Adrenal Cushing Syndrome Diagnosed During Pregnancy: Successful Medical Management With Metyrapone (original) (raw)
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Sensor …, 2010
A 32-year-old female, gravid two, para one, with Cushing's syndrome (CS) was admitted to our hospital at 25 week of gestation with severe hypercortisolism. Basal urinary free cortisol (UFC) was elevated about 10 times above the upper limit of normal in two separate times and plasma cortisol failed to suppress after an overnight 1mg dexamethasone suppression test but Adrenocorticotropic hormone (ACTH) level was suppressed. An abdominal non-contrast magnetic resonance imaging (MRI) disclosed a 3-cm right adrenal mass . Due to her critical general condition, the adrenalectomy was not performed. At 30 week of gestation, by the diagnosis of severe preeclampsia she underwent an emergent cesarean section. Two weeks later, right adrenalectomy was performed via laparotomy. Pathologic examination of the gland showed a benign adrenocortical adenoma. The newborn was a healthy male who weighed 1850 gram. There was no clinical or biochemical suppression of adrenocortical function in child and they were discharged after 40 days.[GMJ. 2012;1(1):38-41]
Adrenocorticotropic Hormone-Independent Cushing Syndrome Manifesting During Pregnancy
Endocrine Practice, 2010
A 48-year-old woman was incidentally found to have bilateral adrenal masses, 2.8 cm in diameter on the right, and 2.3 cm and 1.7 cm in diameter on the left, by abdominal computed tomography. The patient had a medical history of hypertension, which was not being controlled by carvedilol, at a dose of 25 mg daily. She presented with signs and symptoms that suggested Cushing Syndrome. We diagnosed adrenocorticotropic hormone (ACTH)-independent Cushing Syndrome based on the results of basal and dynamic hormone tests. Adrenal vein sampling (AVS) was performed to localize a functioning adrenal cortical mass. AVS results were consistent with hypersecretion of cortisol from both adrenal glands, with a cortisol lateralization ratio of 1.1. Upon bilateral laparoscopic adrenalectomy, bilateral ACTH-independent adrenal adenomas were found. The patient's signs and symptoms of Cushing Syndrome improved after surgery just as the blood pressure was normalized. After surgery, the patient was started on glucocorticoid and mineralocorticoid replacement therapy.
A case of Cushing's syndrome in pregnancy
Endokrynologia Polska, 2011
Cushing's syndrome (also known as hypercortisolemia) is rare in pregnant women due to the menstrual disturbances and infertility in women with hypercortisolism. A diagnosis of pathological hypercortisolism in pregnant women is often difficult as some symptoms of the disease may be associated with a complicated pregnancy. Hypercortisolemia leads to serious complications for mother and foetus, and is associated with premature labour and high foetal mortality. Hormonal and radiological diagnostics in pregnancy are limited. The results of hormonal measurements and dynamic tests are difficult to interpret due to the physiological changes in the hypothalamo-pituitaryadrenal axis connected with pregnancy. The optimal time and method of treatment should be chosen cautiously case by case because of the possibility of maternal and foetal complications. In this paper, we present a case of Cushing's syndrome secondary to adrenal adenoma in which the diagnosis was made in the 22 nd week of pregnancy. Due to the advanced gestational status and mild symptoms of hypercortisolism, only symptomatic treatment was introduced. The patient was under continuous obstetric and endocrinological care. At 35 weeks of gestation, the pregnancy was terminated by emergency caesarean section because of premature detachment of the placenta. A male infant weighing 2,450 g was delivered; neither adrenal insufficiency in the child nor hypercortisolemia complications in the mother were observed.
Cushing's syndrome during pregnancy: personal experience and review of the literature
Journal of Clinical …, 2005
Cushing's syndrome (CS) occurs rarely during pregnancy. We investigated and treated four patients with pituitary-dependent Cushing's syndrome during pregnancy over a 15-yr period at the National Institutes of Health. Except for preservation of menses before conception, our patients presented with typical clinical features, increased urinary free cortisol, and loss of diurnal variation of cortisol. The diagnosis was facilitated, without complications, by the use of CRH testing and inferior petrosal sinus sampling in three women. Transsphenoidal pituitary surgery achieved remission in three women, but there were two fetal/neonatal deaths. This experience and review of 136 previous reports suggest that: 1) urinary free cortisol in CS patients overlaps the normal pregnant range; 2) ACTH levels are not suppressed in adrenal causes of CS, which may be identified by the 8-mg dexamethasone test; 3) inferior petrosal sinus sampling and transsphenoidal pituitary surgery, the optimal diagnostic test and treatment for nonpregnant patients with pituitary-dependent Cushing's syndrome, can safely facilitate the management of pregnant patients; and 4) surgery may achieve remission during pregnancy, but the prognosis for the fetus remains guarded. It is likely that earlier recognition and treatment would improve outcome. There is a need for development of criteria for interpretation of diagnostic tests and increased consideration of CS in pregnancy. (
MANAGEMENT OF ENDOCRINE DISEASE: Management of the pregnant patient with Cushings
European journal of endocrinology / European Federation of Endocrine Societies, 2015
The progress in diagnosis and treatment of endocrine diseases turned pregnancy into reality for women with such medical disorders, including Cushing´s syndrome (CS). Nevertheless, despite its rarity, pregnancy in patients with CS can be troublesome due to maternal-fetal complications. Therefore hipercortisolism, if present, should be surgically or medically controlled in most cases. Moreover, changes in the hypothalamic-pituitary-adrenal axis during normal pregnancy may mislead the diagnosis of CS during this period, as many laboratory assessments suggestive of CS may be present in normal pregnancy, in a setting of clinical features mimicking those seen in patients with CS. The aim of this review is to update the diagnostic approach to this medical condition, mainly for pregnant women without previous diagnosis of CS, and to describe the therapeutic strategies for CS during pregnancy in order to minimize complications for both mother and fetus.
