Victor Godel

Further evidence for an autosomal dominant form of oculoauriculovertebral dysplasia

A family with oculoauriculovertebral dysplasia is reported in which there are nine affected members spanning three consecutive generations. It is concluded that despite the known genetic heterogeneity in this disorder, there is an autosomal dominant form. Thus, genetic counseling can only be given after proper completion of all the necessary clinical and family studies.

Congenital Ocular Motor Apraxia – Familial Occurrence

The occurrence of congenital oculomotor apraxia in two children of the same family is described. The improvement of the ocular dysfunction in the older sister gave some hints to the benign nature of the condition and its tendency to recover with age. The possible pathophysiological mechanism underlying this anomaly is discussed.

Hereditary vitreoretinal degeneration, cleft lip and palate, deafness, and skeletal dysplasia.

Two brothers and their mother had vitreoretinal degeneration associated with cleft palate, a peculiar physiognomy, skeletal anomalies, and sensorineural deafness. The pattern of inheritance in this family suggested autosomal dominant transmission.

Hypotony as a Late Complication of Extracapsular Cataract Extraction

Persistent hypotony after extracapsular cataract extraction is an uncommon but significant complication. We observed an unusual late complication of extracapsular cataract extraction—detachment of the ciliary body and the choroid caused by traction of lenticular remnants adherent to the intact posterior capsule.

Computed tomography in ethmoid cell trauma.

Clinical and radiological evaluation of 19 patients with injury to the ethmoid bones following cranio-facial trauma is presented. The correlation of ethmoid trauma and damage to neighbouring structures is discussed. Two cases with damage to the orbits are demonstrated. These findings indicate that orbital involvement is relatively infrequent in association with ethmoid cell trauma. However, when it does occur, severe complications, such as infection and loss of vision may result. Early evaluation by computed tomography is the key to accurate diagnosis and prevention of these complications. Computed tomography is of inestimable value in the detection of minute and subtle bone fragmentation and sequelae.

WAGNER'S VITREORETINAL DEGENERATION WITH GENERALIZED EPIPHYSEAL DYSPLASIA

Wagners vitreoretinal degeneration, midline facial defects and a moderate generalized epiphyseal dysplasia defined radiologically were encountered in a boy and his father. The familial nature of the condition and its autosomal dominant inheritance are described. Suggestions are made that patients said to have Wagners disease differ in no way radiologically from cases diagnosed as having Sticklers syndrome.

ANTERIOR PUPILLARY BLOCK

Two patients with post‐operative adhesions between the iris and cornea are presented. The term of anterior pupillary block is suggested, and its clinical picture is described. The irido‐corneal adherences appeared to be the trigger mechanism of the intraocular pressure rise and deepening of the posterior chamber. The tension was fully controlled following the rupture of these adherences. The beneficial result of a simple procedure of posterior chamber tap is mentioned.

Computerized tomography in conditions concomitantly involving the orbits and the paranasal sinuses

Forty-three patients with computerized tomographic (CT) findings demonstrating concomitant orbital and paranasal sinus involvement are presented. Exophthalmos was the most common presenting symptom. Ethmoid and maxillary sinuses were most commonly affected. CT findings were of help in evaluating the extent of the various disease processes and are mandatory for treatment planning. Attention is drawn to the common association of paranasal lesion and ophthalmic manifestations. The combination of clinical presentation and radiological findings might indicate certain etiologies.

Cataracts Due to Repeated Osmotic Insults

The long-term effect of repeated osmotic insults were investigated and the lenticular changes due to hyperosmosis were described. The results of these experiments demonstrated that subsequent hyperosmotic insults to hamster lenses by exposure of the eye and to rabbit lenses by intracameral injection of hypertonic solution did not cause permanent lenticular damages being completely reversible and not harmful.

Best’s Vitelliform Macular Dystrophy Subretinal Neovascularization

Best’s Vitelliform Macular Dystrophy (BVMD) is one of the most pleomorphic of the hereditary macular degenerations with respect to its manifestation in the ocular fundus(1). The presence of the disease is heralded by the awareness of the typical egg-yolk abnormality in the macula and the pathog nomonic electrooculogram. This macular dystrophy with its autosomal dominant inheritance is characterized by a progressive temporal course that may have an awkwardly broad spectrum of appearances in various morphologic stages.