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\begin{document}

\title{Meningitis and Endocarditis as a Sequela of Streptococcus Pneumoniae
Mastoiditis: A Case Report}



\author[1]{Mitchell Peebles}%
\author[1]{Mehnaz Roshani}%
\author[2]{Kumaraman Srivastava}%
\affil[1]{Texas Health Harris Methodist Hospital Fort Worth}%
\affil[2]{Texas Christian University}%


\vspace{-1em}



  
  \date{May 27, 2024}


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\textbf{Meningitis and Endocarditis as a Sequela of Streptococcus
Pneumoniae Mastoiditis: A Case Report~}

\textbf{Running Title: Meningitis, Endocarditis, Mastoiditis}
~\textbf{as a Variant of Austrian Syndrome}

Dr. Mitchell Peebles, DO, PGY-1 Internal Medicine Resident at Texas
Health Harris Methodist Hospital, Fort Worth, Texas

Email Address: mitchellpeebles@texashealth.org

Dr. Mehnaz Roshani, MD, Internal Medicine Core Faculty at Texas Health
Harris Methodist Hospital, Fort Worth, Texas

Email Address: mehnazroshani@texashealth.org

Kumaraman Srivastava, MS3 at Anne Marion Burnett School of Medicine at
TCU, Fort Worth, Texas

Email Address: k.srivastava@tcu.edu

\textbf{Corresponding Author:} Kumaraman Srivastava

\textbf{Ethical Statement:} The authors of this study had the required
consent from the patient involved in this case report.

\textbf{Data Availability Statement:} All data can be found in the
article and no other data or materials were used.

\textbf{Acknowledgements:} N/A

\textbf{Conflicts of Interest:} Authors state no conflicts of interest.

\textbf{Background:} \emph{Streptococcus pneumoniae} is responsible for
more than 50\% of all bacterial meningitis and has a case fatality rate
of 22\% in adults\textsuperscript{1}. In addition, \emph{Streptococcus
pneumoniae} is also one of the most common causes of acute otitis media,
a known cause of mastoiditis. However, in conjunction with bacteremia
and endocarditis, limited evidence is able to be
identified\textsuperscript{2}. This sequence of infections also closely
relates to Austrian syndrome. Otherwise known as Osler's triad, Austrian
syndrome is a rare phenomenon of meningitis, endocarditis, and pneumonia
secondary to \emph{Streptococcus pneumoniae} bacteriemia that was first
delineated by Robert Austrian in 1956\textsuperscript{3}. The incidence
of Austrian syndrome is reported to be less than \textless{}0.0001\% per
year and has decreased significantly since the initial usage of
penicillin in 1941\textsuperscript{4}. Despite this, the mortality rate
of Austrian syndrome is still around 32\%\textsuperscript{5}. Despite an
extensive literature review, we were unable to find any reported cases
of variants of Austrian syndrome that include mastoiditis as the primary
insult. As such, we present a unique presentation of Austrian syndrome
with mastoiditis, endocarditis, and meningitis with complex medical
management that led to resolution for the patient.

\textbf{Objective:} To discuss the presentation, progression, and
complex medical management of a rare triad of mastoiditis, meningitis,
and endocarditis occurring in a patient.

\textbf{Key Clinical Message:} Austrian Syndrome classically consists of
meningitis, endocarditis, and pneumonia due to \emph{Streptococcus
pneumoniae} bacteremia. A literature review, however, does not show
variants of this triad. Our case highlights a unique variant of Austrian
Syndrome with mastoiditis, meningitis, and endocarditis which requires
immediate recognition and treatment to prevent devastating patient
outcomes.

