The patient was admitted to the Neurosciences Intensive Care Unit with a presumptive diagnosis of pituitary apoplexy. Pituitary hormone measurements and other indicators of endocrine function were normal for the immediate postpartum period. The initiation of successful breastfeeding, the absence of excessive thirst/urination, and a normal serum sodium concentration, serum, and urine osmolality were consistent with normal anterior and posterior pituitary gland function. Based on presentation and radiological findings, a presumptive diagnosis of LAH was made, and therapy was initiated with prednisone 55 mg daily (1 mg/kg postpartum weight). This led to complete resolution of her HA within 2 days. Formal visual field testing demonstrated no deficits. The patient was discharged home on day 3 of her readmission for HA on a prolonged taper of steroids. Interval follow-up indicates that her HA remains in remission and her hormonal indices remain normal except for an elevated prolactin serum concentration consistent with continued breastfeeding; there has been interval involution of her pituitary gland by 33% (although this is still larger than the normal size of <0.9 cm in the craniocaudal direction; Table; Figure).
LAH is a rare clinical entity with incidence estimated at 1 case per 9 million.2 It is more common in females (5–8:1) who are more likely to present at an earlier age (mean age at diagnosis is 34.5 years for females and 44.7 years for males) and has a temporal association with pregnancy with 50% to 75% of cases occurring during the peripartum period.2–4 Presentations of LAH include bifrontal, retro-orbital, or temporal HA (53%–70%), visual disturbances (40%–50%),2–4 and they can be associated with nausea/vomiting in up to 25% of cases.4 Partial or total hypopituitarism is common, with adrenocorticotropic hormone being the hormone most likely decreased (60%–65%).2,4 Interestingly, prolactin may either be decreased (34%) or increased (14%–38%).3,4 Hyperprolactinemia is thought to result from interference with the release, transport, or action of hypothalamic dopamine, the prolactin release-inhibiting factor.2,4 Diabetes insipidus (9%–20%) can occur with neurohypophyseal involvement.3,4 MRI, the imaging modality of choice, demonstrates a symmetrically enlarged pituitary gland with an intact sellar floor without contrast, whereas with contrast there is strong homogeneous enhancement of the gland.2–4 Definitive diagnosis requires a biopsy demonstrating infiltration and destruction of pituitary acini by infiltrates of plasma cells and lymphocytes.3 However, presumptive diagnosis can be made by history, imaging, and hormonal findings, and a therapeutic response to corticosteroid therapy coupled with a reduction in pituitary gland size.
Postpartum HA is a common clinical diagnosis ranging in cause from benign entities such as tension HA to more insidious disorders such as intracranial tumors. PDPH is a well-known iatrogenic complication of neuraxial anesthesia. Our patient clearly had many of the attributes associated with the diagnosis of PDPH including a history of dural puncture with a large bore needle, a strongly positional HA, throbbing pain, and photophobia. However, some features of her HA were less characteristic of PDPH including its early onset (despite no large loss of CSF) and relatively prolonged duration (about 9 days between initial dural puncture and readmission for HA). While it is common practice to repeat an epidural blood patch within 48 hours in symptomatic patients and only obtaining neurological consultation after failure of a second blood patch,5 this patient’s presentation was unusual enough to warrant further workup.
Initial evaluation suggested a diagnosis of Sheehan syndrome (postpartum hypopituitarism), based primarily on the interpretation of the head CT without contrast (Table) along with symptoms of an apparently new retro-orbital HA. The absence of severe hemorrhage or hypotension in the peripartum period, the lack of apoplexia or other neurologic symptoms, the initiation and maintenance of successful breastfeeding, and continued laboratory evidence of normal postpartum endocrine parameters argued against this diagnosis.6 The CT and MRI findings ultimately suggested that the apparent pituitary hemorrhage was more consistent with the presence of an incidental Rathke’s cleft cyst (RCC), a benign, epithelium-lined, intrasellar cyst originating from remnants of Rathke’s pouch. However, this lesion does not explain the patient’s presentation because the RCC was small, no association with RCC and pregnancy exists, and treatment of a symptomatic RCC is surgical rather than medical.7 The early positional HA after a known dural rent with an appropriate response to epidural blood patch, followed later by the presence of a secondary retro-orbital HA in the peripartum period, with radiological imaging showing an enlarged pituitary gland along with the rapid response of this second HA to corticosteroids, was felt to be most consistent with a diagnosis of PDPH overlaying a more indolent LAH. Her initial postpartum 18-fold increase in prolactin levels followed by a prolonged 2-fold increase in this hormone might be incorrectly thought to represent the hyperprolactinemia seen with LAH, but instead represents levels which are consistent with those seen postpartum followed by an extended period of breastfeeding.8 Unlike many cases of LAH, her other pituitary hormone levels were initially normal and remained so during prolonged follow-up.
The clinical conundrum discussed above illustrates the need to approach the differential diagnosis of HA in the postpartum patient with a known dural puncture in a cautious manner. Both PDPH and LAH more commonly occur in young parturients, and both share some common symptomatology. This case indicates that Occam’s razor is not always an adequate guide to diagnosing a clinical problem and that Hickam’s dictum (“patients can have as many diseases as they damn well please”) may provide a more appropriate diagnostic model.9
Name: James A. Dolak, MD, PhD.
Contribution: This author was the attending anesthesiologist for the case and helped write the manuscript.
Name: Constantinos G. Hadjipanayis, MD, PhD.
Contribution: This author the attending neurosurgeon and helped write the manuscript.
Name: Linda J. Demma, MD, PhD.
Contribution: This author helped write the manuscript and in stylistic input of the manuscript.
This manuscript was handled by: Hans-Joachim Priebe, MD, FRCA, FCAI.
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