Prognostic Role of Unusual Clinical Presentation in

Acute Mastoiditis
María Suárez-Bustamante Huélamo
Hospital Infantil Universitario Niño Jesús
Jose Antonio Alonso-Cadenas

Hospital Infantil Universitario Niño Jesús

María Pérez Moreno
Hospital Infantil Universitario Niño Jesús
Sonia Clavero Sánchez
Hospital Infantil Universitario Niño Jesús
Daniel Sánchez González
Hospital Infantil Universitario Niño Jesús
Mercedes de la Torre Espí
Hospital Infantil Universitario Niño Jesús

Short Report

Keywords: acute mastoiditis, intracranial, sequelae, atypical, neurologic symptoms

Posted Date: May 2nd, 2024

DOI: https://doi.org/10.21203/rs.3.rs-4281613/v1

License:   This work is licensed under a Creative Commons Attribution 4.0 International License.
Read Full License

Additional Declarations: No competing interests reported.

Page 1/8
Abstract
Purpose

To determine if unusual manifestations of acute mastoiditis (AM) have a worse outcome than when the
disease presents characteristically. As a secondary objective, we describe the patients with an atypical
clinical presentation of AM included in the series.

Methods

Retrospective single-center observational study of patients younger than 18 years diagnosed with AM
from June 1, 2018 to May 31, 2023.

Unusual or atypical presentation is defined as any clinical manifestation in the absence of retroauricular
erythema and/or ear protrusion observed on physical examination.

Results

A total of 110 children with AM were included in the registry. The clinical presentation was usual in 104
patients (94.5%) and unusual in 6 (5.5%). The signs and symptoms on physical examination of children
with an unusual presentation were as follows: fever, otalgia, and altered level of consciousness (Patients
1 and 2), ataxia (Patients 3 and 4), fever, ataxia, and vomiting (Patient 5), and headache and vomiting
(Patient 6).

Poor outcomes were more frequent in children with an unusual presentation (P < 0.001), mainly due to
intracranial complications. In this series, only one patient with a usual clinical presentation developed
sequelae (P > 0.05).

Conclusions

While AM with an unusual presentation is an uncommon finding, it should be considered in the

differential diagnosis of young children with neurologic symptoms. Our results do not suggest a delay in
diagnosis, but do show a greater risk of poor outcome.

INTRODUCTION
Acute mastoiditis (AM) is an infection of the mastoid air cells of the temporal bone secondary to
prolonged suppurative conditions of the middle ear. Commonly preceded by acute otitis media (AOM),
AM initially spreads to the periosteum and may progress to osteitis and bone destruction. Complications
of AM vary widely and can be extracranial or intracranial. The estimated incidence is 1.8/100,000
children since the introduction of the pneumococcal conjugate vaccine (PCV), PCV13.1

The diagnosis of AM is clinical. Patients usually present with fever, otalgia, retroarticular erythema,
and/or ear protrusion. In children, the most common associated organisms are Streptococcus
Page 2/8
pneumoniae, Streptococcus pyogenes, and Pseudomonas aeruginosa.1,2

Patients without the hallmark signs or symptoms are considered to have an unusual clinical
presentation, although only isolated reports have appeared in the literature.3 This study aims to
determine if unusual manifestations of AM have a worse outcome than when the disease presents
characteristically. As a secondary objective, we describe the patients with an atypical clinical
presentation of AM included in the series.

MATERIALS AND METHODS

We performed a retrospective single-center observational study of patients younger than 18 years
diagnosed with AM from June 1, 2018 to May 31, 2023. Data were collected through a standardized
online form, which included the following variables: age, sex, vaccines administered, history of recurrent
AOM or tympanostomy tube placement, previous AOM diagnosis, number of consultations until AOM
diagnosis, presence and grade of fever, accompanying symptoms and physical examination findings,
laboratory and image test results, diagnosis, management, and outcome.

