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Occam’s Razor Could Not Cut It: Tale of 2 Headaches in a Postpartum Patient: A Case Report

Dolak, James A. MD, PhD; Hadjipanayis, Constantinos G. MD, PhD; Demma, Linda J. MD, PhD

doi: 10.1213/XAA.0000000000000580
Case Reports: Case Report
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Not all postpartum headaches are caused by dural puncture, and it is possible for postpartum patients to have >1 cause for headache. After neuraxial block with an incidental large-gauge dural puncture, our patient developed a severe, classic postdural puncture headache which initially responded to an epidural blood patch. The patient was readmitted 2 days after discharge complaining of recurrent headache less characteristic of a postdural puncture headache, now being bifrontal/retro-orbital and without clear positional component. Computerized tomography and magnetic resonance imaging revealed an enlarged pituitary gland with a possible hemorrhagic focus; all endocrine parameters were normal. The patient was ultimately diagnosed with lymphocytic adenohypophysitis, an autoimmune inflammation of the anterior pituitary gland.

From the Department of Anesthesiology, Emory University School of Medicine, Atlanta, Georgia.

Constantinos G. Hadjipanayis, MD, PhD, is currently at Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York.

Accepted for publication April 25, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Linda J. Demma, MD, PhD, Bassett Healthcare, 1 Atwell Rd, Cooperstown, NY 13326. Address e-mail to ldemma@gmail.com.

The differential diagnosis of postpartum headache (HA) includes both primary HA disorders such as migraine or tension HA and a variety of secondary causes such as intracranial vascular disorders, nonvascular intracranial disorders, homeostatic disorders, or infection.1 Postdural puncture headache (PDPH), with loss of cerebrospinal fluid (CSF) leading to intracranial hypotension, is a common iatrogenic cause of postpartum HA in parturients who have received neuraxial blocks complicated by dural puncture. This type of HA typically presents 24 to 48 hours postpartum, is throbbing in nature, and may be associated with visual or aural symptoms; its strong positional component, worse with sitting/standing and mitigated by lying flat, helps differentiate it from other causes of postpartum HA. In the patient discussed below, an HA consistent with PDPH developed after a known dural puncture. Occam’s razor (or diagnostic parsimony, which states that one should not make more diagnoses than the minimum needed) argues that her symptoms represented a PDPH. However, the patient also had a coexisting HA unrelated to dural puncture, diagnosed clinically and with supporting images as lymphocytic adenohypophysitis (LAH), an autoimmune inflammation of the anterior pituitary gland. We present this case to expand the differential diagnosis of postpartum HAs with a heretofore not previously reported clinical entity in the anesthetic literature. The patient has reviewed this case report and has given written permission for publication.

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CASE DESCRIPTION

A 34-year-old gravida 2 para 0, with a height of 158 cm and a body weight of 66 kg presented in labor at 36 3/7 weeks estimated gestational age with a twin pregnancy. Past medical history consisted of laparoscopy for endometriosis and in vitro fertilization for the current pregnancy. Medications included prenatal vitamins, FeSO4, and docosahexaenoic acid supplements. At 3 cm dilation, a combined spinal-epidural (CSE) was attempted at the L3-L4 interspace using a 17-gauge Weiss needle with loss of resistance to 1 mL air. Immediately upon loss of resistance, CSF flowed into the syringe. This was reinjected into the patient, and the needle was withdrawn. A second CSE attempt was successful at the L2-L3 interspace and after dural puncture with a 27-gauge Whitacre needle; 20 µg fentanyl was injected into the subarachnoid space resulting in rapid pain relief. An epidural catheter was inserted, and a standard test dose proved negative for either intravascular or intrathecal placement. Labor analgesia was maintained with an infusion of 0.2% ropivacaine (9 mL/h). Two females were delivered vaginally in excellent condition 11 hours after placement of the CSE.

The patient complained of HA within 4 hours of CSE placement. She was seen by an anesthesiologist postdelivery. She was afebrile, normotensive, and had a normal neurological examination but had a severe PDPH (occipital pain associated with nausea, photophobia, and improvement when supine). The patient initially elected initial conservative therapy with IV caffeine (600 mg) and oxycodone/acetaminophen (10/650 mg). While she improved temporarily, by the next morning she was again complaining of a severe positional PDPH. She then requested an epidural blood patch, which was performed in the usual manner with 20 mL sterile autologous blood at the L2-L3 level, and resulted in a marked improvement of her HA.

The patient was discharged on postpartum day 3, but 1 day later presented to the emergency department complaining of a persistent bifrontal, retro-orbital HA. Again, vital signs and neurologic examinations were normal. Computerized tomography (CT) showed an enlarged pituitary gland with an internal hyperdensity concerning for hemorrhage. Magnetic resonance imaging (MRI), with and without contrast, showed an enlarged pituitary gland causing chiasmal compression and an abnormal hyperintensity on T1-weighted images and hypointensity on T2 images within the posterior, dependent portion of the pituitary gland (Table; Figure).

Table.

Table.

Figure.

Figure.

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).

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DISCUSSION

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

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DISCLOSURES

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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REFERENCES

1. Wlody DL. Atlee JL. Postpartum headache other than post-dural puncture headache. In: Complications of Anesthesia. 2007:2nd ed. Philadelphia, PA: Saunders Elsevier; 795798.
2. Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR. Autoimmune hypophysitis. Endocr Rev. 2005;26:599614.
3. Foyouzi N. Lymphocytic adenohypophysitis. Obstet Gynecol Surv. 2011;66:109113.
4. Rivera JA. Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy. Pituitary. 2006;9:3545.
5. Baysinger CL, Pope JE, Lockhart EM, Mercaldo ND. The management of accidental dural puncture and postdural puncture headache: a North American survey. J Clin Anesth. 2011;23:349360.
6. Keleştimur F. Sheehan’s syndrome. Pituitary. 2003;6:181188.
7. Larkin S, Karavitak N, Ansorge O. Fliers E, Korbonits M, Romijn JA. Rathke’s cleft cyst. In: Handbook of Clinical Neurology. Vol 2014:124. Amsterdam, the Netherlands: Elsevier BV; 255270.
8. Stefos T, Sotiriadis A, Tsirkas P, Messinis I, Lolis D. Maternal prolactin secretion during labor. The role of dopamine. Acta Obstet Gynecol Scand. 2001;80:3438.
9. Mani N, Slevin N, Hudson A. What three wise men have to say about diagnosis. BMJ. 2011;343:d7769.
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