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Epidermolysis bullosa (EB) is a rare disorder that presents with urological complications. We present a 6-year-old boy admitted with urological symptoms that revealed an inherited EB misdiagnosed. We also review the literature on this disorder and management of the common urological complications.

Urethra duplication is a rare anomaly with numerous anatomic variations. These fall into four main groups: epispadiac, hypospadiac, fusiform, and Y-type. We report on one case of sagittal incomplete hypospadiac anterior duplication of the urethra. Surgical repair consisted of incision of the septum between the two urethras and hypospadias repair at the same setting. Follow-up was characterized by the occurrence of some complications. [Copyright &y& Elsevier]

Severe sustained hypertension occurs in only 0.1% of the pediatric population and only about 2% of these patients will have an underlying endocrine cause. Pheochromocytoma as a catecholamine secreting tumour causing severe hypertension is exceedingly rare in children. A high index of suspicion and an awareness of the clinical spectrum are therefore necessary to make the diagnosis. We report two cases of pheochromocytoma occurring in two a 12 year-old boys who''s presented with sustained hypertension, headache, sweating, and visual blurring. Measurement of 24 hour urinary catecholamines showed a marked increase and localization of adrenal unilateral pheochromocytoma was determined by ultrasonography and computed tomography. The approaches to the adrenal gland were transverse transperitoneal and the excision of the tumors was followed of return to the normal of tensional numbers. [Copyright &y& Elsevier]

Objective: The objective of this study was to evaluate the efficacy of extracorporeal lithotripsy for the treatment of urinary stones in children., Material and Methods: From November 2002 to November 2004, 34 children from the department of paediatric urology of Rabat children's hospital, aged 3 to 15 years (mean age: 6 years), including 15 children under the age of 6 years, were treated for symptomatic urinary stones in the extracorporeal lithotripsy centre with a Lithostar Multiline lithotriptor adapted to the treatment of adults as well as children, even very young children. At the time of treatment, all 34 children had normal blood pressure, normal blood urea and creatinine, normal clotting parameters, sterile urine and no urinary tract obstruction. All children under the age of 6 years were treated under ketamine sedation., Results: Treatment required a variable number of sessions from 1 to 3 (mean: 1.5) and concerned 38 stones: 30 renal stones including 6 staghorn and 8 ureteric stones. The number of impacts delivered per session ranged from 1,500 to 3,500 for renal stones (mean: 2,500 impacts), with a maximum of 5,000 impacts for ureteric stones (mean: 3,250 impacts). Three months after the last lithotripsy session, 30 patients presented no fragments. No lesion of the treated kidney or adjacent organs was demonstrated on follow-up ultrasound performed 3 to 6 months after treatment., Conclusion: Evaluation of the results confirms the efficacy of extracorporeal lithotripsy for the treatment of even very large urinary stones in children, even in very young children. Extracorporeal lithotripsy is now the first-line treatment for-urinary stones in children.

Duplicate bladder exstrophy is an extremely rare congenital malformation. Two forms have been described, the anteroposterior form and the collateral form, which is the less common. We report a case of bladder exstrophy in association with a duplicated lower urinary tract, omphalocele, colic duplication and diphallia. Fewer than 30 cases of duplicate bladder exstrophy have been described previously, emphasizing the embryologic significance of this case. To our knowledge it is the first reported case of such an association of anomalies with the exstrophy-epispadias complex.

We report the case of an unusual genitourinary anomaly in a 5-year-old patient. The malformation consisted of a para-anal persistent urogenital sinus in a 46XX patient associated with a scrotum-like structure in the presumed clitoral location. A duplicate urethra arising from the anterior aspect of the bladder neck drained into the scrotal pouch. Two hemivaginas drained into the urogenital sinus. The patient was totally continent through the urogenital sinus but the duplicate urethra was incontinent. No hormonal problems were encountered. The girl underwent total urogenital sinus mobilization, scrotal pouch ablation, and duplicate urethra resection.

