Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder characterized by capillary malformation, varicose veins, and limb hypertrophy. Women with KTS face increased risks of thromboembolism and hemorrhage during pregnancy, delivery, and the postpartum period. Intrahepatic cholestasis of pregnancy (ICP), the most common pregnancy-related liver disorder, is associated with adverse perinatal outcomes. However, coexistence of KTS and ICP has not been previously reported. We describe the case of a 29-year-old pregnant woman with known KTS who developed ICP in the third trimester. She was managed with ursodeoxycholic acid and prophylactic anticoagulation. Despite therapy, her serum bile acids remained elevated, and fetal monitoring revealed decelerations, prompting cesarean delivery at 35 weeks of gestation. The newborn was preterm but otherwise healthy. Postpartum, the patient experienced progressive coagulopathy with markedly elevated D-dimer levels, superficial vein thrombosis, and clinical signs of disseminated intravascular coagulation (DIC). With adjustment of anticoagulation and administration of fresh frozen plasma and fibrinogen, her coagulation profile gradually normalized. She was discharged on rivaroxaban and remained stable during follow-up. This case highlights the complexity of managing pregnancy in women with KTS complicated by ICP. The coexistence of two high-risk conditions poses challenges in balancing thrombosis and bleeding risks, as well as safeguarding fetal outcomes. Multidisciplinary care, close monitoring, and individualized anticoagulation strategies are essential to optimize maternal and neonatal prognosis.

Peripartum cardiomyopathy (PPCM) is a rare but serious form of heart failure that occurs toward the end of pregnancy or in the earlypostpartum period. We present the case of a 28-year-old woman who developed severe biventricular heart failure and cardiac arrest shortly after childbirth. Despite treatment with mechanical circulatory support with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and intra-aorticballoon pump (IABP), her condition did not improve, and she was urgently referred for heart transplantation. Histological examination of theexplanted heart revealed noncompaction cardiomyopathy (NCC), a rare structural heart disease that may mimic PPCM. This case highlights the challenges in diagnosing PPCM and the importance of early intervention, including advanced support and transplant, in cases with poor recovery prospects.

Factor (F)XI deficiency is a rare bleeding disorder with a poor correlation between bleeding tendency and FXI level. Management of pregnant women with FXI deficiency is not clearly established, especially regarding neuraxial analgesia (NA). A retrospective multicenter observational study was conducted in French hemostasis centers on pregnant women with FXI of <60 IU/dL. Data to report were (i) FXI levels before pregnancy and at time of delivery, (ii) type of NA and delivery management modalities, and (iii) possible complications related to NA and bleeding complications. Three hundred fourteen pregnancies in patients with FXI deficiency of <60 IU/dL were reported (from 20 centers); among them, 199 NA procedures have been completed (137 epidurals and 61 spinals, 1 had both). The period of childbirth was mostly from 2014 to 2020 (281/314; 89.5%). Congenital FXI deficiency was established with certainty by investigators in 32.8% patients (n = 103). Previous bleedings were described in 20.4% of the patients (64/314; 45.3% cutaneous, 31.3% gynecologic, and 15.6% postsurgical). Thirteen deliveries had an NA procedure with FXI of <30 IU/dL, 42 with FXI of 30-40 IU/dL, and 118 with FXI of 40-60 IU/dL. Median FXI levels at delivery in the epidural and spinal groups were not significantly different but were significantly lower in the group without NA by medical staff contraindications. There were no complications related to NA. A 17.5% postpartum hemorrhage or excessive postpartum bleeding incidence was reported, which is consistent with previous data. Our data support the use of a 30 IU/dL FXI threshold for NA, as suggested by the French proposals published in August 2023.

Pregnancy and breastfeeding are associated with bone density loss. Fracture occurrence during pregnancy and post-partum, and its determinants, remain poorly known in Osteogenesis Imperfecta (OI). The aim of this study was to characterize fractures that occurred during pregnancy and post-partum in OI patients. We conducted a retrospective multicentric study including a total of 50 previously pregnant OI women from 10 Bone Centers in France. Among these patients, 12 (24%) patients experienced fractures during pregnancy or in the 6 months following delivery, and 38 (76%) did not experience any fracture. The most frequent localizations were: proximal femur (25%), spine (25%), distal femur (12.5%), and pelvis (12.5%). Fractures during pregnancy occurred during the third trimester and post-partum fractures occurred with a mean delay of 2 months following delivery. No fractures occurred during childbirth. We next compared the 12 patients with pregnancy or post-partum fractures with the 38 patients without fractures. Mean age at pregnancy was 32.7 ± 3.1 years-old in the fractured group, vs 29.3 ± 5.0 years-old in the non-fractured group (p = 0.002). Breastfeeding was reported in 85.7% of patients in the fractured group, vs 47.1% in the non-fractured group (p = 0.03). All patients with post-partum fractures were breastfeeding. Bone mineral density was significantly lower in patients with pregnancy-related fractures compared with other patients: spine Z-score - 2.9 ± 1.6DS vs - 1.5 ± 1.7DS (p = 0.03), and total hip Z-score - 2.0 ± 0.7DS vs - 0.5 ± 1.4DS (p = 0.04). At least one osteoporosis-inducing risk factor or disease other than OI was identified in 81.8% vs 58.6% of fractured vs non-fractured patients (not significant). Fracture during pregnancy or post-partum was not associated with the severity of OI. Bisphosphonates before pregnancy were reported in 16.7% and 21.1% of patients with pregnancy-related fractures and non-fractured patients, respectively (not significant). OI management during pregnancy and post-partum should aim for optimal control of modifiable osteoporosis risk factors, particularly in patients with low BMD. Breastfeeding should be avoided.

For a healthy parturient, a cardiopulmonary collapse that suddenly occurs shortly after an uneventful caesarean section is a relatively rare event and presents a significant challenge for the anesthesia provider. Amniotic fluid embolism (AFE) is characterized by acute and rapid collapse and is well known to the obstetric team. Our patient experienced sudden cardiovascular collapse, severe respiratory difficulty and hypoxia, in the absence of other explanations for these findings at the time, and thus AFE was immediately become the focus of the consideration. However, there is no quick, standard laboratory test for AFE, therefore the diagnosis is one of exclusion based on presenting symptoms and clinical course. After given symptomatic treatment, the patient made an uneventful initial recovery in a short period and developed a rash. We recognized that the postpartum shock was associated with delayed anaphylaxis of antibiotics. These observations have implications for understanding whenever administering drugs in surgery, which may affect the anesthesiologist's judgment regarding the complications of anesthesia. Even though serious complications of common perioperative drugs may rarely occur, anesthesia providers should be aware of the consideration. Early recognition and effective treatment are more important than prompt diagnosis.