[Marfan syndrome and pregnancy. Apropos of 4 cases]. / Syndrome de Marfan et grossesse. A propos de quatre cas.

OBJECTIVES: To create a follow-up protocol for pregnant patients with Marfan syndrome. PATIENTS AND METHODS: We retrospectively reviewed the charts of patients who delivered in the Jeanne de Flandre University Hospital between June 1996 and June 1999. Four pregnant patients with Marfan syndrome were identified. RESULTS: Three of these patients had Bentall procedure. One of them had vaginal delivery and the two others underwent cesarean section. One of these two patients developed aortic valve thrombus at 14 weeks of amenorrhea. The fourth patient did not have surgery and had two vaginal deliveries. DISCUSSION: According to our results and after reviewing literature pregnant patients with Marfan syndrome were divided into two groups. The 1st group was comprised of patients who underwent Bentall procedure. The 2nd one was comprised of patients who did not undergo any surgical procedure. The possibility of vaginal delivery for patients who underwent Bentall procedure (one case) and the interest of Propanolol and anticoagulant treatment are emphasized. CONCLUSION: The multivariant approach of pregnant patients with Marfan syndrome is stressed out with special reference to the potential complications of this syndrome such as aortic dissection and to the problems related to the anticoagulant treatment.

[Severe maternal and fetal situations during delivery]. / Accouchement dans les situations extrêmes maternelles et foetales.

If pregnancy is very frequently normal, severe complications can appear for fetus, mother or both. The etiologies are various but preeclampsia and its complications remain one of the leading causes. The management is discussed according to the etiology and the severity of the disease and also the level of maternity and neonatal unit which can accept the newborn. However, all situation is a case apart and any decision will not be taken without concertation between obstetricians and neonatologists. In France, a regionalization policy is taking place to improve the maternal and neonatanal management.

[Diabetes before pregnancy, apropos of 143 cases]. / Diabète antérieur à la grossesse, à propos de 143 observations.

OBJECTIVE: The authors want to appraise the management of diabetes prior to pregnancy in a local population treated in the Lille University Hospital. METHOD: This is a retrospective study of 143 pregnancies occurring in 111 patients with diabetes prior to pregnancy, between 1987 and 1997, in the Obstetrics Department at the Lille University Hospital. RESULTS: Only one-third of the patients benefited from preconception management; the stability of diabetes during the first trimester was satisfactory in 50% of the cases. The maternal complications are represented by preeclampsia (20%), metabolic complications specific to diabetes (hypoglycemia, ketoacidosis), the aggravation or the emergence of a retinopathy (10%) and polyhydramnios (19%). Concerning the termination of the pregnancies, of the 147 fetuses (four twin pregnancies), 140 newborns in good health, two neonatal deaths, three in-utero deaths and two therapeutic terminations of pregnancy were observed. The fetal malformation rate was 9.5% (14 cases/147). The cesarean section rate was 63%, whereas the fetal macrosomatia rate was 35%, with dystocia in 26% of the deliveries (outside of planned cesareans). Three shoulder dystocia were observed (two requiring the Jacquemier's maneuver and one with transitory plexus brachial palsy for a newborn weighing 5,650 g). CONCLUSION: The authors conclude that preconception management (one-third of the patients in this series) and management of during the first trimester of pregnancy (50% in this series) was insufficient. This fact is perhaps due to the confusion, for many practitioners, with gestational diabetes, which is a very mediatized affection, though much less severe for the fetus and mother.

Intravenous nitroglycerin for internal podalic version of the second twin in transverse lie.

