Lethal acute aortic dissection complicated with ischemic bowels in the third trimester of pregnancy: A case report

ObjectiveAortic dissection is a rare but lethal disease. We demonstrate a rare case of acute complicated Stanford type A aortic dissection in the third trimester.Case reportA 41-year-old primiparous patient with chronic hypertension was brought to our emergency department at 34 weeks of gestation. She presented with acute tearing chest pain radiating to her back. Computed tomography revealed a Stanford type A aortic dissection. Primary cesarean section followed by open cardiovascular surgery was performed. However, ischemic bowels and bowel perforation occurred on the 9th and 11th postoperative days, respectively, and the patient died of septic shock on the 12th postoperative day.ConclusionAortic dissection must be taken into consideration for pregnant women with chronic hypertension who present with acute tearing chest pain and pulseless unilateral extremities. Bowel ischemia may occur in patients with complicated Stanford type A aortic dissection.     

A novel nonsense mutation of TGFBR1 in a fetus with untypical Loeys-Dietz syndrome 1

ObjectiveWe present a rare untypical Loeys-Dietz syndrome 1 case in prenatal setting and report a novel mutation in the TGFBR1 gene.Case reportA pregnant woman came for medical attention due to the fetal ultrasound anomaly. The fetus was found to have short long bones. Trio-based WES was applied to the family. A novel de novo nonsense mutation c.1237C > T was detected in the TGFBR1 gene. A diagnosis of Loeys-Dietz syndrome 1 (LDS1) was plausible, but the fetus did not demonstrate the characteristic phenotype of the syndrome.ConclusionIn prenatal setting, fetal phenotypes are difficult to be fully observed, putting stress on the utility of molecular techniques. LDS1 in fetuses could present untypical features such as skeletal dysplasia.     

Turner syndrome and pregnancy: clinical practice. Recommendations for the management of patients with Turner syndrome before and during pregnancy

Following the death in France by acute aortic dissection of two women with Turner syndrome who were pregnant following oocyte donation, the Director of the French Biomedicine Agency (Agence de la biomédecine) sent a letter to the President of the French College of Obstetricians and Gynaecologists (FCOG). He requested the College's expertise in reviewing point-by-point the cases and risk factors and in determining whether there are grounds to propose additional measures complementary to the recommendations made by the Haute autorité de santé or French National Authority for Health (HAS) in 2008 in terms of indication and monitoring of patients. A joint practice committee of the FCOG, the French Cardiologic Society, the French Chest and Cardiovascular Surgery Society, the French Society of Anaesthesia and Intensive Care, the French Endocrine Society, the French study group for oocyte donation, and the Biomedicine Agency defined the exact questions to be put to the experts, chose these experts, followed them up and drafted the synthesis of recommendations resulting from their work.The questions concerned the check-up before pregnancy of Turner patients, contraindication and acceptance of pregnancy, information for the patients, and recommendations for antenatal care, delivery and postnatal follow-up.     

Recurrent immature teratoma of the ovary: a case report of radical secondary cytoreduction with replacement of the aortic bifurcation

BACKGROUND: Composing of less than 1% of all ovarian cancers, immature teratoma is a malignancy that mainly affects the young. Although standard treatment protocol for primary disease has improved over the past decades, the role of secondary cytoreduction in the setting of recurrence remains controversial. CASE: A 20-year-old Chinese female presented with recurrent immature teratoma at the level of the aortic bifurcation. Patient was status post a right salpingo-oophorectomy at time of her initial diagnosis 8 years ago, and subsequent exploratory laparotomies and chemotherapy when recurrence that developed at the before-mentioned site. She ultimately underwent a radical cytoreduction with en-block excision of the aortic mass and simultaneous artificial aortic bifurcation graft. CONCLUSION: There may be a beneficial role of radical secondary cytoreduction in the setting of recurrent immature teratoma.     

Pregnancy and childbirth in a patient with multiple sclerosis: A case report of successful management

Objective

Multiple sclerosis (MS) preferentially affects females of reproductive age, making reproduction an important issue for women with MS. An increased incidence of poor labor progress often results in assisted vaginal delivery or a cesarean section. However, with good pre-pregnancy counseling and management, women with MS can conceive and give birth safely. Here, we present a case of pregnancy with MS, which was carried to term uneventfully and ended with unassisted vaginal delivery.

Emergent cesarean section in a preterm pregnant woman with severe COVID-19 pneumonia in Taiwan: A case report

ObjectiveThe pandemic Coronavirus Disease 2019 (COVID-19) is a global public health crisis. Many maternity units worldwide are currently establishing the management protocols for these patients.Case reportWe report the first critically ill pregnant woman with COVID-19-induced respiratory failure undergoing emergent caesarean delivery at 32 weeks of gestation, in the setting of a positive pressure operating room (OR) with negative pressure anteroom in Taiwan.ConclusionMultidisciplinary planning and collaboration are necessary to achieve satisfactory clinical outcomes in pregnancies with critical COVID-19 pneumonia. The combinations of comprehensive evaluation, timely treatment as well as establishment of rigorous protocol and safe environment for the emergent delivery are important.     

