[Partial facial duplication (a rare diprosopus): Case report and review of the literature]. / Duplication faciale partielle (un diprosope rare): à propos d'un cas et revue de littérature.

INTRODUCTION: Diprosopus, or partial facial duplication, is a very rare congenital abnormality. It is a rare form of conjoined twins. Partial facial duplication may be symmetric or not and may involve the nose, the maxilla, the mandible, the palate, the tongue and the mouth. OBSERVATION: A male newborn springing from inbred parents was admitted at his first day of life for facial deformity. He presented with hypertelorism, 2 eyes, a tendency to nose duplication (flatted large nose, 2 columellae, 2 lateral nostrils separated in the midline by a third deformed hole), two mouths and a duplicated maxilla. Laboratory tests were normal. The cranio-facial CT confirmed the maxillary duplication. DISCUSSION: This type of cranio-facial duplication is a rare entity with about 35 reported cases in the literature. Our patient was similar to a rare case of living diprosopus reported by Stiehm in 1972. Diprosopus is often associated with abnormalities of the gastrointestinal tract, the central nervous system, the cardiovascular and respiratory systems and with a high incidence of cleft lip and palate. Surgical treatment consists in the resection of the duplicated components.

[Congenital afibrinogenemia: a case report]. / Afibrinogénémie congénitale: à propos d'une nouvelle observation.

Afibrinogenemia is a rare autosomal recessive disease. Its clinical manifestations vary in severity, ranging from minimal bleeding to cataclysmic hemorrhage, and can begin at birth or, sometimes, later. We report a case of a female infant, 10 months of age, hospitalized in the pediatrics department because of a postvaccination hematoma. Biologic exploration found congenital afibrinogenemia. Through this case, we review the clinical features of this disease and its management.

[Comparison of 2 therapeutic strategies for severe endometriosis, in young women counsulting for sterility or pain. Results in cases of chronic pelvic pain]. / Comparaison de deux stratégies thérapeutiques dans les endométrioses sévères, chez des femmes jeunes consultant pour stérilité ou douleurs. Résultats en cas de douleurs pelviennes chroniques.

AIM OF THE STUDY: Compare two medical strategies associated to surgery in women requiring for chronic pelvic pain due to stage III-IV endometriosis. MATERIAL AND METHODS: Two different patient groups, A (N 27) and B (N 41), requiring for chronic pelvic pain, associated with AFS stage III-IV endometriosis, operated on from 1992 to 1997, were compared. The medium age was 35 and 34 years, respectively. Pelvic pain, classified in three stages, was similar in both groups but they were more AFS stage IV in group A, 67% than in group B, 46% (p < 0.01). Both groups had similar operative procedure: laparoscopic resection of deep endometriotic nodules or endometriomas, plus destruction of small superficial lesions using CO2 laser (A) or bipolar coagulation (B). Associated medical strategy was different: group A: operative laparoscopy without preoperative treatment and in 25% a second laparoscopy taking place after two-three months of LHRH analogues; no postoperative treatment; group B, operative laparoscopy taking place after ovarian blockage with three-six weeks of Diane (Androcur + ethinyl estradiol), then two-three months of analogue postoperative treatment immediately followed by long term progestoid treatment in order to prevent recurrences in women without pregnancy desire. RESULTS: After one year, 6/27 (22%) of A and 3/41 (7%) of B had no follow-up. Of the followed patients, a complete improvement was observed in 18/21 (86%) A, 33/38 (87%) B, moderate pelvic pain continued in 2/21 (10%) A, 4/38 (11%) B, and the treatment was in failure in 1/21 (5%) A, 1/38 (3%) B, without significant difference. After two years, 67% of A and 76% of B had a follow-up and the corresponding rates of complete improvement are 72% (A), 87% (B), incomplete improvement: 22% (A), 10% (B) and failure: 6% (A), 3% (B). The difference is lightly significant (p < 0.05) and remains so if patients without follow-up are considered as failures. There was no persistence nor recurrence of endometriosis nor endometrioma two years after the surgery was completed. CONCLUSION: Since there were more stage IV endometriosis in group A than in B, the different medical strategies and particularly the long term postoperative treatment used in B seem have little influence on results. However, these data was obtained in women of medium age > 30, with a relatively short follow-up. It should be of interest to compare in a prospective multicentric study the long term follow-up of two cohorts of young women operated on for stage III-IV endometriosis, receiving or not a long term medical treatment after surgery in order to prevent recurrences.

