Dataset Information

Boston type 1 keratoprosthesis: the New York Eye and Ear experience.

ABSTRACT: PURPOSE: The Boston keratoprosthesis has had variable success rates in the past. However, significant modifications to design and management have recently led to successful outcomes. This study was undertaken to evaluate the outcomes of the Boston type 1 keratoprosthesis at our institution. METHODS: A retrospective chart review was performed of all Boston type 1 keratoprosthesis procedures conducted at a single practice at the New York Eye and Ear Infirmary from December 2006 to August 2010. Outcome measures included visual acuity, retention rates, and complications. RESULTS: In all, 58 eyes of 51 patients who received a Boston type 1 keratoprosthesis were included. The most common indication for the keratoprosthesis was failed penetrating keratoplasty (PK) (81.0%; mean 2.4±1.3 PKs per eye). Glaucoma was the most common comorbidity (75.9%). Pre-operative best corrected visual acuity (BCVA) was <20/400 in 87.9% of eyes. At last follow-up, 43.1% of eyes had a BCVA of 20/200. Retention rate was 87.9% over an average follow-up of 21.5±11.4 months (median 22 months, range 3-47 months). Complications increased with time, with 65.5% of eyes experiencing at least one event by 6 months and 75.9% by 1 year. The most common post-operative complication was retroprosthetic membrane formation (50.0%). CONCLUSIONS: The Boston type 1 keratoprosthesis provides visual recovery for eyes with multiple PK failures or with poor prognosis for primary PK, showing excellent retention rates. However, there is a trend towards a decline in visual acuity with time and the development of late complications, highlighting a need for longer-term studies.

SUBMITTER: Patel AP

PROVIDER: S-EPMC3298984 | biostudies-other | 2012 Mar

REPOSITORIES: biostudies-other

ACCESS DATA

Similar Datasets

Project description:PurposeTo describe the process and assess outcomes for the first 2 years of newborn screening for severe combined immunodeficiency (SCID NBS) in New York State (NYS).MethodsThe NYS algorithm utilizes a first-tier molecular screen for TRECs (T-cell receptor excision circles), the absence of which is indicative of increased risk of immunodeficiency.ResultsDuring the first 2 years, 485,912 infants were screened for SCID. Repeat specimens were requested from 561 premature and 746 non-premature infants with low or borderline TRECs. A total of 531 infants were referred for diagnostic evaluation leading to identification of 10 infants with SCID and 87 with a clinically significant non-SCID abnormality based on flow cytometry or CBC results (positive predictive value 20.3 %). Nine infants were diagnosed with typical SCID and one with leaky SCID. SCID diagnoses included two patients with adenosine deaminase deficiency, three patients with typical and one with leaky IL2RG-related SCID, one patient with IL7Rα-related SCID, and three cases of typical SCID, etiology unknown. TRECs were undetectable in eight of the nine babies with typical SCID. Infants with other non-SCID conditions included 27 patients with a syndrome that included T-cell impairment, 18 of which had DiGeorge syndrome. Seventeen infants had T-cell impairment secondary to another clinically significant condition, and 13 were classified as 'other'. Among 30 infants classified as idiopathic T-cell lymphopenia, 11 have since resolved, and the remainder continues to be followed. One infant with undetectable TRECs had normal follow-up studies. Molecular studies revealed the presence of two changes in the infant's DNA.ConclusionsOverall, ten infants with SCID were identified during the first 2 years of screening in NYS, yielding an incidence of approximately 1 in 48,500 live births, which is consistent with the incidence observed by other states screening for SCID. The incidence of any clinically significant laboratory abnormality was approximately 1 in 5,000; both estimates are higher than estimates prior to the onset of newborn screening for SCID. Improvements to the NYS algorithm included the addition of a borderline category that reduced the proportion of infants referred for flow cytometric analysis, without decreasing sensitivity. We identified a large number of infants with abnormal TRECs and subsequent idiopathic T-cell lymphopenia. Long-term follow-up studies are needed to determine the prognosis and optimal treatment for this group of patients, some of whom may present with previously unrecognized, transient lymphopenia of infancy.

Dataset Information

Boston type 1 keratoprosthesis: the New York Eye and Ear experience.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets