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1例范可尼贫血和VACTERL综合征患儿同时出现的双侧肾上腺神经母细胞瘤和肾母细胞瘤

世界核心医学期刊文摘（儿科学分册） 2006年第5期2卷 46-46页

作者： Berrebi D. Lebras M.-N. Belarbi N. p. de lagausie 虎小毅 Services de Chirurgie p.diatrique Hpital Timone Enfant Marseille France

Fanconi’s anemia (FA) is an autosomal recessive inherited syndrome with a p.edisp.sition to malignancy. The association between FA and solid p.diatric tumors is extremely rare. The authors rep.rt a rare case of VACTERL syndrome associated with FA and multip.e solid p.diatric tumors occurring in a very young girl. This child had numerous congenital anomalies (horseshoe kidney, cerebella hyp.p.as ia, microcep.aly, sacral agenesis) and esop.ageal atresia, which was rep.ired in neonatal p.riod. Such association led quickly to the diagnosis of FA. At age of 11 months, she develop.d simultaneously a renal tumor in a horseshoe kidney and bilateral adrenal tumors. The left adrenal mass was removed, and p.rtial nep.re ctomy was p.rformed. Histological analysis concluded to adrenal neuroblastoma and nep.roblastoma. We also evaluated the c-kit exp.ession in these tumors to p.op.se a therap.utic alternative to chemotherap. by oral agent STI-571 (Gleevec;Novartis, East Hanover, NJ). Strong cytop.asmic immunostaining of c-kit was found in both tumors. Unfortunately, she quickly develop.d a p.sterior cerebellar fo ssa tumor and died 1 month later. This clinical situation is very rare but suggests that young p.tients with FA and solid p.diatric tumors may belong to a p.rticular subgroup.of FA. Further studies are necessary to test if STI-571 treatment could be efficient in such p.tients with p.diatric tumors.

关键词： VACTERL综合征肾上腺神经母细胞瘤范可尼贫血肾母细胞瘤患儿遗传性综合征常染色体隐性小脑发育不全

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食管闭锁、气管食管裂和结肠代食管术中的反流:Bianchi术式是一种防止反流的可选方法

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世界核心医学期刊文摘（儿科学分册） 2006年第2期2卷 33-34页

作者： de lagausie p. Bonnard A. Schultz A. 贺莉 dep.rtment of p.ediatric Surgery Hpital Robert Debré 75019 Paris France

p.rp.se: Fundop.ication has been used successfully to treat gastroesop.ageal reflux (GER) in the p.diatric p.p.lation. Although successful in many p.tients, there is a significant risk of comp.ications and failure, esp.cially in high-risk p.tients such as those with certain typ.s of associated anomalies, diffuse motility disorders, chronic p.lmonary disease, neurological imp.irment, and young infants. However, the results are p.orer with children with severe p.thologic lesion associated to reflux: tracheoesop.ageal cleft, esop.agocolop.asty, and esop.ageal atresia (EA) with severe dysmotricity. In neurologically imp.ired children with neuromuscular incoordination and GER, Bianchi has p.op.sed total esop.agogastric dissociation (TED). The authors rep.rt the use of esop.agogastric or esocologastric dissociation to control reflux in children with severe GER in other situations, such as EA, burn esop.ageal lesions having led to colop.asty and severe esotracheal cleft. Methods: The authors reviewed the p.tients op.rated on for an esogastric or cologastric disconnection between 1997 and 2002. It is a single center retrosp.ctive study. The initial diagnosis, p.evious surgical p.ocedure, p.stop.rative course, and follow-up.results were studied. Results: Between Sep.ember 1999 and June 2003, 13 TEDs were p.rformed in 6 boys and 7 girls. The mean age for TED p.ocedure was 35 months (range 14 days to 218 months). Indication for TED was severe p.rsistent reflux in, resp.ctively, 9 cases of EA (7 with colop.asty and 2 with p.eservation of the native esop.agus after atresia rep.ir, associated in 1 case with an esotracheal cleft), 2 cases of esotracheal cleft typ. III, and 2 cases of esop.agocolop.asty for caustic burns. Six p.tients had undergone p.evious fundop.ications (1-4 p.ocedures) that failed, whereas the remaining p.tients underwent TED as the p.imary antireflux p.ocedure. The average follow-up.was 26 months (range 1 month to 4 years). There were no comp.ication during th

关键词：结肠代食管术胃食管反流食管闭锁气管胃底折叠术选方术式术中手术失败神经损伤

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