All analyses were conducted using STATA V.17. Numerical variables were represented using median and
interquartile range (IQR) when the data were dispersed. Categorical variables were expressed as counts
and percentages. Comparisons between groups were performed using the Fisher exact test for
categorical variables and the Mann-Whitney U-test for numerical variables after the Kolmogorov-Smirnov
goodness-of-fit test indicated that the data did not follow a normal distribution. P values < 0.05 were
considered statistically significant. The sample size was too small to carry out a multivariate analysis.

Definitions
Unusual or atypical presentation is defined as any clinical manifestation in the absence of retroauricular
erythema and/or ear protrusion observed on physical examination.

Normal blood test values: white blood cell count (WBC) 5000–15,000/mm3, absolute neutrophil count
(ANC) 1500–10,000/mm3, C-reactive protein (CRP) < 40 mg/L, and procalcitonin (PCT) < 0.5 ng/mL.

Sepsis: We classified patient as septic based on the international consensus criteria for pediatric sepsis
and septic shock, published by Schlapbach et al.4

Poor outcome: extracranial (ie, subperiosteal abscess, facial paralysis, osteomyelitis, labyrinthitis,
hypoacusia) or intracranial complications (ie, bacterial meningitis, subdural empyema, sinus thrombosis,
intracranial abscess), sequelae, or death.

RESULTS

Page 3/8
A total of 110 children with AM were included in the registry. Among them, 72 (65.5%) were male and the
median age was 1.8 years (IQR, 0.9–3.9 years).

The clinical presentation was usual in 104 patients (94.5%) and unusual in 6 (5.5%). The signs and
symptoms on physical examination of children with an unusual presentation were as follows: fever,
otalgia, and altered level of consciousness (Patients 1 and 2), ataxia (Patients 3 and 4), fever, ataxia, and
vomiting (Patient 5), and headache and vomiting (Patient 6). An otoscopic evaluation revealed presence
of hyperemia in at least one tympanic membrane in all children studied, although with no bulging. Table 1
shows the patient characteristics according to usual or unusual clinical presentation. AM was diagnosed
during the first visit in 43.6% of patients (n = 48).

Page 4/8
 Table 1
Patient characteristics according to the form of clinical presentation of acute mastoiditis.
Clinical presentation

Usual Unusual P-value

N = 104 N=6

Age, median (IQR) 2.9 (0.9–3.8) 3.1 (1.9–4.3) n.s.

Recurrent AOM, n (%) 20 (19.2) 0 n.s.

Tympanostomy tube, n (%) 10 (9.6) 0 n.s.

< 2 weeks previous AOM diagnosis, n 33 (31.7) 1 (16.7) n.s.

(%)

Number of care visits until AM 1.4 (1–2) 1.5 (1-2.5) n.s.

diagnosis, median (IQR)

Length of admission (days), median 7.7 (5–8) 7 (6.3–25) n.s.

(IQR)

Blood test

• WBC, median (IQR) 15.200 (11.050– 15.150 (13.350– n.s.

19.670) 16.800)

• ANC, median (IQR) 8.020 (6.100- 9.590 (8.100– n.s.

11.650) 12.200)

• CRP, median (IQR) 87.8 (42.3-141.3) 98.9 (74.4–142) n.s.

• PCT, median (IQR) 0.13 (0.08–0.88) 0.28 (0.14–0.56) n.s.

CT scan, n (%) 18 (17.3) 6 (100) < 0.001

PICU admission, n (%) 5 (4.8) 2 (33.3) n.s.

Poor outcomes, n (%) 18 (17.3) 6 (100) < 0.001

• Sepsis, n (%) 1 (1.0) 0 n.s.

• Complications, n (%) 16 (15.4) 6 (100) < 0.001

o Extracranial complications, n (%) 12 (11.5) 2 (33.3) n.s.

o Intracranial complications, n (%) 4 (3.9) 4 (66.7) < 0.001

• Sequelae, n (%) 1 (1.0) 0 n.s.

