Endocrine Abstracts

Introduction: Sheehan syndrome (SS) is a hemorrhagic necrosis of the pituitary gland responsible of anterior pituitary insufficiency. It occurs in a context of cerebral hypo flow due to a rapid blood pressure drop and/or acute hemorrhagia. We report a case of SS revealed by acute corticotropic insufficiency.

Case report: A sunday morning 34 years-old woman consulted the emergency department for of a fatigue and diarrhea. She reported a 7 kg weight-loss in the late 2 months associated with a lack of appetit. Her blood pressure 96/56 mmHg. Biologic assessment showed a natremia at the low range and a normal kaliemia. Hypoglycemia at 0.38 g/l was objectified. An acute adrenal insufficiency was suspected. Intravenous hydrocortisone and glucosed fluid infusion immediately after cortisol blood sample. The hormonal assesment confirmed a corticotrope insufficiency with a low level of both plasma cortisol 113 nmol/l (<138) and ACTH <0.2 nmol/l. Bacterial infection by Aeromonas punctata was confirmed. Oral antibiotic treatment using Ciprofloxacine 500 mg twice a day stoped diarrheas. Three months before, the patient reported an hemorrhagic delivery, a lack of lactation and persistance of amenorrhea. Other typical symptomes were also found: lack of pubic and axillairy pilosity and vaginal dryness. In this clinical context the diagnostic of SS was retained. A screening of other pituitary dysfunction showed, a thyreotrope insufficiency (low fT4 and fT3 with low normal TSH level), a low FSH and LH levels and low prolactinemia. IGF1 level was normal. After 48 h glucocorticoid substituition the patient presented a polyuric poludipsic (PUPD) syndrome. The fluid restriction test was rapidly stopped because of hypernatremia at 150 mmol/l. The treatment by desmopressin 90 μg/d was initiated with resolution of the PUPD syndrome and natremia normalisation. Pituitary MRI showed a sequelae-like changes involving the entire pituitary gland but respecting its stem. Post hypophyse was reduced to punctiform hyposignal T1. The diagnosis of central insipidus diabetes was confirmed.

Comments and conclusions: SS is due to a necrosis of the pituitary gland in the context of reduced brain output. Sometimes the diagnosis of this rare syndrome may be delayed notably when the first typical symptômes (lack of lactation, post-partum amenorrhea, lack of pubis hairs in the waxed women) are not spontanously reported by the patient. Acute adrenal gland insufficiency and even diabetes insipidus may occur lately with a severe clinical presentation in the course of common minor illness like bacterial diarrhea.