Gestion du péri-partum de la patiente conductrice d’une hémophilie

Women who are carriers for hemophilia are usually considered as safe carriers. However, they can present hemorragic symptoms associated with low factor VIII or IX levels. During pregancy, factor VIII increases whereas factor IX does not. The peripartum period is at risk of increased bleeding in these women. Here are presented reports of clinical data concerning two hemophilia carriers with low factor VIII or IX (30–40%) during the peripartum period. They received remifentanil and ketamine for labor pain management because of contraindication of epidural and spinal analgesia. Delivery occured quickly but they presented immediate moderate postpartum haemorrage. They did not necessitate blood transfusion. The one with hemophilia A received desmopressin just after delivery and the other one received factor IX when she arrived in delivery room. Blood factor VIII or IX has to be assessed in these women with familial history of hemophilia and bleeding. During pregnancy, factor VIII increases and can be assessed many times during pregnancy expecting a level over 50%. Factor IX does not really increase during pregancy and hemorrage can occur. Epidural and spinal anesthesia seem to be contraindicated as far as recommandations are concerned. Coagulation factor substitution is a mean of increasing factor level before these anaesthesias and can be discussed for each case.     

Transabdominal cervico-isthmic cerclage in the management of cervical incompetence in high risk women

Objectives

Transabdominal cervico-isthmic cerclage is an alternative to vaginal route for patients with severe cervical incompetence. The purpose of this study is to describe our experience with this technique during pregnancy for high risk women.

Materials and methods

We performed a retrospective study including 12 transabdominal cerclages performed between 1988 and 2005. All patients had an history of repeated midtrimester fetal losses or preterm delivery, and 82% already had a prior failed transvaginal cerclage. Fourteen pregnancies were reported.

Results

The median gestational age at cerclage placement was 14 weeks (range: 12 to 17). All patients underwent a caesarean section at a mean gestational age of 35 weeks gestation (range: 23 to 38 WG). The fetal survival rate was 93 compared to 17% before the cerclage. There were no significant perioperative or neonatal complications.

Conclusion

Transabdominal cervico-isthmic cerclage remains a reliable technique for the management of cervical incompetence after a prior failed transvaginal cerclage when vaginal access is difficult. Laparoscopic approach is under development.     

Hypokaliemic periodic paralysis and pregnancy: perinatal management. A case report

Familial hypokalemic periodic paralysis (FHPP) is a rare inherited disease characterized by a dysfunction of the membrane ion channels. Clinical manifestations are attacks of hypokaliemia with flaccid muscle paralysis. Paralysis is sometimes severe but always reversible with symptomatic treatment. Pregnancy and delivery have been reported to exacerbate FHPP. Authors report a case of FHPP during pregnancy with a favourable outcome. Vaginal delivery is usually possible with monitoring and epidural analgesia, avoiding active maternal expulsive efforts (passive descent of the fetus and elective outlet forceps) and other stimulating factors (carbohydrate loads, maternal stress, betamimetics, epinephrine...). Administration of IV potassium supplementation is often necessary.     

Prise en charge anesthésique d’un accouchement chez une patiente atteinte d’un déficit en inhibiteur de C1 estérase

Hereditary and acquired angioedema (HAE/AAE) are the clinical translation of a qualitative or a quantitative deficit of C1 esterase inhibitor (C1 INH). The frequency and severity of clinical manifestations vary greatly, ranging from a moderate swelling of the extremities to obstruction of upper airway. Anaesthesiologists and intensivists must be prepared to manage acute manifestations of this disease in case of life-threatening laryngeal edema. Surgery, physical trauma and labour are classical triggers of the disease. The anaesthesiologists should be aware of the drugs used as prophylaxis and treatment of acute attacks when considering labour and caesarean section. Androgens are contraindicated during pregnancy. If prophylaxis is required, tranexamic acid may be used with caution. The safest obstetric approach appears to be to administer a predelivery infusion of C1 INH concentrate. It is important to avoid manipulation of the airway as much as possible by relying on regional techniques. We report the case of a patient suffering from an HAE discovered during pregnancy. The management included administration of C1 INH during labor and early epidural analgesia for pain relief. A short review of the pathophysiology and therapeutic options follows.     

Myasthenia gravis and pregnancy: clinical course and management of delivery and the postpartum phase

OBJECTIVES: To study influences of pregnancy on the time-course of myasthenia gravis (MG) and of MG on pregnancy, delivery, postpartum and newborn. METHODS: We retrospectively collected data from 100 women affected with MG, hospitalized between 1994 and 2003 in departments of Neurology of Lille University Hospital. RESULTS: Eighteen patients had a total of 36 pregnancies, occurring 7.2 years on average after MG onset. MG exacerbation occurred in 7 patients (26 percent) during pregnancy and in 4 (14.8 percent) during postpartum. One patient died of acute respiratory failure during postpartum. Delay between the onset of MG and pregnancy was the only variable significantly associated with MG exacerbation: 5.8 years when exacerbation and 9.5 years when no exacerbation (p=0.03). Seven miscarriages, two therapeutic abortions and no death at birth were reported. Levels of anti-acetylcholine receptor antibodies were abnormal in 3 of 27 newborns (11 percent), but only one (3.7 percent) developed seronegative transient neonatal myasthenia gravis. DISCUSSION: During pregnancy, the clinical course of MG is variable but exacerbations were associated with a shorter delay between MG diagnosis and pregnancy. The risk of transient neonatal myasthenia gravis is relatively small but exists even when the parturient has stable MG without elevated levels of anti-acetylcholine receptor antibodies. CONCLUSION: Our study confirms pregnancy is more difficult to manage at the beginning of MG. Given the unpredictable course of MG during pregnancy, we recommend women affected with MG to begin a pregnancy when the disease is stable.