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Single stage surgery for Blepharophimosis syndrome.

Bhattacharjee K, Bhattacharjee H, Kuri G, Shah ZT, Deori N - Indian J Ophthalmol (2012 May-Jun)

Bottom Line: There was a statistically significant decrease of astigmatism following ptosis correction (P<0.05), improvement of telecanthus (P<0.0001) in terms of IICD (inner intercanthal distance), and HPFL (horizontal palpebral fissure length) (P=0.019) along with improvement of the superior visual field.There was also a significant postoperative improvement of ptosis (P< 0.01), as measured by IPFH (vertical interpalpebral fissure height).All the patients had a stable functional and cosmetic result after a mean follow-up period of 3 years.

View Article: PubMed Central - PubMed

Affiliation: Department of Orbit, Ophthalmic Plastic and Reconstructive Surgery, Sri Sankaradeva Nethralaya, Guwahati, Assam, India.

ABSTRACT

Purpose: The purpose of this study was to report the functional and cosmetic outcome of single stage surgical procedure for correction of the classic components of Blepharophimosis syndrome.

Materials and methods: We report a retrospective case file review of 11 patients with Blepharophimosis syndrome operated between July 2004 and April 2008. Each patient had undergone the correction of epicanthus inversus, telecanthus, palpebral phimosis, and bilateral ptosis as a single-stage surgical procedure. Patients were examined and photographed before and after surgery. The mean follow-up was 3 years (range 2-6 years).

Results: A total of 11 patients (8 males, 3 females) with a mean age of 9 years (range 6--22 years) were reviewed. The surgical outcome was assessed both functionally and cosmetically. The mean preoperative visual acuity was 0.729 ± 0.316 SD and the mean postoperative visual acuity was 0.856 ± 0.277 SD (P <0.0428). There was a statistically significant decrease of astigmatism following ptosis correction (P<0.05), improvement of telecanthus (P<0.0001) in terms of IICD (inner intercanthal distance), and HPFL (horizontal palpebral fissure length) (P=0.019) along with improvement of the superior visual field. The mean preoperative and postoperative IICD was 3±0.33 SD and 2.418 ± 0.189 SD, respectively. There was also a significant postoperative improvement of ptosis (P< 0.01), as measured by IPFH (vertical interpalpebral fissure height). All the patients had a stable functional and cosmetic result after a mean follow-up period of 3 years.

Conclusion: Single-stage surgical correction of the classic anomalies of Blepharophimosis syndrome provides stable and successful long-term results.

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Related in: MedlinePlus

Patient of Blepharophimosis syndrome planned for correction of epicanthal fold
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Figure 1: Patient of Blepharophimosis syndrome planned for correction of epicanthal fold

Mentions: In Mustarde's double Z-plasty the flaps were fashioned at an angle of 60° to the line joining the present medial canthus and the site of the proposed medial canthus [Figures 1 and 2]. After fashioning the flaps, the underlying subcutaneous tissue and muscle were excised and the medial canthal tendon was exposed. The periosteum and the medial canthal tendon attachment were dissected from the anterior lacrimal crest and the medial wall of the lacrimal fossa was exposed. In the case of very prominent anterior lacrimal crest, excess bony tissues were removed with the bone punch [Figure 3]. The same surgical procedure was performed bilaterally and a bone drill was then used to make an opening just anterior to the posterior lacrimal crest and drilled across the nose from one side to emerge in a similar position on the other side. A loop of stainless steel wire was passed through the opening to which the medial canthal tendons on either side were sutured along with the subcutaneous tissues, and pulled medially toward the nose. The stainless steel wire was fixed and tightened after titration of midline shift of medial canthus and subcutaneous tissue [Figure 4]. The subcutaneous tissues were sutured with 6-0 polyglactin sutures and skin flaps transposed and sutured with 6-0 silk sutures. Finally, the bilateral ptosis was corrected by frontalis brow suspension with autologous fascia lata harvested from the thigh [Figure 5].


