Evaluation of the management of pregnancies and infants at risk for congenital syphilis: La Réunion, 2008 to 2014.

OBJECTIVE: To evaluate the maternal-fetal management and follow-up of infants at risk for congenital syphilis. STUDY DESIGN: Monocentric retrospective cohort study at the Félix Guyon Hospital, Saint-Denis, La Réunion between January 2008 and December 2014. Management of 38 pregnancies (35 women, 39 fetuses) with a positive syphilis serology was evaluated according to the Centers for Disease Control and Prevention (CDC) guidelines. RESULTS: A total of 68% (n=26) of pregnancies were screened before 16 weeks of gestation, and 26% (n=10) had appropriate treatment. Adverse outcomes were noted in 45% of pregnancies. Neonatal serology was not performed in 7 live-born neonates (19%) and 33 infants were classifiable according to the four CDC scenarios. In the infants, adequate complementary evaluation and appropriate treatment were noted in 42% (n=13) and 55% (n=17) of cases, respectively. Rate of infant follow-up was 76% (n=16). No treatment failure was observed in infants. CONCLUSION: Reinforcement of public health policies and a better sensitization, training and collaboration among perinatal caregivers are warranted given the gaps revealed in our study.

[An atypical Demons-Meigs syndrome]. / Un syndrome de Demons-Meigs réellement atypique.

Meigs'syndrome is defined as a hydrothorax with ascites and benign ovarian tumor, both of which resolve on removal of the tumor. Pseudo-Meigs'syndrome or atypical Meigs'syndrome occurs when a pelvic mass other than an ovarian fibroma is present with hydrothorax and ascites. Both these syndromes should be nevertheless considered in women who present hydrothorax and ascites. This case concerns a 50-year-old woman who presented a pseudo-Meigs'syndrome (Hydrothorax and uterine leiomyoma). The distinction between these two syndrome is useless, because tumour removal is the only treatment.

[Prenatal diagnosis of omphalopagus conjoined twins at 13 weeks of amenorrhea]. / Découverte anténatale de jumeaux conjoints omphalopages à 13 semaines d'aménorrhée.

Conjoined twins are a rare occurrence. We present a case of conjoined twins at 13 weeks' gestation. This case demonstrated the possibility of making an accurate diagnosis of conjoined twins and delineating the extent of organ sharing in the first trimester. This analysis and the discover of another anomalies can help the parents with the option for pregnancy termination. Early diagnosis and precise delineation of the shared organs of conjoined twins are essential for optimal obstetric and postnatal management.

[Fallopian tube torsion in a 15-year-old adolescent]. / Torsion tubaire isolée chez une adolescente de 15 ans.

UNLABELLED: Isolated Fallopian tube torsion in teenagers is extremely rare and diagnosis is difficult. Coelioscopy is necessary, but surgery often occurs too late for tube conservation. CASE REPORT: A case of Fallopian tube torsion bringing about a cyst is presented. Diagnostic difficulties, echography, treatment and cause are discussed. CONCLUSION: Early diagnosis and surgical intervention is mandatory when isolated Fallopian tube torsion is suspected.

[Prenatal diagnosis of exencephaly at 10 weeks gestation, confirmed at 13 weeks gestation]. / Diagnostic anténatal d'une exencéphalie à 10 SA, confirmé à 13 SA.

First trimester diagnosis of the exencephaly anencephaly sequence has been reported since the introduction of transvaginal sonography, the earliest being described at 10 weeks 5 days gestation. We report the discovery of exencephaly at 10 weeks gestation. The diagnosis was confirmed by repeat ultrasonography at 13 weeks.

[Fallopian tube torsion: five cases with no other element]. / Torsion tubaire isolée: à propos d'une série de cinq cas.

