[Toxoplasmosis in pregnancy: Practical Management]. / Toxoplasmose pendant la grossesse : proposition actuelle de prise en charge pratique.

The burden of congenital toxoplasmosis has become small in France today, in particular as a result of timely therapy for pregnant women, fetuses and newborns. Thus, the French screening and prevention program has been evaluated and recently confirmed despite a decline over time in the incidence of toxoplasmosis. Serological diagnosis of maternal seroconversion is usually simple but can be difficult when the first trimester test shows the presence of IgM, requiring referral to an expert laboratory. Woman with confirmed seroconversion should be referred quickly to an expert center, which will decide with her on treatment and antenatal diagnosis. Although the level of proof is moderate, there is a body of evidence in favor of active prophylactic prenatal treatment started as early as possible (ideally within 3 weeks of seroconversion) to reduce the risk of maternal-fetal transmission, as well as symptoms in children. The recommended therapies to prevent maternal-fetal transmission are: (1) spiramycin in case of maternal infection before 14 gestational weeks; (2) pyrimethamine and sulfadiazine (P-S) with folinic acid in case of maternal infection at 14 WG or more. Amniocentesis is recommended to guide prenatal and neonatal care. If fetal infection is diagnosed by PCR on amniotic fluid, therapy with P-S should be initiated as early as possible or continued in order reduce the risk of damage to the brain or eyes. Further research is required to validate new approaches to preventing congenital toxoplasmosis.

[Effects of deformational plagiocephaly during the first 12 months on the psychomotor development of prematurely born infants]. / Effets de la plagiocéphalie posturale au cours des 12 premiers mois sur le développement psychomoteur à 4 ans des enfants nés très prématurément.

AIMS: The link between deformational plagiocephaly and psychomotor development is a recurrent question in medical publications. Main publications concentrate on term infants, but there is a lack of data on the impact of deformational plagiocephaly on the long-term neurodevelopment of premature infants. We attempted to establish a possible relation between deformational plagiocephaly during the 1st year of life and the psychomotor score at 4 years in prematurely born infants. Other risk factors potentially impacting the psychomotor score were also studied. MATERIAL AND METHODS: A retrospective study of the files of the children followed by the "Naître et Devenir Région PACA Ouest Corse Sud" healthcare network and included in the database allowed us to select a cohort of 594 infants born prematurely at under 33 weeks of gestational age. These children were developmentally evaluated during the 1st year of life and at 4 years or age using the "EVAL Mater" test. The "Naître et Devenir" network is following up infants born prematurely at under 33 weeks of gestation in the West Provence Alpes Côte d'Azur and South Corsica region, from discharge to 7 years. A group of 170 specially trained pediatricians follow these infants developmentally at term, 3, 6, 9, 12, 18, and 24 months of corrected age and 3, 4 5, 6, and 7 years. Data are collected in a specially designed database. RESULTS: There was no significant link between deformational plagiocephaly during the 1st year of life and a pathological psychomotor score at age 4, but some risk factors were demonstrated: male gender, birth at under 28 weeks of gestational age, weight at birth under 1000g, having a Latal and Ferriero neuromotor score equal to or greater than 2 at 3 months of corrected age, and to a lesser extent having a prescription for physiotherapy during the 1st year. CONCLUSION: The research on deformational plagiocephaly in the full-term infant suggests a relation between deformational plagiocephaly and developmental delay predominantly on the motor side, with an increased rate of special needs services at school age. The question is raised of whether deformational plagiocephaly is the cause of the delay or an early sign of cerebral anomaly with an early motor delay in full-term infants. The results suggest that deformational plagiocephaly in the prematurely born infant may not be related to neurodevelopmental delay but simply to the extended time spent in the supine position because of the early birth associated with physiological hypotonia and axial extension. Other risk factors such as male gender, birth before 28 weeks of gestation, weight less than 1000g, a Latal and Ferriero neuromotor score greater than 2 at 3 months of corrected age, and having a prescription for physiotherapy during the 1st year of life are strongly related to delayed psychomotor development at age 4.

Long-term ocular outcome in congenital toxoplasmosis: a prospective cohort of treated children.

