The authors present the case of a postpartum 29-year-old woman with intracranial hypertension (IH) in association with Sheehan syndrome (SS). The clinical manifestations of IH resolved with corticosteroid replacement. The authors conclude that the patient's SS led to hypocortisolism, which, in turn, contributed to IH. They say that corticosteroid replacement is essential in the treatment of this potentially life-threatening condition.
They are unaware of previous reports of SS-related hypocortisolism leading to IH. The patient presented seven weeks postpartum with a two-day history of left periorbital swelling, fever, nausea and vomiting. She had consulted with her primary care doctor a week earlier complaining of fatigue, occipital headache, blurred vision and insufficient lactation.
She was found to be hypotensitve and have hypothyroidism. There was left periorbital edema and ciliary injection. She had visual acuity of 20/40 in the right eye and 20/200 in the left eye, bilateral optic disc edema and left sixth nerve palsy.
She was treated with intravenous benzylpenicillin and flucloxacillin and subcutaneous enoxaparin for presumed periorbital cellulitis and possibly cavernous sinus thrombosis. After 48 hours, she was switched to intravenous ceftriaxone and clindamycin because of inadequate resolution of periorbital edema and fever. Her condition deteriorated over the next five days and she became inattentive, disorientated, and somnolent with intractable nausea, requiring continuous parenteral antiemetics, and developed a right sixth nerve palsy.
She was transferred to a tertiary neurosciences center, where upon arrival she was hypoglycemic, hypotensive and tachycardic but afebrile. Acetazolamide (500 mg twice a day) was begun in addition to intravenous saline. Blood tests revealed an undetectable cortisol level < 30 nmol/L, growth hormone level < 1.0 mg/L, and insulin growth factor 1 level < 25 mg/L, with hypoglycemia and an undetectable prolactin level, establishing the diagnosis of hypopituitarism.
MRI showed a slightly enlarged pituitary gland with a central hypointense area with subtle enhancement and cerebellar tonsils 5 mm below the level of the foramen magnum. The optic nerve sheaths were distended with flattening of the back of the globes. Magnetic resonance venography showed reduced flow signal within the lateral portions of the transverse sinuses.
The patient was begun on intravenous hydrocortisone (100 mg every six hours). Her level of arousal improved. Three days later, both sixth nerve palsies had resolved and acetazolamide was stopped. Visual acuity returned to 20/30, right eye, and 20/40, left eye. The confusional state resolved over the next two weeks, and four months later, there was evidence of bilateral optic disc pallor with retinal pigment epithelial changes in the macula of each eye. Repeat MRI showed reduction in bulk of the pituitary gland and resolution of cerebellar tonsillar descent with less crowding of structures at the craniocervical junction.
The authors say that it is very likely that the patient had SS complicated by IH, with cortisol deficiency being the main etiological factor producing IH given the rapid and dramatic response to steroid replacement therapy. Thyroxine administration, the co-occurrence of infection, and ongoing vomiting-related fluid loss contributed to the precipitation of an adrenocortical crisis.
They write that in retrospect, agalactia and secondary hypothyroidism were early indicators of anterior pituitary failure. Typical T1 MRI features of SS include an enlarged pituitary gland with central hypointensity and peripheral enhancement with or without central heterogenous enhancement.