[Correlation of retinal thickness with the extent of Purtscher's retinopathy]. / Korrelation der Netzhautdicke mit dem Verlauf der Purtscher-Retinopathie.

BACKGROUND: The course of Purtscher's retinopathy (PR) or Purtscher-like retinopathy (P-lR) is dependent on time, length, and expression of confluent cotton-wool spots. To correlate the course of the disease with findings of optical coherence tomography (OCT), we present two extreme courses of PR and P-lR. METHODS: Complete ophthalmological examination plus electroretinography (ERG) and OCT were performed. In the first case the follow-up was carried out until complete resorption of the edema and in the second case 8 years after the occurrence of P-lR. RESULTS: An increase of the central retinal thickness (308-430 microm was observed during the acute phase of PR. Normalization of visual acuity and central retinal thickness to 210-273 microm with an ERG within normal limits (35 ms) was achieved after 8 weeks. In the course of P-lR the marked edema was visible even after 6 months and a markedly reduced neuroretina (112-120 microm) was measured after 8 years. CONCLUSIONS: Retinal thickness analysis correlates well with organic functions in the cases of PR or P-lR. Fast reduction of the edema was associated with a good prognosis for visual acuity.

[Retinal vein branch occlusion and palsy of the N. abducens in protein S deficiency]. / Venenastverschluss und Abduzensparese bei Protein-S-Mangel. Eine genealogische Untersuchung.

BACKGROUND: Protein S deficiency, which exists in 0.7% of the population, is a risk factor for retinal vein branch occlusions and is inherited in an autosomal dominant manner. METHODS: A genealogical study was carried out on three generations of one family who exhibited different venous occlusions and subsequent complications. RESULTS: Four members of the family, spanning three generations, suffered from complications of venous thrombosis. In the first generation a great uncle died of complications from a deep leg venous thrombosis. In the second generation, the mother underwent a venous branch thrombosis at the age of 41 with a protein S activity of 18%. Subsequently, a palsy of the N. abducens developed with multiple cerebral lesions (presumably post-thrombotic) in the MRI. Fluorescein angiography showed a typical picture of a venous branch occlusion which had been treated by laser. In the third generation, the 16-year-old daughter developed iliac venous thrombosis and a pulmonary embolism with a protein S activity of 0%. The fluorescein angiography showed distinctively engorged veins. A 28-year-old daughter, with a protein S activity of 16%, remained asymptomatic, although fluorescein angiography demonstrated engorged veins. Protein C activity and APC resistance of all family members were normal. The chromosomal analysis of the family members revealed no morphological aberrations. CONCLUSION: Protein S deficiency increases the risk of congenital thrombosis in young and middle-aged heterozygous individuals.