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Title

Correction of the lower eyelid malpositioning in the blepharophimosis, ptosis and epicanthus inversus syndrome (BPES).

Purpose	Blepharophimosis, 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.
Methods	An 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.
Results	All 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.
Conclusion	Lateral 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.

Author 1

Last name	DECOCK
Initials	CE
Department	Ghent University Hospital
City	ghent

Author 2

Last name	Claerhout
Initials	I
Department	Ghent University Hospital
City	ghent

Author 3

Last name	Kestelyn
Initials	ph
Department	Ghent University Hospital
City	ghent

Author 4

Last name	Leroy
Initials	BP
Department	Ghent University Hospital
City	ghent

Author 5

Last name	Debaere
Initials	E
Department	Ghent University Hospital
City	ghent