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Pedunculated episcleral choristoma without ocular involvement

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Eye is the official journal of the Royal College of Ophthalmologists. It aims to provide the practising ophthalmologist with information on the latest clinical and laboratory-based research.
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       
 
   

    
    
 
      
        
 
    
     
   
 

 
 

 


 


 

 
  
 
 


 
  
 

    



  
  
 


 


  
  

 
 

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

  

  
 

  
   

 
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 
 


 

 


 

  
     


 
  

 

      
     
       
   
   
 
   
      
      

 
 
 
 
 
   
 

 

     

 
  
    
 

    

    

  
  

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The simultaneous ipsilateral presence of an epibulbar choristoma and microphthalmia has rarely been reported. We present two such cases, one of which is associated with bone formation, and we consider a possible pathogenetic mechanism.
Article
Choristomas are congenital lesions representing normal tissue(s) in an abnormal location. They are the most common epibulbar and orbital tumors in children. Epibulbar choristomas affect the cornea, limbus or subconjunctival space, and range in appearance from a small, flat lesion to a large mass filling most of the epibulbar region. Astigmatism is often present. Choristomas may be associated with coloboma, Goldenhar syndrome or epidermal nevus syndromes; those associated with the latter are often bilateral and extensive. Choristomas are occasionally familial. Surgery may be indicated to improve vision or cosmesis, or to impede growth. Although choristomas most commonly involve the epibulbar area, they can affect many areas of the eye and orbit, and often affect more than one area.
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We encountered an infant with nevus sebaceus of Jadassohn who was born with a striking pedunculated mass on the superotemporal aspect of the anterior sclera and limbus of the left eye. In addition, two small choroidal colobomas were seen in the papillomacular bundle area of the same eye. Histopathologic examination of the excised mass revealed a mixture of ectodermal and mesodermal elements, including cartilage and bone. These findings led to the rare diagnosis of epibulbar complex choristoma.