[Elastolysis of the eyelids. A rare cause of ptosis]. / Elastolyse der Lider. Seltene Ursache einer Ptosis.

BACKGROUND: Mid-dermal elastolysis is a rare disorder of elastic tissue which is characterized clinically by wrinkling of the skin and histologically by the selective absence of elastic fibers in the mid dermis. Two patients with severe ptosis and a loss of the lateral tarsal suspension are reported. PATIENTS AND METHODS: A 36-year-old male patient presented with bilateral ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. With normal clinical and routine laboratory investigations, punch biopsy revealed clear signs of mid-dermal elastolysis. Plastic surgery of both eyelids (levator advancement) was performed with good success after a 1-year interval without progression of ptosis. Another 37 year old patient suffered from mild ptosis, skin atrophy of the eyelids and a loss of lateral tarsal suspension. As the patient chose not to have surgical treatment, he was treated with oral corticosteroids. To differentiate between an acute and a chronic type of elastolysis a punch biopsy is required. In addition, we want to highlight the surgical option in chronic and non-progressive cases. CONCLUSIONS: In contrast to formerly reported localized forms of acute acquired cutis laxa with complete loss of elastic fibres in the reticular and papillary dermis, we report a chronic type of elastolysis in these patients. In our experience in cases without any apparent progression of symptoms a surgical repair can provide long-term success.

[Unilateral blepharochalasis with IgA-deposits]. / Unilaterale Blepharochalasis mit läsionalen IgA-Ablagerungen.

A 36-year-old male patient presented with unilateral periocular skin atrophy. The blepharochalasis developed without any obvious inflammation of the eyelids over the past 10 years. Interestingly, elongated blood vessels and microaneurysmatic vessel changes were found in the tarsal conjunctiva. A punch biopsy revealed a nearly complete loss of elastic fibres in the papillary and superficial reticular dermis. The contralateral side was histopathologically normal. On immunohistology IgA-deposits could be observed especially on perifollicular elastic fibres. Immunoelectronmicroscopy confirmed the diagnosis and suggested fibulin and fibronectin as potential binding sites for the autoantibodies. This further report of elastolysis in association with IgA-autoantibodies defines the autoantibody binding site in more detail and suggests that the immune mechanisms may also play a role in vessel changes of the conjunctiva.

CPEO associated with a single nucleotide deletion in the mitochondrial tRNA(Tyr) gene.

In the muscle biopsy of a female patient with chronic progressive external ophthalmoplegia (CPEO), myopathy, and exercise intolerance, the heteroplasmic deletion of a single nucleotide (DeltaT5885) in the mitochondrial tRNA tyrosine gene (tRNA(Tyr)) was found. The mutation was associated with the mitochondrial phenotype of individual muscle fibers, suggesting a causal association of DeltaT5885 with the mitochondrial disease phenotype. The microdeletion was absent from the patient's and her relatives' blood, indicating a spontaneous somatic origin.

Recurrent keratoconus in a patient with Leber congenital amaurosis.

PURPOSE: Clinical history of a 17-year-old patient with Leber congenital amaurosis (LCA) with histologically proven recurrent keratoconus (KC) two years after corneal transplantation in one eye and a recurrence-like appearance with a more global contour on the other eye four years after corneal grafting is reported. The possible mechanisms for this recurrence are discussed in light of the fact that this is, to the best of our knowledge, the first penetrating keratoplasty reported in LCA. METHODS: Computerized videokeratography (CVKG) and specular microscopy were performed preoperatively. The patient underwent regrafting, and the excised corneal button was examined by light microscopy and transmission electron microscopy. RESULTS: Analysis of CVKG showed a keratoconus-like pattern on the right eye, with the left eye demonstrating the aspects usually seen in keratoglobus. Histologic examination revealed the features usually observed in progressed keratoconus. CONCLUSION: Recurrence of keratoconus in a graft has not yet been described after such a short time until now. A "true" recurrence of the disease is postulated; it could be caused by an "aggressive" genetic factor that also leads to the frequent KC in patients with LCA. This mechanism also could explain the high incidence and rapid progress of KC in this disease.

