Comparison of totally implanted and external catheters in paediatric oncology patients.

From June 1982 until December 1989, 93 permanent central venous catheters [59 external catheters (ECs) and 34 implanted catheters (ICs)] were placed in 69 patients. The median age of these patients at placement was 5.6 years for ECs and 8.8 years for ICs (P less than 0.05). Follow-up evaluation was possible on 86 catheters (58 ECs and 28 ICs). The median time of insertion was 236 days and 316 days for ECs and ICs, respectively (P less than 0.05). The median number of open days was 58 for ECs and 66 for ICs (not significant). 17 catheters (6 ECs and 11 ICs) were transiently obstructed (P less than 0.005). 30 episodes of bacteraemia were documented in 20 patients. The incidence of catheter sepsis and bacteraemia of unknown source was one in 278 and 283 open days for ECs and ICs, respectively (not significant). In this retrospective study, ECs appeared to be as safe as ICs when infection was correlated with use of the catheter, but this finding should be confirmed in a randomised design.

Gelatinous drop-like dystrophy. A form of primary corneal amyloidosis.

A keratoplasty was performed on the right eye of a 12-year-old boy affected by gelatinous drop-like corneal dystrophy. This rare form of primary corneal amyloidosis has been more often reported in Japanese than in occidental literature. In the case presented here, the occasional fusiform deposits of amyloid observed in the stroma suggest a relationship between gelatinous drop-like corneal dystrophy and lattice dystrophy. A new classification of the different types of primary corneal amyloidosis is proposed.

Corneal trauma from projection of metallic mercury into the eyes.

A 24-year-old woman sustained bilateral ocular lesions due to projection of mercury into the eyes during an explosion in a chemical laboratory. A lamellar keratectomy was performed on the right eye four hours after the accident, and 12 days later, the same procedure was performed of the left eye. Studies by light and electron microscopy were done on both specimens. The essential findings were total loss of epithelium, necrosis of keratocytes, absence of inflammatory reaction, and absence of superficial stromal repair in the specimen that was obtained 12 days after the accident. These findings indirectly confirm the importance of epithelium and normally vascularized conjunctiva in healing wounds of the cornea.

In vivo effects of glycine on retinal ultrastructure and averaged electroretinogram.

Glycine, when injected intravitreally, has an inhibitory action on the electroretinogram (ERG) of the rabbit resulting in a transient loss of the oscillatory potentials of the b-wave. This inhibitory action is reversible within 24 h after glycine injection. In autoradiographs, after [3H] glycine administration, the radioactive label is mainly found over the inner nuclear layer (INL) and inner plexiform layer (IPL) without predilection of specific cell types and/or synapses. Electron microscopy reveals cytopathological changes in amacrine cells, in particular their cell membranes. These changes are conspicuous especially 1-2 h after the injection and the cells become normal again within 24 h. It is concluded that glycine has an inhibitory action upon the rabbit ERG in vivo. This action, on the basis of our morphological observations under our experimental conditions, may be due to an overall somatic membrane action rather than to an action as inhibitory neurotransmitter at the synaptic level. The reversible cellular lesions of amacrine cells after glycine administration with a concomitant, transient loss of oscillatory potentials (OP) supports the hypothesis that the cellular origin of OP is situated in amacrine cells.

A pilot study of the relationship between experts' ratings and scores generated by the NBME's Computer-Based Examination System.

This pilot study evaluates the consistency of experts' ratings of students' performances on the National Board of Medical Examiners' Computer Based Examination (CBX) cases and the relationship of those ratings to the CBX's scoring algorithm. The authors were investigating whether an automated scoring algorithm can adequately assess an examinee's management of a computer-simulated patient. In 1989-90, at the Michigan State University College of Human Medicine, eight students, completing a surgery clerkship, each managed eight CBX cases and took a computer-administered, multiple-choice examination. Six clerkship coordinators rated the students' performances in terms of overall management, efficiency, and dangerous actions. The ratings correlated highly with scores produced by the CBX's scoring system.

Intravascular papillary endothelial hyperplasia of the orbit.

Intravascular papillary endothelial hyperplasia is a rare vascular benign tumour bearing some similarities to malignant angiosarcoma. The case reported here in a 20-year-old girl is of particular interest because the orbital tumour, despite its entirely benign nature, invaded the temporal fossa through the lateral bone of the orbit.

[Geographic and helicoid choroidopathies. Clinical and angiographic study; attempted classification]. / Les choroïdopathies géographiques et hélicoïdales. Etude clinique et angiographique; essai de classification.

