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|Year : 2011 | Volume
| Issue : 6 | Page : 891-894
Unique case of postpartum cerebral angiopathy requiring surgical intervention: Case report and review of literature
Jai Deep Thakur, Prashant Chittiboina, Imad Saeed Khan, Anil Nanda
Department of Neurosurgery, Louisiana State University Health Science Center, Shreveport, Louisiana, USA
|Date of Submission||12-Sep-2011|
|Date of Decision||16-Sep-2011|
|Date of Acceptance||28-Sep-2011|
|Date of Web Publication||2-Jan-2012|
Department of Neurosurgery, Louisiana State University Health Sciences Center - Shreveport, 1501 Kings Highway, Shreveport, Louisiana 71103
Postpartum cerebral angiopathy (PPCA) is a rare form of reversible cerebral vasoconstriction syndrome. A 39-year-old female presented with a 5×6 cm left frontal intracerebral hemorrhage (ICH) and following an emergent neurosurgical evacuation of ICH, she developed a contralateral ICH and intraventricular hemorrhage. Her postoperative course was highlighted by the finding of diffuse bilateral segmental vasospasm on angiography and trans-cranial Doppler, left anterior cerebral artery region infarct and intractable elevated intracranial pressure (ICP). Medical management including osmotic therapy, nimodipine, corticosteroids, and phenytoin was unable to control ICP. Intractable elevated ICP led to evacuation of the right-sided ICH. After 3.5 years, the patient appears to be cognitively intact but continues to have right lower extremity weakness and is wheelchair-bound. Our case suggests that PPCA can rarely manifest as a life-threatening emergency for which neurosurgical intervention may be life-saving.
Keywords: Call Fleming syndrome, intracerebral hemorrhage, isolated cerebral vasculopathy, postpartum cerebral angiopathy, reversible cerebral vasoconstriction syndrome
|How to cite this article:|
Thakur JD, Chittiboina P, Khan IS, Nanda A. Unique case of postpartum cerebral angiopathy requiring surgical intervention: Case report and review of literature. Neurol India 2011;59:891-4
|How to cite this URL:|
Thakur JD, Chittiboina P, Khan IS, Nanda A. Unique case of postpartum cerebral angiopathy requiring surgical intervention: Case report and review of literature. Neurol India [serial online] 2011 [cited 2013 May 19];59:891-4. Available from: http://www.neurologyindia.com/text.asp?2011/59/6/891/91372
| » Introduction|| |
Postpartum cerebral angiopathy (PPCA) is a rare clinical entity, ill-categorized in literature as Call Fleming syndrome, isolated cerebral vasculopathy, benign variant of isolated central nervous system (CNS) vasculitis; all of which are generally clubbed under the spectrum of reversible cerebral vasoconstriction syndrome (RVCS). ,, To our knowledge, only one previous case of neurosurgical evacuation of intracerebral hemorrhage (ICH) in PPCA has been reported.  We report a case of PPCA with an aggressive clinical course leading to ICH and ischemic stroke necessitating multiple neurosurgical interventions in a short span of time.
| » Case Report|| |
A previously healthy 39-year-old female (Gravid 4, Para 3, Abortus 1) underwent an elective normal vaginal delivery. She had an uncomplicated obstetrical history except for gestational diabetes mellitus which was controlled with lifestyle modification. On postpartum Day (PPD) 13, she experienced a thunderclap headache followed by drowsiness. Computed tomography (CT) head scan done at another facility revealed a left frontal lobe 3 × 3.5 cm ICH. On admission at our hospital, she was confused but neurologically intact with Glasgow Coma Scale (GCS) score of 11 (E3V2M6), blood pressure of 162/91, and bilaterally reactive 3 mm pupils. Soon after the initial assessment, the patient became unresponsive with a precipitous drop in oxygen saturation below 60%, bilateral pupillary dilatation and GCS score 3. Repeat CT head revealed a massive progression of the ICH to 5 × 6.4 cm with a 7 mm midline shift [Figure 1]a. The patient underwent an emergent craniotomy for evacuation of the hematoma on PPD-13. A postoperative CT head revealed a complete evacuation of left-sided hematoma and a new 2.5 cm × 3.8 cm contralateral frontal lobe ICH [Figure 1]b. Four-vessel cerebral angiogram on PPD-16 revealed abnormalities in the anterior cerebral artery territory with bilateral diffuse segments of stenosis and dilatations [Figure 2]. A CT head also revealed a recent infarct in the left anterior cerebral artery (ACA) territory [Figure 1]c. Oral nimodipine (60 mg PO every 4 h) and intravenous dexamethasone therapy (4 mg IV every 6 h) was initiated. Laboratory investigations forAnti-neutrophil cytoplasmic antibodies, Lupus anticoagulant, Serum immunoglobulins, Rapid plasma reagin, Cerebrospinal fluid VDRL, Anti-histone antibodies and viral screen were negative, and mildly increased ESR and C-reactive protein was noted. Intractable intracranial hypertension led to a right-sided craniotomy for evacuation of the clot on PPD-18. After extensive discussion with the family, noninvasive modality (trans-cranial Doppler) was chosen to monitor the vasculopathy. Eventually the patient recovered clinically not needing an additional neuro-interventional procedure. At discharge patient had weakness in right lower extremity. On the last follow-up, 3.5 years after the surgery, she was grossly cognitively intact and able to converse well. She had full strength bilaterally in her upper extremities and was wheelchair-dependent with residual right lower extremity weakness.
