Effectiveness of levator muscle resection combined with Mustarde’s double Z-plasty for blepharophimosis-ptosis-epicanthus inversus syndrome_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

QU Chun’an , LIU Yue , WANG Xiaoyu , LI Min , LI Dongyue ,  TANG Shengjian

Institute of Reconstructive Plastic Surgery, Weifang Medical University, Weifang Shandong, 261000, P.R.China;

Corresponding author：

TANG Shengjian, Email: tsj3676@163.com

Keywords：

Blepharophimosis-ptosis-epicanthus inversus syndrome; levator muscle resection; Mustarde’s double Z-plasty; child

DOI：

10.7507/1002-1892.201712065

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ObjectiveTo investigate the effectiveness of levator muscle resection combined with Mustarde’s double Z-plasty to correct blepharophimosis-ptosis-epicanthus inversus syndrome (BPES).MethodsBetween March 2015 and June 2017, one-stage operation of levator muscle resection combined with Mustarde’s double Z-plasty were performed on 26 children with bilateral BPES. There were 16 boys and 10 girls with an average age of 7 years (range, 4-14 years). All patients marked the four typical signs of BPES. There were 7 cases accompanied with a low nasal bridge, and 20 cases with amblyopia and strabismus. The length of eye fissure was (19.5±4.5) mm, the width of eye fissure was (2.5±1.6) mm, the diameter of inner canthus was (42.1±6.5) mm, and the muscular strength of levator palpebrae superioris was (5.5±1.3) mm.ResultsAll the incisions healed by first intention. Twenty-three patients were followed up 2-12 months, with an average of 10 months. Among which, 2 cases were less corrected, 3 cases were over corrected, 6 cases had poor curvature of the eyelid. No eyelid internal and external pronation or keratitis occurred. Amelioration of blepharoptosis and epicanthus was achieved in the other patients, and the double eyelid fold was naturally smooth. At 7 days after operation, the length of eye fissure was (27.2±1.9) mm, the width of eye fissure was (12.5±1.3) mm, and diameter of inner canthus was (29.4±2.6) mm, which were superior to preoperative values (t=0.127, P=0.042; t=0.341, P=0.029; t=0.258, P=0.038). There was no angular deformity caused by the width and length regressions of eye fissures.ConclusionThe levator muscle resection combined with Mustarde’s double Z-plasty can effectively correct BPES and obtain good effectiveness.

Citation： QU Chun’an, LIU Yue, WANG Xiaoyu, LI Min, LI Dongyue, TANG Shengjian. Effectiveness of levator muscle resection combined with Mustarde’s double Z-plasty for blepharophimosis-ptosis-epicanthus inversus syndrome. Chinese Journal of Reparative and Reconstructive Surgery, 2018, 32(6): 714-717. doi: 10.7507/1002-1892.201712065 Copy

1.	敖丽颖, 刘勇智. 先天性感音神经性耳聋合并先天性小睑裂综合征的临床诊断. 临床耳鼻咽喉头颈外科杂志, 2015, 29(18): 1660-1663.
2.	Bouman A, van Haelst M, van Spaendonk R. Blepharophimosis-ptosis-epicanthus inversus syndrome caused by a 54-kb microdeletion in a FOXL2 cis-regulatory element. Clin Dysmorphol, 2018, 27(2): 58-62.
3.	Tronina SA, Bobrova NF, Khrinenko VP. Clinical and anatomical substantiation of levator resection in the complex surgical treatment of BPES. Orbit, 2006, 25(1): 5-10.
4.	Nuruddin M, Osmani M. Two-stage correction of blepharophimosis syndrome: analysis of surgical outcome. Asia Pac J Ophthalmol (Phila), 2012, 1(6): 345-348.
5.	Park JI. Modified Z-epicanthoplasty in the Asian eyelid. Arch Facial Plast Surg, 2000, 2(1): 43-47.
6.	Allen CE, Rubin PA. Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES): clinical manifestation and treatment. Int Ophthalmol Clin, 2008, 48(2): 15-23.
7.	Li H, Li D, Jie Y, et al. Multistage correction of blepharophimosis: our rationale for 18 cases. Aesthetic Plast Surg, 2009, 33(4): 576-581.
8.	Chen L, Pi L, Ke N, et al. The protective efficacy and safety of bandage contact lenses in children aged 5 to 11 after frontalis muscle flap suspension for congenital blepharoptosis: A single-center randomized controlled trial. Medicine (Baltimore), 2017, 96(36): e8003.
9.	Zhou M, Jin R, Li Q, et al. Frontalis muscle flap advancement for correction of severe ptosis under general anesthesia: modified surgical design with 162 cases in China. Aesthetic Plast Surg, 2014, 38(3): 503-509.
10.	Medel R, Molina S, Vasquez LM, et al. Frontalis muscle flap versus maximal anterior levator resection as first option for patients with severe congenital ptosis. Ophthal Plast Reconstr Surg, 2018. [Epub ahead of print].
11.	Tu Y, Gao F. Dexmedetomidine-based monitored conscious sedation combined local anesthesia for levator resection in a 10-year-old child with Marcus Gunn jaw-winking synkinesis: A case report. Medicine (Baltimore), 2017, 96(51): e9369.
12.	Mawatari Y, Fukushima M, Kawaji T. Changes in sunken eyes combined with blepharoptosis after levator resection. Plast Reconstr Surg Glob Open, 2017, 5(12): e1616.
13.	Yamaguchi K, Imai K, Fujimoto T, et al. Cosmetic comparison between the modified uchida method and the mustarde method for blepharophimosis-ptosis-epicanthus inversus syndrome. Ann Plast Surg, 2015, 75(5): 518-521.
14.	Nakajima T, Yoshimura Y, Onishi K, et al. One-stage repair of blepharophimosis. Plast Reconstr Surg, 1991, 87(1): 24-31.
15.	Karacaoğlan N, Sahin U, Ercan U, et al. One-stage repair of blepharophimosis: a new method. Plast Reconstr Surg, 1994, 93(7): 1406-1409.
16.	Wu SY, Ma L, Tsai YJ, et al. One-stage correction for blepharophimosis syndrome. Eye (Lond), 2008, 22(3): 380-388.
17.	Sebastiá R, Herzog Neto G, Fallico E, et al. A one-stage correction of the blepharophimosis syndrome using a standard combination of surgical techniques. Aesthetic Plast Surg, 2011, 35(5): 820-827.
18.	Cogrel O. Mustardé flap lower eyelid reconstruction following lentigo maligna excision. Ann Dermatol Venereol, 2016, 143(2): 167-168.