[Comparison of 2 therapeutic strategies in severe endometriosis, in young women consulting for sterility or pain. II. In the case of infertility, value of ovarian stimulation with intrauterine insemination after surgery]. / Comparaison de deux stratégies thérapeutiques dans les endométrioses sévères, chez des femmes jeunes consultant pour stérilité ou douleurs. II. En cas d'infertilité, intérêt des stimulations ovariennes avec inséminations intra-utérines après la chirurgie.

AIM OF THE STUDY: Define the best medico surgical strategy in infertile women with stage III-IV endometriosis. MATERIAL AND METHODS: Two groups, A (N26) and B (N 37), treated for infertility associated or not with pelvic pain, due to stage AFS III or IV endometriosis, were compared. They had similar surgical procedure: operative laparoscopy including resection of endometriotic lesions, more particularly endometriomas and rectovaginal septum nodules. Associated medical strategy was different: group A, operative laparoscopy without preoperative treatment and in 40% a second laparoscopy taking place after 2-3 months of LHRH analogues; no post operative treatment; group B, operative laparoscopy taking place after ovarian blockage with 3-6 weeks of Diane (Androcur + ethinyl estradiol), then 2-3 months of analogue postoperative treatment immediately followed by ovarian stimulation (OS) + intrauterine insemination (IUI) in women more than 30 years old with operative tubes (N 22), no treatment for six months in similar cases less than 30 (N 5), and IVF in women with damaged tubes (N 5) or after OS + IUI failure (N 4). One patient refused two patients with high FSH level had oocyte donation. RESULTS: Two years evolutive pregnancy rate was significantly higher (p < 0.01) in group B (59%) versus group A (23%) and was higher after OS + IUI (68%) than after IVF (55%) or without any treatment in women < 30 (43%). The difference is equally significant by age (p < 0.05), for endometriomas (p < 0.01) and for recurrences (p < 0.01). CONCLUSION: Similar results obtained for pelvic pain (see chapter I) suggest that both strategies are similarly successful in treating endometriosis. These results confirm the interest of an ART after surgery for stage III-IV endometriosis and show that OS + IUI, a less costly than IVF technique, can be used successfully in selected cases with operative tubes.

[Bourneville's tuberous sclerosis]. / Sclérose tubéreuse de Bourneville.

We present a case of tuberous sclerosis, classified as phacomatosis. The patient is a young woman admitted to hospital for a tumor in the right eye. The diagnosis of tuberous sclerosis was based on the presence of the various symptoms of this disease. The patient's ophthalmological symptoms were severe. Ophthalmologists must look for these symptoms in all subjects at risk, particularly in patients with epilepsy but also in the presence of cutaneous or visceral symptoms.

[Ophthalmic manifestations of acute leukemia]. / Manifestations ophtalmologiques des leucémies aiguës.

Orbital and ocular lesions are the third most frequent extramedullar locations of acute leukemia after the meninges and testicles. These lesions are treated as a central nervous system lesion; therefore diagnosis is essential to therapeutic adjustment. We present a retrospective study on charts of children treated for acute leukemia between 1996 and 1998. Ophthalmic examination was carried out when there were ocular symptoms. One hundred ninety-six children were treated for acute leukemia. Twelve children (6.1%) had an ocular and orbital lesion: 7 boys and 5 girls, with an average age of 6 years. Six had acute lymphoblastic leukemia, 6 had acute myeloid leukemia. Visual acuity was 1/10 in 9 children. Four children had an initial orbital lesion with a rapidly progressing exophthalmos. The hemogram and myelogram showed a granulocytic sarcoma. Two children who had been previously treated for acute leukemia presented a bilateral anterior uveitis with hypopyon; anterior chamber paracentesis showed blast cells and confirmed the ocular relapse. A corneal lesion was found in 2 children and in 2 cases, the optic nerve had edema; a decrease in visual acuity was the sign of the optic nerve lesion. Two patients had retinal infiltration with hemorrhages in the posterior pole. Prophylaxis consists of intrathecal injection of methotrexate and reinforcement of chemotherapy. Central nervous system irradiation is seldom used in children. Orbital and ocular lesions carry a poor prognosis according to the majority of authors. Two years after diagnosis of the ocular lesions, only 1 of the children studied remains alive.