\textbf{Case report:} A 78-year-old man with past medical history of
resected prostate cancer, pheochromocytoma, and obstructive sleep apnea
presented to the emergency department due to altered mental status,
right-sided whole-body weakness, and expressive aphasia. The patient's
wife reported that the patient was shivering and lethargic and his last
known normal was determined to be 14 hours prior to presentation. The
patient's recent medical history was remarkable for planned procedure to
undergo bilateral eustachian tube placement. The patient developed a
fever up to 103 F in the emergency department and initial labs revealed
a leukocytosis of 12 with lactic acid of 1.67. Further imaging included
CT scan of the head as well as CTA head and neck which did not reveal
any acute abnormalities other than a right-sided tympanomastoid effusion
FIGURE 1. The physical exam on admission was significant for altered
mental status, agitation, and subjective nuchal rigidity. Moreover, the
patient was incoherent, a significant decline from his baseline
independence according to his wife. The patient received a fluid bolus
and Cefepime in the ED prior to hospital admission. An attempt to obtain
a lumbar puncture in the ED was also unsuccessful due to the patient's
increased agitation. Upon admittance to the hospital, Vancomycin and
Ceftriaxone at central nervous system dosing were administered to the
patient for empiric coverage of meningitis. Furthermore, blood cultures
were positive for \emph{Streptococcus pneumoniae} . On hospital day 2,
another attempt at lumbar puncture under fluoroscopy was attempted but
was unsuccessful due to repeated patient agitation. Lumbar puncture was
finally obtained on hospital day 3 under sedation with the following CSF
findings consistent with bacterial meningitis: FIGURE 2

CSF cultures showed no growth and ENT was consulted for further
evaluation of right tympanomastoid effusion secondary to mastoiditis.
Patient underwent right mastoidectomy with bilateral myringotomy with
ear tube insertion on hospital day 3. The mastoid bone was noted to be
sclerotic and the mastoid air cells were filled with inflammatory
tissue. Vancomycin was also discontinued on hospital day 4 after
cultures revealed sensitivity to Ceftriaxone.

Further workup included a transthoracic echocardiogram which revealed an
immobile mitral valve echo-density measuring 0.7 cm in diameter
associated with chordal structures of the anterior mitral leaflet
without evidence of mitral regurgitation or mitral stenosis FIGURE 3.
This strongly suggested endocarditis and infectious diseases and
cardiology were consulted and determined transesophageal echocardiogram
was not required to confirm the diagnosis. As such, the decision was
made to extend the patient's ceftriaxone for 6 weeks for treatment of
endocarditis. However, the patient developed significant surgical site
bleeding on hospital day 6 which was presumed to be due to
ceftriaxone-induced thrombocytopenia, so ceftriaxone was stopped, and
Vancomycin was restarted. The patient also had elevated fibrinogen
suggesting possible disseminated intravascular coagulation so a dose of
cryoprecipitate was given, and the patient's platelet and fibrinogen
levels stabilized after hospital day 9. The patient had deconditioned
significantly from his baseline status so physical therapy was initiated
and the patient responded very well. On hospital day 10, a peripherally
inserted central catheter line was placed so that the patient could
continue to receive IV antibiotics at home. The patient was stable for
home discharge on hospital day 11.

\textbf{Discussion/Conclusion:}

Austrian's syndrome has been typically described as meningitis,
endocarditis, and pneumonia with multiple case reports and case series
describing the difficulties of managing this complex disease. There is,
however, no published cases that demonstrate a variant of Austrian
syndrome with the triad of mastoiditis, endocarditis, and meningitis. In
this patient's case, the tympanomastoid effusion may have been the
initial insult that led to mastoiditis. Moreover, meningitis has been
described in several cases as a rather uncommon complication of
mastoiditis\textsuperscript{6,7}. Furthermore, the etiology of the
patient's endocarditis was likely due to meningitis\textsuperscript{8},
but there are some case reports that suggest that mastoiditis may also
directly cause endocarditis\textsuperscript{9}. Our patient initially
presented with altered mental status with multiple failed attempts at a
lumbar puncture due to agitation, so we initiated empiric coverage of
meningitis with vancomycin and ceftriaxone\textsuperscript{10}. Notably,
we did not add ampicillin because our clinical suspicion
of\emph{Listeria monocytogenes} was extremely low. Initial CT scans
revealed a right tympanomastoid effusion which we believed was the nidus
of infection. Prompt ENT intervention surgically removed the right
mastoid bone to alleviate the probable bacterial source, but our patient
unfortunately developed post-operative DIC which is a known complication
of both meningitis and head/neck surgery\textsuperscript{11,12}.
Ceftriaxone was stopped due to incidences of ceftriaxone-induced
thrombocytopenia and cryoprecipitate was given with resolution of the
patient's uncontrolled bleeding. Our patient endured several
complications due to his complex interaction of diseases, but we were
able to treat him effectively. As such, this case report presents an
exceptionally distinct variant of a rare phenomenon in Austrian syndrome
with complex medical management that resulted in complete recovery of
the patient.

\textbf{Author Contributions:}

Author 1: Dr. Mitchell Peebles provided direct medical care to the
patient, helped write the initial manuscript, and reviewed/edited the
final draft before submission.