[Rapid eye fatigue--causes and therapy]. / Rasche Ermüdung der Augen--Ursachen und Therapie.

Tired eyes even after a short period of strain are well known as asthenopia. This term derives from a Greek expression meaning 'weak eye'. The following reasons are to be taken into account: uncorrected refractional errors such as hyperopia and astigmatism, accommodative and fusional defects and heterophoria, or combinations there of. With many cases of asthenopia the clinical symptomatology may resemble an organic eye disease or an affection of the brain [dry eye, Graves' disease, myasthenia, special form of essential blepharospasm, neurogenic latent eye muscle palsies, narcolepsy]. It is the aim of a precise patient's history to differentiate between functional and organic causes. By means of case reports and diagnostic tools, the development of symptomatology and the therapy are discussed.

[Unusual forced head postures in congenital paralysis of the superior oblique muscle]. / Ungewöhnliche Kopfzwangshaltungen bei kongenitaler Parese des M. obliquus superior.

BACKGROUND: With congenital superior oblique palsy, an abnormal head posture which is different from the usual one (head tilt) has hardly been described. We performed this study to point out the spectrum of atypical head posture and its operative treatment procedures. PATIENTS: The charts of all 103 patients with congenital superior oblique palsy which were seen at our clinic between 1983 and 1993 were reviewed. RESULTS: 13 patients (13%) had an atypical head posture. Group 1: patients with a face turn to the non-involved side (n = 5). The vertical deviation increased abruptly starting from the primary position; is was comitant in adduction. Combined surgery of the obliques muscles was most often performed (n = 3). Group 2: patients with a vertical abnormal head posture. 3 Patients presented with a chin elevation. Their vertical deviation was incomitant, it was smallest in downgaze. An isolated recession of the inferior oblique muscle was performed in all cases. One patient had a chin depression; she also had an esotropia in downgaze. A recession of the inferior oblique muscle was performed. Group 3: patients with a face turn and a chin elevation (n = 4). Vertical deviation was maximal in adduction and was smallest in the lower and temporal field of gaze. We performed first a recession of the inferior oblique muscle and then-if necessary-a tuck of the superior oblique muscle or a recession of the contralateral inferior rectus muscle. CONCLUSIONS: An atypical head posture can occur in about 10% of cases. Its cause can be explained after checking the incomitance of the vertical deviation and the motility disorder. These parameters also determine the operative procedures.

[Orbital wall fracture from the ophthalmologic viewpoint]. / Die Orbitawandfraktur aus ophthalmologischer Sicht.

The fracture of the orbital wall is characterized by a typical ophthalmological symptomatology, which is repeated more or less completely with each patient. Beside the optic nerve lesion, which is rare, the motility disturbance of the globe is the severest consequence of an orbital wall fracture. It is the ophthalmologist's task to correctly interpret the motility disturbance caused by an orbital wall fracture and to distinguish it from ocular motility disturbances of other origin. Thus he has decisive influence on the indication of an operation. After a short presentation of how an orbital wall fracture comes into being and its connection with a restricted motility of the globe, the diagnostic steps are described. Two questions important for the daily practice are discussed with the help of 161 case histories: 1. the connection between the development of the motility disturbance and the interval between the day of the accident and the day of the operation; 2. the influence of the type of the fracture on the kind and the extent of the motility disturbance and the time required for its complete recovery. It has been observed that the factor time, that is the fastest possible operative revision, does not, as assumed before, play the most important role for the normalisation of the globe's motility. Even late reconstructions of orbital wall fractures achieve good functional results. Only a fracture of the zygomatic bone healed in dislocation cannot be brought back to its proper anatomical position after a period of 4 to 5 weeks. Functionally it is, however, of minor importance. But there is an obvious connection between the type of the fracture and the extent as well as the regression of the motility disturbance of the globe. With isolated fractures of the zygomatic bone there are practically no motility restrictions, with combined fractures of the orbital entrance and the orbital floor there can but there need not be a motility disturbance. Severe motility disturbances are almost always caused by isolated blow out-fractures, especially with the trap-door mechanism and with fractures of the medial orbital wall. Even after an exact anatomical operative reconstruction motility disturbances in connection with medial orbital wall fractures remain for months, whereas they disappear 5 weeks after the operation at the latest with the other types of fractures.(ABSTRACT TRUNCATED AT 400 WORDS)

