Surgical outcome of epicanthus and telecanthus correction by C-U medial canthoplasty with lateral canthoplasty in treatment of Blepharophimosis syndrome

Abstract

Purpose: To evaluate the surgical outcome of epicanthus and telecanthus correction by C-U medial canthoplasty with lateral canthoplasty in Blepharophimosis Syndrome.

Patients and methods: This was a retrospective single arm interventional study including 18 eyes of 9 patients with Blepharophimosis-ptosis-epicanthus inversus syndrome who presented to oculoplastic clinic, ophthalmology department, Qena university hospital in the period of between July 2020 to April 2021. All the patients had BPES with epicanthus and telecanthus. All cases were subjected to by C plasty with medial and lateral canthoplasty for correction of epicanthus and telecanthus correction followed by frontalis suspension surgery to correct the co-existing blepharoptosis.

Results: The study included 9 cases of BPES, 6 boys and 3 girls, the mean age was 5.4 ± 1.5 in the study group, all patients had a positive family history for BPES. After surgery, the mean IICD decreased from 38.44 mm preoperatively to 32.8 mm postoperatively, with a mean difference of 6.2 mm (P < 0.001). Likewise, the mean PFL increased from 20.78 mm preoperatively to 26.63 mm postoperatively, with a mean difference of 5.8 mm (P < 0.001). Epicanthus skin fold disappeared in all cases and medical canthus could be seen with well healed difficulty seen scars.

Conclusion: C-U medial canthoplasty with lateral canthoplasty in Blepharophimosis Syndrome was found to be an effective procedure in the correction of epicanthus and telecanthus.

Keywords: Blepharophimosis Syndrome; C-U medial canthoplasty; Epicanthus; Telecanthus; lateral canthoplasty.

Fig. 1

Four points (A-D) drawn to determine the C and U-shaped incisions

Fig. 2

Steps of surgery from A to P for epicanthus and telecanthus correction by C-U with medial canthoplasty and lateral canthoplasty. A Marking of points A and B in right eye, B Marking of points C and D right eye and points A, B in left eye, C Marking of the C and U arms of C-U medial canthoplasty in Left eye, D excision of the skin and orbicularis muscle in between the C and U arms of the C-U medial canthoplasty, E exposure of the medial canthal tendon, F Plication of the medial canthal tendon, G Shortening of the medial canthal tendon with lengthening HPFL, H Complete of the medial wound closure, I Marking of the skin for lateral canthoplasty, J Lateral canthal wound to lengthen the HPFL, K Suturing the undermined conjunctiva to the new lateral canthal angle, L making the sharp lateral canthal angle

Fig. 3

3 cases of BPES before and after one year of surgery

Fig. 4

A Changes in mean IICD and mean PFL within one year follow up. B Changes in the IICD/IPD ratio and the IICD/HPFL ratio within year follow up