OBJECTIVE: To report our experience with high doses (0.1-0.2 mg per 10 kg pregnant weight) of intravenous (IV) nitroglycerin as a uterine relaxing agent for managing internal podalic version of the second twin in transverse lie with unruptured membranes. METHODS: Between August 1994 and December 1997, we managed 22 cases of internal podalic version of the second twin with the administration of high doses of IV nitroglycerin. RESULTS: Twenty internal podalic versions were completed successfully, and two cases failed. One failure was considered not related to IV nitroglycerin because the patient had a panic attack, requiring general anesthesia for sedation. The internal podalic version then succeeded. The patient with true failure of IV nitroglycerin required emergency cesarean because of acute fetal bradycardia and a nonrelaxed uterus. This was the only nontransverse lie, but with a very high face presentation. One internal podalic version was complicated by hemorrhage (2000 mL). CONCLUSION: Intravenous nitroglycerin to induce uterine atonia, with epidural analgesia, avoids general anesthesia and makes internal podalic version easier. In 22 cases (with success in 20) of internal podalic version of the second twin in transverse lie with unruptured membranes, IV nitroglycerin induced transient and prompt uterine relaxation without affecting maternal and fetal outcomes.

[Breech presentation: management (304 cases)]. / Présentation du siège: conduite à tenir (à propos de 304 observations).

UNLABELLED: BUT: Try to precise the optimal management in 1997 face a breech presentation. METHOD: Between January 1991 and December 1995, 304 cases of breech presentations were listed at the maternity of Pavillon Victor Olivier (Lille). From these 304 breech presentations, all parities blended, the authors have analysed the mode of delivery of these patients, distinguishing the para one from the multiparous women and the preterm babies from the other babies. From this study and a review of the literature, were discussed the criterions which can help the practitioner to determine the mode of delivery of these fetus. RESULTS: The results show a cesarean section (cs) rate of 51% (41% of first intention cs and 10% of second intention cs were realized only for primiparity + breech presentation and 67% of the para one women benefited from a first intention cs 8.72% of vaginal deliveries were complicated, and 5 fetal injuries were noted without sequel. On 296 live new-borns, only 2 cases of fetal death were perhaps due to the vaginal delivery. Fetal mortality is principally reliable at the prematurity and the congenital malformations, but finally, not due to mode of delivery. CONCLUSION: The difficulty is to determine rigorous criterions of selection to authorize a vaginal delivery without spoil the fetal pronostic. The major criterions are a perfect radiopelvimetry, an estimated fetal weight < 3800 g for the para one women and < 4300 g for the multiparous woman, a well flexed fetal head, favorable obstetric conditions ond the absence of maternal or fetal complications. The primiparity is not an indication of systematic cesariean section.

Fetal macrosomia: risk factors and outcome. A study of the outcome concerning 100 cases >4500 g.

OBJECTIVE: Because difficult vaginal delivery is more frequent with macrosomic fetuses, some authors recommend routine caesarean section for the delivery of fetuses >4500 g. The purpose of this study was to evaluate the appropriateness of this recommendation, in particular, to analyze maternal and fetal complications according to the mode of delivery. METHOD: Maternal and neonatal records of 100 infants with weights of at least 4500 g were identified retrospectively from January 1991 to December 1996. Outcome variables included the mode of delivery and the incidence of maternal and perinatal complications. RESULTS: The study sample consisted of 100 infant and mother pairs. Macrosomic fetuses represented 0.95% of all deliveries during this period and only ten were >5000 g. Mean birth weight was 4730 g (maximum, 5780 g). Gestational diabetes was present in nineteen patients. Diabetes was present in three patients. A trial of labour was allowed in 87 women, and elective caesarean delivery was performed in thirteen patients. The overall cesarean rate, including elective caesarean delivery and failed trial of labour, was 36%. Of those undergoing a trial of labour, 73% (64/87) delivered vaginally. Shoulder dystocia occurred fourteen times (22% of vaginal deliveries) and it was the most frequent complication in our series. There were five cases of Erb's palsy, one of which was associated with humeral fracture, and four cases of clavicular fracture. By three months of age, all affected infants were without sequelae. There was no related perinatal mortality and only two cases of birth asphyxia. Maternal complications with vaginal delivery of macrosomic infants included a high incidence of lacerations requiring repair (eleven cases). No complications were noticed in the patients who had a caesarean section. CONCLUSION: Vaginal delivery is a reasonable alternative to elective cesarean section for infants with estimated birth weights of less than 5000 g and a trial of labour can be offered. For the fetuses with estimated birth weight >5000 g, an elective caesarean section should be recommended, especially in primiparous women.

