Mast cell activation syndrome-anesthetic challenges in two different clinical scenarios.

Mast cell activation syndrome (MCAS) includes a group of disorders that result in the inappropriate release of inflammatory mediators from mast cells. These mediators can affect multiple organ systems and lead to significant morbidity, and possible fatality. Although reactions, typically in response to various nonspecific stimuli, are usually mild, they may put those with MCAS at increased risk of anaphylaxis. In this case report, we present two clinical scenarios of MCAS, and identify possible factors triggering mast cell mediator release. We also define a preoperative preventive pathway, outline anesthetic considerations, and discuss the management of immediate hypersensitivity reactions in patients with MCAS. Meticulous preoperative preparation, avoidance of triggers, and development of a plan to treat possible adverse organ responses are paramount of good outcomes.

Intrahepatic Persistent Right Umbilical Vein and Associated Outcomes: A Systematic Review of the Literature.

The aim of this study was to provide a comprehensive review of the current data surrounding an intrahepatic persistent right umbilical vein in the fetus, including associated anomalies and outcomes, and to assist practitioners in counseling and management of affected pregnancies. We performed a MEDLINE, Embase, Cochrane Central Register of Controlled Trials, and Northern Light database search for articles reporting outcomes on prenatally diagnosed cases of a persistent right umbilical vein. Each article was independently reviewed for eligibility by the investigators. Thereafter, the data were extracted and validated independently by 3 investigators. A total of 322 articles were retrieved, and 16 were included in this systematic review. The overall prevalence of an intrahepatic persistent right umbilical vein was found to be 212 per 166,548 (0.13%). Of the 240 cases of an intrahepatic persistent right umbilical vein identified, 183 (76.3%) were isolated. The remaining cases had a coexisting abnormality, including 19 (7.9%) cardiac, 9 (3.8%) central nervous system, 15 (6.3%) genitourinary, 3 (1.3%) genetic, and 17 (7%) placental/cord (predominantly a single umbilical artery). In summary, a persistent right umbilical vein is commonly an isolated finding but may be associated with a coexisting cardiac defect in 8% of cases. Therefore, consideration should be given to fetal echocardiography in cases of a persistent right umbilical vein.

A Case Report and Review of Postural Orthostatic Tachycardia Syndrome in Pregnancy.

Purpose Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance characterized by an increased heart rate upon transition from supine to standing, and head-up tilt without orthostatic hypotension. Its etiology is multifactorial, and no clear cause has been identified. Common symptoms include light-headedness, blurred vision, weakness, cognitive difficulties, and fatigue and are often accompanied by palpitations, shortness of breath, syncope, or gastrointestinal symptoms. Management includes volume expansion, physical counter maneuvers, and pharmacological agents such as fludrocortisone, midodrine, propranolol, and pyridostigmine. The course of POTS in pregnancy is variable and POTS has not been directly implicated in any adverse outcomes for the mother or fetus. Methods Two cases of POTS in pregnancy are presented, along with a review of the literature for reports of POTS in pregnancy. Results Along with our 2 cases, 10 other case reports were identified and included. Conclusion The course of POTS in pregnancy is variable, and not directly linked to increase perinatal morbidity or mortality. Women can safely undergo regional anesthesia, and vaginal delivery with close monitoring of hemodynamic changes.

Isaacs' syndrome in pregnancy.

Isaacs' syndrome is a rare neuromuscular disorder of continuous muscle fibre activity resulting from peripheral nerve hyperexcitability. Symptoms commonly include myokymia (muscle twitching at rest), pseudomyotonia (delayed muscle relaxation), muscle cramps and stiffness. It is caused by voltage-gated potassium channel dysfunction and may be inherited or acquired. Treatment commonly includes anticonvulsants, immunosuppressive therapy and plasma exchange. To date only two cases of Isaacs' syndrome in pregnancy have been reported. We present a case of maternal Isaacs' along with a review of the literature. There are few reports of Isaacs' syndrome in pregnancy, but all are associated with favourable outcomes. Given the autosomal dominant inheritance pattern, genetic counselling of the gravida is recommended. Anticonvulsant may have to be used in pregnancy, and given the potential teratogenicity with several of these agents; preference should be given to newer drugs such as lamotrigine.