[Congenital ectropion in ichthyosis congenita mitis and gravis]. / Angeborenes Ektropium bei Ichthyosis congenita mitis und gravis.

BACKGROUND: Congenital ichthyosis is a generalized hyperkeratinization of the skin at birth. Depending on clinical aspects and severity, three forms of congenital ichthyosis have been defined: mitis, tarda, and gravis. Desquamation of the parchment-like hyperkeratinized skin begins shortly after birth and may require several weeks to complete. Skin alterations in the eyelid cause shortening of the anterior lamella, subsequently resulting in ectropion. This affects the upper eyelid more often than the lower and can lead to complications such as chronic palpebral or bulbar conjunctivitis and keratinization or exposure keratopathy. Here we present two case reports illustrating the course of ichthyosis congenita mitis and gravis. PATIENTS AND METHODS: Patient 1 (ichthyosis congenita mitis): a male baby prematurely born at 34+2 weeks of gestation was delivered by cesarean section. The entire body was covered by a parchment-like hyperkeratinized skin. Both eyes showed ectropion of the upper and the lower eyelid, which was more obvious with enforced lid closure. Frequent application of external ointment and spontaneous desquamation led to resolution of the ectropion. Patient 2 (ichthyosis congenita gravis): a male baby prematurely born at 35+4 weeks of gestation was delivered by cesarean section. At birth the child showed the signs of a collodion baby with ectropion of all four eyelids in combination with a characteristic "fish mouth" and rudimentary external ears. The child died on the 14th day of life of septicaemia. CONCLUSION: In mild forms of congenital ichthyosis surgical treatment of eyelid ectropion is not required. In more severe cases a skin graft may become necessary. Various although limited sources of graft material which are discussed can be considered.

Senile schisis detachment with posterior outer layer breaks.

BACKGROUND: Senile retinoschisis with associated retinal detachment in the presence of multiple or large posterior outer layer breaks is rare. Despite many and significant developments in vitreoretinal surgery for this situation, no definitive therapeutic guidelines exist. In an observational study three different approaches performed by two of the authors are presented. METHODS: Eighteen eyes of 18 patients with symptomatic schisis detachment and large posterior outer layer breaks were analyzed retrospectively. The fovea was detached in six eyes. Four eyes had laser photocoagulation and/or transscleral cryopexy (Group I), and five eyes had extensive scleral buckling (Group II). In nine eyes primary vitrectomy and gas endotamponade were performed, including inner layer resection in two eyes (Group III). The median follow-up period was 13 months. RESULTS: Initial anatomic success, which was defined as complete reattachment of the outer retinal layer, was achieved in two of four eyes of Group I, in four of five eyes of Group II, and in seven of nine eyes of Group III. Inner layer separation persisted in 11 of 16 eyes. Visual acuity improved in 3 of 18 eyes, remained unchanged in 9 of 18 eyes, and worsened in 6 of 18 eyes. Complications included in Group I were persistent outer retinal layer detachment (two eyes); in Group II, proliferative vitreoretinopathy (PVR) detachment (one eye), CME (one eye), diplopia (one eye), late redetachment (one eye); in Group III, secondary rhegmatogenous detachment (one eye) PVR detachment (two eyes), macular pucker (one eye), preexisting CME (two eyes), subretinal perfluorocarbon liquid (one eye), transient central serous detachment (one eye), and nuclear sclerosis (five of eight eyes). CONCLUSION: Photocoagulation alone may be considered a first approach in selected cases of schisis detachment complicated by large posterior outer layer breaks. By use of present surgical techniques, today pars plana vitrectomy is the most rational approach allowing an exact evaluation of the complex retinal architecture, a controlled coagulation, and an adequate tamponade of the involved area. Resection of the inner layer seems to increase the risk for epiretinal membrane formation.

Results of vitrectomy and the no-touch-technique using autologous adjuvants in macular hole treatment.

BACKGROUND: Anatomic and functional results of macular holes using vitrectomy and platelet-concentrate (PC) or autologous whole blood (WB) were investigated without peeling any epiretinal membrane in order to minimize retinal trauma. METHODS: 44 patients with macular holes stage II, III and IV underwent a vitrectomy with removal of posterior cortical vitreous (in stage II and III) and C2F6 gas endotamponade. No peeling of any epiretinal membrane or the ILM was performed. In 14 patients autologous WB was injected and in 30 patients autologous PC. The mean follow-up time was 9.3 months. RESULTS: An anatomic closure was observed in 36.4% of patients in the WB-group and in 93.9% of patients in the PC-group. SLO-microperimetry showed a reduction of absolute scotomas in 80% of the WB- and in 75% of the PC-group. In the WB-group 1 retinal detachment and 1 branch vein occlusion occurred; in the PC-group 3 retinal detachments, 1 central artery occlusion, 1 macular pucker, 1 cystoid macular edema, 1 late reopening and 1 secondary glaucoma were observed. In both groups no endophthalmitis occurred. CONCLUSION: If autologous platelet concentrate is available, high anatomic success rates can be achieved without aggressive membrane removal, which may cause retinal damage. Autologous whole blood cannot be recommended as an alternative adjunct for the treatment of macular holes without membrane removal.