Pyogenic brain abscess associated with an incidental pulmonary arteriovenous malformation
- 1 Internal Medicine, Weill Cornell Medicine, New York, New York, USA
- 2 Geriatrics, Weill Cornell Medical College, New York, New York, USA
- Correspondence to Dr Kara Morgan Ryan; kmr9020@nyp.org
Abstract
Pulmonary arteriovenous malformations (PAVMs) are rare and often asymptomatic vascular anomalies that can be associated with serious neurological consequences due to right-to-left shunting. We report a case of a woman in her 80s without substantial medical history who presented with a headache, weakness and personality changes, and was found to have a pyogenic brain abscess requiring emergent neurosurgical evacuation. The abscess grew oral flora, suspected to have reached the brain via an incidentally discovered PAVM. With drainage and antibiotics, the patient achieved a full recovery and the PAVM was embolised. To our knowledge, this is the oldest presentation of a PAVM-associated brain abscess in the published literature. Older patients may present without the typical signs and symptoms of a given illness, which complicates accurate diagnosis and treatment. Primary care physicians can help facilitate timely care and positive clinical outcomes.
Background
A pulmonary arteriovenous malformation (PAVM) is an aberrant communication between the arterial and venous supply of the pulmonary vasculature, bypassing the normal capillary bed between them.1 PAVMs can present with or without symptoms and can exist as solitary, multiple or part of a syndrome such as the genetic condition hereditary haemorrhagic telangiectasia (HHT).
Streptococcus intermedius is a bacterium commonly found in the normal microbiome of the gastrointestinal, respiratory and genitourinary tracts; however, it has been also recognised as one of the most common pathogens in abscesses of the central nervous system.2 Dental infections or cleanings can be a source of initial infection with subsequent seeding into the brain.
We report a case of a S. intermedius brain abscess, dental infection and an incidentally discovered asymptomatic PAVM that potentially linked the odontogenic infection to cerebral abscess formation.
Case presentation
A woman in her late 80s presented to the emergency department with a new and progressively worsening right-sided headache. The headache began 1 week prior to presentation and was described as a throbbing pain. The headache was associated with global weakness, fatigue, gait imbalance and behaviour changes. Friends and family noted she was less interactive, and her speech was slowed. She had no relevant medical history. At baseline, she lived alone, was independent in all instrumental and basic activities of daily living, and regularly engaged in activities in the community, including music lessons. She had no recent trauma or falls. She had no associated vision or hearing changes, numbness or weakness of her extremities, nausea, vomiting or urinary incontinence.
On presentation, she was afebrile with a blood pressure of 134/77 mm Hg, a pulse of 52 beats per minute and an oxygen saturation of 96% on room air. On physical examination, she was alert and fully oriented with a normal cardiopulmonary examination. Neurological examination was notable for 4+/5 weakness of the left upper and lower extremities, left hemianopsia and a wide-based, unsteady gait. Sensory, reflex, cranial nerve and cerebellar examinations were normal. There were no mucosal or skin telangiectasias.
Investigations
Initial laboratory data included a normal white cell count of 8.2 x10∧9/Lwith 75% neutrophils. The patient’s haemoglobin, comprehensive metabolic panel, and urinalysis were within normal limits and an HIV test was negative. Chest radiograph was normal (figure 1). CT of the head demonstrated two hypodense masses in the right temporal lobe measuring 2.5×3.4 cm and 1.5×1.5 cm with surrounding vasogenic oedema, effacement of the right lateral ventricle and 5 mm of leftward midline shift. MRI of the brain was ordered. The neurosurgery service was consulted and recommended a CT of the chest, abdomen and pelvis to evaluate for an underlying malignancy, with a working diagnosis of a brain metastasis. This imaging study showed a right lower lobe PAVM and a uterine mass consistent with, but not diagnostic of, a fibroid (figure 2).
Initial chest radiograph showing lung fields without abnormality.
Pulmonary arteriovenous malformation (blue arrow) noted in the right lower lobe on CT imaging.
Differential diagnosis
Considering the CT findings, metastatic malignancy was initially of highest concern due to the patient’s advanced age and the presence of an uncertain pelvic mass. A primary malignancy of the central nervous system was less likely epidemiologically. The diagnosis of brain metastases from a presumed gynaecological origin was shared with the patient and her son, and she was admitted to the geriatrics service where her outpatient primary care provider (PCP) would be the attending physician. The leading diagnosis was rapidly abandoned when shortly following admission, MRI of the brain showed a 4.8 cm right temporal lobe lesion with irregular margination, peripheral enhancement with marked homogeneous central diffusion restriction, a ‘dual rim sign,’ and surrounding vasogenic oedema—a constellation of findings indicative of a brain abscess (figures 3 and 4).
Sagittal T1-weighted sequence of MRI brain demonstrating a 4.8×4.5×2.8 cm irregularly marginated, centrally T2 hyperintense lesion with associated marked homogenous central diffusion restriction and thick peripheral enhancement.
