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   Table of Contents      
BRIEF REPORT
Year : 2003  |  Volume : 51  |  Issue : 2  |  Page : 185-186

Post traumatic subconjunctival dislocation of lens in Ehlers-Danlos syndrome.


Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India

Correspondence Address:
Y Sharma
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi
India
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Source of Support: None, Conflict of Interest: None


PMID: 12831154

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  Abstract 

Ehlers-Danlos Syndromes are a rare group of inherited connective tissue disorders of defective collagen synthesis. They predominantly involve the skin, joints and vessels. Ocular involvement in the form of blue sclera, microcornea and susceptibility to trauma is a common feature of EDS type VI. A minor ocular trauma in these patients can cause globe rupture. We herein report simple and successful management of a case of traumatic subconjunctival dislocation of lens in a patient of EDS type VI.

Keywords: Ehlers-Danlos Syndrome, blue sclera, scleral rupture, subconjunctival lens dislocation


How to cite this article:
Sharma Y, Sudan R, Gaur A. Post traumatic subconjunctival dislocation of lens in Ehlers-Danlos syndrome. Indian J Ophthalmol 2003;51:185-6

How to cite this URL:
Sharma Y, Sudan R, Gaur A. Post traumatic subconjunctival dislocation of lens in Ehlers-Danlos syndrome. Indian J Ophthalmol [serial online] 2003 [cited 2019 Aug 23];51:185-6. Available from: http://www.ijo.in/text.asp?2003/51/2/185/14704

Systemic manifestations of Ehlers-Danlos Syndrome (EDS) include skin fragility, skin hyperextensibility, joint hypermobility and excessive bruising. Significant ocular manifestations are present in EDS type I, VI and VII. Myopia, looseness of the lids with easy eversion of upper eyelids (Metenier's sign) and strabismus are common findings. Epicanthal folds and blue sclera are other less common manifestations. Microcornea, retinal detachment, keratoconus, angioid streaks, corneal haze at the Bowman membrane, secondary glaucoma, and vitreoretinal degeneration have also been reported in patients with Ehlers-Danlos Syndrome. [1],[2],[3]

Spontaneous dislocation of lens is rare in this condition and has been reported only twice.[4] We describe a case of Ehlers-Danlos Syndrome with dislocation of lens, related to minor trauma, into the subconjunctival space. To the best of our knowledge this is the first such report of successful surgical management.


  Case report Top


A 16-year-old girl presented complaining of impaired vision in her right eye, which she noticed on accidentally closing her left eye, a week before presentation. She reported minor blunt trauma to the right eye with a wooden stick three months earlier. Visual acuity in the left eye was 6/18, improving to 6/6 with -2.00 DSph, and in the right eye it was 3/60 improving to 6/9 with +7.00 DSph and +1.00 DCyl at 180°. An external examination of the right eye showed a subconjunctival mass, inferonasally close to the limbus [Figure - 1]. The pupil was jet black, irregular and slightly peaked in the inferonasal direction. A slitlamp examination revealed aphakia. Gonioscopic examination of the angle was normal except for pigment dispersion. Intraocular pressure (IOP) was 18mmHg in the right eye and 16mmHg in the left eye. Fundus examination of both eyes with the slitlamp and indirect ophthalmoscope revealed vitreous strands and some retinal haemorrhage inferonasally in the right eye. It seemed that the inferonasal periphery at the pars plana region was the likely site of scleral rupture. The rupture did not extend beyond the ora serratta, which was confirmed by indirect ophthalmoscopy and gentle indentation. These preoperative impressions were confirmed during the surgery.

The girl's parents reported easy bruising since childhood and recurrent dislocation of wrist and digital joints. A general examination showed thin skin with diffuse bruising of the arms and legs. There was considerable extensibility of the skin along with digital and wrist joint hyperextensibility. The patient also had long thin fingers and a severe kyphoscoliosis since birth. The family history­­­ revealed an affected sibling with similar systemic features and simple myopia (-2DSph both eyes) but both parents were clinically unaffected. The skin biopsy was positive for Ehlers-Danlos Syndrome. The diagnosis of Ehlers-Danlos Syndrome type VI was further confirmed by low levels of lysyl hydroxylase activity in cultured fibroblast. This test is diagnostic for EDS type VI.