1. 敖丽颖, 刘勇智. 先天性感音神经性耳聋合并先天性小睑裂综合征的临床诊断. 临床耳鼻咽喉头颈外科杂志, 2015, 29(18): 1660-1663.
2. Bouman A, van Haelst M, van Spaendonk R. Blepharophimosis-ptosis-epicanthus inversus syndrome caused by a 54-kb microdeletion in a FOXL2 cis-regulatory element. Clin Dysmorphol, 2018, 27(2): 58-62.
3. Tronina SA, Bobrova NF, Khrinenko VP. Clinical and anatomical substantiation of levator resection in the complex surgical treatment of BPES. Orbit, 2006, 25(1): 5-10.
4. Nuruddin M, Osmani M. Two-stage correction of blepharophimosis syndrome: analysis of surgical outcome. Asia Pac J Ophthalmol (Phila), 2012, 1(6): 345-348.
5. Park JI. Modified Z-epicanthoplasty in the Asian eyelid. Arch Facial Plast Surg, 2000, 2(1): 43-47.
6. Allen CE, Rubin PA. Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES): clinical manifestation and treatment. Int Ophthalmol Clin, 2008, 48(2): 15-23.
7. Li H, Li D, Jie Y, et al. Multistage correction of blepharophimosis: our rationale for 18 cases. Aesthetic Plast Surg, 2009, 33(4): 576-581.
8. Chen L, Pi L, Ke N, et al. The protective efficacy and safety of bandage contact lenses in children aged 5 to 11 after frontalis muscle flap suspension for congenital blepharoptosis: A single-center randomized controlled trial. Medicine (Baltimore), 2017, 96(36): e8003.
9. Zhou M, Jin R, Li Q, et al. Frontalis muscle flap advancement for correction of severe ptosis under general anesthesia: modified surgical design with 162 cases in China. Aesthetic Plast Surg, 2014, 38(3): 503-509.
10. Medel R, Molina S, Vasquez LM, et al. Frontalis muscle flap versus maximal anterior levator resection as first option for patients with severe congenital ptosis. Ophthal Plast Reconstr Surg, 2018. [Epub ahead of print].
11. Tu Y, Gao F. Dexmedetomidine-based monitored conscious sedation combined local anesthesia for levator resection in a 10-year-old child with Marcus Gunn jaw-winking synkinesis: A case report. Medicine (Baltimore), 2017, 96(51): e9369.
12. Mawatari Y, Fukushima M, Kawaji T. Changes in sunken eyes combined with blepharoptosis after levator resection. Plast Reconstr Surg Glob Open, 2017, 5(12): e1616.
13. Yamaguchi K, Imai K, Fujimoto T, et al. Cosmetic comparison between the modified uchida method and the mustarde method for blepharophimosis-ptosis-epicanthus inversus syndrome. Ann Plast Surg, 2015, 75(5): 518-521.
14. Nakajima T, Yoshimura Y, Onishi K, et al. One-stage repair of blepharophimosis. Plast Reconstr Surg, 1991, 87(1): 24-31.
15. Karacaoğlan N, Sahin U, Ercan U, et al. One-stage repair of blepharophimosis: a new method. Plast Reconstr Surg, 1994, 93(7): 1406-1409.
16. Wu SY, Ma L, Tsai YJ, et al. One-stage correction for blepharophimosis syndrome. Eye (Lond), 2008, 22(3): 380-388.
17. Sebastiá R, Herzog Neto G, Fallico E, et al. A one-stage correction of the blepharophimosis syndrome using a standard combination of surgical techniques. Aesthetic Plast Surg, 2011, 35(5): 820-827.
18. Cogrel O. Mustardé flap lower eyelid reconstruction following lentigo maligna excision. Ann Dermatol Venereol, 2016, 143(2): 167-168.

Chinese Journal of Reparative and Reconstructive Surgery

Effectiveness of levator muscle resection combined with Mustarde’s double Z-plasty for blepharophimosis-ptosis-epicanthus inversus syndrome

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