2024/12/31 by Sfar MS., Belghith M, Chahed J +8
#atrésiepylorique #chirurgie #dermatologie #dermatology #epidermolysis bullosa #neonate #nouveau-né #pyloricatresia #surgery #épider- molyse bulleuse
paper · doi:10.71566/pist-rmp-194875
Introduction:Hereditary epidermolysis bullosa are rare genodermatoses with skin fragility leading to the bubble forma- tion and diffuse erosions affecting the skin and mucous membranes. Arare and particular association with pyloric atresia is seen especially in the junctional form of epidermolysis bullosa. Method:Wereporttwonewbornswithpyloricatresiaasso- ciatedwithepidermolysisbullosahospitalizedinthe Pediatric Surgery department of Fattouma Bour- guiba Hospital between January 1990 and Octo- ber2010. Bothpatientsweretreatedsurgically. The firsthadaresectionofpyloricdiaphragmfollowed bypyloroplasty. The second had apylorectomyfol- lowed by a duodeno-antral anastomosis. Results:Inthefollow-up,wenoticedthedisappearanceof vomiting but alsothe appearance ofbullous lesions atthesecondandfifthpostoperativedayforboth newborns. At the age ofthree months, aprofuse diarrhea caused the death ofthe two patients. Conclusion:The epidermolysis bullosa associated to pyloric atresia represent a heterogeneous disease with viable forms and lethal forms in few months after birthdespitethesurgicalcorrectionofatresia.