Objective: The authors report their experience of xanthogranulomatous pyelonephritis in children and emphasize the diagnostic and therapeutic difficulties supported by several published series., Materials and Methods: This was a retrospective based on ten patients treated for xanthogranulomatous pyelonephritis., Results: Treatment was surgical in 90% of patients. Total nephrectomy was performed in all diffuse forms (80%) and one of the 2 localized forms (10%). Treatment was exclusively medical in only one patient (10%). Five subcapsular nephrectomies, three total nephrectomies and one radical total nephrectomy were performed. The course after treatment was excellent in 100% of cases., Conclusion: The treatment of xanthogranulomatous pyelonephritis is surgical, although cure can sometimes be achieved by conservative treatment.

Multilocular cystic nephroma (MCN) is a relatively rare, benign tumor of uncertain etiology. It typically arises from the renal parenchyma and is often treated with nephrectomy. We report a case of MCN with unusual prominent renal pelvis extension. A left nephrectomy was performed. A multilocular cystic lesion was found in the removed specimen, which had a direct tumor extension into the renal pelvis. There were no signs of malignancy. The tumor extension into the renal pelvis has been found in very few reports. This growth pattern might be one of the characteristics of this benign renal tumor.

We report the case of a 3 year old boy with a combination of anorectal malformation, sacral agenesia and anterior meningocele (Currarino's triad) and provide a review of the literature of this rare syndrome.

Peritoneal hydatidosis is a rare localisation of hydatic disease, most often secondary to a hydatic cyst of the liver. Its primitive form is considered due to a haematogenous diffusion through arterial vessels. We report a historical case of peritoneal hydatidosis concomitant to a hepatic localisation, in a 13-year-old girl. Diagnosis was performed by ultrasounds and CT scan, and a strong positive serology. Surgical cure has been completed by pharmacologic treatment with the aim of avoiding a relapse. A brief reminder of the therapeutic management of the disease is provided.

The study focuses on 70 vesicals lithiasis, with a sample consisting of 60 boys (86%) and ten girls (14%). The age varies from one year to 14 years. The revealing signs are dominated by mictional troubles in 47 cases (67%), macroscopic hematurie in 26 cases (37%), the pyurie in 12 cases (17%) and acute retention of urine in 7 cases (10%). The most frequent germs are the staphylococcus aureus with 28% of cases, the protéus mirabilis with 24% of cases and the Echerichia coli with 24% of the cases. Associated malformations were observed in 9% of the cases. The treatment was essentially surgical (99%). The evolution was simple in 97% of the cases.

Nephrectomy in childhood is an ultimate indication. It is realised in case of severe deterioration of the renal function or neoplastic affection of the kidney. We report a retrospective study on a series of 80 patients. Mean age was 5 years with extremes from 2 months to 14 years. Abdominal mass, pain, infection and haematuria were the mostly encountered revealing symptoms. The main aetiology was nephroblastoma. No laparoscopic nephrectomy was realised. Follow-up was uneventful except for the troubles existing prior to surgery.

Giant cell fibroblastoma (GCF) is a rare neoplasm occurring mostly in children. It has a tendency to recur locally after treatment. We report a new case noticed in an five year-old child who developed recurrence a first time three years later with the picture of GCF and at a second time with the picture of dermatofibrosarcoma protuberans 17 months later.

Unlabelled: Digestive duplications are a rare condition in children, characterized by an important anatomoclinical polymorphism. Diagnosis suspected on clinics and evoked by radiology, require histological confirmation., Patients and Methods: Nineteen children with duplications were managed in our department, from 1989 to 2001: their hospital chart was retrospectively studied., Results: Ten of our patients were less than one year of age, ten were boys and nine were girls. Clinical signs consisted of pain and abdominal mass, with some transit disturbs, and these signs led to ultrasonography and tomodensitometry. In one case alimentary tract duplication was revealed by digestive hemorrhage, leading to scintigraphic study. The digestive duplications were localized on the stomach in one case, the duodenum in two cases, the jejuno-ileum in twelve cases, the colon in two cases, and the rectum in two cases. Five digestive duplications were tubular, with one communicating duplication. All cases benefited from surgical treatment, and resection procedure was chosen according to duplication type and site. Histological study showed eight cases of ectopic mucosa, six ectopic gastric mucosa and two pancreatic ectopic mucosa., Conclusion: Diagnosis and surgical precocious treatment of digestive duplications are the only way to warn complications of this benign pathology.