Axial T1-weighted sequence of MRI brain demonstrating a 4.8×4.5×2.8 cm lesion with radiographic features consistent with a brain abscess as well as 5 mm leftward midline shift secondary to mass effect.
Treatment
On establishing the diagnosis of a cerebral abscess, the neurosurgery team updated the patient and her son of this new diagnosis and explained the need for emergent surgical evacuation. The patient and her son were initially hesitant to proceed but agreed after a discussion with her PCP that reinforced the diagnosis and the risks of delaying intervention. The patient was retriaged to the neurosurgical service and underwent emergent craniotomy and abscess evacuation. Culture of the abscess yielded S. intermedius and Fusobacterium nucleatum, both common oral bacteria. Multiple sets of blood cultures remained negative and a transthoracic echocardiogram was without evidence of endocarditis. She received empiric broad-spectrum antibiotics that were narrowed to ceftriaxone and metronidazole. Because of the presence of oral flora in the abscess, a full dental examination was completed. There was mobility of the right upper second premolar. Maxillofacial CT demonstrated lucencies and mucosal thickening of the adjacent maxillary sinus concerning for infection. There was no radiographic evidence of direct communication between the tooth or maxillary sinus and brain abscess. This tooth was extracted on postoperative day three. The pulmonology service was consulted due to concern that the PAVM may have facilitated abscess formation; they recommended embolisation of the PAVM after completion of the antibiotic course.
Outcome and follow-up
She recovered well from surgery and was discharged to acute rehabilitation on hospital day 9. After a short acute rehabilitation stay, she was discharged home. She attended outpatient infectious disease and neurosurgery follow-up appointments and completed a 10-week course of intravenous antibiotics. Subsequent imaging demonstrated resolution of the abscess. The patient’s PCP remained involved in the case throughout inpatient admission, rehabilitation, and outpatient treatment and ensured follow-up with the pulmonology, infectious disease and interventional radiology subspecialists. The postoperative course was complicated by a deep vein thrombosis for which the patient was started on anticoagulation, which delayed PAVM embolisation. Approximately 6 months after her initial hospitalisation, she underwent successful coil embolisation of the PAVM by the interventional radiology team (figures 5 and 6). She has done well, experiencing resolution of her headaches, weakness and personality changes. She has been able to resume all of her prior activities.
Angiography demonstrating a right lower lobe pulmonary arteriovenous malformation prior to intervention.
Angiography following coil embolisation of the right lower lobe pulmonary arteriovenous malformation.
Discussion
This case offers three lessons, namely: (1) A brain abscess necessitates a search for PAVMs, even in older individuals or those without a history of HHT; (2) Embolisation is the recommended treatment for PAVMs to avoid future complications and (3) Preliminary diagnoses based on imaging alone, even if corrected, can lead to adverse consequences, including fostering mistrust at a time when intervention is urgently needed.
PAVMs are abnormal connections between the pulmonary artery and vein that create a right-to-left shunt. This disrupts the ability of the pulmonary capillaries to adequately filter thromboembolic material including bacteria, potentially facilitating the spread of infection.1 3 The incidence of brain abscess in the setting of PAVM is up to 9%.4 5 In this case, we postulate that an odontogenic infection was able to spread hematogenously, facilitated by a solitary PAVM. Embolisation to the brain led to abscess formation.
Odontogenic bacteria can access the brain in four ways: (1) haematogenous spread, (2) contiguous infection, (3) direct venous drainage and (4) lymphatic spread.6 7 Based on a systematic review of intracranial bacterial infections of presumed oral origin, haematogenous spread is proposed to be the most likely pathogenesis. A key finding supporting this assertion was the discordant laterality noted between the intraoral infection and the brain abscess, where only 26% had abscesses ipsilateral to the oral infection.7 In addition, both maxillary and mandibular infections were observed in approximately equal incidences.7 These observations favour haematogenous spread. Our patient’s head CT, brain MRI and maxillofacial CT were without evidence of a contiguous infection, further supporting this hypothesis. Prior defined criteria for an odontogenic brain abscess include: (1) a lack of an alternative source of bacteraemia, (2) microbiological studies revealing organisms typically found in oral microflora and (3) clinical or radiographic signs of active dental or periodontal disease.8 Our patient meets all three criteria, with the caveat that the PAVM creates an even higher risk of haematogenous spread through bypassing a host defence mechanism in the lung capillary bed. This case highlights the pathophysiology underlying PAVM-associated cerebral abscesses, which are recognised entities in the literature.9 10
The microbiological aetiologies of brain abscesses in immunocompetent hosts are often streptococci including S. intermedius, a common oral flora that was seen in our patient’s brain abscess.6 In a cohort evaluating 37 patients with PAVM-associated cerebral abscesses, the predominant organisms that were cultured were of periodontal origin including streptococci (Streptococcus milleri, Streptococcus anginosus and non-haemolytic streptococci) as well as Actinomyces species, S. intermedius and other unspecified anaerobic species.5
As nearly 70% of PAVMs are associated with HHT, there are multiple case reports in the literature of brain abscesses related to PAVMs in the setting of HHT.11–13 Our patient did not have any respiratory symptoms, and she did not display any other components of the Curaçao Diagnostic Criteria for HHT. The criteria include internal organ AVMs, epistaxis, skin or mucosal telangiectasias and a family history of HHT.14 There are far fewer reports of solitary, asymptomatic PAVMs leading to brain abscess, particularly in patients with advanced age. In fact, to our knowledge, this would be the oldest person with such occurrence in the published literature. In this case, the diagnosis of PAVM was incidentally discovered prior to the diagnosis of a brain abscess. In a patient with a new brain abscess, it is prudent to search for a PAVM as a contributing factor to prevent future harm.
