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Unlabelled: In recent years good results after treatment of different superficial corneal pathologies with the excimer laser 193 nm have been reported. Thiel-Behnke dystrophy is a hereditary form of anterior stromal dystrophy, a promising layer for successful phototherapeutic keratectomy (PTK). This type of dystrophy was first described in 1967 in Kiel., Patients and Methods: Ten members of the original family of 1967 were re-examined at the University Eye Hospital, Kiel. A phototherapeutic keratectomy was performed in six eyes because of the typical honeycomb corneal surface irregularity and superficial haze. Two of these eyes had a recurrence of the primary disease on the graft. Four eyes had been untreated so far. The best spectacle corrected visual acuity preoperatively was between 20/200 and 20/40. For all treatments the MEL 60 excimer laser (Aesculap Meditec) was used. To even out the peaks and valleys of the irregular surfaces, modulating agents were applied in decreasing concentrations. The follow-up period is now between 8 months and 4 years., Results: All eyes showed significant visual improvement postoperatively with a best spectacle corrected visual acuity of 20/32 and higher. In one of the postkeratoplasty eyes with the longest follow-up after PTK, the original disease has begun to recur., Conclusions: Phototherapeutic keratectomy is a successful therapy for Thiel-Behnke dystrophy; it results in quick recovery of patients and their vision. Stable visual acuity and corneal transparency have been observed that last for years. Recurrences after penetrating keratoplasty or after PTK can be positively retreated. A (re-) keratoplasty may be postponed or avoided.

Background: Combined malformations (hamartomas) of the retina and the retinal pigment epithelium are extremely rare. Confusion of these pigmented lesions with choroidal melanomas or other malignancies is possible., Materials and Methods: We present the cases of a 7-year-old girl who developed accommodative convergent strabism and the case of an 8-year-old boy with deterioration of visual acuity in the left eye. On examination the anterior segments were normal in both cases. Funduscopically both eyes showed a centrally located large, partly pigmented and slightly prominent tumor, which extended fanlike into the normal-appearing retina. Both lesions were partly obscured by grayish-white, semitranslucent and thickened retina with retinal folds and overlying tortuous vessels. On follow-up examination one lesion seemed to be slightly more pigmented whereas the other lesion remained unchanged., Results and Discussion: Combined malformations of the retina and the retinal pigment epithelium were originally described as clinical entities (hamartomas) by Gass in 1973. A few additional cases were subsequently published by McLean in 1976, by Laqua and Wessing in 1979 and some other authors. Laqua and Wessing were the first to give a report of a histologic study in one case with a macular lesion that showed two distinguishable components. The outer pigmented portion consisted of hypertrophied retinal pigment epithelium; the inner unpigmented portion consisted of a malformed and thickened retina which, however, showed normal layering. The clinical characteristics are usually diagnostic. Fluorescein angiography is helpful in establishing the diagnosis and ruling out differential diagnoses., Conclusion: Although long-term follow-up did not show evidence of growth patients with combined malformation of the retina and retinal pigment epithelium should be followed up regularly.