Hepatic rupture after cesarean section in a patient with HELLP syndrome: a case report and review of the literature

Background Spontaneous hepatic rupture in pregnancy is rare and carries a high maternal and perinatal mortality. Case report We describe a case of hepatic rupture that occurred after emergency caesarean section performed in a pregnant woman at 38 weeks of gestation with Hemolysis, Elevated Liver enzymes and Low Platelet count (HELLP) syndrome. The patient was discharged after 30 days of intensive therapy and four explorative laparotomies. Today, 2 years after delivery, the patient and her baby are doing well. Conclusion Hepatic rupture in pregnancy is a rare event that may complicate HELLP syndrome. In this case, we were able to achieve an excellent clinical outcome by the performance of an immediately explorative laparotomy.     

无症状伴肺部影像学改变的新型冠状病毒肺炎孕妇一例

本文为孕晚期无症状新型冠状病毒肺炎(COVID-19)一例报告,孕妇分娩前有胸部CT典型肺炎表现,母儿结局良好。患者孕37周+3因新型冠状病毒核酸检测阳性7 h余于2020年2月12日由外院转入宜春市人民医院。患者孕期定期产前检查无异常,入院前无任何不适。患者的丈夫和婆婆分别于患者入院前8 d和当日确诊为COVID-19。入院第2天胸部CT示右肺下叶见斑片状模糊影,胎心监测发现基线微小变异,考虑胎儿窘迫可能,在做好防护的情况下行,急诊剖宫产娩出一活女婴。术后产妇及新生儿分别送至隔离病区进行单间隔离观察治疗。参与剖宫产手术相关医护人员术后均采取单间隔离。术后给予患者抗病毒和抗感染治疗,患者恢复良好,仍无发热、乏力、咳嗽等症状,术后3和5 d咽拭子新型冠状病毒核酸检测均为阴性,术后4 d胸部CT提示右肺下叶炎症、术后7 d复查示右肺下叶炎症稍吸收。新生儿分别于出生当天、4日龄和7日龄取外周血和咽拭子标本行新型冠状病毒核酸检测,结果均为阴性。母女术后9 d顺利出院。参与手术的相关医护人员术后7和14 d咽拭子新型冠状病毒核酸检测结果均阴性。     

Maternal and fetal outcomes of the pregnant woman with COVID-19: The first case report in Taiwan

ObjectiveA real-Taiwan experience to deal with near-term pregnant woman infected by severe acute respiratory syndrome coronavirus 2, SARS-CoV-2 (coronavirus disease 2019, COVID-19) is extremely limited. We described the first case in Taiwan.Case reportA 30-year-old woman, primigravida had a laboratory-confirmed COVID-19 infection at 36 gestational weeks (GW). She was asymptomatic. Ten days later, she was hospitalized and receive a selective cesarean section with a term baby weighted 3142 gm (Apgar score 8 and 9 at 1st and 5th minute, respectively) at 38 GW. No evidence of in utero and direct transmission was found and newborn was free of COVID-19.ConclusionIt is still uncertain whether timing or mode of delivery is appropriate in SARS-CoV-2 infected pregnant woman in near term, but we suggested that a selective delivery time at 38 GW or later, regardless of which mode of delivery is finally decided, can be considered.     

Cerebral haemorrhage in a pregnant woman with a multiple endocrine neoplasia syndrome (type 2A or Sipple's syndrome)

We present the case history of a 30-yr-old woman who suffered a cerebral haemorrhage toward the end of pregnancy (at 35 wk). The pregnancy was terminated by Caesarean section because aggravation of cerebral bleeding was feared. Extensive studies revealed that the patient was suffering from a multiple endocrine neoplasia syndrome (type 2A or Sipple's syndrome) with bilateral pheochromocytomas and a medullary thyroid carcinoma. She made an uneventful recovery. The relevant literature is discussed.     

Group A streptococcal toxic shock syndrome associated with pelvic peritonitis following diagnostic hysteroscopy in a healthy woman: a case report and review

We describe a case of group A streptococcal toxic shock syndrome (TSS) associated with pelvic peritonitis, occurring after a diagnostic hysteroscopy and curettage in a healthy woman. At laparotomy, performed to rule out bowel perforation, the diagnosis of pelvic inflammatory disease and pelvic peritonitis was confirmed. Her general condition deteriorated postoperatively needing supportive care in the intensive care unit, followed by gradual recovery. Although it is difficult to avoid a laparotomy in most cases following a surgical procedure, it is important to consider the diagnosis of streptococcal TSS associated with pelvic peritonitis from ascending sepsis in rapid onset, severely shocked cases.     

