First Emergency Department Visit
A 41‐year‐old female patient who was 6 days postpartum, gravida 5, para 5, presented to the emergency department (ED) at an outlying hospital with reports of lower chest and abdominal pain for 5 days. The pain was constant and dull, and it worsened while she was breast‐feeding. She had a previous history of migraines and tobacco use but had not smoked for the past year. There were no reports of eclampsia or hypertension during this or previous pregnancies, and she had no complications during this or previous deliveries. An abdominal and pelvic computed tomography (CT) scan, abdominal X rays, and a complete blood count were all within normal limits. She was given oxycodone/acetaminophen (Percocet), which decreased her pain, and she was discharged home with the prescription and instructions to return to the ED if symptoms worsened.
Second Emergency Department Visit and Transfer
Three days later, she returned to the ED via ambulance after experiencing a sudden onset of a severe headache and a possible seizure; she also reported nausea, vomiting, and numbness and tingling of her lower extremities. She was pale, restless, diaphoretic, and yelling from the pain. At the time, it was noted that she had experienced a persistent, migraine‐like headache since her delivery, for which she was taking acetaminophen/isometheptene/dichloralphenazone (Midrin) and ibuprofen with minimal relief. Upon examination, she was confused but able to move all of her extremities. At one point, while the patient was describing her headache as “really bad,” the nurse noted that the patient's eyes rolled back, she began to posture, and then she became apneic. She immediately was given 100% oxygen via a face mask, and her respiratory status quickly returned to baseline. A CT scan of her head and chest revealed a small subarachnoid hemorrhage (SAH) and a pulmonary embolus.
She was then transferred to our hospital for further management. She continued to have seizure‐like activity (described as eye deviation and posturing), as well as altered mental status. Her admitting blood pressure was 213/116 mm Hg with a pulse of 156 beats per minute. She was quickly intubated for airway protection and taken for a head CT scan, which revealed a large subarachnoid and intraventricular hemorrhage, a dilated segment of the right vertebral artery with a possible blister‐like aneurysm, and a large posterior communicating (PCOM) artery. A ventriculostomy was performed and a shunt placed to monitor her intracranial pressure (ICP) and drain excess cerebrospinal fluid (CSF). The protein level in her CSF was elevated, and her ICP initially was 24‐25 mm Hg. At this time the patient opened her eyes to deep stimulation, withdrew her right arm, and extended her left arm to pain. A repeat CT scan the next day (hospital day 2) revealed the same extent of SAH and the presence of a small lacunar infarct in the basal ganglia. The consulting physicians noted that her pulmonary embolus was stable and that, based on the severity of her SAH, anticoagulation was not appropriate.
On hospital day 3, the patient's neurological examination worsened. Her pupils were intermittently unequal and nonreactive, and she no longer opened her eyes to painful stimulation or had spontaneous trace movement of her extremities. A magnetic resonance angiogram scan was ordered; it revealed severe turbulence effects (rather than severe vasospasm) of the internal carotid artery (ICA), anterior cerebral artery, and the middle cerebral artery without evidence of aneurysm. She also had multiple vascular territory and watershed infarcts in both cerebral hemispheres.
Hospitalization and Diagnosis
During her lengthy hospitalization (67 days), this patient underwent various diagnostic procedures. Multiple electroencephalograms were ordered; initially they revealed nonconvulsive status epilepticus and later showed generalized slowing. A Doppler ultrasound examination of the lower extremities revealed bilateral deep‐vein thrombosis; an inferior vena cava filter was placed to prevent further pulmonary emboli. Transcranial Doppler ultrasounds, performed several times, revealed high flow velocities in the major cerebral arteries. An angiogram was performed and revealed mild stenosis of the left ICA at the PCOM junction with poststenotic dilation and moderate stenosis of the right ICA at the PCOM junction with both poststenotic dilation and irregular dilation of the right vertebral artery just distal to the posterior inferior cerebellar artery. The irregular dilation, initially believed to be an aneurysm, was the possible cause of her SAH. Although the angiographic findings were characteristic of vasculitis arising from an inflammatory process such as infection or as a result of medication, they were also consistent with the description of postpartum cerebral angiopathy. Because the rest of the workup was negative for vasculitis, infection, or any congenital deformities, the patient was ultimately diagnosed with postpartum cerebral angiopathy.