A Pregnant Woman Who Underwent Laparoscopic Adrenalectomy due to Cushing’s Syndrome
Case Reports in Endocrinology, 2014
Cushing's syndrome (CS) may lead to severe maternal and fetal morbidities and even mortalities in pregnancy. However, pregnancy complicates the diagnosis and treatment of CS. This study describes a 26-year-old pregnant woman admitted with hypertensioninduced headache. Hormonal analyses performed due to her cushingoid phenotype revealed a diagnosis of adrenocorticotropic hormone-(ACTH-) independent CS. MRI showed a 3.5 cm adenoma in her right adrenal gland. After preoperative metyrapone therapy, she underwent a successful unilateral laparoscopic adrenalectomy at 14-week gestation. Although she had a temporary postoperative adrenal insufficiency, hormonal analyses showed that she has been in remission since delivery. Findings in this patient, as well as those in previous patients, indicate that pregnancy is not an absolute contraindication for laparoscopic adrenalectomy. Rather, such surgery should be considered a safe and efficient treatment method for pregnant women with cortisol-secreting adrenal adenomas.
The hypothalamic-pituitary-adrenal axis in pregnancy: challenges in disease detection and treatment
Endocrine reviews, 2005
Pregnancy dramatically affects the hypothalamic-pituitary-adrenal (HPA) axis leading to increased circulating cortisol and corticotropin (ACTH) levels during gestation, reaching values in the range seen in Cushing's syndrome (CS). The cause(s) of increased ACTH may include placental synthesis and release of biologically active corticotropin-releasing hormone (CRH) and ACTH, pituitary desensitization to cortisol feedback, or enhanced pituitary responses to corticotropin-releasing factors. In this context, challenges in diagnosis and management of disorders of the HPA axis in pregnancy are discussed. CS in pregnancy is uncommon and is associated with fetal morbidity and mortality. The diagnosis may be missed because of overlapping clinical and biochemical features in pregnancy. The proportion of patients with primary adrenal causes of CS is increased in pregnancy. CRH stimulation testing and IPSS can identify patients with Cushing's disease. Surgery is a safe option for treatment in the second trimester; otherwise medical therapy may be used. Women with known adrenal insufficiency (AI) that is appropriately treated can expect to have uneventful pregnancies. While a fetal/placental source of cortisol may mitigate crisis during gestation, unrecognized AI may lead to maternal or fetal demise either during gestation or in the puerperium. Appropriate treatment and management of labor are reviewed. I.
Cushing'ssyndromeinpregnancy: Reportofacaseandreviewoftheliterature
cushing's syndrome (cs) during pregnancy is a rare nosology with only a few cases reported in the literature. Misdiagnosis is common, as the syndrome may be easily confused with preeclampsia or gestational diabetes. cs during pregnancy is usually associated with severe maternal and fetal complications. A high degree of clinical awareness is therefore required to avoid miscarriage or premature delivery. We report an 18-year old female referred to our institution with amenorrhea and truncal obesity. Physical examination revealed cushingoid characteristics, including mild hypertension (130/100 mmHg). she was also found to be 8 weeks pregnant. A provisional diagnosis of cs was made based on plasma cortisol and adrenocorticotropin hormone (ActH) measurements but the patient did not receive any relevant therapy. she eventually gave birth to a healthy full-term infant via vaginal delivery. A right adrenal adenoma was diagnosed post-labor and was subsequently treated with surgical resection. the patient's condition remained stable and 19 months after the adrenalectomy she gave birth to a second healthy full-term infant. Hydrocortisone (30 mg/day) was administered throughout the second gestation. six months post-labor the treatment was discontinued after a normal hypothalamic-pituitary-adrenal (HPA) axis was ascertained.
Cushing’s syndrome in pregnancy: a case report and mini review of the literature
Gynecological Endocrinology, 2014
Adrenal diseases in pregnant women are diagnosed relatively rarely. The main cause of hypercortisolemia during pregnancy is Cushing's syndrome related to adrenal adenoma. It is important to diagnose Cushing's syndrome in pregnant women because it can lead to significant maternal and foetal complications and morbidity. However, due to physiological endocrine changes and symptoms in pregnant women the diagnosis of this disorder can be a challenge. One current case describes a 38-year-old pregnant woman with hypertension, oedema and an adrenal tumour. At the beginning, Conn syndrome was suspected, but after careful analysis Cushing's syndrome (with an adenoma of the right adrenal gland) was diagnosed. After delivery and 5 weeks of pharmacological treatment the patient underwent right side adrenalectomy by laparoscopy.