Embolisation of the PAVM is recommended to prevent neurological complications including cerebral abscess and stroke. It is also indicated for prevention of lung haemorrhage and to improve exercise tolerance. Embolisation should be offered to both asymptomatic and symptomatic patients, provided they have a PAVM suitable for intervention, which originally was 3 mm or more in diameter.15 However, the recommendation based on size was withdrawn as embolisation of smaller PAVMs may also confer protection against complications.15 16 There are case reports of recurrent brain abscess in patients with PAVM that were either not discovered or not embolised initially, highlighting the importance of intervention.17
Another lesson from this case is the impact of the initial misdiagnosis of the intracranial lesion as a brain metastasis rather than an abscess. This led to inappropriate triage and urgent reassessment, as well as confusion and mistrust by family members. The initial differential diagnosis should have captured a spectrum of pathology broader than just malignancy. Other causes of a new brain lesion include cerebral infarction or haemorrhage, demyelinating disease and various infections including pyogenic abscess, tuberculosis, toxoplasmosis, cysticercosis, cryptococcus and more.6 18 Diagnostic discrepancies in patients being admitted via the emergency department can increase mortality and length of stay.19 Older patients are at risk for misdiagnosis for a variety of reasons, including attributing symptoms to normal ageing, having multiple complex comorbidities and using diagnostic criteria determined from a younger population.20 Perhaps advanced age led to the premature assumption that the brain lesion was malignant, since an abscess is considerably rarer in this population.
Connection with the geriatrics team, led by the patient’s own PCP, was fortuitous in re-establishing trust and enabling a timely, critical neurosurgical intervention. PCP involvement facilitated continuity from the initial presentation to the emergency department, through the postoperative course, acute rehabilitation and outpatient appointments. In the outpatient setting, the patient’s PCP offered counselling to the patient and family, while collaborating with the infectious disease and interventional radiology subspecialists to ensure outpatient PAVM embolisation.
Patient’s perspective
It is now more than 9 months since my operation, but I am still under the care of my doctors who regularly monitor my recovery. Though I feel fine and have been able to resume all my previous activities, I am grateful to know that I am being “watched” so that another serious incident could be identified more quickly and with greater efficiency.
The perspectives I had while in the hospital varied. When I was admitted, I really had no idea of what was going on, or the seriousness of my condition. I have always trusted my doctors without reservation, so that was a constant throughout. The only time that I felt frightened was during the first MRI. For some reason, perhaps because of what had infected my brain, I found that experience terrifying. I have had MRIs since and never had the same reaction.
After my operation, I began to understand my diagnosis, and as I progressed from one area of the hospital to another, I felt that the nurses, doctors, and therapists engaged me in my own recovery and understood me as an individual.
During this entire process, my two sons were in constant attendance, and they were consulted and advised by the doctors on the team as decisions were being made. When I was discharged, my sons oversaw the care I needed at home, and they administered the necessary intravenous antibiotics every day. Their involvement and their communication with my doctors gave me great confidence in my ongoing care, treatment, and complete recovery.
Learning points
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Although less common in older people, a brain abscess should be included in the differential diagnosis for patients with acute-onset headache, neurological deficits and a brain lesion on imaging.
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Clinicians should evaluate for pulmonary arteriovenous malformations (PAVMs) in patients with a brain abscess even in the absence of respiratory symptoms, because silent PAVMs can precipitate abscess formation via right-to-left shunting, allowing bacteria to bypass the lung’s innate immune system.
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Embolisation is the standard management of PAVMs to prevent future complications.
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Primary care providers, especially those with geriatric training, can help facilitate the care of older hospitalised patients in many ways, including guiding nuanced decision making and providing reliable continuity of care.
Ethics statements
Patient consent for publication
Footnotes
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Twitter @drkararyan
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Contributors All authors listed on the title page contributed equally. The patient was under the care of KR and ES. Report was written by KMR and ES.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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