Pott’s Puffy Tumor Managed in Infant with von Willebrand Disease

A clinical report published in the Egyptian Journal of Neurosurgery describes the successful surgical and medical management of Pott’s puffy tumor in a one-year-old boy diagnosed with von Willebrand disease. Pott’s puffy tumor is characterized by a subperiosteal abscess accompanied by osteomyelitis of the frontal bone, representing a rare and severe complication that typically arises from fronto-ethmoidal sinusitis or untreated cranial trauma. The authors noted that this presentation is the first documented case in medical literature combining Pott’s puffy tumor with an underlying bleeding disorder, as well as potentially the youngest documented patient with the condition in Pakistan.

Although termed a tumor, the condition does not represent a true neoplasm; rather, the historical designation refers directly to the localized inflammatory swelling resulting from infectious destruction. Medical literature indicates that Pott’s puffy tumor develops in fewer than 0.5 percent of acute and chronic sinusitis cases, exhibiting a recognized predilection for adolescent males. This demographic distribution has historically been hypothesized to stem from increased trauma frequency, though investigators emphasize that such age and gender associations lack a definitive scientific basis. Furthermore, while the frontal sinuses typically begin development around two years of age and become radiographically apparent at six years, infection can still arise in infancy through the frontal sinus recess, the ethmoid sinuses, or hematogenous dissemination.

The patient presented to an outpatient neurosurgery department in May 2023 with a six-month history of progressive swelling across the forehead that had slowly enlarged over time. Clinical history revealed that the child had previously received intravenous antibiotic therapy during hospitalization for a chest infection, at which point no cranial swelling had been observed, though further medical records from that stay were unavailable. At the outpatient evaluation, the one-year-old patient was alert, vitally stable, and presented without signs of systemic infection, fever, photophobia, seizures, or prior head trauma.

Physical examination identified a firm, non-tender, immobile mass measuring approximately three by three centimeters located over the frontal region. The overlying skin displayed mild erythema without any drainage or discharge, and a neurological assessment was completely unremarkable with a full Glasgow Coma Scale score. Baseline laboratory evaluations showed normal values for the child’s complete blood count, C-reactive protein, and prothrombin time, whereas the activated partial thromboplastin time was notably elevated, consistent with the patient’s known underlying von Willebrand disease.

Diagnostic neuroimaging confirmed the structural extent of the cranial lesion. An initial non-contrast computed tomography scan revealed a hypodense extracranial lesion in the frontal region accompanied by focal erosion of the underlying frontal bone. A subsequent contrast-enhanced magnetic resonance imaging study demonstrated a soft tissue mass alongside an interhemispheric ring-enhancing lesion with surrounding perilesional edema, confirming an intracranial abscess originating from the frontal infectious process.

Given the diagnosis of Pott’s puffy tumor and the life-threatening risk of perioperative hemorrhage linked to defective platelet adhesion and factor VIII stabilization in von Willebrand disease, clinicians carried out targeted preoperative hematologic optimization. The child received transfusions of packed red blood cells alongside fresh frozen plasma dosed at 10 milliliters per kilogram. The surgical team then proceeded with a bicoronal incision, identifying and excising hardened soft tissue that contained purulent material. Due to extensive bone destruction from osteomyelitis, surgeons performed a frontal craniectomy, preserved the pericranium, executed a complete capsulectomy, obtained biopsy specimens for laboratory evaluation, and secured hemostasis before layered closure with a temporary surgical drain.

The postoperative course remained uneventful, allowing the surgical drain to be safely removed on the first postoperative day following minimal fluid output. The child maintained an intact neurological status without fever or focal deficits. Empiric postoperative antimicrobial coverage commenced with intravenous ceftriaxone at 100 milligrams per kilogram daily and intravenous co-amoxiclav at 30 milligrams per kilogram daily. A repeat non-contrast computed tomography scan demonstrated the expected postoperative surgical changes at the frontal base with no evidence of residual fluid collections or intracranial hemorrhage.

Histopathological analysis of the resected tissue demonstrated chronic inflammatory changes, whereas microbiological cultures and sensitivity testing yielded no bacterial growth. Investigators explained that while literature reviews identify polymicrobial, anaerobe-predominant microbiomes dominated by Streptococcus species as typical in Pott’s puffy tumor, the absence of isolated pathogens in this patient likely stemmed from the broad-spectrum antibiotics administered during the preceding chest infection. Following guidance from infectious disease consultation, the child was discharged after three days of inpatient care on intravenous meropenem at 30 milligrams per kilogram daily and cefixime at 10 milligrams per kilogram daily, supplemented with short courses of oral levetiracetam and paracetamol syrups.

At the two-week outpatient evaluation, clinicians noted that the bicoronal surgical site was healing appropriately and the child remained completely asymptomatic. Having achieved satisfactory surgical source control and a positive clinical response, the care team discontinued intravenous antimicrobial therapy and transitioned the patient to an additional two-week regimen of oral cefixime. A follow-up examination at one month revealed complete resolution of the initial symptoms with no clinical indicators of persistent local or systemic infection.

The authors highlighted several clinical considerations and study limitations in their report, noting specifically that the patient resided in a distant geographic area and was subsequently lost to extended follow-up due to logistical constraints. They concluded that Pott’s puffy tumor must remain an important differential diagnosis in young pediatric patients presenting with unexplained forehead masses, even prior to the complete anatomical pneumatization of the frontal sinuses. The authors emphasized that successful management in patients with concurrent coagulopathies depends directly on prompt imaging, multidisciplinary hematologic preparation, meticulous surgical debridement, and appropriate broad-spectrum antimicrobial coverage.

In broader clinical contexts, Pott’s puffy tumor is recognized to arise in settings involving specific underlying predispositions. Clinical literature documents diverse systemic risk factors including rhinitis, aplastic anemia, diabetes mellitus, chronic renal failure, and general immunocompromised states. When evaluating suspected presentations, computerized tomography serves an essential role in delineating the structural integrity of cranial bone, whereas magnetic resonance imaging provides detailed visualization capable of identifying subperiosteal inflammation, epidural or subdural abscesses, and septic thrombi. In this infant, detailed multiplanar magnetic resonance sequences, including axial, coronal, and sagittal T1-weighted contrast imaging alongside axial T2-weighted and fluid-attenuated inversion recovery scans, demonstrated extracranial and frontal base hypointensity with marked perilesional hyperintensity, confirming the presence of an irregularly shaped ring-enhancing intracranial abscess and guiding surgical margins.

Exploring pediatric medical literature reveals how extraordinarily unusual this presentation is in very early life. Frontal sinuses begin initial formation at two years of age, appear on radiographs around six years, and reach full pneumatization only upon puberty. Consequently, infantile presentations remain exceptionally rare. A notable 2022 case documented by Karadaghy and colleagues described Pott’s puffy tumor in a twenty-three-month-old child, which had represented the youngest recorded patient in published literature. By comparison, the twelve-month-old patient evaluated in this report represents a distinctly younger presentation, establishing potentially the earliest documented case in Pakistan. Furthermore, while the child reported by Karadaghy and colleagues developed forehead swelling preceded by croup, and a case reported by Viana and colleagues followed clinical sinusitis, this infant presented after receiving therapy for a chest infection, demonstrating the diverse antecedent pathways that may precede pediatric cranial osteomyelitis.

Pathophysiological mechanisms of intracranial dissemination in Pott’s puffy tumor generally proceed through two distinct anatomical routes. Infectious spread may occur via direct erosion traversing the posterior table of the frontal sinus, as occurred in this patient, or through hematogenous propagation along the draining veins of the forehead and frontal sinus. Depending on the anatomical extent of disease, surgical options detailed in the literature include open or endoscopic frontal sinus trephination, sinus exenteration, ethmoidectomies, and maxillary antrostomies. For patients with intracranial collections, prompt debridement and abscess excision remain vital to prevent severe complications, including subdural empyemas, extensive epidural collections, or septic venous thrombosis.

The presence of underlying von Willebrand disease introduces critical considerations during invasive neurosurgical procedures. As an essential clotting glycoprotein, von Willebrand factor mediates platelet adhesion and stabilizes procoagulant factor VIII. Inherited deficiencies are classified into quantitative defects, designated as type 1 or type 3, and qualitative defects categorized as type 2. In patients with known coagulopathies undergoing surgery, factor replacement therapy or desmopressin is standardly deployed to mitigate life-threatening intraoperative hemorrhage and prevent delayed wound healing. Although von Willebrand factor contributes to platelet binding and microthrombi formation, which connects to the pathophysiology of septic thrombi, the authors emphasized that no causal association links von Willebrand disease to the initiation of Pott’s puffy tumor. The patient’s isolated coagulopathic abnormality was an elevated activated partial thromboplastin time, necessitating careful preoperative optimization before surgical debridement.

Comparative laboratory patterns also distinguish this case from conventional presentations described in clinical reports. In published series, diagnostic evaluations typically reveal pronounced elevations in systemic inflammatory markers, specifically elevated white blood cell counts, neutrophilia, and increased C-reactive protein levels. In this infant, all such inflammatory parameters remained within normal limits, a finding attributed to the broad-spectrum intravenous antimicrobial therapy previously administered for his chest infection. Systematic reviews by Daloiso and colleagues indicate that cultures from Pott’s puffy tumor cases generally yield polymicrobial anaerobic flora, particularly Streptococcus species, supporting standard empirical triple regimens consisting of vancomycin, metronidazole, and ceftriaxone. However, because colonization with Gram-negative organisms such as Escherichia coli and Actinomyces species has also been documented, selecting broad-spectrum coverage such as meropenem based on institutional microbial resistance profiles remains crucial when surgical cultures yield no organism, ensuring optimal source control in complex pediatric patients.

Subject of Research: Pediatric Pott’s puffy tumor complicated by von Willebrand disease

Article Title: Pott’s puffy tumor in a one-year-old boy with von Willebrand disease: a case report

Article References: Khan, F. I., Abid, A., Khan, H., Qadri, H. M., Khizar, A., & Bashir, A. (2026). Pott’s puffy tumor in a one-year-old boy with von Willebrand disease: a case report. Egyptian Journal of Neurosurgery, 41(1), Article 184. https://doi.org/10.1186/s41984-026-00692-x

Image Credits: AI Generated

DOI: 10.1186/s41984-026-00692-x

Keywords: Pott's puffy tumor, von Willebrand disease, pediatric neurosurgery, frontal osteomyelitis, subperiosteal abscess, craniectomy, case report, Pott, puffy, tumor, one-year-old, Willebrand