Throughout the diagnosis process, treatment consisted of intravenous dexamethasone, cyclophosphamide, and nimodipine with little improvement in her mental status. She required a tracheostomy to maintain her airway and a gastric tube for nutrition. After developing communicating hydrocephalus, she received a ventriculoperitoneal shunt. The patient's mental status slowly improved to the point that she opened her eyes spontaneously, her pupils were equal and reactive, and she had spontaneous movement of her right arm. She would frequently raise her head off the pillow and grimace to painful stimuli, but she did not follow commands or track the nurse around the room. She had frequent high fevers, which were thought to be central in origin, and responded well to the placement of a clonidine transdermal patch.
Near the end of her hospitalization, a follow‐up head CT scan revealed formation of a new aneurysm in the right vertebral artery. The patient underwent coiling of the aneurysm and stent placement without complications. She was discharged to a long‐term care facility, 67 days after admission, with prescriptions for cyclophosphamide and prednisone to continue for 30 more days and esomeprazole (Nexium) for ulcer prophylaxis. She continued to require a tracheostomy and gastric tube. Although she opened her eyes spontaneously, she did not respond to her environment, was unable to communicate her needs, and required total nursing care for activities of daily living. Several months later, at the time of a follow‐up contact, the patient continued to reside in a long‐term facility, but she was able to follow commands and communicate some basic needs.
Postpartum cerebral angiopathy is a rare condition for which no incidence rate is cited in the literature (Konstantinopoulos, Mousa, Khairallah, & Mtanos, 2004). It can cause ischemic or hemorrhagic stroke, or both, and usually occurs within the first week following a normal pregnancy and uncomplicated delivery. Although the pathophysiology is unclear, it is believed to be caused by an inflammatory process, such as vasculitis or transient vasospasm (Ursell et al., 1998). Two points in favor of a vasculitic origin are that the angiographic findings are similar to vasculitis and that symptoms respond to steroid therapy and reoccur after tapering. Furthermore, it has been proposed that prolonged vasoconstriction may result in vascular changes (Kubo, Nakata, Tatsumi, & Yoshimine, 2002).
However, vasospasm is more likely to be the cause of postpartum cerebral angiopathy, because of angiographic and transcranial Doppler findings, lack of abnormalities in blood and CSF, and usually rapid resolution of neurological symptoms. Some authors have suggested the hormonal changes that occur during pregnancy and the postpartum period may result in changes to the intima of the blood vessels, and when combined with acute hypertension, contribute to vasospasm (Ursell et al., 1998). In addition, there are several medications that, if given during the delivery or postpartum period, may contribute to postpartum cerebral angiopathy because they have a vasospastic effect on the cerebral vessels. These medicines include ergot derivatives: ergonovine and sumatriptan, which are usually given for headaches, and bromocriptine, which is given to suppress lactation (Kubo et al., 2002). In one case report, a patient was diagnosed with migraine headaches and after being treated with dihydroergotamine, developed worsening neurological symptoms and subsequently was diagnosed with postpartum cerebral angiopathy (Calado, Vale‐Santos, Lima, & Viana‐Baptista, 2004). The ingredient isometheptene, found in Midrin, an over‐the‐counter medication used to treat migraines, was also reported to cause postpartum cerebral angiopathy when it was taken in excess of the recommended amount (Raroque, Tesfa, & Purdy, 1993). The patient in this case study took this medication for her headaches after delivery of her baby. Although the dosage taken was unknown, taking this drug may have precipitated the onset of postpartum cerebral angiopathy.
The patient in this case study presented with the classic signs and symptoms of postpartum cerebral angiopathy. The most common initial sign of the condition is the sudden onset of a severe headache. Other symptoms include nausea, vomiting, seizures, visual changes, and a decreased level of consciousness or focal neurological deficits or both (Ursell et al., 1998). CT and magnetic resonance scans are usually normal initially but later show cerebral ischemia, hemorrhage, or edema, or all three. The CSF is usually normal and transcranial Dopplers reveal high flow velocities. One of the most beneficial diagnostic tests in patients suspected of having postpartum cerebral angiopathy is the cerebral angiogram. This test usually reveals areas of stenosis and dilation in multiple intracranial vessels (Ursell et al.). The patient's angiographic findings were consistent with the literature's explanation of postpartum cerebral angiopathy.
