2024/12/31 by Fatou LY., CisséF D, Fadjri E +7
#AdamsOliversyndrome #SyndromeAdamsOliver #newborn #nouveau-né
paper · doi:10.71566/pist-rmp-194722
AdamsOliversyndrome(AOS)isararecongenitaldiseasecharacterizedbytheassociationofcongenitalskin aplasiamostoftenofthescalp, transverseabnormalitiesofthelimbsassociatedwithvascularabnormalities. The firstcasehasbeenreportedsince1945byForrestH. AdamsandCPLiver. Sporadiccaseshavebeenreported. Thepurposeofourobservationwastorecallarareaffectionanddescribeitsclinicalandradiologicalmanifesta- tions in anewborn. We reportthe observation ofamale newborn, bornto a42-year-old mother4th gesture 3rd parewithnospecific pathological history. The pregnancywaswell followed. The deliverywas bytransverse low caesareansectionattheendofaprolongedpregnancyof41weeks+5days. Thechildhadscreamedatbirth withoutanynotionofresuscitation. Clinically,theexaminationfoundapolyformativesyndromewithdiffuse neonataltelangiectasiaassociatedwith skin aplasiaofthe scalp,venous malformationwith cerebral masstype, complete agenesis ofthe righttoes, hypoplasiaofthe lefttoes, bilateral hexadactyly, right cryptorchidism and harmonious intrauterine growth retardation, diagnosingAdams Oliversyndrome. X-rays ofthe hands and feet hadshownboneagenesisandanappendageoftheulnaredgeofthelefthand. Theshort-termevolutionwas favourableunderlocaltreatment,markedbyahealingofscalplesionswithasatisfactoryaestheticresultandin terms ofpsychomotordevelopment, adelayin psychomotordevelopment. This casewe described is most likely sporadic,astherewerenootherknowncasesinthesiblings.