Urethra duplication is a rare anomaly with numerous anatomic variations. These fall into four main groups: epispadiac, hypospadiac, fusiform, and Y-type. We report on one case of sagittal incomplete hypospadiac anterior duplication of the urethra. Surgical repair consisted of incision of the septum between the two urethras and hypospadias repair at the same setting. Follow-up was characterized by the occurrence of some complications.

Severe sustained hypertension occurs in only 0.1% of the pediatric population and only about 2% of these patients will have an underlying endocrine cause. Pheochromocytoma as a catecholamine secreting tumour causing severe hypertension is exceedingly rare in children. A high index of suspicion and an awareness of the clinical spectrum are therefore necessary to make the diagnosis. We report two cases of pheochromocytoma occurring in two a 12 year-old boys who's presented with sustained hypertension, headache, sweating, and visual blurring. Measurement of 24 hour urinary catecholamines showed a marked increase and localization of adrenal unilateral pheochromocytoma was determined by ultrasonography and computed tomography. The approaches to the adrenal gland were transverse transperitoneal and the excision of the tumors was followed of return to the normal of tensional numbers.

Hydatid cysts rarely present as a cervical tumor. We report a case of hydatid cyst of the neck in a child with multiple hydatid cysts in the liver, which was revealed by laryngeal dyspnea. Symptoms occurred in a six year-old country dweller, who presented with progressive laryngeal dyspnea. The chest radiographs showed extrinsic tracheal compression. A cervical ultrasonography showed a liquid filled mass evocative of hydatid cyst type 1. Explorative cervicotomy confirmed the diagnosis. The treatment was conservative after destruction of the parasite using a hypertonic saline solution. The immediate result was the disappearance of the laryngeal dyspnea.

Pleuropulmonary blastoma is an uncommon malignant lung tumor observed in children. Outcome is often unfavorable. Two boys and two girls, mean age 4.5 years, were admitted for nonspecific respiratory signs. Oriented by radiology findings, the diagnosis of pleuropulmonary blastoma was confirmed at pathology examination of a pneumonectomy specimen. Three of the children were given postoperative adjuvant chemotherapy. There were three deaths and one child was lost to follow-up. We discuss the clinical features of pleuropulmonary blastoma. No optimal treatment has been defined for this often fatal tumor.

The value of ultrasonic tomography exploration of the abdomen in non-Hodgkin's lymphoma of the small intestine is illustrated by findings in 9 children. Performed as the initial examination for an abdominal mass (sometimes after a straight radiograph), a positive diagnosis was established by ultrasonography in all cases. Moreover, extensions of the lesion were identified in the liver in two cases, in the two kidneys in four cases, and retroperitoneal adenopathies detected in one case. Ultrasonic findings in the case of intestinal localizations were a hypoechogenic mass with a hyperechogenic zone or a gaseous acoustic shadow in its center. Kidney, liver, and retroperitoneal lesions were seen as hypoechogenic nodules. In one case, there was an increase in echogenicity of both kidneys, these being more echogenic than the liver. This atypical appearance is emphasized as it does not appear to have been reported previously in the published literature. Results of ultrasonic and conventional radiological examinations are compared, and ultrasonic differential diagnosis discussed.

The authors report one new case of thymolipoma of child. Rare affection, its clinically latent and fortuitous discovery. The plain radiographic can orient the diagnosis in pré-operative. The recurrences after a complete surgical traitement are exceptional, witch confer these disease a good prognosis.