[Pelvic trauma and pregnancy. Literature review and case report]. / Traumatisme du bassin et grossesse. Revue de la littérature à propos d'un cas.

The authors are dealing with a case of a pelvic fracture (right superior pubic ramus) after a road traffic accident to a patient who arise a pregnancy with twins at twenty five weeks pregnant. Seven per cent of a road traffic accident affect pregnancy with a maternal death rate from eight to sixteen per cent and a fetal death rate up to fifty seven per cent. Pelvic trauma are more deleterious during the pregnancy because of the gravide uterus, of the abdominal injuries binding more often, and of pregnancy secondary maternal physiology which lead to delay diagnosis and therapeutics. Blunt fetomaternal consequences are ruled by pelvic haematomas, uterine rupture, prematurity, acute fetal distress, fetal injuries and in utero death. At mid and long range arise the problem of child birth way and the risk of mechanical dystocy. Cesarean is store in case of vesical and urethral injuries, or perineal injury, several pelvic fractures or in case of the pelvic belt fracture moved and not reduced, bringing to a surgical unsymmetrical pelvis. In the other cases, the obstetrical prognoses will be done after a dialogue with all medical staff and a full synthesis of the file based on the fetopelvic comparisons (obstetrics previous, clinic, fetal biometry, pelvimetry X ray).

[Ultrasonic diagnosis of placenta percreta. Apropos of a case with hemorrhagic complication]. / Diagnostic ultrasonore du placenta percreta. A propos d'une complication hémorragique.

Placenta percreta is a rare but severe disease, which is more and more frequent. The reported case shows that diagnosis can be made with B mode and color Doppler ultrasonography. Extension of high-vascularized placenta to the myometrium, abnormal placental-subplacental complex and vascular flow through the myometrium were suggestive of the diagnosis. Early diagnosis should decrease mortality and morbidity.

Myotonic dystrophy and pregnancy. A report of two cases and a review of the literature.

Myotonic dystrophy is a rare disease (1/8000), that is rarely associated with pregnancy, due to the fact that parents carrying the disease often encounter hypogonadism. Myotonic dystrophy is a neuro-endocrinian 'heredo-degenerative' dystrophy, with dominant autosomic transmission. Its association with pregnancy can lead to several problems. The myotony is often aggravated which leads to obstetrical complications turning into fetal loss, premature term delivery, hydrops, in-utero death, difficulties in expulsion, haemorrhage during delivery and/or anaesthetic accidents. The following signs during the pregnancy can diagnose fetal damage: presence of a hydrops, rare active fetal movements, and low fetal cardiac rhythm. They signify serious fetal damage leading to a diagnosis of myotonic dystrophy. Personal and family antecedents as well as an important hypotony and respiratory distress discovered in the new born are equally evocative elements. In congenital cases (6-30% of the time) the prognosis of the child is pessimistic. For all of the above elements, transmission is of maternal origin. The diagnosis of the congenital form is difficult because the disease is often unknown by the mother. The appearance of molecular tools permits a diagnosis to be formed much more rapidly in a new-born suspected to carry the illness of neonatal Steinert. Two observations illustrate this pathology. The occurrence of congenital myotonic dystrophy in a new-born allows us to diagnose the disease within the mother.

[Panorama of uropathies discovered during the prenatal period. 147 cases]. / Panorama des uropathies dépistées en période anténatale. A propos de 147 observations.

