Literature DB >> 23704443

Porphyria: varied ocular manifestations and management.

Alok Sati1, Virender S Sangwan, Sayan Basu.   

Abstract

On review of past 10 years medical records, we could find four typical cases of porphyria with rare ocular manifestations. Cases 1, 2 and 4 have presented with features suggestive of acute scleritis. Based on clinical, biochemical and dermatological evaluation, all these three cases were diagnosed to have congenital erythropoietic porphyria. Case 1 was initially managed with scleral patch graft which on subsequent melt was managed with double layered amniotic membrane grafting along with conjunctival advancement and lateral paramedian tarsorrhaphy in both the eyes. Cases 2 and 4 were managed conservatively with artificial tear drops and general protective measures. Case 3 was presented with multiple failed grafts due to repeated ulceration and infection. Owing to multiple failed grafts, Boston keratoprosthesis was done and the patient is doing well with stable kertaoprosthesis at the last follow-up visit.

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Mesh:

Year:  2013        PMID: 23704443      PMCID: PMC3669952          DOI: 10.1136/bcr-2013-009496

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  9 in total

Review 1.  Congenital porphyria with necrotizing scleritis in a 9-year-old child.

Authors:  P Venkatesh; S P Garg; E Kumaran; H K Tewari
Journal:  Clin Exp Ophthalmol       Date:  2000-08       Impact factor: 4.207

2.  Photodamage of the conjunctiva in patients with porphyria cutanea tarda.

Authors:  H Hammer; I Korom
Journal:  Br J Ophthalmol       Date:  1992-10       Impact factor: 4.638

3.  Sight threatening complications in porphyria cutanea tarda.

Authors:  A G Zaborowski; G H Paulson; A L Peters
Journal:  Eye (Lond)       Date:  2004-09       Impact factor: 3.775

4.  Porphyria cutanea tarda.

Authors:  Patrick Yachimski; Nirali Shah; Raymond T Chung
Journal:  Clin Gastroenterol Hepatol       Date:  2007-02       Impact factor: 11.382

5.  Cicatricial ectropion and lacrimal obstruction associated with the sclerodermoid variant of porphyria cutanea tarda.

Authors:  A J Sober; A S Grove; J E Muhlbauer
Journal:  Am J Ophthalmol       Date:  1981-03       Impact factor: 5.258

6.  Scleral necrosis in a patient with congenital erythropoietic porphyria.

Authors:  Sana S Siddique; Luis A Gonzalez-Gonzalez; Pranjal Thakuria; Peter Y Chang; C Stephen Foster
Journal:  Cornea       Date:  2011-01       Impact factor: 2.651

7.  Ocular complications in 2 cases with porphyria.

Authors:  Ugur E Altiparmak; Yusuf Oflu; Fatma Akbas Kocaoglu; Yasemin Aslan Katircioglu; Sunay Duman
Journal:  Cornea       Date:  2008-10       Impact factor: 2.651

8.  Boston keratoprosthesis for visual rehabilitation in porphyria cutanea tarda.

Authors:  Alok Sati; Virender S Sangwan; Sayan Basu; Parthasarathi Kalaiselvan
Journal:  BMJ Case Rep       Date:  2013-02-01

9.  Acute scleritis as a manifestation of congenital erythropoietic porphyria.

Authors:  M P Veenashree; Virender S Sangwan; Geeta K Vemuganti; Anchala Parthasaradhi
Journal:  Cornea       Date:  2002-07       Impact factor: 2.651

  9 in total
  3 in total

1.  Allogeneic corneoscleral limbus tissue transplantation for treatment of the necrosis in porphyria eye disease.

Authors:  Feng Yan; Yan Lu; Jie Yin; Feng Jiang; Zhen-Ping Huang
Journal:  Int J Ophthalmol       Date:  2014-08-18       Impact factor: 1.779

2.  Scleral necrosis in congenital erythropoietic porphyria: A case report and review of the literature.

Authors:  Shweta Agarwal; Parthopratim Dutta Majumder; Bhaskar Srinivasan; Geetha Iyer
Journal:  Oman J Ophthalmol       Date:  2015 Sep-Dec

3.  Scleral Compromise in Hereditary Porphyria Cutanea Tarda.

Authors:  Eugenia Andrea Paez Soria; Federico Andres Cremona; Francisco Lucero Saá; Pablo Chiaradía
Journal:  J Curr Ophthalmol       Date:  2021-03-26
  3 in total

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