The effect of ascorbic acid on the facility of outflow in normal and buphthalmic rabbits.
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Calcium oxalate crystals, identified morphologically, chemically and histochemically, were found in the eyes of two patients, one with congenital buphthalmia and one with proliferative diabetic retinopathy. The deposits had not been observed clinically or by macroscopic examination during sectioning of the eyeballs. Generalized oxalosis could be ruled out as a source of the oxalates. The crystals were localized in the detached, degenerated retina, subretinally and in the retinal pigment epithelium. Previously, deposition in the last-named layer has been reported only once and only in a case of generalized oxalosis. The literature is reviewed, and suggestions concerning the formation of localized oxalate crystals are advanced. It is concluded that the pigment epithelium is probably involved in the production, perhaps through a changed metabolism.
Thirty-eight children with congenital infantile glaucoma (hydrophthalmia) were examined using electrophysiological methods. In contrast with adult glaucoma, hydrophthalmia is characterized by early changes in the electro-retinogram and rise of the threshold of electrical phosphene, which points to serious pathological changes in the retinal optic nerve. In such cases surgical intervention is indicated.
A family from Turkey with congenital ectopia lentis, likely occurring as an autosomal recessive trait, is presented. No systemic disorders such as homocysteinuria, Marfan's or Weill-Marchesani's syndromes were found in any of the patients. However, all patients except one were less than 2 years old when first examined so that lens luxation must have happened very early in life. Besides almost total lack of zonular threads the patients presented anomalies of ocular dimensions with increased corneal diameters and axial lengths, and in several cases glaucomatous distension of the optic discs could also be found. A possible explanation for these buphthalmic changes and their relation to lens luxation taking place very early in life is proposed. The importance of early diagnosis and treatment is discussed.
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AIM: The role of neoadjuvant chemotherapy was studied when first line enucleation cannot be safely performed in unilateral extensive retinoblastoma (major buphthalmia or radiologically detectable optic nerve involvement). METHODS: Six patients, referred for unilateral retinoblastoma, presented with major buphthalmia (two) or optic nerve invasion (four): they were treated by neoadjuvant chemotherapy using etoposide and carboplatin. RESULTS: Good tumour response was observed in the two patients with buphthalmia and in three of four cases with optic nerve involvement. Meningeal progressive disease was observed in the last patient. The five patients without disease progression were then operated on: anterior enucleation in the patients with buphthalmia and enucleation via a double neurosurgical and ophthalmological approach with prechiasmatic optic nerve section in the other three cases. Postoperative chemotherapy was performed in these five patients. Local radiotherapy to the chiasmatic region and posterior part of the optic canal was necessary in only one patient. The non-operated patient died with disease progression 6 months after the diagnosis. The other five patients are alive with a follow up of 12, 15, 21, 36, and 40 months after stopping treatment. CONCLUSION: Neoadjuvant chemotherapy can be useful in extensive unilateral retinoblastoma with buphthalmia and/or radiological optic nerve invasion at diagnosis.
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