Prompt diagnosis is necessary to initiate early treatment. Treatment includes corticosteroids, such as intravenous methylprednisolone, and calciumchannel blockers, such as nimodipine. Most events resolve quickly without permanent neurological deficits. However, fatalities resulting from postpartum cerebral angiopathy have been reported. In cases where the medications mentioned were ineffective, some physicians have used long‐term immunosuppressive therapy, such as cyclophosphamide (Konstantinopoulos et al., 2004). Although not diagnosed immediately with postpartum cerebral angiopathy, the patient in this case study was started on the recommended medications early during her hospitalization. Unfortunately, she did not have rapid resolution of her neurological symptoms, as her recovery was complicated by continued vasospasm, status epilepticus, persistent subarachnoid hemorrhage, and hydrocephalus.
Because of the potentially devastating nature of this disorder, it is important for the nurse to recognize the symptoms of postpartum cerebral angiopathy early and be knowledgeable about the possible causes, common symptoms, and usual treatments required to prevent neurological deficits. Obtaining a thorough history and performing a detailed baseline neurological assessment is essential to identify deterioration that may follow the patient's initial presentation. Should the patient present with a headache, avoid the use of ergot derivatives or other vasospastic drugs, because they may aggravate the cerebral vessels. Airway protection should be considered and the patient's safety maintained if seizures develop. The nurse must administer antiepileptic medications, such as lorazepam or diazepam, to prevent status epilepticus, which would further compromise the patient's neurological status.
The nurse must also take special care to prevent secondary brain injury because of poor oxygenation, increased intracranial pressure, and cerebral edema.
One of the most beneficial diagnostic tests in patients suspected of having postpartum cerebral angiopathy is the cerebral angiogram. This test usually reveals areas of stenosis and dilation in mulitple intracranial vessels.
The nurse should closely monitor vital signs, intracranial pressure, and pulse oximetry. Increased intracranial pressure can be managed by keeping the head of the bed at 30 degrees, decreasing stimulation, and administering osmotic diuretics. To prevent further neurological damage, the nurse must perform frequent neurological assessments and watch for signs of vasospasm, which include decreased level of consciousness, confusion, coma, and hemiparesis. Calcium‐channel blockers and triple‐H therapy (hypervolemia, hypertension, hemodilution) should be administered, and the patient should be monitored with transcranial Dopplers.
The nurse must provide adequate nutrition and monitor intake and output. Dehydration can worsen the cerebral infarction, increase the risk of vasospasm, and cause systemic secondary complications. In addition, the nurse must monitor electrolytes, paying special attention to sodium; low sodium levels can cause seizures and further neurological damage and increase the risk of vasospasm. To prevent increased cerebral edema and bleeding, blood glucose levels must be kept within the normal range.
Use of aseptic technique when caring for the patient is necessary to prevent nosocomial infections, such as urinary tract infections and pneumonia. If the patient has neurological deficits, it is important to begin the rehabilitation process as early as possible to prevent such secondary complications as immobility, contractures, decubitus ulcers, and deep‐vein thrombosis. Important interventions are mobilizing the patient (if she is medically stable), performing range of motion to the extremities, and turning the patient every two hours. The nurse should ensure that the patient wears properly sized sequential compression device or thromboembolic stockings as ordered and administer prophylactic anticoagulants if ordered.
Psychological support is also necessary, because this condition occurs at a time when families should be rejoicing the arrival of a new baby. Other family members may be feeling guilty or be overwhelmed with the uncertain prognosis and resulting lifestyle changes. The nurse must also provide continued reassurance and education regarding the disease progress and patient outcome. The patient and family members may become frustrated if complications arise or symptoms do not resolve quickly. Preparing them for the possible need for rehabilitation or longterm care may be a necessary component of patient education. Counseling regarding avoiding future pregnancies should also be considered, because postpartum cerebral angiopathy has been shown to recur (Ursell et al., 1998).
This patient closely resembled the classic picture of postpartum cerebral angiopathy as evidenced by her presenting symptoms, neurological presentation, magnetic resonance angiogram, and angiographic findings. Unfortunately, hers was not a transient episode, unlike most cases in the literature, and it was complicated by a pulmonary embolus, subarachnoid hemorrhages, hydrocephalus, and status epilepticus. She was discharged with instructions to continue immunosuppressive therapy for 30 more days. Follow‐up on the patient several months after discharge was promising. Although she continues to reside in a long‐term facility, she is now following commands and able to communicate some needs. It is important for the neuroscience nurse to be knowledgeable about rare disorders such as postpartum cerebral angiopathy and to be cognizant of early symptoms and treatments to minimize permanent neurological damage.