• Death, n (%) - - -

IQR, interquartile range; AOM, acute otitis media; n, number of patients; AM, acute mastoiditis; PICU,
pediatric intensive care unit; %, percentage; WBC, white blood cell count; ANC, absolute neutrophil
count; CRP, C-reactive protein; PCT, procalcitonin; CT: computed tomography; n.s., not significant.

Page 5/8
A blood test was performed in 108 children (98.2%), revealing a high median CRP level (88 mg/L; IQR,
43.8–142 mg/L); no other relevant abnormalities were found in the remaining blood values (ie, WBC,
ANC, PCT). The ear culture was positive in 14 cases (12.7%), identifying 4 patients in whom S. pyogenes
was the causative organism, 4 Haemophilus influenzae, 3 P. aeruginosa, 1 S. pneumoniae in a correctly
vaccinated child (all patients in the registry except 2 (98.2%) had received PCV13), 1 Staphylococcus
aureus, and 1 Stenotrophomonas maltophilia. An imaging study (CT scan) was performed in 24 patients
(21.8%).

Sepsis was diagnosed in 1 patient with a usual presentation and none with an unusual form (P > 0.05).

The admission rate was 100%: 98 (89.1%) were admitted to the ward, 7 (6.3%) to the pediatric intensive
care unit (PICU), and 5 (4.6%) directly from the pediatric emergency department to the hospital at home
program. Poor outcomes were more frequent in children with an unusual presentation (P < 0.001), mainly
due to intracranial complications. In this series, only one patient with a usual clinical presentation
developed sequelae (P > 0.05). No patients died.

DISCUSSION
AM with an unusual presentation is a rare finding, representing fewer than 1 in 10 of all cases of AM.
Although uncommon, atypical manifestations of AM should be included in the differential diagnosis
given the presence of neurologic symptoms found in our pediatric population, independently of fever. As
these children do not seek care more frequently than those with a usual presentation, the signs and
symptoms appear insidiously. Their general condition resembled that of patients with a typical
presentation, yet the outcome was worse (21.2% vs 100%; P < 0.001). However, length of hospital stay
was similar in the 2 groups (P > 0.05).

Since the advent of universal PCV access, and especially PCV13, the incidence of AM has decreased,
although it remains the most common complication of AOM.1 The 2008 systematic review by van den
Aardweg et al.5 reported an average of 42% of children with a history of otologic disease, compared to
30% (n = 34) in our series. This lower percentage may be due to the retrospective study design used here,
as some of the charts may have been incomplete. Also, the authors found a mean duration of symptoms
suggestive of AM of 9.8 days. Although this information was often not included in the medical records
analyzed, we suspect that it is lower in our series, as the median number of previous visits to the
pediatric emergency department or primary care until diagnosis was 1.5 times for both groups. Given
that patients are instructed to seek medical care every 2 or 3 days if symptoms persist, we estimate a
mean duration of symptoms before AM diagnosis of 4 or 5 days.

Most existing reports describe individual cases of AM with an unusual presentation,3,6 which precludes
any comparison between our results and previous research.

In our sample, children in the 2 groups were of similar age and otorhinolaryngological history, which
suggests that neither of these 2 variables effectively predict the clinical presentation of mastoiditis (P >
Page 6/8
0.05).

Blood testing showed no significant differences between the 2 groups. CRP was the biomarker with the
highest sensitivity (approximately 75% of patients had a high CRP level), as described in the literature.2

PICU admission rates did not show statistically significant differences between groups (P = 0.054).
However, patients with an unusual presentation had a 7-fold higher risk for PICU admission (33.3% vs
4.8%), mainly due to the higher percentage of poor outcome.

