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TitreCorrection of the lower eyelid malpositioning in the blepharophimosis, ptosis and epicanthus inversus syndrome (BPES).
ButBlepharophimosis, ptosis, epicanthus inversus syndrome (BPES) is an autosomal dominant complex eyelid malformation. We aim to offer an explanation for the lower eyelid malformation and propose a novel surgical approach to correct it.
MéthodesAn observational and interventional case series of ten consecutive, molecularly proven, BPES patients who underwent surgical repair of the lower eyelid malformation. During surgery detailed anatomical examination and surgical repositioning of the medial canthal tendon was performed.
RésultatsAll patients exhibited a marked asymmetry in the attachment of the lower and upper eyelid to the medial canthal tendon, with the lower eyelid being much less attached. This resulted in an abnormal downward concavity with a temporal ectropion and a temporally displaced lower eyelid. Consequently, the inferior punctum was displaced temporally. All patients underwent a novel surgical technique to remediate this, namely inserting 4.0 nylon suture between the tarsal plate of the lower eyelid and the medial canthal tendon during telecanthus surgery. This simple additional surgical step not only corrected the position of the lower eyelid, but also its abnormal downward concavity, the temporal ectropion and the lateral displacement of the inferior punctum. None of our patients had lasting epifora.
ConclusionLateral displacement of the inferior punctum is an important hallmark in the diagnosis of BPES. We demonstrate an anatomical explanation for the complex lower eyelid malformation and also propose a novel surgical technique to correct this.
Auteur 1
NomDECOCK
InitialesCE
InstitutGhent University Hospital
Villeghent
Auteur 2
NomClaerhout
InitialesI
InstitutGhent University Hospital
Villeghent
Auteur 3
NomKestelyn
Initialesph
InstitutGhent University Hospital
Villeghent
Auteur 4
NomLeroy
InitialesBP
InstitutGhent University Hospital
Villeghent
Auteur 5
NomDebaere
InitialesE
InstitutGhent University Hospital
Villeghent
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