Genitourinary complications as initial presentation of inherited epidermolysis bullosa.

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.

[Portal vein thrombosis after splenectomy in childhood: report of 4 cases]. / Thrombose portale après splénectomie chez l'enfant: à propos de 4 observations.

Portal vein thrombosis is a major complication of splenectomy. Its frequency is underestimated because of asymptomatic cases. Mesenteric occlusion with intestinal infarcts is the first cause of mortality. Secondarily, in the absence of repermeabilisation, a portal hypertension can occur. We present in this study 4 cases of portal vein thrombosis in childhood. Portal vein thrombosis is frequent (8% of splenectomies) and may be asymptomatic. Doppler postoperative surveillance is justified. Thrombocytosis seems to be a determinant factor. Early diagnosis and treatment may reduce lethal outcome.

[Cystic adenomatoid malformations of the lung: a retrospective study of 12 cases]. / Malformations adénomatoïdes kystiques du poumon : étude rétrospective de 12 observations.

The authors present a retrospective study of 12 cases of cystic adenomatoid lung malformations in clinical surgery unit "A" at the Rabat Children's hospital, between 1999 to 2006. Two pregnancies with lung malformation were identified. All of the patients were symptomatic. The treatment was surgical in all cases (lobectomy: 11 cases; pneumonectomy: one case). The evolution was good after an average of three years. In the light of these 12 malformations, the authors try and demonstrate the value of early diagnosis in order to surgically treat the anomaly in optimum conditions. Moreover, the authors insist on the exeresis not only of the malformation, but of the entire lobe involved in anomaly to guarantee a recurrence-free result.

[Peritoneal hydatidosis in children. Report of a historical case]. / L'hydatidose péritonéale chez l'enfant. A propos d'un cas historique.

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.

[Pediatric vesical lithiasis. 70 case reports]. / Lithiase vésicale de l'enfant. A propos de 70 cas.

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.

[Total nephrectomy in children: 11 years of experience in 80 cases]. / Les néphrectomies totales chez l'enfant: une expérience de 11 ans sur 80 cas.

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.

[Gastrointestinal system duplications in children (19 cases)]. / Les duplications digestives chez l'enfant (à propos de 19 cas).

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.

[Anterior urethral duplication in a male child. A case report]. / Duplications sagittales antérieures de l'urètre chez le garçon. A propos d'un cas.

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.

[Pheochromocytoma in children. Two case reports]. / Le phéochromocytome de l'enfant. A propos de deux cas.

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.

[Laryngeal dyspnea caused by cervical hydatidosis: apropos of a case]. / Dyspnée laryngée révélatrice d'une localisation cervicale d'hydatidose: à propos d'un cas.

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.

[Pleuro-pulmonary blastoma. Report of 4 cases]. / Le blastome pleuro-pulmonaire. A propos de quatre observations.

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.

[A new case of thymolipoma in a child]. / Un nouveau cas de thymolipome chez l'enfant.

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.

[Ultrasonography of the whole abdomen in non-Hodgkin's lymphoma of the small intestine in children. Apropos of 9 cases]. / Apport de la pansonographie abdominale dans le lymphome non Hodgkinien du grêle de l'enfant. A propos de 9 cas.

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.