**5. Pheochromocytoma**

Pheochromocytoma is a rare cause of hypertension during pregnancy, with clinical features similar to those in the general population (Keely, 1998). The prevalence is estimated at 1 in

Adrenal Disease and Pregnancy 57

the pheochromocytoma is not recognized until the second half of gestation, increasing

Successful laparoscopic excision of a pheochromocytoma in the second trimester of

Other options include combined cesarean delivery and tumour resection or delivery followed by tumour resection at a later date. Delivery is generally delayed until the fetus reaches sufficient maturity to reduce postpartum morbidity, providing successful medical management exists. Although successful vaginal delivery has been reported, it has been associated with higher rates of maternal mortality than caesarean section (Schenker &

It occurs in a family of monogenic inherited enzymatic defects of adrenal steroid biosynthesis, with manifestations secondary to an accumulation of precursors proximal to the enzymatic deficiency. The most common form of CAH is 21-hydroxylase deficiency,

Classic, severe 21-hydroxylase deficiency is associated with ambiguous genitalia, and inadequate vaginal introitis, and progressive postnatal virilization including precocious adrenarche, advanced somatic development, central precocious puberty, menstrual irregularity , a reduced fertility rate, and possible salt wasting (White & Speiser, 2000). The spontaneous abortion rate is twice that in the normal population, and congenital anomalies are more frecuent. Conception requires adecuate glucocorticoid therapy, which then continues at stables rates during gestation, except at labor and delivery. Nonclassical 21-hydroxilase deficiency patients present with pubertal and postpubertal hirsutism and menstrual irregularity, and may have improved fertility with glucocorticoid therapy ( Krone

ACTH stimulation testing to measure 17-OH progesterone demonstrates overlap between heterozygotes for CAH and the normal population. Ideally CYP21 genotyping should be performed. Virilization is not seen in the female fetus with non classical 21-hydroxylase deficiency, but occurs in a fetus with classic 21-hydroxylae unless fetal adrenal androgen

Dexamethasone most readily crosses the placenta as it is not bound to CBG and is not metabolize at dose of 20 mcg/kg maternal body weight per day to a maximum of 1.5 mg daily in 3 divided doses beginning at recognition of pregnancy before the 9th week of gestation, though lower doses are recommended by some (Coleman MA, Honour, 2004). Maternal plasma and/or urinary estriol levels reflect fetal adrenal synthesis and are monitored to assess efficacy. Maternal cortisol a DHEA-S levels will represent maternal

As only 25% of female fetuses are affected in a family with CAH, it is important to discontinue therapy as soon as possible in the male fetus and unaffected female fetus. Chorionic villus sampling at 9-11 weeks gestation may be used for gender determination

Side effects of dexamethasone therapy are potentially significant, including excessive weight gain, sever striae with scarring, edema, irritability, gestational diabetes mellitus, hypertension, and gastrointestinal intolerance. In affected pregnancies, dexamethasone may

uterine size makes surgical exploration difficult (Sarathi et al,. 2010).

seen in more than 90% of the CAH cases in pregnancy (Forest, 2004).

pregnancy has been described (Finkensted et al, 1999).

**6. Congenital Adrenal Hyperplasia (CAH)** 

Granat, 1982).

et al., 2001).

adrenal suppression.

productions is adequately suppressed.

and direct ADN analysis for the 21-hydroxilase gene CYP21.

54000 pregnancies (Botchan et al, 1995). As the uterus enlarges and an actively moving fetus compresses the neoplasm, maternal complications such as severe hypertension, hemorraghe into the neoplasm, hemodynamic collapse, myocardial infarction, cardiac arrhythmias, congestive heart failure, and cerebral hemorraghe may occur. Extra-adrenal tumours which occur in 10%, such as in the organ of Zuckerkandl at the aortic bifurcation, are particularly prone to hypertensive episodes with changes in position, uterine contractions, fetal movement, and Valsalva maneuvers. Unrecognized pheochromocytoma is associated with a maternal mortality rate of 50% at induction of anesthesia or during labor (Lau et al., 1996). There is a minimal placental transfer of catecholamines likely due to high placental concentrations of catechol-O-methyltransferase and monoamine oxidase. Adverse fetal effects such hypoxia are a result of catecholamine-induced uteroplacental vasoconstriction and placental insufficiency, and of maternal hypertension, hypotension, or vascular collapse (Saarikoski, 1974).
