[Bilateral wilms tumors: diagnosis and therapeutic management. about 12 cases]

Bilateral Wilms tumor [WT] is uncommon malignancy, the incidence is about 5% of all nephroblastoma. It shows association with some congenital anomalies. there is no scheduled regimen for bilateral WT and their management offers a great challenge. To evaluate the clinical characteristics and the treatment result. 12 patients with bilateral WT diagnosed between 1997 and 2005, were retrospectively reviewed. Data collected: patients characteristics, chemotherapy, modality of surgical resection, renal function and survival. 11 patients [pts] had synchronous tumor and one metachronous lesions. The median age at primary diagnosis was 17 months [range 6m - 72m]. Congenital malformation were present in 4 children. 1 pt had hemi-hypertrophy, genitourinary horse kidney, 3 had hypertension.. All pts received preoperative prolonged chemotherapy [CT] for 25 to175 days [median 45 days] 7 received double agent chemotherapy. [VCR + Actinomycine], 2 triple agents: one [VCR + Actino + Adriblastine], the others [Etoposide, Carboplatine, Cyclophosphamied] for tumor progression. Two pt died before surgery due to infection. Bilateral partial nephrectomy 1 case total unilateral nephrectomy with partial nephrectomy on the other side in 3 cases. 9 pts are alive without disease and normal renal function 87 months after. The 10th in course of treatment after unilateral nephrectomy.Prolonged preoperative chemotherapy permit conservative surgical treatement of favorable histology bilateral Wilm's tumor and may improve the preservation of renal masses and function without impairing patient survival

[Surgical aspects of intussusception due to lymphoma in children]

Introduction: Bowel's intussuception is a rare kind of child Burkitt lymphoma revelation mode. The management of this situation is full of traps. The retrospective study of 6 observations aims to analyse the clinical, evolutionary and therapeutic side of this revelation mode

Results: Four boys and two girls, aged from three to seven years with an average of 5 years, had Burkitt lymphoma revealed by a Bowel's intussusception. The abdominal ultrasound find the secondary cause in 3 cases .One patient had a full-column barium enema All the cases had a laparotomy. Three cases had a surgical resection. A biopsy of mesenteric lymph nodes was done in 3 cases. A chemotherapy was initiated as soon as we made the diagnosis .There was no after effects in all the cases. No recurrence was noted until two years and a half after the treatment

Conclusion: The diagnosis of lymphoma must be suspected when the bowel's intussuception occurs in more than 5 years old children. A biopsy must be done in the event of suspicion of lymphoma. The surgical procedure must be adapted to each case

[Neurological diseases in the "triple a" syndrome]

Introduction: The 3A syndrome or Allgrove syndrome is an autosomic recessive disease characterized by the association of oesophagus achalasia, alacrymie and suprarenal insufficiency. Neurological disorders can appear at any time of the course's disease and would be due to a neuropathy affecting the central nervous system as well as the peripheral nervous system

Patient and methods: From1991 to 2005, we noticed 22 cases of Allgrove syndrome. The follow-up of these patients enabled us to notify the neurological abnormalities appear later in the evolution

Results: neurological symptoms appear at any time of the course's disease. All cases were backward pupil. 8 patients had a nasal voice due to palate paralysis. 7 patients had xerostomia wich is included in the dry syndrome due to the degeneration of salivary glands. One patient had a neurological motor disorder and 3 patients had a sensitive disorders with loss of cold feeling and vasomotor disorders in the hands and feet. These symptoms appear after 4 years on average after diagnosis. An orthostatics hypotension was noticed among three patients as a dysautonomic sign

Conclusion: All of these data approve that neurological disorders appear at a variable time of the course's disease. This polyneuropathy affects the oesophagus and always leads to the recurrence of the digestives signs

[Crossed renal ectora : a two cases report]

Crossed renal ectopia is a rare congenital anomaly, in which, the two kidneys are in the same side with one of the ureters which crosses the midline to be brought together in the bladder on the opposite side. Generally asymptomatic and of fortuitous discovery, this anomaly can appear by abdominal pains, urinary tract infection or a hematuria. The diagnosis rests on the radiological examinations in particular the ultrasonography, the intravenous urography and the CT scann. The treatment is surgical and is reserved for the complicated forms. We report two observations of children with a crossed renal ectopia The first observation is that of a 5 years old girl, presenting urinary tract infections at repetition whose etiologic assessment comprising a renal ultrasonography, a voiding cystourethrography as well as a three-dimensional tomodensitometry objectified a vesico-ureteral reflux grade HI, on left kidney in crossed ectopia. After sterilization of the urines, this patient profited from a surgical cure of her left vesicoureteral reflux with simple continuations.The second observation concerns a 10 years old boy, carrying a malformation anorectale, operated at birth, and at which the malformatif assessment [renal ultrasonography, vertebral radiography] objectified a left kidney in crossed ectopia with vesicoureteral ipsilateral reflux grade Ill associated with complex vertebral anomalies. This child was operated according to the same technique with simple operational continuations. From these two observations and after review of the literature, we recall the clinical, radiological and therapeutic characteristics of this malformation and discuss the pathogenic assumptions