The patient was operated for removal of the dislocated lens and strengthening of the underlying scleral wound. The possible need for future vitreo-retinal surgery was explained. A small nasal conjunctival peritomy was done and lens aspiration using a 22G Simcoe irrigation/aspiration cannula was initiated. Gentle aspiration at a low irrigation rate was initiated to slowly remove the pultaceous lens matter with a transverse planar motion of the cannula tip. Lens removal en masse was not attempted because of the fear of possible full thickness scleral defect beneath. However, to our pleasant surprise, we were able to successfully accomplish this. After lens removal, an underlying area of scleral thinning was observed [Figure - 2]. The scleral wound had healed and there was no tissue breach. Conjunctiva was then sutured. Postoperatively, the patient continues to maintain a vision of 6/9 with contact lens in follow-up of over two-years. There have been no postoperative complications. Aphakia and vitreous strands in the wound area at the inferonasal pars plana remain the only findings.


  Discussion Top


Though Ehlers-Danlos syndrome is a rare disorder occurring in approximately 1 per 200,000 persons,[1] ophthalmologists should be aware of the ocular manifestations. Minor ophthalmological changes including prominent epicanthal folds (25%), myopia (8%), and strabismus (7%) are not infrequent but serious ocular complications are rare.[1] However there are few reports of patients with impaired vision from retinal detachment[3],[5] and corneal or scleral perforation.[6] Spontaneous dislocation of lens in Ehlers-Danlos Syndrome is very rare and has been reported only twice.[4] Several reports of subconjunctival dislocation of crystalline lens exist in the literature.[7],[8] Our report of subconjunctival dislocation of lens in a patient of Ehlers-Danlos Syndrome following minor trauma, and its management is the first of its kind. We wish to highlight that in a case of serious ocular trauma (dislocated lens, retinal detachment or corneal/scleral perforation) following minor trauma, the possibility of an underlying connective tissue disorder like Ehlers-Danlos Syndrome should be considered.

These patients present a surgical challenge because of tissue weakness that leads to difficulty in suturing, excessive bleeding, and very slow wound healing. Fragility of the globe may make ocular surgery risky for these patients. Extreme care during surgery, avoiding unnecessary manoeuvers is mandated. Globe fragility also makes scleral buckling risky. Detachments are usually repaired with pars plana vitrectomy and laser photocoagulation. In our case, the scleral wound had self-healed adequately in 3 months, albeit with some scleral thinning. This suggests that in Ehlers-Danlos Syndrome, at least in type VI, scleral healing can take place. It is heartening that in over two years' follow-up, the patient has not developed any surgery-related complication. Of course, evidence based on one case cannot be conclusive but in extremely rare cases such as EDS type VI any observation may be worthwhile.

 
  References Top

1.
Mc Nelis M. Ehlers-Danlos Syndrome. In: Marks ES, Adamczyk DT, Thomann KH, editors. Primary Eye Care in Systemic Disease . Norwalk: Appleton & Lange, 1995. pp. 309-12.  Back to cited text no. 1
    
2.
Hyams SW, Neumann E. Blue sclera and keratoglobus. Ocular signs of a systemic connective tissue disorder. Br J Ophthalmol 1969; 53:53-8.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.
Pemberton JW, Freeman HM, Schepens CL. Familial retinal detachment and the Ehlers-Danlos Syndrome. Arch Ophthalmol 1966; 76:817-24.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.
Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol 1982; 27:143-60.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.
Beighton P. Serious ophthalmological complications in the Ehlers-Danlos Syndrome. Br J Ophthalmol 1970; 54:263-68.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.
Parc C, Legeais JM, Renard G. Posttraumatic scleromalacia in an Ehlers-Danlos Syndrome. J Fr Ophthalmol 1998; 21:761-63.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.
Kramar PO,Brandt DE. Subconjunctival dislocation of the lens. Arch Ophthalmol 1976;94:110-11.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.
Satish S, Chakarbarti A, Pranja V. Traumatic subconjunctival dislocation of the crystalline lens and its surgical management. Opthalmic Surg Lasers 1999; 30:684-86.  Back to cited text no. 8
    


    Figures

  [Figure - 1], [Figure - 2]


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