Corneal rupture management with amniotic membrane graft in a patient with Brittle cornea syndrome

Purpose. This case series demonstrate diagnostic features, treatment options, and challenges for Brittle Cornea Syndrome. Observations. Three cases presented with bluish sclera and extremely thin cornea. Genetic workup was performed and confirmed the diagnosis of Brittle Cornea Syndrome, a rare autosomal recessive disorder characterized by corneal thinning and blue sclera. Case 1 was a 4-year-old boy who developed cataract and glaucoma after undergoing right tectonic penetrating keratoplasty (PK) secondary to a spontaneous corneal rupture. Glaucoma was controlled medically. Later, the kid underwent right transcorneal lensectomy and vitrectomy with synechiolysis. After 6 weeks, he sustained graft dehiscence that was repaired using onlay patch graft. Case 2 was a 7-year-old boy who underwent PK in the right eye, then a pericardial patch graft in the left eye following spontaneous corneal rupture. Glaucoma in both eyes was controlled medically. Case 3 was the 2-year-old sister of the 2nd case. She had a pachymetry of 238 μm OD and 254 μm OS. In the 3 cases, parents were instructed to take protective measures for both eyes and to continue with follow-up visits. Also, they were instructed to have regular screenings for late-onset hearing loss, dental abnormalities, and bone deformities. Conclusions. Long-term follow-up of children diagnosed with Brittle Cornea Syndrome is paramount to minimize the morbidity of corneal rupture and late-onset extraocular conditions.

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Brittle Cornea Syndrome: Case Report with Novel Mutation in thePRDM5Gene and Review of the Literature

Case Reports in Ophthalmological Medicine ◽

10.1155/2015/637084 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 3

Author(s):

Georgia Avgitidou ◽

Sebastian Siebelmann ◽

Bjoern Bachmann ◽

Juergen Kohlhase ◽

Ludwig M. Heindl ◽

...

Keyword(s):

Novel Mutation ◽

Clinical Findings ◽

Corneal Scarring ◽

Brittle Cornea Syndrome ◽

Exon 1 ◽

Corneal Hydrops ◽

The Right ◽

Almost All ◽

Pr Domain ◽

Corneal Rupture

A 3-year-old boy presented with acute corneal hydrops on the left eye and spontaneous corneal rupture on the right eye. A diagnosis of brittle cornea syndrome was confirmed by molecular analysis. A novel mutation, the homozygous variant c.17T>G, p.V6G, was found in the gene for PR-domain-containing protein 5 (PRDM5) in exon 1. Brittle cornea syndrome is a rare connective tissue disease with typical ocular, auditory, musculoskeletal, and cutaneous disorders. Almost all patients suffer from declined vision due to corneal scarring, thinning, and rupture. The most common ophthalmologic findings include keratoconus, progressive central corneal thinning, high myopia, irregular astigmatism, retinal detachment, and high risk for spontaneous corneal or scleral rupture. In addition to describing the case with a novel mutation here we review the current literature on brittle cornea syndrome pathogenesis, clinical findings, and therapy.

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Bilateral Spontaneous Corneal Rupture in Brittle Cornea Syndrome

Cornea ◽

10.1097/00003226-199909000-00019 ◽

1999 ◽

Vol 18 (5) ◽

pp. 621 ◽

Cited By ~ 12

Author(s):

Luis Izquierdo ◽

Mark J. Mannis ◽

Peter B. Marsh ◽

Samuel P. Yang ◽

J. Martin McCarthy

Keyword(s):

Brittle Cornea Syndrome ◽

Corneal Rupture

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PENGGUNAAN SELAPUT AMNION SEGAR PADA INSISI LUKA OPERASI SEKSIO SESAREA

JOURNAL OBGIN EMAS ◽

10.25077/aoj.1.2.47-55.2017 ◽

2019 ◽

Vol 1 (2) ◽

pp. 47-55

Author(s):

Syahredi Syaiful Adnani ◽

Hafni Bachtiar

Keyword(s):

Wound Healing ◽

Caesarean Section ◽

Amniotic Membrane ◽

Healing Time ◽

Cost Of Care ◽

Control Group ◽

P Value ◽

Control Groups ◽

And Control ◽

Wound Healing Time

In the last few decades, the incidence of caesarean section is increasing in the world, especially in Indonesia. One of the way to treat tissue scar is through biologic and synthetic dressing where nowadays, amnion has been used as biologic dressing frequently. This study was conducted to determine the effect of the use of fresh amniotic membrane on wound incision Caesarean section compared with Caesarean section incision wound covered using regular gauze bandages and fixated with plaster in RS. Dr. Reksodiwiryo Padang. The design of this study is an experimen-tal study with Post test design with control group design. Sampling was done using a formula consecutive sampling two different test samples obtained an average of 72 people for each group. The analysis used include univariate and bivariate analyzes. The average wound healing time the difference was statistically significant (p value <0.05) in the treatment and control groups. There was highly significant difference in the proportion of local infection on day 3 between the treatment and control groups (p value <0.05). There were very significant differences in the proportion of local allergic reactions at day 3, and 5 between the treatment and control group (p <0.05). There are significant differences in terms of the cost of care per day between treatment and control groups (p <0.05). From this study, the average wound healing time has a very significant difference.Keywords: Fresh Amniotic Membrane, Wound Cesarean Section, Wound Healing

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