Among the helicoid and geographic (or serpiginous) choroidopathies, several entities can be distinguished which differ in their clinical evolution, morphology, angiographic appearance and pathophysiology. The entity is a chorioretinal heredodystrophy characterized by tonguelike strips of choroidal atrophy radiating starlike, from the optic disc. The evolution lasts many years (up to 20 or more), with slow progression and no inflammatory stages. Other types originate from occlusion of one or several short ciliary vessels, or at least of the corresponding choriocapillary network. Inflammation is always present initially. In the acute stage, there are localized or lobular areas of exudation. After an evolution of variable duration, up to 4-5 years, exudation is followed by atrophy and formation of scars at times helicoidal in configuration but somewhat more irregular than the dystrophic entity. Similar scars around the optic disc or in the posterior pole sometimes arise from a generalized vasculopathy e.g. a giant cell arteritis. Several typical cases are presented to demonstrate the variety of manifestations that constitute tentative system of classification.

[The retinopathy of ophthalmoplegia plus (Kearn's syndrome)]. / La rétinopathie de l'ophtalmoplégie plus ou syndrome de Kearns.

The retinopathy of ophthalmoplegia plus (Kearns syndrome) is characterised by an extreme variability and a relative mildness of the visual disturbances, contrasting with the gravity of the ophthalmoscopic lesions. A study of three new cases, as well as an analysis of the literature, tend to demonstrate that the retinal lesions are mainly localised in the pigment-epithelial cells (mitochondrial anomalies?). This peculiar pathogenesis could explain why the retinopathy in this syndrome differs from classical pigmentary retinopathy.

[Sturge-Weber-Krabbe syndrome with unusual symptomatology (author's transl)]. / Maladie de Sturge-Weber à symptomatologie inhabituelle a propos de 2 cas.

Two cases of Sturge-Weber-Krabbe's syndrome with unusual symptomatology are described. In the first case 13 years old boy the naevus flammeus was missing. A convulsive fit at the age of 2 1/2 years led to the correct diagnosis from the very characteristic radiological findings. At 13 years, intracortical calcifications were bulky and diffuse, and associated with optic nerve hypoplasia and anomalies of ocular motricity, their relation with the syndrome being difficult to establish. In the second patient (aged 17) the naevus flammeus was extensive, with numerous naevi on the whole body, and was associated with a retinal vascular malformation and glaucoma. A choroidal angioma was not observed. In these two patients the disease was isolated, without familial character, and there were no other neurological symptoms such as a pathological EEG. No evidence of mental retardation was noted.

[Visual evoked potentials and ophthalmoscopy redfree light in subclinical retrobulbar neuritis of multiple sclerosis (author's transl)]. / Potentiels évoqués visuels et ophtalmoscopie à la lumière anérythre dans les formes subcliniques des névrites rétro-bulbaires de la sclérose en plaques.

Combined study using pattern and flash VER as well as fundoscopy with redfree light shows an increased number of axonal degeneration of the retinal fiber layer in patients suffering from multiple sclerosis without ocular symptoms. Discussion of differential diagnosis of other neuropathies (toxical, compressive, etc.).

[Simultaneous cutaneous and choroidal melanomas (author's transl)]. / Mélanomes simultanés de la choroïde et de la peau.

In patients with both cutaneous and ocular malignant melanomas it is most important for prognosis and therapy to know whether they are distinct tumors developing simultaneously or whether one of the tumors represents a metastasis of the other. In the case reported here, two distinct tumours developed independently from pre-existing benign melanocytic lesions. Cases of this type, which are extremely rare, are discussed as well as the differential diagnosis between primary choroidal malignant melanoma and choroidal metastases from melanomas of different origin.

[Microangiopathy and retinal dystrophy]. / Microangiopathie et dystrophie rétinienne.

Two cases are described of an association of retinal dystrophy and a vasculopathy similar to Coat's disease. The first case was a female adult in whom the two sets of symptoms appeared almost simultaneously. In the second case the disease appeared in infancy, evolved rapidly during adolescence and led to blindness. In both cases, the vasculopathy developed over several years and then stabilised; in contrast, the dystrophy, which was of the paucipigmentary type, continued to get worse. From these two cases, and others described in the literature, it appears that many causes can be involved in the association of the two syndromes. A genetic factor that has been demonstrated in several families was not present in these two patients; there appears to have been an immunological or inflammatory process, not yet identified, that became active at the time of the rapid evolution of the dystrophy.

[The Brown's syndrome: an anatomic variation (author's transl)]. / Une variation anatomique du syndrome de Brown.

In a young woman with an anomaly of the ocular motility evoking Brown's syndrome, an insertion anomaly of the superior oblique muscle, situated in the superior nasal quadrant 10 mm of the limbus, was found during operation. A stair-like tenotomy restored an almost normal motility.

[Degenerative choroidal atrophy (author's transl)]. / Les atrophies choroïdiennes dégénératives.

Based on of 10 personal observations, the authors discuss the modes of onset and the various clinical, functional and genetic aspects of primary degenerative choroidal atrophies. Central areolar atrophy may be the only lesion or is sometimes associated with other signs of a more extensive degeneration (fundus flavimaculatus, degeneration of the posterior pole). Among the diffuse forms a familial case is reported which can be interpreted as a sectorial hypoplasia, and a case similar to a choroideremia, but with recessive transmission.