Author 2: Dr. Mehnaz Roshani supervised the patient's medical care and
reviewed/edited the final draft before submission.

Author 3: Kumaraman Srivastava helped write the initial manuscript,
reviewed/edited the final draft before submission, and also served as
the corresponding author.

\textbf{The authors of this study obtained written consent from the
patient prior to submission of this case report.}

\textbf{Key Words:} \emph{Streptococcus pneumoniae} , Austrian syndrome,
mastoiditis, meningitis, endocarditis

\textbf{References:}

1. Clinical features of pneumococcal disease. Centers for Disease
Control and Prevention. January 27, 2022. Accessed May 10,
2023.\emph{https://www.cdc.gov/pneumococcal/clinicians/clinical-features.html}.

2. Gregory J, Chohrach N, Iyengar D (August 29, 2022) Invasive
Pneumococcal Disease Secondary to Acute Otitis Media in a High-Risk
Patient: A Case Report and Review of Recent Changes to Pneumococcal
Immunization Guidelines. Cureus 14(8): e28557. doi:10.7759/cureus.28557

3. Shin YI, Papyan N, Cede\selectlanguage{ngerman}ño H, Stratidis J. Austrian syndrome: The
deadly triad. IDCases. 2020;22:e00948. doi: 10.1016/j.idcr.2020.e00948.
Epub 2020 Sep 4. PMID: 32923368; PMCID: PMC7473259.

4. Rodríguez Nogué M, Gómez Arraiz I, Ara Martín G, Fraj Valle MM, Gómez
Peligros A. Austrian syndrome: A rare manifestation of invasive
pneumococcal disease. Case presentation and literature review. Rev Esp
Quimioter. 2019 Apr;32(2):98-113. Spanish. Epub 2019 Mar 15. PMID:
30880376; PMCID: PMC6441982.

5. Akram A, Kazi A, Haseeb A. The Deathly Hallows of the Austrian Triad.
Cureus. 2020 Jan 5;12(1):e6568. doi: 10.7759/cureus.6568. PMID:
32047709; PMCID: PMC6999727.

6. Barry C, Rahmani G, Bergin D. Pneumocephalus and Meningitis as
Complications of Mastoiditis. Case Rep Radiol. 2019 Feb 19;2019:7876494.
doi: 10.1155/2019/7876494. PMID: 30915252; PMCID: PMC6399545.

7. Felisati G, Di Berardino F, Maccari A, Sambataro G. Rapid evolution
of acute mastoiditis: three case reports of otogenic meningitis in
adults. Am J Otolaryngol. 2004 Nov-Dec;25(6):442-6. doi:
10.1016/j.amjoto.2004.06.005. PMID: 15547816.

8. Lucas MJ, Brouwer MC, van der Ende A, van de Beek D. Endocarditis in
adults with bacterial meningitis. Circulation. 2013 May
21;127(20):2056-62. doi: 10.1161/CIRCULATIONAHA.113.001545. Epub 2013
Apr 17. PMID: 23596007.

9. Smith CP, Jackson C, Stewart R. Subacute bacterial endocarditis
secondary to mastoiditis: a rare complication. BMJ Case Rep. 2012 Nov
30;2012:bcr2012007247. doi: 10.1136/bcr-2012-007247. PMID: 23203179;
PMCID: PMC4543826.

10. Tunkel AR, Hartman BJ, Kaplan SL, Kaufman BA, Roos KL, Scheld WM,
Whitley RJ. Practice guidelines for the management of bacterial
meningitis. Clin Infect Dis. 2004 Nov 1;39(9):1267-84. doi:
10.1086/425368. Epub 2004 Oct 6. PMID: 15494903.

11. Ozaki H, Tachibana H, Ishikawa S, Yusa K, Kitabatake K, Iino M.
Disseminated Intravascular Coagulation after Surgery for Facial Injury.
Case Rep Dent. 2016;2016:6053652. doi: 10.1155/2016/6053652. Epub 2016
May 30. PMID: 27313913; PMCID: PMC4904073.

12. Mertens R, Peschgens T, Granzen B, Heimann G. Diagnosis and
stage-related treatment of disseminated intravascular coagulation in
meningococcal infections. Klin Padiatr. 1999 Mar-Apr;211(2):65-9. doi:
10.1055/s-2008-1043767. PMID: 10407813.\selectlanguage{english}
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