Management of parturients with Factor XI deficiency-10year case series and review of literature.

This is an article reviewing the management of pregnant women with factor XI (FXI) deficiency. Retrospective review of the electronic records of 67 pregnancies in 25 women with FXI deficiency over a ten-year period was undertaken. All women received care at St Mary's Tertiary Referral Obstetric/Haematology Clinic for some or all of their pregnancies. Outcome measures included antenatal complications, mode of delivery, anaesthesia provided and postpartum haemorrhage (PPH) and management required. A positive bleeding history was identified in 50% of women prior to pregnancy. Fifteen pregnancies (22%) ended in first trimester miscarriage; there was 1 termination of pregnancy. Two pregnancies were complicated by Antepartum haemorrhage. Of the remaining 51 pregnancies there were 50 live births - 2 preterm and 48 at term. There was one antenatal (34 weeks gestation) stillbirth of a growth restricted baby and one neonatal death secondary to severe prematurity (24 weeks gestation). Twenty -five babies delivered vaginally (20 spontaneous and 5 instrumental). The remaining 26 were delivered by Caesarean section (9 elective and 17 emergency). A sub-analysis of 22 operative deliveries was reviewed; this suggested that regional anaesthesia was safe in selected women with FXI deficiency - a selection that was based on FXI level/range, presence/absence of bleeding history and intended operative intervention.Solvent detergent treated Fresh Frozen Plasma (SD-FFP/Octaplas) and Tranexamic Acid (TXA) were given to those considered vulnerable -an individualised decision made by the multidisciplinary team in accordance with BCSH guidance. Primary PPH complicated 10/51 (15%) deliveries. The commonest cause of PPH was due to atony. Secondary PPH was only seen in only one case. Bleeding in women with FXI deficiency is highly variable and, whilst it does not directly correlate with Factor XI levels, provision of replacement therapy is required if FXI levels are <15 IU/dL as per BCSH guidance. Women with Factor XI levels >40 IU/dL are considered safe for regional anaesthesia following prophylactic FFP as suggested by sub group analysis. Treatment of women with rare bleeding disorders during pregnancy should be by a multidisciplinary team of specialists, to include Haematologist, Anaesthetist and Obstetrician, all of whom have an interest in bleeding disorders in pregnancy. Decisions should then be individualised, based on the presence/absence of a bleeding history and the third trimester FXI levels. Delivery does not have to be by Elective Caesarean. With appropriate care both operative vaginal delivery and regional anaesthesia can be facilitated.

A rare case of primary ovarian carcinoid.

We present a rare case in an elderly Caucasian lady of primary ovarian carcinoid tumor, causing symptoms of the carcinoid syndrome and severe carcinoid heart disease. Carcinoid tumors are the commonest neuroendocrine tumors, the majority of which are found in the gastrointestinal tract and lungs. Ovarian carcinoids represent only 0.1% of all ovarian neoplasms and 1% of all carcinoid tumors. Carcinoid syndrome can present with flushing, diffuse watery diarrhea, wheezing and breathlessness and in advanced cases lead to carcinoid heart disease. This involves damage to the right heart structures and pulmonary hypertension, which can cause significant breathlessness and peripheral edema, and is associated with a worse prognosis. Diagnosis involves multi-modality radiological imaging and biochemical analysis of neuroendocrine tumor markers, and the first line treatment should always be resection of the tumor where possible. Prognosis is generally favorable, except in some cases with metastasis.

Recurrence of phaeochromocytoma in pregnancy in a patient with multiple endocrine neoplasia 2A: a case report and review of literature.

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant inherited condition with a prevalence of one in 40 000 individuals. It causes the development of tumours in endocrine glands, such as medullary thyroid cancer, pheochromocytomas, as well as primary hyperparathyroidism. MEN 2A in pregnancy is very rare with only 29 cases reported in the literature. The presence of pheochromocytoma is a rare cause of hypertension during pregnancy with an incidence of 0.007% of all pregnancies. This has severe implications on both mother and the foetus. This case report describes a 22-year-old nulliparous Caucasian woman with known MEN2A syndrome, who underwent thyroidectomy for medullary thyroid carcinoma in childhood and excision of left sided pheochromocytoma at the age of 19. She was found to have a recurrence of pheochromocytoma in the right adrenal gland during pregnancy at 16 weeks of gestation and was oddly normotensive. Catecholamine effects were blocked with phenoxybenzamine and she delivered by an uneventful elective caesarean section at 36 weeks gestation. She underwent a laparoscopic right adrenalectomy six weeks postpartum, followed by lifelong corticosteroid replacement.

Benign thyroid disease in pregnancy: A state of the art review.

Thyroid dysfunction is the commonest endocrine disorder in pregnancy apart from diabetes. Thyroid hormones are essential for fetal brain development in the embryonic phase. Maternal thyroid dysfunction during pregnancy may have significant adverse maternal and fetal outcomes such as preterm delivery, preeclampsia, miscarriage and low birth weight. In this review we discuss the effect of thyroid disease on pregnancy and the current evidence on the management of different thyroid conditions in pregnancy and postpartum to improve fetal and neonatal outcomes, with special reference to existing guidelines on the topic which we dissect, critique and compare with each other. Overt hypothyroidism and hyperthyroidism should be treated appropriately in pregnancy, aiming to maintain euthyroidism. Subclinical hypothyroidism is often pragmatically treated with levothyroxine, although it has not been definitively proven whether this alters maternal or fetal outcomes. Subclinical hyperthyroidism does not usually require treatment and the possibility of non-thyroidal illness or gestational thyrotoxicosis should be considered. Autoimmune thyroid diseases tend to improve during pregnancy but commonly flare-up or emerge in the post-partum period. Accordingly, thyroid auto-antibodies tend to decrease with pregnancy progression. Postpartum thyroiditis should be managed based on the clinical symptoms rather than abnormal biochemical results.