Although the estimated incidence of complications in AM varies, recent reports have found adverse
events in 13–38% of cases.7 Intracranial complications are particularly common in the literature, ranging
from 4–16%2; meanwhile, ≈20% (n = 22) of the patients in our population had complications and ≈7% (n
= 8) had intracranial involvement. Thus, a combined analysis of both groups produces results like those
described in the literature. A study by Vergardi et al.8 of AM complicated by cerebral venous sinus
thrombosis, which is considered an intracranial complication, revealed that the most important finding in
these children was an atypical clinical presentation (5/5, 100%), which included 2 or more neurologic
signs and symptoms. Also, the authors use the term “silent mastoiditis” to refer to cases identified by
imaging studies in the absence of auricle protrusion, postauricular swelling, and postauricular erythema.
In our sample, the group with an atypical presentation had the highest number of intracranial
complications (66.7%, or 4/6 children with an atypical presentation vs 3.9%, 4/104 children in the usual
presentation group; P < 0.001).

Our study has certain limitations. Firstly, retrospective chart studies such as this one entail a loss of
data. Secondly, the low incidence of AM with unusual presentation makes it difficult to fully characterize
children with these symptoms.

While AM with an unusual presentation is an uncommon finding, it should be considered in the

differential diagnosis of young children with neurologic symptoms. Our results do not suggest a delay in
diagnosis, but do show a greater risk of poor outcome.

Abbreviations
AM, acute mastoiditis; ANC, absolute neutrophil count; AOM, acute otitis media; CRP, C-reactive protein;
IQR: interquartile range; PCV, pneumococcal conjugate vaccine; PCT, procalcitonin; PICU, pediatric
intensive care unit; WBC, white blood cell count

Declarations
STATEMENTS AND DECLARATIONS

The authors have not declared a specific grant for this research from any funding agency in the public,
comercial or not-for-profit sectors.

Page 7/8
Author Contributions

María Suárez-Bustamante Huélamo and Jose Antonio Alonso Cadenas conceptualized and designed the
brief report, drafted the initial manuscript and critically reviewed and revised the text.

María Pérez Moreno, Daniel Sánchez González and Sonia Clavero Sánchez collected information for the
database.

Jose Antonio Alonso Cadenas and Mercedes de la Torre Espí coordinated and supervised the redaction
of de manuscript, critically reviewed and revised the manuscript.

All authors approved the final manuscript as submitted and agree to be accountable for all aspects of
the work.

Ethics approval

This is an observational study. The Hospital Niño Jesús Research Ethics Committee has confirmed that
no ethical approval is required. This study was performed in line with the principles of the Declaration of
Helsinki.

References
1. Edwards S, Kumar S, Lee S, et al. Epidemiology and variability in management of acute mastoiditis in
children. American Journal of Otolaryngology-Head and Neck Medicine and Surgery.
2022;43:103520.
2. Cassano P, Ciprandi G, Passali D. Acute mastoiditis in children. Acta Biomed. 2020;91:54–9.
3. Velasco Zúñiga R, Fernández Arribas JL, Puente Montes S, et al. Unusual forms of mastoiditis: a
presentation of two cases. An Pediatr (Engl Ed). 2011;75:219–20.
4. Schlapbach LJ, Watson RS, Sorce LR, et al. International Consensus Criteria for Pediatric Sepsis and
Septic Shock. JAMA. 2024;331:665-674.
5. Van Den Aardweg MTA, Rovers MM, De Ru JA, et al. A systematic review of diagnostic criteria for
acute mastoiditis in children. Otology and Neurotology. 2008;29:751–7.
6. Lammers TH, Krieser DM. Unusual presentation of acute otomastoiditis with petrositis. J Paediatr
Child Health. 2013;49:E457-60.
7. Krishnan M, Walijee H, Jesurasa A, et al. Clinical outcomes of intracranial complications secondary
to acute mastoiditis: The Alder Hey experience. Int J Pediatr Otorhinolaryngol. 2020;128:109675.
8. Vergadi E, Velegrakis S, Raissaki M, et al. Acute mastoiditis complicated by cerebral venous sinus
thrombosis in children. Int J Pediatr Otorhinolaryngol. 2021;141:110508.

Page 8/8