Adrenalectomy for adrenocortical adenoma causing Cushing's syndrome in pregnancy: a case report and review of literature

We present a case of adrenocorticotropic hormone (ACTH)-independent Cushing's syndrome diagnosed in a patient in the third trimester of her pregnancy, with an adrenal mass observed on imaging studies. Laparoscopic adrenalectomy was performed successfully at 32 weeks. To the best of our knowledge, this is the latest gestational age at which laparoscopic adrenalectomy has been reported. We present the various considerations for determining the surgical approach and the optimal timing for surgery. Adrenalectomy during pregnancy for the treatment of Cushing's syndrome caused by adrenocortical adenoma has been reported in 23 patients in the English-language medical literature to date and seems safe and beneficial. According to the data, surgical treatment has led to a reduction in perinatal mortality and maternal morbidity rates, but has not affected the occurrence of preterm birth and intrauterine growth restriction. The best outcome can be achieved by a multidisciplinary approach, with a team comprising a maternal–fetal medicine specialist, an endocrinologist and a surgeon. The timing of surgery and the surgical approach need to be determined according to the surgeon's expertise, the severity of the condition, the patient's preferences, and gestational age. Laparoscopy may prove to be the preferred surgical approach. The small number of cases precludes providing evidence-based recommendations.     

Major depressive disorder in an adolescent with Turner syndrome: a case report

Turner syndrome (TS) is a chromosomal abnormality, of which the presence and impact of coexisting psychiatric morbidity has received little attention. The present report describes an adolescent with mosaic karyotype TS who had major depressive disorder with the predisposing cause of psychosocial burden, and relieved with the treatment of sertraline and complete remission with combined use of estradiol valerate. The report suggests us to pay more attention on the mood disorders in children with TS, especially in adolescents. For treatment aspect, medications for improving the puberty development and short stature should be added to in addition to antidepressants if they had mood disorders.     

Clinical management and obstetric outcome in WFS1 Wolfram syndrome spectrum disorder: A case report and literature review

ObjectiveWolfram Syndrome (WS) is a rare autosomal recessive neurodegenerative disorder caused by mutations in WFS1 or CISD2 (WFS2). We present a rare case report of pregnancy with WFS1 spectrum disorder (WFS1-SD) in our hospital and reviewed literature to provide the management of pregnancy in these patients through multi-disciplinary cooperation.Case reportA 31-year-old (gravida 6, para 1) woman with WFS1-SD conceived naturally. During the pregnancy, she adjusted insulin intermittently to control blood glucose and monitored intraocular pressure changes under the guidance of doctors without any complications. Cesarean section was delivered at 37⁺⁴ weeks of gestation due to breech position and uterine scar and the neonatal weight was 3200 g. Apgar score 10 at 1 min, 10 at 5-min and 10 at 10 min, respectively. This rare case had a good maternal and infant outcome under multidisciplinary management.ConclusionWS is an extremely rare disease. Limited information is available on the impact and management of WS on maternal physiologic adaptation and fetal outcome. This case provide a guide for clinicians to raise awareness of this rare disease and strengthen the management of pregnancy in these patients.     

Septostomy with amniodrainage in the treatment of twin-to-twin transfusion syndrome: a 16-case report

Objective To report the treatment results of 16 monochorionic and diamniotic gestations cases, which had complications due to the twin-to-twin transfusion syndrome (TTTS), the neonatal and other possible complications of the septostomy associated to the amniodrainage. Method Based on ultrasonographic findings, 16 pregnant women were diagnosed with the twin-to-twin transfusion syndrome (TTTS). These cases were divided in two groups: one of them included the fetuses without hydrops and the second included the “recipient” fetuses with hydrops. The therapy measures included septostomy with or without amniodrainage. The following parameters were evaluated: gestational age at the time the septostomy was performed, volume of drained amniotic fluid, gestational age at delivery, birth weight, postnatal evolution, and procedure complications. Results The average gestational age for this procedure was of 23.6 weeks (from 14 weeks and 1 day to 33 weeks). The gestational age for the septostomy until the delivery was of 8.18 weeks (from 1.0 to 21.3 weeks). The survival rate in the group without hydrops was of 68.7%, while in the second group it was of 25%. Some of the complications were as follows: two cases of premature membrane rupture and one case of preterm labor. Conclusion Septostomy with amniodrainage, when performed on the initial stages and on earlier gestational ages has good perinatal results.     

妊娠晚期危重型新型冠状病毒肺炎一例

本文报告一例妊娠晚期合并新型冠状病毒肺炎(COVID-19)危重型孕妇。患者32岁,孕35周+2因"咽痛4 d,发热3 h"入院。入院前曾至湖北孝感应城,回家隔离期间发病。入院后病情进展迅速,7 h后出现左侧胸背部疼痛、气促、头晕,迅即出现呼吸衰竭、感染性休克。考虑病情严重,且有2次剖宫产史,行紧急子宫下段剖宫产术。术前血气分析示呼吸衰竭、呼吸性酸中毒合并代谢性酸中毒。术中娩出一男婴,2700 g,1 min Apgar评分1分(心率20次/min),经复苏抢救,5和10 min评分均为1分,家属放弃抢救后死亡。产妇术后气管插管呼吸机维持,同时给予其他支持治疗。入院时的新型冠状病毒核酸检测结果于分娩后报告为阳性,遂转入定点医院治疗。患者术后26 d体外膜肺氧合脱机成功,术后36 d脱离呼吸机,进入康复阶段。