OBJECTIVES: The aim of this study was to establish the panorama of uropathies discovered during the antenatal period and to analyze the explorations performed. Pregnancy outcome and infant prognosis was also recorded. METHOD: Ultrasonographic imaging revealed dilatation in 62.5% of the cases, parenchymal anomalies in 26.3% and unilateral or bilateral agenesia in 11.2%. The percentage of abnormal karyotypes was 4.76% for all urorenal symptomatologies. These abnormal karyotypes corresponded to 10% of those performed in 17 fetuses, urine puncture was used in order to assess in utero renal function. There were 113 live births, 31 medically termined pregnancies and 3 spontaneous abortions. Among the 113 live infants, 12 died during the post-natal period. Thirty-two infants were considered to be normal and 69 had an urorenal malformation, including 2 infants with pre-end-stage renal failure at 4 and 3 years. CONCLUSION: It is uncommon to discover an urorenal malformation at prenatal ultrasonography. The main problem is antenatal management and evaluation of prognosis. Urine puncture and in utero derivation are discussed. When no other reliable factors affecting fetal prognosis are available, puncture of fetal urine provides useful information for management although the technique remains under debate.

[Prenatal diagnosis of partial trisomy 9p]. / Diagnostic anténatal d'une trisomie 9p partielle.

The prenatal diagnosis of trisomy for the distal half of the short arm of n(o) 9 chromosome (partial trisomy 9p) has been realized from a morphologic ultrasound. A genetic investigation has permitted to establish that this trisomy was due to a bad segregation of a stable translocation present in the patient's mother. To our knowledge, the ultrasound prenatal diagnosis of partial trisomy 9p has never been reported in the literature. The prognosis of this syndrome remains very pejorative and the termination of pregnancy is the most often proposed solution.

[Pregnancy in women with a mechanical heart valve. Review of the literature]. / Grossesse chez les femmes porteuses d'une valve cardiaque mécanique, Revue de la littérature.

AIM: To define therapeutic ways to manage obstetrics at an optimal level for a patient carrying a mechanical prosthetic heart valve. METHOD: From a review of literature and documented cases, we propose an obstetrical and cardiological management scheme for pregnant patients carrying a mechanical prosthetic heart valve. We deal successively with the preventive (anticoagulation) and curative treatment (surgery and thrombolytic therapy) of valvular thrombosis, in fact, a severe -but unfortunately frequent- complication of these pregnancies. RESULTS: Pregnancy concerning patients with mechanical prosthetic heart valves is a high-risk pregnancy. In fact, the risk of thrombo-embolic accidents even with a closely followed anticoagulant treatment seems to come from the existence of the prothese, the state of physiological hypercoagulation and peri-partum hemorrhages. Naturally, multidisciplinary follow-up is indispensable. The prescription of anticoagulant treatments must respect the main principals and the normal counter-indications in order to minimise maternal and fetal complications. CONCLUSION: Pregnancy amongst patients carrying mechanical prosthetic heart valves should be considered rare and highly exceptional cases, from a cardio-vascular surgeon's point of view. For obstetricians, it is sometimes difficult to forbid pregnancy to a nullipara. The discussion whether to authorize a pregnancy will be treated case-by-case, holding into account the socio-cultural environment of the patient, as the principles of an anticoagulant treatment and the underlying risks must be well understood.

Intravenous nitroglycerin for intrapartum internal podalic version of the second non-vertex twin.

OBJECTIVE: Authors report their experience of intravenous nitroglycerin as uterine relaxing agent for managing successfully internal podalic version of the second twin. METHODS: From a retrospective study including nine observations of internal podalic version of the second non vertex twin performed with administration of intravenous nitroglycerin, between August 1994 and February 1996, authors compare their results with those reported elsewhere. RESULTS: Two failures of internal podalic version with nitroglycerin have been observed. But one failure is not considered to be due to the NTG: it was a patient, who had a panic attack necessitating a general anesthesia for sedative purpose. The internal podalic version succeeded. The true failure of NTG needed an emergency cesarean due to acute fetal distress and a non relaxing uterus. One internal podalic version was complicated by hemorrhage. The intravenous NTG used to induce uterine atonia associated with epidural-analgesia to relief pain avoiding general anesthesia makes internal podalic version easier. CONCLUSION: Our results confirmed those already reported. That intravenous nitroglycerin (NTG) injection induces a transient and prompt uterine relaxation required for internal podalic version without affecting maternal and fetal prognosis.

