Endocrine Abstracts

Introduction

Pheochromocytoma(PHEO) occurs in pregnancy with an estimated incidence of 0.007%. Its rarity and overlapping presentation with other pregnancy-related hypertensive disorders make the diagnosis really challenging. If left unrecognized, may result in increased maternal and fetal mortality. There are no guidelines on approaching PHEO during pregnancy. This case highlights the difficulties encountered in managing this condition in pregnancy.

Clinical case

A 33-year-old previously healthy female(G3P2) with clinical features of pulsatile headache and palpitations triggered by physical efforts, without hypertension, starting 4 months before conception, treated with antidepressants. She had a gestational diabetes mellitus history since the 11th week of gestation (WG) which was managed with diet and metformin. Until the 2nd trimester of pregnancy she was normotensive, however, her headaches worsened at 24thWG and related hypertensive peaks were reported (180/100 mmHg) from this time. Methyldopa was initiated at a starting dose of 250 mg, twice daily with a necessary increase to three times daily. Nevertheless, the clinical picture’s worsening prompted further investigation at 36thWG. Laboratory data revealed elevated plasma metanephrine of 201 pg/m(<65) and normetanephrine of 1735 pg/ml (<196); elevated total urinary metanephrine of 10 605 µg/24 h(<785). MRI showed a right adrenal solid mass of 4.6×5.5 cm. She was admitted to the hospital and treated with phenoxybenzamine (increasing doses, 20 mg/day) followed by propranolol(40 mg/day) and nifedipine(10 mg, SOS). Methyldopa was suspended. Maternal dyspnea, chest pain, orthostatic hypotension accompanied by fetal distress emerged. On the sixth day of adrenergic blockade, at 38thWG, she went into spontaneous labour. A multidisciplinary decision (Endocrinology, Internal Medicine, Obstetrics, Anaesthesia, Surgery and Neonatology) was made to perform a caesarean section. Hypertensive peak (220/120 mmHg) was controlled with boluses of IV isosorbide-dinitrate. The newborn had an Apgar score of 10/10 (1`/5`) and was transferred to the intensive care unit (ICU) for surveillance. He was discharged 3 days after birth, without complications. Twenty days after delivery, the patient underwent an uncomplicated laparoscopic adrenalectomy. In the postoperative period she remained in the ICU for 11 days due to difficult to manage hypertensive peaks. Histopathology examination confirmed PHEO.

Conclusions

PHEO should be considered in pregnant patients presenting atypical/resistant hypertension despite treatment. Moreover, methyldopa may cause false high values in urine metanephrine levels, hampering the diagnosis. There are few reports in literature describing the antepartum difficulties in the management of these patients. We emphasize hypotension, decisions concerning timing/method of delivery and the appropriate surgical timing. A multidisciplinary team’s commitment is of utmost importance.