E.Coli, Klebsiella Pneumoniae and Candida albicans. The further voiding cystourethrography showed a vesico-ureteral reflux on graft in 5 cases. Among the 17 patients, 4 lost their graft and are actually on haemodialysis. The urinary tract infection represents the major complication after renal transplantation. Diagnosis ant treatment must be made early to avoid the loss of the graft

Histiocytome fibreux angiomatoide: Une tumeur rare a malignite intermediaire

Angiomatoid fibrous histiocytoma is a rare humour affecting young adults. Unlike conventional malignant fibrous histiocytoma, it's extension is only local thus giving a good prognosis. We report the cases of a 9 years-old girl and a 1 6 years-old boy presenting respectively, with an axiliary tumour 5cm of diameter and a paravertebral subcutaneous tumour 1,5cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection