Introduction
Pott’s puffy tumor (PPT) is an osteomyelitis of the frontal bone accompanied by subperiosteal abscess, which typically arises after head trauma or frontal sinusitis. In addition, it can also be linked to infected insect bites, dental procedures, and nasal polyps (1,2). Prior to 2001, to the authors’ best knowledge, only 30 cases of PPT in the pediatric population had been published worldwide. However, from 2001 through 2022, a total of 292 cases had been published, including 140 pediatric cases (3), indicating an increase in the recognition and documentation of this entity (4-6). This case series with comprehensive literature review provides valuable insights into the epidemiology, clinical presentation, diagnosis, and management of pediatric patients with PPT, particularly from the emergency physician (EP)’s perspective. It highlights the variability in clinical practices from the initial emergency department (ED) visit to hospital discharge, and the lack of standardized guidance in the literature, particularly for EPs.
Methods
1. Study design
This retrospective chart review was conducted at 2 large hospitals in South Florida: Joe Di Maggio Children’s Hospital (JDMCH), a tertiary children’s hospital serving Southern Broward County, and Holtz Children’s Hospital (HCH) affiliated with Jackson Memorial Hospital (JMH), a tertiary regional referral center for Miami-Dade County.
We intended to capture all patients aged 0-18 years who were either hospitalized or discharged from JDMCH and HCH from January 31, 2016 through February 1, 2019. The patients were identified through a comprehensive search using diagnostic codes per the International Classification of Disease 10th Revision, which are associated with frontal bone osteomyelitis, acute sinusitis, epidural or subdural abscess, and/or periorbital or orbital cellulitis. The codes included J01, J01.1, J01.90, J01.91, J01.11, J01.08, J01.81, J01.40, J01.41, and J32.1. The charts of patients with the codes were independently reviewed by 2 pediatric infectious disease specialists and 1 emergency physician, identifying 11 patients meeting the clinical and radiographic criteria for PPT (see case definition below). Collected data included the patients’ demographic data, clinical presentation, imaging studies, details of medical and surgical management, laboratory/microbiological data, and clinical outcomes. The study protocol was approved by the institutional review boards of JDMCH and HCH with a waiver for informed consent (IRB no. JDMCH MHS.2019.41, JMH/HCH: 20191094).
2. Case definition
The following definition, proposed by the study team, was based on a comprehensive literature review (3,4,6,7). PPT is defined as meeting both 1 clinical and 1 radiographic criterion:
1) Clinical criteria: fever, headache, or frontal edema
2) Radiological criteria: frontal bone osteomyelitis, or frontal bone subperiosteal collection or subgaleal abscess, with or without intracranial suppurative complications, as observed on head computed tomography (CT) scan or brain magnetic resonance imaging (MRI).
3. A clinical pathway for diagnosis and initial management of PPT
The clinical pathway (CP) for diagnosing and managing PPT was initially developed by 2 pediatric infectious disease specialists using data from the patient cohort and comprehensive literature review (Fig. 1) (3,4,6-9). This CP was then submitted for multidisciplinary review and feedback from EPs to ensure the pathway’s practicality and effectiveness.
Results
From the total of 11 patients, 8 were treated at JDMCH and the other 3 at HCH. Their demographic data and medical history are summarized in Table 1. The male-to-female ratio was approximately 3:1. Seven patients were African Americans, 3 Hispanics, and 1 Caucasian. Ten out of 11 were previously healthy, though 2 patients had a history of seasonal allergies and one had sickle cell disease (SCD).
The most common symptoms of hospitalization in this cohort were fever, headache, and frontal edema (Table 2). Neurological symptoms were observed in only 3 patients experiencing manifestations such as seizures, right hemiparesis, facial palsy, lethargy, and transient diplopia. Phonophobia was noted in 1 patient. C-reactive protein concentrations were elevated in patients with more extensive intracranial complications. Upon hospitalization, all patients underwent both CT and MRI, revealing intracranial collections in 10 patients, including epidural, subdural and subgaleal abscesses, and cerebritis. Consequently, these 10 patients required endoscopic sinus surgeries, with 6 of them also necessitating craniotomies, all performed within 24 hours of hospitalization. Subsequently, all patients were transferred to the pediatric intensive care units for close monitoring per the hospital protocols, where they underwent placement of a peripherally inserted central catheter in anticipation of a prolonged course of antibiotic therapy (Table 3).
In terms of microbiological findings, among the 10 intraoperative samples collected, 3 yielded negative results. Interestingly, 2 of the 3 patients with negative cultures tested positive for Streptococcus intermedius in their blood cultures. The remaining 7 intraoperative samples showed the presence of various pathogens, including Streptococcus pneumoniae, S. intermedius, Streptococcus pyogenes, Eikenella spp., and methicillin-sensitive Staphylococcus aureus. Empiric broad-spectrum antibiotics were administered intravenous (IV) at doses for meningitis to all patients. The preferred combination consisted of vancomycin and ceftriaxone, except 1 patient who was critically ill upon hospitalization and received cefepime instead of ceftriaxone. Anaerobic coverage was empirically provided to only 2 patients: 1 with head trauma and another with SCD. Additionally, 1 patient required continuous infusion of vancomycin to achieve therapeutic concentrations.
The mean length of stay was 9.8 days (standard deviation, 8.0), during which they received comprehensive care from a multidisciplinary team including the Departments of Otorhinolaryngology, Pediatric Infectious Disease, Pediatric Intensive Care, Ophthalmology, and Neurosurgery. All patients underwent a course of IV antibiotics lasting 6-8 weeks. A shorter length of stay was reported for the patients who were able to be discharged to continue IV antibiotics with home nurse support. When cultures were available, the antibiotic regimen was refined based on susceptibility profiles. Following the hospital discharges, 10 patients received ongoing outpatient care from the pediatric infectious disease specialists for a minimum of 6 months, with no reported sequela or mortality. None of the patients required surgical re-intervention.
Discussion
PPT, initially documented in 1769 by Sir Percivall Pott, delineates a soft subperiosteal abscess of the frontal bone linked to forehead trauma, which he termed a “puffy tumor.” In 1879, Lannelongue suggested a potential progression from frontal sinusitis to osteomyelitis with erosion of the anterior frontal bone as a plausible etiology for PPT. This theory continues to be the primary predisposing factor, particularly in the pediatric patients, where a history of forehead trauma accounts for only 15% of PPT cases (1,2,10,11).
The pathophysiology may originate from the extension of frontal or ethmoidal sinusitis, either through direct infiltration of the sinus walls or retrograde flux via the diploic veins. Areas with the least resistance, such as the floor of the anterior sinus and the anterior wall, are particularly vulnerable to this process, resulting in erosion of the external bone table and subsequent formation of a subperiosteal abscess. Additional predisposing factors include immunosuppression, preceding dental infections, acupuncture, use of nasal methamphetamine and cocaine, mastoiditis, craniotomy or cranioplasty, insect bites, frontal sinus mucocele, acute otitis media, and sebaceous cyst removal (5-7,12).
PPT demonstrates a predilection for male adolescents, attributed to their larger diploic veins, which lead to heightened blood flow and an increased likelihood of frontal sinusitis (9,13). However, rare cases have been described in children aged as young as 3 years despite minimal frontal sinus development at this age (5,11,12). The sex distribution in our cohort aligns with this trend, with 8 of the 11 patients being males, reflecting a male-to-female ratio of 3:1. This sex disparity is noteworthy given that sinusitis, a common precursor to PPT, is more frequently observed in females, constituting up to 60% of cases (4,14). Conversely, pediatric studies demonstrate a higher incidence of head trauma in males, suggesting a complex interplay of factors contributing to PPT development in the pediatric male population (15).
The current literature does not suggest any specific association with race or ethnicity. The distribution of the race or ethnicity in our series likely mirrors the local demographics of both hospitals. Miami-Dade County’s population consists of 71% white individuals, of whom 89% were self-identified as Hispanic, and 16.7% African American. In contrast, Broward County has a higher proportion of African American residents at 20% but a lower Hispanic population, accounting only for 27%, compared to Miami-Dade County (16). As part of the critical evaluation of social determinants of health in our population, it is worth mentioning the following facts. In Miami-Dade County, 93% of children have health insurance, but 39.1% below the poverty line are African American and 22.9% are Latinx. This demographic feature possibly hampers access to health care, which could result in delayed diagnosis and treatment of sinusitis, associated with an increased risk of PPT (17).
The etiology of PPT is usually polymicrobial in nature. In approximately two-thirds of cases, cultures of the secretions reveal the presence of multiple microorganisms. The most common pathogens include Streptococcus anginosus group (S. anginosus, S. constellatum, and S. milleri), Streptococcus viridans, S. pyogenes, S. pneumoniae, and S. intermedius. Additionally, S. aureus, Streptococcus epidermidis, Enterococcus spp., Haemophilus influenzae, and Pseudomonas aeruginosa, as well as anaerobes linked to cavities and dental work, such as Peptostreptococus micros, Pasteurella multocida, and Fusobacterium necrophorum (1,2,4,5,11,18-20). Candida spp., Aspergillus spp., and Mucor spp. are rarely involved (21,22). In our cohort, 4 of the 11 patients had positive cultures of the peripheral blood. Two of the cultures were from patients with negative intraoperative cultures, both positives for S. intermedius, which could be attributed to intraoperative culture sampling bias or prior exposure to antibiotics decreasing yield of intraoperative cultures, or represent a blood culture contaminant. This highlights the importance of obtaining blood cultures prior to antimicrobials, usually in EDs, in patients where hematologic dissemination is a concern, as supported by previous studies (23,24).
Initial symptoms of PPT are insidious, starting with inflammation of the subgaleal space that evolves into a phlegmon and eventually progresses into an abscess (2). During this progression, some patients may experience headaches or nasal congestion, while others may already exhibit systemic symptoms (18). EPs are often the first to assess children with this cluster of unspecific symptoms, indicating a need for a high index of suspicion. Subperiosteal abscess is frequently misdiagnosed as a scalp abscess. Use of antibiotics at this stage may mask symptoms, potentially delaying diagnosis. However, as fever subsides, frontal edema becomes more pronounced, prompting medical evaluation.
Neurological manifestations are observed in up to 40% of PPT cases, including seizures, dyspraxia, cranial nerve palsy, visual deficit, intracranial hypertension, pneumocephalus, and coma (1,2,25). In our series, only 1 patient exhibited seizure, which subsequently progressed to hemiparesis and facial palsy, indicating the deterioration of a subdural collection. Orbital involvement, documented in 30% of cases, was observed in just 2 of the 11 patients in our study.
A recent systematic review on adult PPT showed an incidence of up to 30% for the occurrence of intracranial complications (26). In our cohort, 90.9% reported intracranial complications, exceeding the 72% previously described in adolescents, with epidural abscess being most prevalent, aligning with literature findings (2,26,27). Furthermore, the communication between the diploic veins and the dural venous plexus allows the development of additional, less common intracranial complications, such as subdural or intracerebral abscess, cerebritis, meningitis, venous sinus thrombosis, and sinocutaneous fistula (2,5,6,25,28). None of our patients developed septic shock although sepsis could arise as infection progresses or intracranial complications develop (2,6).
Several underlying conditions have been associated with an elevated risk of PPT, including diabetes mellitus, aplastic anemia, and dialysis (2,4) (see the risk factor section in Fig. 1). In our series, only 1 patient had co-morbidity, SCD, while the remaining cases occurred in healthy children.
In general population, CT scan remains the gold standard for diagnosis of PPT, with findings including involvement of 1 or more sinuses, cortical bone erosion, and frontal soft tissue swelling (10,26). When intracranial or infraorbital involvement is suspected, MRI is warranted (1,2,18). CT scan is particularly useful for assessing local extension, whereas MRI is valuable for ruling out the presence of intracranial collections (25,29). An adult case report introduced the value of point-of-care ultrasound to identify patients with suspected PPT in the acute setting, and prioritized further imaging modalities (27). While it may offer some utility in identifying subperiosteal collections and bone defects, its efficacy in pediatric cases necessitates further research and validation (2). The proposed CP clearly presents a way to navigate different imaging modalities and avoid delays in diagnosis and treatment.
As part of diagnostic assessment, cerebrospinal fluid cytology may show abnormalities if the meningeal space is involved. Lumbar puncture should be considered once an intracranial mass has been ruled out (25). However, it is crucial to note that neither white blood cell count nor cerebrospinal fluid cytology alone should be relied upon to diagnose or assess response to treatment (18). Biopsy from tissue samples obtained from patients with PPT typically reveals signs of local inflammation, granulation tissue, and new bone formation (6). Nonetheless, in our series, there were no pathology samples available to review.
Prompt IV administration of broad-spectrum antibiotics is recommended to reduce morbidity on patients with PPT (25). Common combinations include third-generation cephalosporin, metronidazole, or vancomycin (1,25). Endonasal endoscopy has emerged as the predominant surgical approach, although the choice ultimately depends on the extent of the infection (6). Antibiotic or surgical therapy alone may yield inferior outcomes compared to the combined treatment, raising the risk of recurrence (1,25,30,31). The duration of antibiotic therapy has been described as 6-8 weeks. In the study cohort, a long length of stay probably reflects the current diversity in management or the high rate of intracranial complications.
In recent studies, major neurological sequelae are relatively uncommon with seizure disorders being most frequently reported (2,25). Recurrent abscesses represent the primary cause for re-intervention (25). While mortality rates of up to 17% have been described in patients with PPT, our study did not observe any patient experiencing sequelae or mortality. The favorable outcomes might be related to the advances in diagnostic tools, heightened awareness, or prompt interventions.
This CP is designed to screen for potential PPT and enable early detection, even before overt clinical signs emerge, with the goal of minimizing the risk of intracranial complications and reducing the need for surgical interventions. It underscores the critical role of a detailed clinical history. The age cutoff of 6 years was selected given that PPT usually begins to present in patients older than that. This cutoff coincides with the natural development of the frontal sinus, and aligns with the American guidelines for pediatric imaging studies in patients presenting with headaches (32). Imaging in children younger than 6 years often requires sedation, particularly for MRI, at the cost of additional risks. For the younger patients, a case-by-case evaluation is recommended to determine the need for imaging, as this age group has a higher prevalence of stabbing headaches (12.4% vs. 3% in older children), often idiopathic (33). The CP also highlights multidisciplinary care and ensures thorough follow-ups with regular imaging to monitor recovery processes.
As part of the limitations of this study, its retrospective nature introduces inherent biases, such as incomplete documentation and variability in clinical management practices across the 2 institutions. The reliance on the International Classification of Disease codes for case identification may have excluded cases not coded accurately. Additionally, while the proposed CP is based on available data and expert consensus, further prospective validation is necessary to confirm its effectiveness in diverse clinical settings.
Ultimately, we propose a CP underscoring the pivotal role of EPs in the timely identifying development of PPT. In addition, we emphasize the need for EPs to recognize social factors delaying the diagnosis, particularly in underserved populations. The structured approach proposed with this CP aims to improve the outcomes through prompt diagnosis, timely intervention, and coordinated care, starting from EDs.
PDF Links
PubReader
ePub Link
XML Download
Full text via DOI
Download Citation 
