Sahebpor Alireza Alam, Ghafari Vajihe, Shokohi Leila
Department of Pediatric Surgery, Mazandaran University of Medical Science, Sari, Iran.
Indian Pediatr. 2008 Oct;45(10):849-51.
We present 5 cases of pyloric atresia associated with junctional epidermolysis bullosa, from 2003 to 2005. Patients underwent laparatomy after stabilization. Four neonates had gastroduodenostomy, and the other had excision of membrane and pyloroplasty. Four survived and one died from fulminant septicemia. Although the association of pyloric atresia with epidermolysis bullosa has been reported to be fatal, our study showed good survival rate.
我们呈现了2003年至2005年间5例与交界性大疱性表皮松解症相关的先天性幽门闭锁病例。患者在病情稳定后接受了剖腹手术。4例新生儿接受了胃十二指肠吻合术，另1例接受了隔膜切除术和幽门成形术。4例存活，1例死于暴发性败血症。虽然先天性幽门闭锁与大疱性表皮松解症的关联据报道是致命的，但我们的研究显示了良好的存活率。