Isolated Fallopian tube torsion is extremely rare and diagnosis is difficult. Laparoscopy is necessary, but surgery is often performed too late to preserve the tube. Five cases of Fallopian tube torsion with no other element are presented. Diagnostic difficulties, causes, ultrasonographic findings and treatment are discussed. Early diagnosis and surgical intervention is mandatory when exceptional isolated Fallopian tube torsion is suspected particularly in a nulliparous young woman.

[Intrauterine device appendicitis: an exceptional complication]. / Stérilet appendiculaire: une exceptionnelle complication.

We report a case of migration of an intrauterine contraceptive device to the appendix. In this patient with a clinical presentation suggestive of appendicitis, transvaginal ultrasonography visualized a device located outside the uterus, near the small bowel. The device, which was partially embedded in the appendix, was removed via laparoscopy and laparotomy. Appendicular migration of an intrauterine device is exceptional. A literature review spanning the past years only revealed a few reported cases. Most of the authors recommend removal because of the potential for inflammatory reactions that can cause bowel obstruction and perforation.

[Ureteral suture by laparoscopy: a new case. Review of the literature]. / Suture urétérale par voie coelioscopique: à propos d'un nouveau cas. Revue de la littérature.

A wound to the ureter is a possible complication of laparoscopy and is usually repaired by suture requiring laparotomy. We report a case of a laparoscopic stitch and review the pertinent literature.

[Antenatal discovery of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) at 12 weeks gestation]. / Découverte anténatale à 12 semaines d'aménorrhée d'un syndrome "intestinal hypoperistalsis mégacystis-microcolon" (MMIHS).

Megacystis on antenatal scan in female fetuses is rare and has diagnostic implications. We report a case of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) in a female infant. The antenatal scan revealed abnormalities, but the diagnosis was not established until after delivery. MMIH is a rare autosomal recessive condition which is usually lethal in the first year of life. Prenatal diagnosis is hampered by the lack of specific diagnostic ultrasound findings and the absence of an identified genetic locus.

[Subependymal pseudocysts in the fetal brain revealing Zellweger syndrome]. / Pseudo-kystes subépendymaires au sein du cerveau foetal: découverte d'un syndrome de Zellweger.

Prognosis of subependymal pseudocyst is poor when associated with other anomalies. They can be caused by infectious, vascular, chromosomal or metabolic disorders but are rarely described in the antenatal period. We report the prenatal diagnosis of subependymal pseudocyst by MRI after prenatal detection of isolated ventriculomegaly at 23 weeks gestation. The karyotype was normal. The diagnostic of Zellweger syndrome was suspected and was confirmed after birth by metabolic studies. Metabolic studies with culture of chorionic villus cell is indicated for subsequent pregnancies.

[Prenatal diagnosis at 25 weeks gestation and neonatal management of a vallecular cyst]. / Découverte anténatale à 25 semaines d'aménorrhée et prise en charge néonatale d'un kyste de la vallécule.

Due to the anatomical location, vallecular cyst is a rare but well-recognized cause of upper airway obstruction and death in newborn. This cyst can be accurately diagnosed by echography in utero and by MR imaging. Prenatal diagnosis allows for early consultation with surgical specialist, so that the time and place of the delivery can be addressed for neonatal preoperative planning. We report the first prenatal diagnosis of a vallecular cyst at 25 weeks of gestation. At birth, the cyst was drained and then marsupialized. We believed that, in cases of oropharyngeal tumors discovered in utero, elective delivery should be realised in a tertiary referral center in which emergency ventilation and tracheostomy are possible.

[About six observations of double discrepancy of the heart: feasibility of prenatal diagnosis?]. / À propos de six observations de double discordance cardiaque : faisabilité du diagnostic anténatal ?

The double discrepancy (DD) is an exceptional cardiopathy, manifested by atrioventricular and ventriculo-arterial discordances. DD is rarely diagnosed in the antenatal period and is often found in adults when cardiac complications occur. We describe six cases of DD, the different forms, their ultrasound semiology, and the assessment of prognosis, correlated with the existence of associated cardiac anomalies.