|Figure 1: (a) Preoperative CT scan showing massive ICH with a clear component of acute bleed and a significant midline shift. (b) Postoperative CT scan done few hours after the surgery shows the development of contralateral ICH. (c) CT scan done on PPD 3 revealing left ACA territory infarct primarily corresponding to the vasculopathy revealed in the cerebral angiography|
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|Figure 2: Four-vessel cerebral angiography done on PPD 16 revealing a pattern of focal stenosis in dilatation represented by white arrows|
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| » Discussion|| |
The etiology of hemorrhagic stroke in postpartum women includes PPCA in addition to those listed for general population.  PPCA is a diagnosis of exclusion which is commonly characterized by acute onset of neurological symptoms within four to six weeks of delivery and cerebral vasoconstriction diagnosed by cerebral angiography. , The differential diagnoses considered prior to diagnosing PPCA included postpartum preeclampsia/eclampsia, vasculitic syndromes and common etiologies of ICH and subarachnoid hemorrhage (SAH) (hypertensive, traumatic and vascular pathologies).
In most cases of PPCA, the onset of headache precedes neurological deterioration by a few days. ,,, PPCA is often a self-limiting disease with complete or near complete recovery of neurological deficits. Our case report highlights a unique aggressive clinical course requiring surgery, with a good long-term functional recovery. PPCA can present with a transient ischemic attack,  ischemic stroke,  ICH,  SAH , or a combination of the above.  Only 11 cases of PPCAwith ICH have been documented in the literature. 
The literature on PPCA is scant; consequently, no major associations with other phenotypic or genetic abnormalities have been identified. Nevertheless, patients present with hypertension irrespective of space-occupying lesions in the brain.  Additionally, use of sympathomimetic or vasoactive medication (VAM) during the peri-partum period has been associated with PPCA even though there was no history of VAM in our patient.  Advanced maternal age has also been associated with worse outcomes including disabling permanent neurological deficits and deaths. ,, Many authors hypothesize that the postpartum hormonal milieu induces persistent vasospasm leading to remodeling of arterial walls through alteration of vascular connective tissue balance. , The common denominator in all the cases is vasoconstriction, reflected clinically by the onset of headache. Alternatively, an unknown genetic predisposition might lead to hypersensitivity reactions to the post-pregnancy physiology or VAM-related environment, inducing cerebral vasoconstriction through the alfa-adrenergic receptors distributed on the cerebral vasculature.
Since vasospasm, brain edema and vasculitis-like features are the highlights of PPCA and most cases are self-limiting, nimodipine, osmotherapy, high-dose corticosteroids and seizure prophylaxis are popular treatment strategies although recent studies have found that neither calcium channel blockers nor glucocorticoids are able to prevent significant clinical deterioration.  Prior to this report, only one case requiring surgical evacuation has been reported in the literature [Table 1]. Vasospasm has also been managed through hypervolemic/hemodilution therapy and intracranial balloon angioplasty. , Geocadin et al.,  reported a case in which they managed a parieto-occipital hematoma with elevated ICP, mass effect, transtentorial herniation, with barbiturate coma and the patient recovered without any neurological deficits. Since our patient had a massive ICH, mass effect, and recent deterioration of GCS, we opted for a surgical option although given the self-limiting nature of PPCA in majority of the cases, the threshold for surgery should be high.
|Table 1: Postpartum cerebral angiopathy cases requiring surgical evacuation|
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In conclusion PPCA is a rare clinical entity and may manifest aggressively. Our patient developed bilateral ICH, ischemic stroke and required surgical clot evacuation on two separate occasions. Although ultimately non-fatal, this disease left our patient with a permanent deficit. A clear elucidation of pathophysiological mechanisms and management protocols is needed to improve the patient outcomes.
| » References|| |
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[Figure 1], [Figure 2]