[Ocular manifestations in Ehlers-Danlos Syndromes: Clinical study of 21 patients]. / Manifestations ophtalmologiques des syndromes d'Ehlers­Danlos : à propos d'une cohorte de 21 patients.

INTRODUCTION: The goal of this study was to describe and analyze the ophthalmological manifestations found in 21 patients followed for Ehlers-Danlos Syndrome in our department. METHODS: This retrospective study analyzed 21 consecutive patients (17 women and 4 men) with Ehlers-Danlos syndrome seen in the Necker hospital, Paris, between April 2016 and November 2017. The mean age was 25.95 years (12-47). A complete evaluation was performed searching for symptoms, orthoptic evaluation and complete ophthalmologic examination with slit lamp examination of the anterior segment, pachymetry and fundus examination with fundus photography and OCT. RESULTS: Nineteen patients presented ophthalmological signs (90.5%). The most frequent ophthalmological signs were: ocular motility disorders in 15 patients (71.4%), with convergence insufficiency in 13 of them, blue sclera in 8 patients (38%) and dry eye syndrome in 7 patients (33%, with 2 patients with reduced Break-Up Time<10seconds and 5 with very reduced Break-Up Time<5seconds). Mean pachymetry was 539.25µm (365-612). One patient presented with bilateral keratoglobus (4.8%). High myopia was present in 2 patients (9.5%) and associated with retinal tears in one patient (4.8%). No patients presented with angioid streaks. DISCUSSION: In this study, the main ophthalmological sign was convergence insufficiency present in more than 60% of the patients. This highlights the importance of an orthoptic examination in patients with Ehlers-Danlos syndrome. Dry eye syndrome with tear film instability was frequent, even though the patients were young. Blue sclera was seen in 38% of the patients. We reported two patients with high myopia and one patient with keratoglobus in our cohort. No patients presented with angioid streaks, and mean pachymetry was normal in our series. CONCLUSION: An ophthalmological and orthoptic evaluation should be performed in all patients with Ehlers-Danlos syndrome to detect and treat ocular manifestations. If Ehlers-Danlos syndrome is suspected, ophthalmological examination can also provide support for the diagnosis.

The determination of fenspiride in human plasma and urine by liquid chromatography with electrochemical or ultraviolet detection.

A selective and sensitive method for the determination of fenspiride in biological fluids is described. The method involves liquid-liquid extraction followed by separation on a reversed-phase column with electrochemical detection for low levels of the drug in plasma (less than or equal to 100 ng ml-1) or UV absorption for higher concentrations in plasma or urine. The method is suitable for pharmacokinetic analyses and drug monitoring studies.

[Radiographic analysis of the orientation of the distal articular surface of the first metatarsal in the horizontal plane]. / Analyse radiographique de l'orientation de la surface articulaire distale du premier métatarsien (AADM) dans le plan horizontal.

PURPOSE OF THE STUDY: The lateral obliquity of the distal metatarsal articular surface is a predominant anatomical factor in hallux valgus pathology. It is important to know its normal angular value. MATERIAL-METHODS: The study included 105 feet without any pathology (48 females and 57 males; 53 left and 52 right between 15 and 72 year old). Weight-bearing, anteroposterior X-rays of the foot was made. We measured the distal metatarsal articular angulation (DMAA), the intermetatarsal angle, the metatarsophalangeal angle, the obliquity of the metatarsocuneiform joint and the length of the first and second metatarsal, and the length of the hallux. RESULTS: The main value of the DMAA was 5.78 degrees in our study, 6.79 degrees for women and 4.92 degrees for men. There was no difference between left and right side, no correlation between intermetatarsal angle and DMAA, neither between biometrics parameters and DMAA. DISCUSSION: Our results confirm the Richardson study on cadaver with a main value of 6 degrees for the DMAA. The DMAA value increases in women and it does not increase proportionaly to the intermetatarsal angle; it increases with the value of the metatarsophalangeal angle. All these points have to be taken in consideration in hallux valgus evaluation. CONCLUSION: The distal metatarsal articular surface has a lateral obliquity of 5.78 degrees. It must be evaluated before surgical correction of hallux valgus, in order to decrease recurrence rate of this pathology and iatrogenic deformity.