ABSTRACT
INTRODUCTION: Blepharophimosis Syndrome (BPES) is a complex and rare disease characterized by epicanthus inversus, telecanthus, lateral ectropion, narrowed or shortened inter-palpebral 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.
ABSTRACT
Epidermoid cyst within the lid and orbit is extremely rare. Epidermoid tumours are inclusion of ectodermal elements in the site not normally containing these structures. It is of two types; primary type related to implantation of ectoderm at the time of closure of the neural groove and secondary type caused by post traumatic inclusion of the surface epithelium. A 45-year-old male had complaint of swelling on the right upper lid and orbital region. It first appeared two years back. It was painless progressively increased in size and shape. There was difficulty in opening of eye lid due to large swelling, feeling of heaviness in the right upper lid and occasional headache. There was history of right eye ocular infection following vegetative matter injury three years back. Evisceration of right eye was done for aforementioned reason. USG report shows cystic encapsulated mass with calcification foci in right upper lid-orbital region with fat component. MRI right orbit shows fairly large hyperintense cystic lesion seen involving right orbit with posterior extension up to optic canal. No intracranial extension. FNAC confirmed epidermal inclusion cyst. We did upper lid reconstruction with removal of mass. We have successfully removed the mass without any complication and with one year follow-up there is no recurrence.
ABSTRACT
Rhinosporidiosis is a granulomatous disease of the mucous membrane caused by Rhinosporidium seeberi, an organism of uncertain taxonomic position, most probably a fungus. It usually infects mucous membrane of the nose and nasopharynx but may infect mucous membrane of all other parts even the skin. Inoculation of the eye and related structures are grouped as oculosporidiosis. In this case of a 50 years old male agriculturist from remote village had complaints of a mass protruding out from the palpebral fissure since last one year. It was gradually increasing in size and shape. There was recurrent redness, watering and discharge. This mass caused dragging of lower lid to produce mechanical ectropion. On examination there was a pedunculated polypoidal broad based conjunctival mass originated from inferior bulbar conjunctiva. It is chemosed with the evidence of necrosis and old haemorrhagic spot, engorged conjunctival vessel and in the exposed pert there is keratinisation. Conjunctival rhinosporidosis has predilection for agriculture workers so that contact with contaminated soil may be the causative factor. The only effective treatment is radical surgical excision followed by thermal and chemical coagulation, nevertheless relapse is the rule rather the exception. No such medical treatment has proved to be effective. Griseofulvin and amphotericin have been used without success.
