Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

Users Online : 32497

Original article / research
Year : 2017 | Month : March | Volume : 11 | Issue : 3 | Page : NC05 - NC08

Surgical Outcome of Epicanthus and Telecanthus Correction by Double Z-Plasty and Trans-Nasal Fixation with Prolene Suture in Blepharophimosis Syndrome

Salil Kumar Mandal, Aparna Mandal, James Christian Fleming, Tara Goecks, Andrew Meador, Brian T Fowler

1. Associate Professor, Department of Orbit and Oculoplasty, Regional Institute of Ophthalmology Medical College, Kolkata, West Bengal, India. 2. Assistant Professor, Department of Physiology, College of Medicine and Sagore Dutta Hospital, Kolkata, West Bengal, India. 3. Lewis Professor and Chairman, Department of Ophthalmology, University of Tennessee, Hamilton Eye Institute, Memphis, United States. 4. Clinical Instructor, Department of Ophthalmology, University of Tennessee, Hamilton Eye Institute, Memphis, United States. 5. PGY-4, Resident, Department of Ophthalmology, University of Tennessee, Hamilton Eye Institute, Memphis, United states. 6. Assistant Professor, Department of Ophthalmology, University of Tennessee, Hamilton Eye Institute, Memphis, United states.

Correspondence Address :
Dr. Salil Kumar Mandal,
Associate Professor, Department of Orbit and Oculoplasty, Regional Institute of Ophthalmology Medical College, Kolkata-700073,
West Bengal, India.
E-mail: salil_dum@live.com

Abstract

Introduction: Blepharophimosis Syndrome (BPES) is a complex and rare disease characterized by epicanthus inversus, telecanthus, lateral ectropion, narrowed or shortened interpalpebral fissure distance and ptosis. It is mostly bilateral and may or may not be symmetrical. It is typically inherited as an autosomal dominant trait. In sporadic cases, the disease may occur without a prior family history as a genetic mutation from a deletion or translocation of the FOXL2 gene, which maps to chromosome 3q23. Surgical treatment of this disease poses an oculoplastic challenge due to multiple complex eyelid deformities.

Aim: To evaluate the functional and cosmetic outcome of telecanthus and epicanthus correction by a Mustarde’s rectangular double Z-Plasty and trans-nasal fixation using 1-0 prolene suture in BPES.

Materials and Methods: This was prospective, interventional study of 16 patients over a period of three years. In this study, all patients had BPES with prominent epicanthus and telecanthus. Mustarde’s double Z-plasty and trans-nasal fixation with 1-0 prolene suture was performed in the first of a two-stage operation. If ectropion was present, the lateral ectropion was corrected by a base-out flap transfer from the upper eyelid to the lower eyelid. After three months, a 2nd stage was undertaken, involving a lateral canthoplasty for horizontal widening of a short palpebral fissure and a tarso frontalis sling with silicone rod for correction of moderate to severe ptosis. Patients were followed up for six months to one year with postoperative ophthalmologic examinations and photographs.

Results: Out of 16 patients, 10 were females and six were males. All the patients had bilateral involvement. In this study preoperative Inner Intercanthal Distance (IICD) ranged from 38 mm to 42 mm and the mean IICD was 41.2±0.57 mm. Postoperative IICD ranged from 31 mm to 34 mm. Horizontal Palpebral Fissure Length (HPFL) ranged from 20 mm to 23 mm and the mean value of HPFL was 21.50 mm preoperatively. Postoperative HPFL ranged from 26 mm to 29 mm and had a mean value of 28.50 mm, which was much improved after a combined correction of telecanthus and lateral canthoplasty. The mean preoperative IICD and HPFL ratio was 1.77 and was reduced to a postoperative value of 1.2. The Marginal Reflex Distance1 (MRD-1) test value improved from +1.25 mm to +3.50 mm postoperatively after placement of a tarsofrontalis sling with silicone rod using the Fox's Pentagon technique. In this study, two eyes had minimal unequal correction but were cosmetically and functionally acceptable. Correction of IICD is possible up to 6 mm. No major complication e.g., CSF rhinorrhea was noted in this series and preoperative prominent epicanthal folds were abolished.

Conclusion: Here we propose a two-staged procedure involving a combined Mustarde’s double Z-plasty with transnasal fixation using a 1-0 prolene suture with a flap transfer from the upper lid to the lower lid in the first stage and a lateral canthoplasty with a tarsofrontalis sling and silicone rod in the second stage. This technique is effective to correct epicanthus, telecanthus, ptosis and lateral ectropion in BPES with good cosmetic and functional outcome.

Keywords

Intercanthal Distance, Lid deformity, Ptosis

How to cite this article :

Salil Kumar Mandal, Aparna Mandal, James Christian Fleming, Tara Goecks, Andrew Meador, Brian T Fowler. SURGICAL OUTCOME OF EPICANTHUS AND TELECANTHUS CORRECTION BY DOUBLE Z-PLASTY AND TRANS-NASAL FIXATION WITH PROLENE SUTURE IN BLEPHAROPHIMOSIS SYNDROME. Journal of Clinical and Diagnostic Research [serial online] 2017 March [cited: 2017 May 25 ]; 11:NC05-NC08. Available from
http://jcdr.net/back_issues.asp?issn=0973-709x&year=2017&month=March&volume=11&issue=3&page=NC05-NC08&id=9496

DOI and Others

DOI: 10.7860/JCDR/2017/25651.9496

Date of Submission: Dec 01, 2016
Date of Peer Review: Dec 22, 2016
Date of Acceptance: Feb 06, 2017
Date of Publishing: Mar 01, 2017

Financial OR OTHER COMPETING INTERESTS: None.

JCDR is now Monthly and more widely Indexed .
  • PubMed Central® (PMC)New
  • Academic Search Complete Database
  • Chemical Abstracts Service
  • Directory of Open Access Journals (DOAJ)
  • EBSCOhostNew
  • Embase & EMbiology
  • Google Scholar
  • HINARI Access to Research in Health Programme
  • Indian Science Abstracts (ISA)
  • Journal seek Database
  • Open J-Gate
  • Popline (reproductive health literature)
  • SCOPUS
  • www.omnimedicalsearch.com