Résumé
Objective To discuss the indication and efficacy for treating hypospadias with ure-throplasty of Pedicled preputial double-faced island flap(PPDIF). Methods Records of 99 children (mean age, 5.1 years; age range, 4 months-12 years)with hypospadias who underwent PPDIF oper-ation were reviewed. Of them, 56 cases were penile type of hypospadias, 24 cases of penile root type, 13 cases of scrotal type and 6 cases of perineal type according to Barcat classification. Based on Duck-ett technique, the partial preputial cortical plate remained and was displaced to gastr-penis with ure-thra. , and finally the new urethra would be covered by the cortical plate. Results During follow-up of average 2 years, 8 patients(8. 1%) developed urethral fistula, 1 (1.0 %) developed mild meatal ste-nosis, and 1(1.0 %) developed flap necrosis. The success rate for total PPDIF procedure was 89.90 % (89/99). And the success rate of simple PPDIF for cases with penile and penile root hypospadias, and PPDIF+Duplay for cases with serotal and perineal hypospadias were respectively 91.25 % (73/80) and 84.21%(16/19). respectively. Conclusions PPDIF is good for treating most hypospadias. Furth-more it make penis better shape. In the severe cases, PPDIF+Duplay prededure is recommended and the effect was affirmative.
Résumé
Objective To study the clinical and pathological features of a case of retroperitoneal desmoplastic small round cell tumor. Methods One case of desmoplastic small round cell tumor was presented with review of the literature. Results A 11 years old child complained of abdominal pain and a 9.0 cm?6.0 cm?3.0 cm cystic tumor located in the left retroperitoneal space near the left kindey was resected.The connective tissue was significantly proliferated around the tumor cell nests. Immunohistochemical staining revealed CD 99 (+) and EWS/WT1 (+). After resection of the tumor,chemotherapy was performed with DDP?ifosfamide and VP16. No tumor recorrence has been noted 16 months after operation. Conclusions Desmoplastic small round cell tumor has specific clinicopathologic stigmata occuring usually in young males and the tumor was characterized by expression of MIC2 gene product CD 99 and the EWS/WT1 protein. Surgical resection with cheomotherapy is the treatment of choice.