[An ophthalmologic diagnostic error leading to a rare systemic diagnosis: Erdheim-Chester disease]. / Une errance diagnostique ophtalmologique aboutissant à un diagnostic systémique rare: la maladie d'Erdheim-Chester.

INTRODUCTION: Erdheim-Chester disease is a rare systemic disease. The diagnosis is difficult due to significant clinical and morphological polymorphism. Orbital involvement is rare, but constitutes a classic means of detection. OBSERVATION: We report the case of a 60-year-old man, who consulted for evaluation of bilateral retro-orbital tumors. These tumors had been discovered on head CT two years previously during work-up of proptosis. Two biopsies were performed. The first one revealed polymorphous inflammatory tissue. The second one revealed intense granulomatous reaction, rich in non-specific foamy histiocytes. Thoracic-abdominal-pelvic CT scan detected peri-aortic and retroperitoneal infiltration. The association of these signs pointed to a diagnosis of Erdheim-Chester disease, confirmed by the re-examination of the histological samples. DISCUSSION: Erdheim-Chester disease is a rare non-Langerhans histiocytosis with a specific tropism for perivascular and fatty connective tissue. The cause is not known. The diagnosis of this systemic disease is histological. CONCLUSION: In the case of bilateral intra-orbital tumors, the diagnosis of Erdheim-Chester disease must be considered.

[Using the superficial temporal fascia flap in orbital surgery]. / Utilisation du lambeau de fascia temporalis superficialis en chirurgie orbitaire.

The superficial temporal fascia flap gives a fine malleable well vascularized tissue and can be used as a pedicled or a free flap to cover large areas of loss of substance. Its dissection needs a period of training. Its use in orbital surgery is rare. However when it is about an anophthalmic socket following radiotherapy with orbital retraction syndrome, it provides tissue of good quality. This could allow later reconstruction by mucous grafts. When used on cavities of exenteration it allows fast re-epithelialisation even post-radiotherapy, while allowing the monitoring of the cavity and in particular the early detection of any tumor recurrence. Its use is advantageous in unfavorable conditions especially after radiotherapy.

[Orbital decompression in Grave's ophtalmopathy]. / Décompression osseuse de l'orbite basedowienne.

Graves disease orbitopathy is a complex progressive inflammatory disease. Medical treatment remains in all cases the proposed treatment of choice. Surgical treatment by bone decompression can be considered as an emergency mainly in cases of optic neuropathy or ocular hypertension not being controlled medically or in post-traumatic exophthalmos stage. Emergency bone decompression eliminates compression or stretching of the optic nerve allowing visual recovery. The uncontrolled ocular hypertension will benefit from decompression. The normalization of intraocular pressure may be obtained by this surgery or if needed by the use of postoperative antiglaucoma drops or even filtration surgery. In all operated cases, the IOP was normalized with an average decrease of 7.71 mmHg and a cessation of eye drops in 3/7 cases. Regarding sequelae, our therapeutic strategy involves consecutively surgery of the orbit, extraocular muscles and eyelids. The orbital expansion gives excellent results on the cosmetic level and facilitates the implementation of subsequent actions.

[Descriptive anatomy of the eyelids: surgical interest]. / Anatomie descriptive des paupières: intérêt chirurgical.

The anatomy of the eyelids is complex. The author describes the limits of the palpebral area as well as the 5 surgical plans of the eyelids of before behind: Skin, the orbicular muscle, the fibro-elastic plan, deep muscles, the tarso-conjunctival plan. The knowledge of this anatomy allows the comprehension of physiology, the realization of the surgery and to approach the potential risks of them.

Massive obesity in adolescents: dietary interventions and behaviours associated with weight regain at 2 y follow-up.

OBJECTIVE: To compare the influence of weight-reducing diets containing different amounts of protein and CHO on body composition in obese adolescents and to examine dietary and physical activity behaviours during follow-up. DESIGN: Prospective randomised study comparing two weight-reducing diets with the same energy (1750 kcal) and fat (31%) content, but different protein and carbohydrate contents: PROT- (15% protein, 54% CHO) vs PROT+ (19% protein, 50% CHO). PATIENTS: Massively obese 11- to 16-year-old children (32 boys and 89 girls). SETTING: A 9-month treatment in a medical centre (boarding school) plus a 2-y follow-up in free-living patients examined at home 1 and 2 y after treatment. MEASUREMENTS: Anthropometry, bioelectrical impedance, nutritional intakes and physical activity. RESULTS: Of the 121 eligible children (61 in PROT- and 60 in PROT+), 82% completed the trial until the end of weight loss treatment and 60% were followed 2 y after treatment. Body mass index (BMI) value at inclusion was 36.3 kg/m(2) or 4.3 z-scores (2.9-5.9). BMI z-score decreased to 1.7 at the end of treatment and went back to 2.8 (0.8-6.1) 2 y after treatment. This corresponded to a weight loss of 30.3 kg and weight regain of 21.3 kg. After treatment, energy intake increased and physical activity decreased. The contribution of energy ingested at breakfast decreased while snacking increased. For all measurements, no dietary group differences existed at baseline or at any time during the intervention and follow-up. CONCLUSION: A higher protein content of the diet did not confer any benefit in the treatment of childhood obesity. Substantial weight loss was obtained with a moderately energy-restricted diet and normal fat content. After weight loss, mean weight increased in spite of moderate energy intake, together with a drift towards obesity-associated behavioural patterns. The causes of the inability to adopt normal weight subjects' behaviour permanently deserve to be investigated further.

