Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management.

The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the ß globin gene resulting in the substitution of glutamic acid by valine at position 6 of the ß globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

Spectral-Domain OPTICAL COHERENCE TOMOGRAPHY of a Macular Fold AFTER Retinal Detachment Repair.

PURPOSE: We report a case with a dry macular fold after retinal detachment repair using spectral-domain optical coherence tomography images (SD-OCT) to evaluate details of the retinal architecture before and after surgical management. METHODS: A 62-year-old man diagnosed with macula-threatening rhegmatogenous retinal detachment underwent primary vitrectomy with an encircling sclera buckle. On post-operative day 5, he was noted to have developed a juxtafoveal retinal fold. SD-OCT was used to image the retinal fold and documented apposition of the retinal layers and discontinuity within the photoreceptor layer in the vicinity of the retinal fold. RESULTS: The attempts to detach and unfold the retina appeared minimally successful intraoperatively. However, as the gas bubble decreased, the retinal fold was noted to have resolved. Repeat SD-OCT documented flattening of the retinal fold with a well-preserved photoreceptor layer. CONCLUSION: This outcome suggests the possibility of delayed postoperative success in flattening a retinal fold despite resistance to manipulation and failed attempts during the course of the surgery. To our knowledge, this is the first report that demonstrates this finding within the macular architecture after retinal detachment surgery in vivo using spectral-domain optical coherence tomography. These findings may have an important role in management of these cases and may become prognostic indicators based on the alterations in retinal anatomy demonstrated by high-resolution imaging.