Glyxambi
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
  • Users Online: 2212
  • Home
  • Print this page
  • Email this page


 
   Table of Contents      
PHOTO ESSAY
Year : 2019  |  Volume : 67  |  Issue : 7  |  Page : 1161-1162

Intercalary staphyloma in Marfan syndrome: A dreaded complication of scleral incision


Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Date of Submission04-Oct-2018
Date of Acceptance11-Mar-2019
Date of Web Publication25-Jun-2019

Correspondence Address:
Dr. Prafulla K Maharana
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1672_18

Rights and Permissions

Keywords: Ectopia lentis, intercalary staphyloma, intracapsular cataract extraction, Marfan syndrome, scleral incision


How to cite this article:
Sahay P, Dhanda S, Maharana PK, Titiyal JS. Intercalary staphyloma in Marfan syndrome: A dreaded complication of scleral incision. Indian J Ophthalmol 2019;67:1161-2

How to cite this URL:
Sahay P, Dhanda S, Maharana PK, Titiyal JS. Intercalary staphyloma in Marfan syndrome: A dreaded complication of scleral incision. Indian J Ophthalmol [serial online] 2019 [cited 2019 Jul 20];67:1161-2. Available from: http://www.ijo.in/text.asp?2019/67/7/1161/261007



A 28-year-old male patient, known case of Marfan syndrome, presented with complaints of low vision in both eyes since childhood. He gave a clinical history of having undergone intracapsular cataract extraction (ICCE) in the right eye 3 years previously. At presentation, the best spectacle corrected visual acuity (BCVA) was 6/12 and 1/60 in the right eye and left eye, respectively. On slit lamp examination, the right eye had an intercalary staphyloma extending from 11 to 2 o' clock with Descemet scar in the superior cornea [Figure 1]. The iris showed atrophic patches, a fixed and dilated pupil and aphakia with no capsular support [Figure 2]. The left eye had iridodonesis with 8 clock hours of nasal subluxation of a clear crystalline lens [Figure 3]. Fundus was noted to be normal on indirect ophthalmoscopy. Intralenticular lens aspiration was planned for the patient's left eye. However, the patient refused surgery.
Figure 1: Slit lamp photograph of right eye showing an intercalary staphyloma, superior descemet scar, and aphakia

Click here to view
Figure 2: Slit lamp photograph of right eye showing 360° iris atrophy with aphakia

Click here to view
Figure 3: Slit lamp photograph of left eye showing nasally subluxated lens with superotemporal stretched zonules and inferotemporal deficient zonules

Click here to view



  Discussion Top


Marfan syndrome is characterized by abnormalities of the fibrillin protein that is responsible for providing strength and elasticity to the connective tissue.[1],[2] “Ectopia lentis” is a common finding in Marfan syndrome that often requires surgery for visual rehabilitation.[3] The ectopic lens can be removed through a corneal, limbal, or scleral incision. However, there are reports in literature that suggest the risk of staphyloma formation following ocular trauma and scleral incisions in these cases.[4],[5] Goldberg and Ryan[4] reported an intercalary staphyloma in a 5-year-old female child of Marfan syndrome following blunt ocular trauma with a stone. Seelenfreund et al.[5] reported occurrence of an incisional staphyloma following retinal detachment surgery in a case of Marfan syndrome. Therefore, this case highlights an important lesson that any form of trauma to the sclera (surgical or mechanical) should be avoided in cases of Marfan syndrome considering the pre-existing scleral thinning and risk of staphyloma formation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Robinson P, Godfrey M. The molecular genetics of Marfan syndrome and related microfibrillopathies. J Med Genet 2000;37:9–25.  Back to cited text no. 1
    
2.
Nelson LB, Maumenee IH. Ectopia lentis. Surv Ophthalmol 1982;27:143–60.  Back to cited text no. 2
    
3.
Miraldi Utz V, Coussa RG, Traboulsi EI. Surgical management of lens subluxation in Marfan syndrome. J AAPOS 2014;18:140–6.  Back to cited text no. 3
    
4.
Goldberg MF, Ryan SJ. Intercalary staphyloma in Marfan's syndrome. Am J Ophthalmol 1969;67:329–35.  Back to cited text no. 4
    
5.
Seelenfreund MH, Freilich DB, Masculli L. Retinal detachment in a 9-year-old girl with Marfan's syndrome and a limbal incisional staphyloma. Ann Ophthalmol 1976;8:967–8.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Discussion
References
Article Figures

 Article Access Statistics
    Viewed141    
    Printed0    
    Emailed0    
    PDF Downloaded33    
    Comments [Add]    

Recommend this journal