OBJECTIVES: Congenital toxoplasmosis remains a public health problem throughout the world. Long-term longitudinal studies are still needed to argument controversial screening and treatment strategies and to enable to accurately counsel parents. METHODS: We conducted a prospective cohort study over 16 years in Marseilles, France. Seronegative pregnant women underwent monthly serological testing. Children were treated antenatally with rovamycine as soon as maternal infection was detected and with pyrimethamine and sulfadoxine in case of positive Toxoplasma PCR on amniotic fluid. Postnatal treatment with pyrimethamine and sulfadoxine was systematically prescribed for one year and possibly continued at the physician discretion. RESULTS: 127 children were included. 24 children (18.9%) presented ocular lesions causing visual impairment in eight cases. Eleven children (8.7%) presented with ocular lesions at birth, mostly macular. Sixteen children (12.6%) developed ocular lesions during follow-up, mostly peripheral. The first ocular lesion could occur as late as 12 years after birth. No significant risk factor of chorioretinitis was identified including gestational age at infection, type of antenatal treatment and shorter postnatal treatment. CONCLUSIONS: These results confirm the overall good prognosis of congenital toxoplasmosis in Europe but highlight though a low risk of late ocular manifestation. Chorioretinitis affected 18.9% of children suffering from congenital toxoplasmosis despite antenatal and neonatal screening associated with early treatment. Long-standing follow-up is needed because first lesion can occur as late as 12 years after birth. Late lesions were less often macular but nevertheless caused sometimes visual impairment.

[Fetomaternal anti-RH3, -4 (anti-E and anti-c) rhesus isoimmunization: a case report]. / Allo-immunisation fœto-maternelle rhésus anti-RH3, 4 (anti-E et anti-c) : à propos d'un cas.

Hemolytic disease of the newborn caused by maternal isoimmunization has been decreasing over the past 10 years because of prophylactic treatment with anti-RH1 (anti-D) immunoglobulin. Nevertheless, there is an increase in the incidence of both relative and absolute numbers of non-RH1 red-cell maternofetal isoimmunizations, essentially anti-RH4 (anti-c), anti-RH3 (anti-E), and anti-Kell. In 8 to 14% of cases, multispecificity antibodies are present, the most common combination being the association of anti-RH3 and -4. Despite absence of specific prophylactic therapy, anti-RH4 isoimmunization could be as severe as anti-RH1 ; as for anti-RH3, it is usually associated with mild to moderate clinical manifestations. Nevertheless, there are few publications on anti-RH3, -4 maternofetal isoimmunization with a bias toward the most severe cases being reported. We report here a case of nonsevere maternofetal anti-RH3, -4 isoimmunization complicated with severe hyperbilirubinemia and delayed profound anemia. Hyperbilirubinemia was controlled using intensive phototherapy. Although anemia was absent at birth, it appeared progressively with a nadir at 7.8 g/dL at 1-month postnatal age. Blood counts were monitored for 3 months but the patient did not require red blood cell transfusion. This report underlines the need for a prolonged and rigorous pediatric follow-up of children born in the context of maternofetal isoimmunization after the acute neonatal period. Furthermore, it stresses the necessity of DAT testing in all pregnant women, even those who are RH1-positive.

Antenatal rupture of a diverticular rectal duplication with neonatal perineal fistulization.

A cystic pelvic malformation was found in a fetus on antenatal sonography (US) at 26 weeks of gestational age that was no longer present 3 weeks later on control US. The male child presented at birth with a right-sided perineal mass that fistulized with meconial drainage. A radiopaque enema showed a low posterior rectal fistula filling a poorly delineated pouch. Surgery performed through a posterior sagittal approach allowed identification and closure of the fistula and pouch drainage. The diagnosis of a diverticular rectal duplication was considered, although no intestinal lining was observed macroscopically or histologically. The child's anorectal function was normal after a 20-month follow-up. Labeling of the malformation and embryological hypotheses are discussed since the case does not fulfill all the criteria of an intestinal duplication. Surgical techniques are discussed, with an emphasis on the sagittal posterior approach.

[The Beals-Hecht syndrome (congenital contractural arachnodactyly) revealed in a neonate]. / Syndrome de Beals-Hecht (congenital contractural arachnodactyly) à révélation néonatale.

A new case of congenital contracture arachnodactyly (CCA) revealed in the neonatal period is reported. CCA is a dominantly inherited syndrome associating arachnodactyly, kyphoscoliosis, multiple congenital joint contractures and crumpled ears. This condition differs from Marfan's syndrome by the usual absence of visceral involvement, although cardiac complications are possible. The neonatal forms result from new mutations are are generally severe.

[Moebus syndrome: apropos of a case]. / Syndrome de Moebius: à propos d'un cas.

The authors describe one case of Moebius syndrome in a neonate; the syndrome is uncommon. It includes congenital oculofacial palsy and limb malformations; other cranial nerves are sometimes involved. The etiology is unknown: genetic or embryopathic (infection or toxic). Pathogeny is unclear: nervous or muscular aplasia; neurocristopathy or dysgenesis of the two first branchial arches. The treatment is medical and chirurgical.