Single stage surgery for Blepharophimosis syndrome.

Bhattacharjee K, Bhattacharjee H, Kuri G, Shah ZT, Deori N - Indian J Ophthalmol (2012 May-Jun)

Patient of Blepharophimosis syndrome planned for correction of epicanthal fold
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3361814&req=5

Figure 1: Patient of Blepharophimosis syndrome planned for correction of epicanthal fold
Mentions: In Mustarde's double Z-plasty the flaps were fashioned at an angle of 60° to the line joining the present medial canthus and the site of the proposed medial canthus [Figures 1 and 2]. After fashioning the flaps, the underlying subcutaneous tissue and muscle were excised and the medial canthal tendon was exposed. The periosteum and the medial canthal tendon attachment were dissected from the anterior lacrimal crest and the medial wall of the lacrimal fossa was exposed. In the case of very prominent anterior lacrimal crest, excess bony tissues were removed with the bone punch [Figure 3]. The same surgical procedure was performed bilaterally and a bone drill was then used to make an opening just anterior to the posterior lacrimal crest and drilled across the nose from one side to emerge in a similar position on the other side. A loop of stainless steel wire was passed through the opening to which the medial canthal tendons on either side were sutured along with the subcutaneous tissues, and pulled medially toward the nose. The stainless steel wire was fixed and tightened after titration of midline shift of medial canthus and subcutaneous tissue [Figure 4]. The subcutaneous tissues were sutured with 6-0 polyglactin sutures and skin flaps transposed and sutured with 6-0 silk sutures. Finally, the bilateral ptosis was corrected by frontalis brow suspension with autologous fascia lata harvested from the thigh [Figure 5].

Bottom Line: There was a statistically significant decrease of astigmatism following ptosis correction (P<0.05), improvement of telecanthus (P<0.0001) in terms of IICD (inner intercanthal distance), and HPFL (horizontal palpebral fissure length) (P=0.019) along with improvement of the superior visual field.There was also a significant postoperative improvement of ptosis (P< 0.01), as measured by IPFH (vertical interpalpebral fissure height).All the patients had a stable functional and cosmetic result after a mean follow-up period of 3 years.

View Article: PubMed Central - PubMed

Affiliation: Department of Orbit, Ophthalmic Plastic and Reconstructive Surgery, Sri Sankaradeva Nethralaya, Guwahati, Assam, India.

ABSTRACT

Purpose: The purpose of this study was to report the functional and cosmetic outcome of single stage surgical procedure for correction of the classic components of Blepharophimosis syndrome.

Materials and methods: We report a retrospective case file review of 11 patients with Blepharophimosis syndrome operated between July 2004 and April 2008. Each patient had undergone the correction of epicanthus inversus, telecanthus, palpebral phimosis, and bilateral ptosis as a single-stage surgical procedure. Patients were examined and photographed before and after surgery. The mean follow-up was 3 years (range 2-6 years).

Results: A total of 11 patients (8 males, 3 females) with a mean age of 9 years (range 6--22 years) were reviewed. The surgical outcome was assessed both functionally and cosmetically. The mean preoperative visual acuity was 0.729 ± 0.316 SD and the mean postoperative visual acuity was 0.856 ± 0.277 SD (P <0.0428). There was a statistically significant decrease of astigmatism following ptosis correction (P<0.05), improvement of telecanthus (P<0.0001) in terms of IICD (inner intercanthal distance), and HPFL (horizontal palpebral fissure length) (P=0.019) along with improvement of the superior visual field. The mean preoperative and postoperative IICD was 3±0.33 SD and 2.418 ± 0.189 SD, respectively. There was also a significant postoperative improvement of ptosis (P< 0.01), as measured by IPFH (vertical interpalpebral fissure height). All the patients had a stable functional and cosmetic result after a mean follow-up period of 3 years.

Conclusion: Single-stage surgical correction of the classic anomalies of Blepharophimosis syndrome provides stable and successful long-term results.

Show MeSH
Related in: MedlinePlus