[Experience with evacuable maxillary sinus endothesis for orbital and maxillary trauma]. / Erfahrungen mit der evakuierbaren anatomischen Kieferhöhlenedothese beim Orbita- und Oberkiefertrauma.

Results with a deflatable anatomically designed Endothesis for the reduction of fractures of the antral walls, especially the orbital floor. For the treatment of the fractures of the antral walls, especially of the extended fractures of the orbital floor, after repositioning through the maxillary sinus, treatment is necessary which supports the maxillary sinus and which, being effective in all directions, protects the ciliated epithelium and guaranties the drainage of secretions. After anatomical preexaminations three differently large hollow models for the maxillary sinus made of thin-walled silicon were developed. These "Endotheses" were implanted through a facial window into the maxillary sinus and are to be filled by way of nasal drain with contrast fluid. 51 patients were subjected to clinical, x-ray and, in selected cases, endoscopic post-control.

[Contact lens correction of post-traumatic unilateral aphakia. Late visual and orthoptic results]. / Die Kontaktlinsenkorrektur der posttraumatischen einseitigen Aphakie. Visuelle und orthoptische Spätergebnisse.

From 1978 to 1981 total of 122 perforating eye injuries were treated in the eye department of the Salzburg provincial clinic. In 25% of the cases treated the opacified lens had to be removed. The authors followed up 35 patients with unilateral post-traumatic aphakia. Of these, 27 proved suitable candidates for contact lenses. Visual function was good in 15 patients, but reduced in 12 due to post-traumatic changes. Ten patients achieved binocular vision, while in 12 it was possible to restore binocular vision by appropriate measures. The paper deals particular with the restoration of binocular vision and maintenance of binocular function in cases of unilateral post-traumatic aphakia.

[Bifocal glasses for non-presbyopic patients (author's transl)]. / Die Bifokalbrille ausserhalb ds presbyopen alters.

A disturbance of the physiological relation between accommodation and convergence which is defined by the AC/C ratio results in a manifest convergent squint. After a short reference to the typical accommodative esotropia, the atypical accommodative esotropia ("convergence excess") and its treatment with bifocal lenses are discussed in greater detail. Bifocal glasses are also used in the following disturbances of binocular vision: in partially accommodative convergent squint with a larger deviation in near fixation, in primary and postoperative microtropia with a larger deviation in near fixation, in esophoria with signs of decompensation in near fixation, with an intermittent exotropia of the divergence excess type. In the last-mentioned case they are use exclusively in preoperative diagnostics. The use of bifocals is simple and does not take much time; contrary to cyclospastic drugs, it has no undesirable side effects. Experience has shown that even after wearing bifocals for years there is no danger of a permanent accommodation insufficiency.

[Is there a typical motility disturbance in orbital wall fractures (author's transl)]. / Wie typisch ist die Motilitätsstörung bei Orbitawandfrakturen.

In case of motility disturbances due to orbital fractures generally a vertical diplopia -- that means a vertical restriction of the motility of the globe -- is found. The classification into four types according to Friemel allows a differentiation between mechanical motility disturbances and those of other origin; furthermore a prediction can be made about the extent of the field of diplopia and about the involved position of gaze. In addition the urgency of a primary reconstruction of the orbit and the prognosis of a late eye muscle surgery can be estimated by this classification. It is of differential diagnostic importance that isolated injuries of the eye muscles and the check ligaments, an intraorbital haemorrhage and a subtotal paresis of the oculomotor nerve can show a similar clinical picture as usually found with orbital fractures. The early diagnosis of ocular motility disturbances caused by orbital trauma is of essential importance for the proper treatment and the recovery of binocular single vision.