Familial calcific band-shaped keratopathy: report of two new cases with early recurrence.
We report two siblings with the rare entity of familial calcific band-shaped keratopathy (BSK). Detailed ophthalmic and systemic investigations failed to reveal any underlying causative pathology. Topical disodium ethylenediamine-tetraacetate (EDTA) was applied for 30 min to all four eyes. In addition the right eye of the younger sibling required a superficial keratectomy. An improvement in corneal clarity was seen in the immediate postoperative period in both siblings. Histopathology of the keratectomy specimen revealed linear extracellular sub-epithelial granular calcium deposits. However, an early recurrence was noted in all four eyes at four weeks postoperatively. We report the second instance in the English literature of this entity. Band-shaped keratopathy presenting without an obvious etiology merits a complete systemic and ophthalmic workup. Patients with familial idiopathic BSK could be cases with poor prognosis for treatment with EDTA due to an early recurrence of the disease.
Arora
Ritu
R
Guru Nanak Eye Centre, Department of Pediatrics, Lok Nayak Hospital, and Department of Pathology, Maulana Azad Medical College, University of Delhi, New Delhi-110 002, India.
Shroff
Daraius
D
Kapoor
Seema
S
Nigam
Sonu
S
Narula
Ritesh
R
Chauhan
Deepender
D
Jain
Priyanka
P
D002114:Calcinosis / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003316:Corneal Diseases / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D004492:Edetic Acid / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D012008:Recurrence; D013997:Time Factors; D014792:Visual Acuity
D002614:Chelating Agents; D004492:Edetic Acid