Giant hepatic cysts: prenatal findings and uncommon postnatal outcome.

With modern prenatal imaging, liver cysts are being diagnosed more often. Although large cysts are usually asymptomatic, they may present as an abdominal emergency requiring surgery in the first weeks of life. We report a series of 3 patients with prenatal diagnosis of isolated cystic liver lesions diagnosed at 22, 31 and 33 weeks of gestational age. The hepatic origin of the cysts was confirmed prenatally by a MRI in 2 cases, with visualization of a normal gallbladder. The prenatal course was uneventful. Postnatal ultrasound confirmed the diagnosis of liver cyst, showed normality of the biliary tract and in one case, rupture of the cyst during delivery. Because of an uncommon rapid increase in size, the 3 children underwent surgical excision of the cysts within the first weeks of life. These were non-bile-containing intrahepatic cysts arising from segment IV. Long-term follow up was uneventful.

[Ruptured uterine artery pseudo-aneurysm treated by embolization for post-partum bleeding: About 2 cases]. / Pseudo-anévrisme rompu de l'artère utérine traité par embolisation pour hémorragie du post-partum : à propos de deux cas.

Pseudo-aneurysms of uterine arteries are rare causes of post-partum hemorrhage. We report two cases of rupture, revealed in one case by a hemoperitoneum and in a second case by a retroperitoneal hematoma. The treatment consisted of embolization of uterine arteries with immediate clinical success, to avoid surgery.

Bruck syndrome: second antenatal diagnosis.

Bruck syndrome is characterized by the association of arthrogryposis and osteogenesis imperfecta. It is a distinct autosomal recessive disorder associated with normal collagen I. The main features are osteoporosis, long bone bowing and scoliosis due to vertebral deformities and congenital joint contractures. We describe a French girl who was born with ankle and wrist contractures (second antenatal discovery).

Antenatal presentation of isolated femoral hypoplasia discovered at 18 weeks of gestation.

Prenatal diagnosis performed by ultrasound scan is now a routine part of antenatal care in France. A case of congenital short femur in an otherwise healthy infant is described. Antenatal diagnosis was made at 18 weeks of gestation and ultrasonic follow-up was performed. The right femur was shorter than the left. Further sonographic exploration demonstrated unilateral femoral hypoplasia without another anomaly. In this case, the occurrence of congenital short femur was sporadic. The diagnosis was confirmed after delivery. Prenatal sonographic features, differential diagnosis, prognosis and management are discussed. This case illustrates the importance of ultrasound as an early detector of certain congenital anomalies and as a useful tool in their follow-up.

Maxillo-nasal dysplasia (binder syndrome): antenatal discovery and implications.

Binder syndrome (BS) or maxillo-nasal dysplasia is an uncommon developmental anomaly affecting primarily the anterior part of the maxilla and nasal complex. The characteristic findings are a failure of development in the premaxillary area with associated deformities of the nasal skeleton and the overlying soft tissues. Affected individuals typically have an unusually flat, underdeveloped midface (midfacial hypoplasia), with an abnormally short nose and flat nasal bridge, underdeveloped upper jaw, relatively protruding lower jaw and/or a 'reverse overbite' (or class III malocclusion). A case of BS was diagnosed at 24 weeks of gestation using two- and three-dimensional ultrasound. The first sign was an isolated flattened fetal nose in the mid-sagittal plane. Further ultrasound imaging showed the absence of the naso-frontal angle, giving impression of flat forehead and small fetal nose. We discuss about this entity.

Antenatal diagnosis and management of a vallecular cyst.

Vallecular cysts are rare. It is important to be aware of their presence as they can cause upper airway obstruction. We describe a case of congenital vallecular cyst diagnosed at 28 weeks of gestation. This early diagnosis enabled us to plan careful perinatal management. We believe that, in cases of suspected oropharyngeal tumors, elective delivery should be carried out in a tertiary referral center in which emergency ventilation and tracheostomy are possible.