Pearls & Oy-sters: Postdural Puncture Headache, Cerebral Sinus Venous Thrombosis, and Reversible Cerebral Vasoconstriction Syndrome in the Peripartum Period

Discussion

We report the case of a young woman who, in the week following delivery, presented with recurrent headaches leading to the discovery of 3 different and possibly unrelated neurologic complications: postdural puncture headache, followed by CSVT complicated by a venous infarct and asymptomatic RCVS, all of which can present with headaches.

Headache is a frequent symptom in the postpartum period, with a prevalence ranging from 11% to 80%, mostly due to primary headaches.² However, the onset of a new or unusual headache in the peripartum period should be treated as a red flag for secondary causes of headaches. This period is associated with an increased risk of several conditions presenting with headaches, including cerebrovascular diseases (subarachnoid hemorrhage, ischemic or hemorrhagic stroke, CSVT, cervical artery dissection, and RCVS) and other causes (posterior reversible encephalopathy syndrome, eclampsia and preeclampsia, PDPH, and pituitary apoplexy).³ On the other hand, the high incidence of headaches in the peripartum period justifies radiologic investigations only in patients with unexpected presentations and using a stepwise approach as in our patient.²

Although PDPH is the most frequent etiology of secondary headaches in the postpartum setting, appearing in up to 5% of pregnancies, other possible etiologies should be kept in mind. Roughly 0.004%–0.01% of pregnancies are complicated by CSVT that causes about 2% of pregnancy-associated strokes.⁴ CSVT occurs mainly in the peripartum period,⁵ probably due to acute changes in coagulation factors and hormones and rapid fluctuations in intracranial pressure during delivery.⁶ CSVT requires acute anticoagulation, which should be initiated despite a hemorrhagic venous infarct as seen in our patient, and the outcome is usually favorable (no or minor disability in 94%).⁷

PDPH and CSVT share common clinical features, which may complicate differential diagnosis. CSVT can present only with headaches, with focal neurologic deficits occurring later or not at all.^7,8 CSVT consequent to dural puncture during epidural catheter placement has been reported,⁸ although a causal relationship is still unclear.⁹ CSVT has also been described in up to 1%–2% of patients with spontaneous intracranial hypotension. In this context, venous engorgement, traction on cerebral sinuses from brain sagging, and increased blood viscosity have been proposed as mechanisms favoring CSVT.^10,11 We could hypothesize similar mechanisms promoting CSVT following dural puncture.

EBP has been suggested as a treatment for CSVT in spontaneous intracranial hypotension¹⁰; therefore, we cannot rule out that the first headache on day 1 postpartum was the result of CSVT and that the 2 blood patches temporarily relieved the symptoms of thrombosis.

Further complicating our patient's case, MRI angiography revealed transient arterial anomalies, and TCD examination exposed blood flow acceleration, indicating RCVS. There are only a few reports of cases of PDPH coexisting with RCVS and CSVT^12,13 with no obvious causal links between them. Currently, a connection between CSF leakage and cerebral vasospasm is not clearly established, despite a few published cases suggesting intracranial hypotension precipitating vasospasm.¹⁴ If this hypothetical association exists, we could suppose that the intracranial hypotension following the lumbar puncture may have been the primum movens of both the RCVS and CSVT seen in our patient, although further studies are needed to ascertain a causal relationship.

RCVS was formerly known as postpartum angiopathy (PPA) occurring in the 6 weeks following delivery, and its pathogenesis remains unknown. Postpartum RCVS is usually self-limiting with resolution of symptoms and angiographic abnormalities within 3 days to 3 months. However, in severe cases, it can cause ischemic stroke, brain hemorrhage, and/or reversible parenchymal edema. RCVS typically presents with recurrent bouts of sudden extreme headaches, sometimes accompanied by focal neurologic symptoms. If serious, postpartum RCVS should be treated with nimodipine and perhaps intra-arterial antispastic drugs and balloon angioplasty.¹⁵ In the present case, we considered the RCVS as an incidental finding, as the characteristics of the initial and later headaches seemed better explained by PDPH and CSVT, respectively, and we did not administer any of the treatments because the vasospasms were asymptomatic and relatively mild.

At the initial evaluation in the emergency department, we considered that the patient had an ischemic stroke because of a small cortical diffusion restriction on the MRI (Figure 1) and treated the patient accordingly with IV thrombolysis. However, a proportional hemiparesis as seen in our patient would be expected from a deep territory stroke, such as stroke in the internal capsule or brainstem, and the ischemic lesion in the cortical territory of Figure 1 would rather provoke a focal motor deficit, limited to the cortical representation corresponding to the affected cortical area.

In summary, our patient presented with postpartum headaches initially attributed to and successfully treated as PDPH. The patient then developed hemiparesis followed by a new, contralateral headache from a cortical venous infarct provoked by CSVT, and finally, we found the presence of radiologic (but asymptomatic) RCVS.

Clinicians should therefore be conscious of the possible co-occurrence of multiple etiologies of postpartum headaches and carefully evaluate and correlate all neurologic symptoms using appropriate diagnostic examinations. Early and correct identification of treatable causes of postpartum headaches should help void complications and long-term sequelae.