Varicella and pregnancy.

OBJECTIVE: To appreciate the risk of embryo-foetopathy in case of maternal varicella occurring before 20 weeks of gestation, as well as the maternal complication risk (notably pulmonary) in case of maternal varicella occurring the third trimester of pregnancy. METHOD: Over the period from January 1987 to February 1995, 20 patients were managed for maternal varicella confirmed during the pregnancy. From these observations, the authors, by studying the literature, attempt to better specify the real fetal and/or maternal complication risk in case of maternal varicella. RESULTS: In their personal series of 20 cases, including 17 before 20 weeks of gestation, the authors have noted no embryo-foetopathy. Similarly, no maternal complication (notably pulmonary complication), has been found. Careful study of the literature allows to specify some points. In case of varicella before 20 weeks, one observes an identical frequency of spontaneous abortions, as compared to the general population and a moderated increase of the frequency of premature delivery. The risk of congenital varicella syndrome reaches about 1.3%. Finally the risk of neonatal varicella consists in a maternal infection which occurs during the perinatal period and which is source of a high perinatal morbidity. The prenatal diagnosis is based essentially and currently, on the amniocentesis with viral research by polymerase chain reaction (PCR) in the amniotic fluid, completed by a ultrasound supervision. CONCLUSION: The occurrence of maternal varicella during the pregnancy is rare (0.7/1000) because more than 90% of women are immunized. The risk of congenital varicella syndrome is limited to the 20 first weeks and seems very weak, authorizing therapists to reassure patients presenting a varicella during their pregnancy. Nevertheless, the risk of pulmonary complications for the mother, in case of varicella during the third trimester, does exist and requires appropriated treatment.

Monoamniotic twin pregnancies. Review of the literature, and a case report with vaginal delivery.

OBJECTIVE: To specify the process of the sometimes difficult diagnosis of monoamniotic twin pregnancies, as well as the best practise for delivery of this type of pregnancy. METHOD: Using their personal observation (a patient with a monoamniotic twin pregnancy, which presented a vaginal delivery at 35 weeks of gestation, two girls, in cephalic presentation, without particular problem, despite an entanglement of the cord and the existence of a knot), the authors established a review of the literature on this subject. RESULTS: Monoamniotic twin pregnancies represent a rare possibility. The prognosis is traditionally somber: 40-60% mortality, mainly due to pathologies of the cord. The review of the recent literature shows that most authors remain in favour of weekly ultrasound supervision from the 23rd week and of caesarean section in principle at 34 weeks (or from fetal pulmonary maturation). CONCLUSION: In the absence of funicular compression signs by colour-doppler, and under the cover of flawless obstetrical conditions, vaginal delivery can only be authorized for cases when both presentations are cephalic.

Prenatal diagnosis of thrombocytopenia-absent radius syndrome.

Thrombocytopenia-absent radius (TAR) syndrome is an autosomal-recessive disorder characterized by a thrombocytopenia and a bilateral radial aplasia with normal thumbs. Only TAR syndrome, out of diseases which may present with radial aplasia, typically has normal thumbs. The prenatal diagnosis is rarely made. We report two observations of TAR syndrome diagnosed in utero in the sibling. The malposition of fetal hands detectable as soon as 11 weeks of gestation requires careful search for longitudinal limb defect of the forearm, especially radial ray defect. The radial aplasia is associated with numerous causes (chromosomal, teratogenic, genetic, multifactorial). The determination of fetal hematologic values revealing a thrombocytopenia allows the prenatal diagnosis of the index case of TAR syndrome.