[Followup of pregnant women in the context of malaria: two case reports]. / Suivi de la grossesse dans le cadre du Paludisme: à propos de deux observations.

Whether or not a pregnant women should travel to regions where malaria is highly endemic will always be open to question as no prophlaxis can guarantee complete protection in every case. No chemoprophylaxis is 100% effective or entirely without side-effects, particularly for pregnant women whose immune status provides a favourable environment for the emergence of this parasitosis. The advice given will depend on the country and the region, a possible resistance of the parasite to treatment, the season, the length and circumstances of the stay and individual factors. The choice of treatment is very limited as many therapies are contra-indicated in pregnancy; in addition it is extremely important to give a sufficiently clear explanation concerning precautions and prophylaxis as well as of how a possible bout of malaria should be treated, particularly in case of extended stay.

[Placental teratoma: differential diagnosis with fetal acardia]. / Tératome placentaire: diagnostic différentiel avec le foetus acardiaque.

The acardiac fetus is a rare congenital malformation observed only in monozygotic twin pregnancies (1/35.000 pregnancies). Placental teratoma is extremely rare (less than 20 cases have been reported in the literature since 1925). A case of placental teratoma, misdiagnosed as fetus acardius amorphus, is presented with ultra-sound follow-up, delivery and outcome of the normal fetus. Pathological findings, histogenesis, differential diagnosis with the acardiac fetus and finally review of the literature will be discussed.

[Loss of visual acuity caused by ethmoidal mucocele cured after endonasal surgery: report of a case]. / Perte d'acuité visuelle par mucocèle ethmoïdale résolutive après chirurgie endonasale. A propos d'un cas.

A posterior and/or ethmoidal mucocele remains a rare occurrence. The condition usually follows a past history of sinonasal surgery. Extension of the lesion may have ophtalmological implications, with the possibility of causing visual impairment. The predilection for a site of origin in the posterior sinuses explains the frequent absence of nasal symptoms, and the usual pattern of discovery by the ophtalmologist. Modern imaging techniques, especially scanning, allows the diagnosis to be made immediately after the first orbital symptoms. By the same token, recent techniques of endoscopic endonasal surgery have radically changed the treatment. Early complete surgical removal holds out hope for remission of the symptoms that led originally to discovery of the mucocele. We report here a case of posterior ethmoidal mucocele causing a visual field defect which reversed completely after endoscopic treatment.

[Congenital toxoplasmosis and seroconversion at the end of pregnancy: clinical observations]. / Toxoplasmoses congénitales et séroconversions de fin de grossesse: observations cliniques.

We report seven cases of subclinical congenital toxoplasmosis secondary to maternal primary infections. Mothers were infected between two and four weeks prior to delivery. The diagnostic criteria of congenital infections included: IgM antibody (Ab) (1 case); IgM and IgA Ab (1 case); a real IgG seroconversion in the neonatal and postnatal samples (3 cases); persistence of IgG Ab beyond 6 months post-delivery (2 cases). A treatment was initiated, including a combination of pyrimethamine + sulfadiazine (6 cases); trimethoprim + sulfamethoxazole (1 case). This retrospective study suggests that it is important to screen the non-immune pregnant women until delivery. We confirmed the usefulness of a combination of isotypes of antibodies for the accurate assessment of congenital infection. Finally, infected infants have to be treated and monitored clinically and immunologically during the first year of life.

[Surgical treatment of myopathic ptosis. Apropos of 20 surgically treated cases]. / Traitement chirurgical des ptosis myopathiques. A propos de vingt cas opérés.

Twenty cases of severe progressive ophthalmoplegia (Myopathic ptosis) with significant ptosis were treated by various procedures, including levator resection with blepharoplasty or frontalis suspension using temporalis fascia. Surgical indications were generally limited to cases with poor or absent levator function. The results are analysed after an average follow-up of 6 months. The particular problems concerning surgery of myopathic ptosis are discussed.