Pheochromocytoma during pregnancy: laparoscopic and conventional surgical treatment of two cases.

Pheochromocytoma is a rare entity. When it presents during pregnancy, rapid recognition and diagnosis are crucial in order to avoid the maternal and fetal morbidity and mortality associated with severe, uncontrolled hypertension. Difficulties arise from the differential diagnosis of pre-eclampsia. We report two patients operated on for pheochromocytoma during pregnancy. The first patient developed new-onset hypertension in early pregnancy. Prior to presentation, there had been non-specific signs of endocrine disorder, namely a mild diabetes, but no hypertension requiring medication. The second patient sought medical care for irregular heartbeat, shortness of breath, and fatigue during the 6th week of pregnancy. She was found to be hypertensive; diagnostic evaluation revealed substantially increased catecholamine levels. The diagnosis was made by elevated urinary catecholamines and by MRI scan as well as ultrasonography, both of which demonstrated an adrenal mass. Both patients underwent surgical extirpation of the affected adrenal gland. The procedure was performed laparoscopically in the case of the second patient. Pre-operatively, both patients were pre-treated with alpha-blockade followed by secondary beta-blockade. Patient two also received methyldopa for control of hypertension. Both patients were readily weaned from antihypertensive medications post-operatively, with complete resolution of hypertension within a few hours. Each pregnancy progressed normally to term.     

Pheochromocytoma and pregnancy: a deceptive connection

A pheochromocytoma in a pregnant patient is one of the most threatening medical conditions for mother, fetus, and physician. Although extraordinarily rare with a frequency of 0.002% of all pregnancies, this tumor is notorious for its devastating consequences. As in non-pregnant patients, the signs and symptoms are quite variable but not specific, with hypertension being one of the most prominent signs. Confusion with the much more prevalent forms of pregnancy-related hypertension is the main cause of overlooking the diagnosis. If undiagnosed, maternal and fetal mortality is around 50%. Conversely, early detection and proper treatment during pregnancy decrease the maternal and fetal mortality to <5 and 15% respectively. For the biochemical diagnosis, plasma or urinary metanephrines are the tests of first choice since they have a nearly maximal negative predictive value. For reliable localization, only magnetic resonance imaging is suitable, with a sensitivity of more than 90%. When the tumor is diagnosed in the first 24 weeks of gestation, it should be removed by laparoscopic adrenalectomy after 10-14 days of medical preparation with the same drugs as in non-pregnant patients. If the tumor is diagnosed in the third trimester, the patient should be managed until the fetus is viable using the same drug regimen as for regular surgical preparation. Cesarean section with tumor removal in the same session or at a later stage is then preferred since vaginal delivery is possibly associated with higher mortality. Despite all technical diagnostic and therapeutic progress over the last decades, the key factor for further reduction of maternal and fetal mortality is early awareness and recognition of the potential presence of a pheochromocytoma in a pregnant patient with hypertension.     

Pneumothorax in pregnancy. Apropos of 3 cases

We report 3 cases of pneumothorax developed during pregnancy and we compare them with the 16 cases previously published. We insist on the late occurrence of pneumothorax during pregnancy and on the possibility of vaginal delivery. Data from literature and our own experience incline us to believe that pneumothorax is not rare during pregnancy but is often misdiagnosed or unreported.     

Plasma Atrial Natriuretic Peptide (ANP) Concentration in Patients with Pheochromocytoma

The interaction between catecholamines (CA) and ANP is not clearly established. The effects of excess endogenous CA on ANP secretion can be investigated in patients with pheochromocytoma. We studied 27 patients with surgically and histologically proven pheochromocytoma (P) aged 19-70 years. In 16 of these patients plasma ANP study was repeated after surgical removal of the tumour. The control group (C) consisted of 20 healthy volunteers aged 21-48 years. Moreover, 42 patients with uncomplicated mild to moderate essential hypertension (EH) aged 18-48 years were also studied. In P higher plasma ANP concentration versus C, EH was found (51.9 ± 8.1; 25.5 ± 1.5; 19.3 ± 1.5 fmol/ml, respectively). In 16 patients with P, increased plasma ANP level (mean 63.3 ± 12.6 fmol/ml) declined after surgical removal of the tumour (mean 22.4 ± 2.9 fmol/ml). In the P patients no relationship was found between plasma ANP and hormonal patterns of the tumour or between plasma ANP and plasma catecholamines, whereas significant positive correlations between plasma ANP and both systolic and diastolic blood pressure and heart rate were demonstrated. These results suggest that excess CA produced by the chromaffin tumour induce ANP secretion via stimulation of adrenergic receptors. However, influence of the haemodynamic changes evoked by CA cannot be excluded. It is suggested that increased secretion of ANP may be of some importance in maintaining blood pressure homeostasis in patients with pheochromocytoma.     

Pheochromocytoma and prazosin

Prazosin was used to control the signs and symptoms of excessive alpha-adrenergic activity in four patients with pheochromocytomas. Long-term dosage requirements were predicted by the blood pressure response to a single 1-mg oral dose. However, surgical management of the pheochromocytomas was inadequate with prazosin alone, and intravenous phentolamine was required to suppress the pressor surges generated by the tumor during surgical manipulation and excision.     

Pheochromocytoma: 25 years of experience. Report of 199 cases

The methods for diagnosing pheochromocytoma have progressed in 25 years, so changing the clinical, biological and tumoral presentations. The authors compare the features of 199 patients with pheochromocytoma operated between 1975 and 2001 by quartiles. The frequency and known duration of hypertension, plasma adrenaline, the tumour size and proportion of cases which were malignant from the outset, have decreased over the observation period (p < 0.01). The average age and proportion of familial cases or associated with diabetes or those of asymptomatic patients (with incidentaloma), has not changed significantly. The pheochromocytoma were adrenal (104 right, 60 left, 12 bilateral) or ectopic (23) and 13 were malignant from the outset. Over a median 5 year follow-up, 35 pheochromocytomas recurred either in the benign or malignant forms. Recurrences of tumours of the right adrenal were more common than those of the left adrenal gland (p = 0.03). In conclusion, pheochromocytomas are diagnosed earlier, at a stage when the tumours are smaller and less secreting. The higher incidence and recurrence rate of right adrenal pheochromocytoma remain unexplained.     

植入型心律转复除颤器治疗Brugada综合征三例

临床资料　例 1　患者男性 ,2 5岁。于 2 0 0 0年 5月 8日晨 6时全身不适 ,随即意识丧失 ,自行清醒片刻后再次意识丧失 ,家属发现其抽搐及大小便失禁 ,被唤醒后自诉头晕、恶心、乏力。无胸闷、胸痛和心悸。即送当地医院 ,心电图报告为心房颤动 (房颤 )、交界性逸搏和ＳＴ Ｔ改变。给予一般治疗 ,无发作 ,7ｄ后出院。 5月 2 3日又因胸闷 4ｄ入本院。入院体检无异常 ,心电图显示Ｖ1 ～Ｖ3 导联ＳＴ段弓背向下抬高 ,呈类右束支阻滞图形 ,而左胸前导联无Ｓ波 ,Ｖ3 ～Ｖ5导联有“Ｊ”波 ,ＱＴ间期正常 (图 1)。心室晚电位阳性 ,超声心动图、Ｘ线…