[Factors associated with fecal incontinence after childbirth. Prospective study in 525 women]. / Facteurs liés à l'incontinence anale du post-partum. Etude prospective chez 525 femmes.

OBJECTIVE: To determine the prevalence of fecal incontinence after childbirth and to identify the risk factors. METHODS: This was a prospective observational study with a consecutive inclusion of 525 women who delivered over a three months period. Women were questioned about their fecal continence four days and six weeks after delivery. RESULTS: The incidence of fecal incontinence four days and six weeks after childbirth was respectively 8.8% and 3.3%. The risk factors for fecal incontinence at 4 days after childbirth were instrumental delivery by forceps (adjusted odds ratio 8.64, 95% confidence interval 3.55-21.0, p < 0.001) and unassisted delivery at home (adjusted OR 8.06, 95% CI 1.30-50.0, p = 0.025). Independent risk factors for the presence of fecal incontinence 6 weeks later were: instrumental forceps delivery (adjusted OR 10.8, 95% CI 2.82-41.3, p = 0.001), unassisted delivery at home (adjusted OR 50.0, 95% CI 3.09-802, p = 0.006), bi-parietal diameter of the newborn > 93 mm (adjusted OR 4.56, 95% CI 1.46-14.1, p = 0.009) and maternal age >30 years (adjusted OR 4.60, 95% CI 1.11-19.1, p = 0.036). CONCLUSION: Fecal incontinence is common after childbirth and its prevalence is predominantly associated with instrumental delivery, unassisted delivery at home, bi-parietal diameter of the newborn and maternal age.

[Enzymologic aspect of piperacillin combinations]. / Aspect enzymologique des associations de pipéracilline.

Like all penicillins, piperacillin exhibits some susceptibility to beta-lactamases of the penicillinase type, including those produced by Staphylococcus aureus and the TEM, OXA and CARB enzymes isolated from Gram-negative bacilli. Piperacillin is very slightly hydrolyzed by cephalosporinases, which makes it similar to 3rd generation cephalosporins. When tested with Enterobacter cloacae GN 5797 and Pseudomonas aeruginosa NTC 8303, two strains which produce inducible cephalosporinases, piperacillin had moderate inductive activity compared to cefoxitin, a potent inducer. Induction was very low with Enterobacter, but the drug was slightly more sensitive to Pseudomonas. Inhibition of this type of beta-lactamase synthesis was very strong when piperacillin was combined with amikacin and weaker when it was combined with pefloxacin. The piperacillin-amikacin combination prevented the development of piperacillin-resistant mutants of Enterobacter and Pseudomonas and, probably, of all Gram-negative bacilli. In our tests, the piperacillin-pefloxacin combination was of interest only against Enterobacter, and probably against all enterobacteria, since Pseudomonas mutants that resist pefloxacin are fairly easily obtained in vitro.

Deprivation, palatability and the micro-structure of meals in human subjects.

Changes in the micro-structure of meals were studied in ten human subjects under different palatability and deprivation conditions. The oscillographic recording of chewing and swallowing movements during standardized meals allowed many meal parameters to be precisely measured. Both increases in deprivation time (from 4 to 15 h) and in food preference produce larger, longer meals. These factor's effects were additive. Eating rate was accelerated in high relative to low preference conditions. The micro-structure of meals proved to be more sensitive to food preferences than to deprivation levels: chewing activity per standard food piece tended to decrease as preference increased, as observed in a previous study. However, in the first quarter of meals, chewing time was affected similarly by deprivation and by preference. A clear deceleration of eating rate was apparent between the beginning and the end of meals. The results are discussed in perspective with other human studies and with reference to preference and deprivation as continua.