Pott puffy tumor (PPT) is a forehead swelling and edema resulting from osteomyelitis of the frontal skull bone with associated subperiosteal abscess. First described by Sir Percival Pott in 1768, a surgeon in London. Initially called a tumor because the word tumor represented one of the four components of inflammation described by Aulus Cornelius Celsus; rubor (redness), tumor (swelling), calor (warmth), and dolor (pain).
When originally described, it was thought to be caused by a complication from direct trauma to the forehead. It is now known that it most frequently occurs as a complication of frontal sinusitis, commonly seen in young adolescents. It is characterized by a circumscribed, tender swelling at the forehead presenting with other associated signs and symptoms, which include fever, headache, nasal discharge, or raised intracranial pressure. Early diagnosis and treatment of this condition are crucial for a good outcome.
Causes of Pott puffy tumor:
Risk factors that affect the normal immune response and can influence the development of PPT include diabetes mellitus, chronic renal failure, and aplastic anemia.
Most of the time, the culture growth is polymicrobial. The most common organisms encountered include non-enterococci streptococci (47%), anaerobic oral bacterial (28%), staphylococci (22%). Less common organisms that have been reported include Fusobacterium, H. Influenza, Enterococcus, Pseudomonas, E. Coli, Pasteurella multocida, Proteus, and Bacteroides.
Pott puffy tumor is a rare clinical entity whose incidence has significantly decreased with the use of modern broad-spectrum antibiotics. Even though it is nowadays rare, rapid recognition and high suspicion is required for prompt management and improved outcomes. It can present in all age groups but is more commonly seen in the pediatric and young adolescent population. Very rarely is seen in adults. The immunocompromised population also has a high risk for the development of PPT.
Infection can develop in the pediatric age group because the pneumatization process of the frontal and ethmoid sinus starts as soon as one year old, but can vary and may be pneumatized by six years of age, reaching adult setting by 15 to 18 years old.
The frontal scalp swelling with edema is caused by underlying osteomyelitis of the frontal bone with an associated subperiosteal abscess, which, if not treated, leads to severe complications. The pathophysiology for the development of frontal skull osteomyelitis can occur due to hematogenous spread of the infection or to direct extension of infection.
The development of the subperiosteal abscess can be explained with the pneumatization process of the frontal and ethmoid sinuses. This process allows sinus mucosa communication with trabecular bone by a local venous system favoring the development of osteomyelitis. The frontal sinus communicates through diploic veins with dural venous plexuses. The pneumatization process starts as early as one year of age and ends approximately at 15 to 18 years of age. PPT most commonly presents in the adolescent period because it is when the peak of vascularity in the diploic circulation is reached during the pneumatization process.
Morphological anomalies during the pneumatization process, such as overpneumatized ethmoid bullae, extensively pneumatized middle turbinate, or enlargement of agger nasi cells may lead to frontal sinus obstruction by the anatomical anomalies or inflamed mucosa leading to dysfunction of the normal ciliary action and stagnation of mucus resulting in an anaerobic environment that stimulates bacterial growth.
Excessive use of intranasal cocaine leads to a compromise of the local blood circulation, leading to bone structure destruction and producing a favorable environment for bacteria.
The presentation is most commonly seen in patients 6 to 15 years old but may include all ages. The patient will show a fluctuating, tender swelling of the frontal scalp. A "doughy" erythematous forehead swelling associated with fever is sometimes considered a pathognomonic finding of pott puffy tumor. The most common presentation is a combination of forehead swelling, headache, fever, and rhinorrhea (nasal congestion or purulent/non-purulent secretion). In some cases, fever can be absent. Other signs/symptoms that may present in PPT include periorbital swelling, nausea/vomiting, cutaneous fistulas, meningitis, or encephalitis.
In the pediatric population, nonspecific symptoms can be present, and they vary depending on the severity of the infection. The indolent course usually presents with headache, rhinorrhea, and fever. The presence of a fluctuant, tender swelling of the frontal scalp should place PPT high in the differential.
Increased intracranial pressure is suspected if the patient presents with nausea/vomiting, photophobia, cranial nerve deficits, seizures, altered mental status, lethargy, or obtundation. Emergent diagnosis, imaging, neurosurgical/otolaryngologist consultation, and treatment is advised.
As soon as there is suspicion of pott puffy tumor, imaging should be done. The image of choice between a head computed tomographic (CT) scan with contrast or a brain magnetic resonance imaging (MRI) is controversial. One of them is necessary, not only to confirm the diagnosis but to identify any intracranial complication. Imaging should not be delayed, since rapid diagnosis and treatment influence the outcome and prognosis.
Bone scintigraphy with Tc-mMP is an option for diagnosis, although not preferred. It is more sensitive than CT for early osteomyelitis but has reduced sensitivity for acute sinusitis.
Laboratory workup includes complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, and C-reactive protein.
The rapid diagnosis and fast treatment of this condition are crucial for reducing the risk of development of complications and have an optimal outcome. Studies have demonstrated that the best strategy for management of pott puffy tumor is the combination of medical and surgical treatment to prevent further complications and improve morbidity and mortality.
Once the patient arrives and PPT is suspected, the patient should be admitted and started on broad-spectrum intravenous (IV) antibiotics, IV hydration, analgesia, and rapid coordination for imaging studies. Once the diagnosis is confirmed, otolaryngologist and neurosurgical consultation should be done.
Broad-Spectrum IV Antibiotics: Should be started as soon as the diagnosis is suspected. Coverage for the most common pathogens, including gram-positive and anaerobes, is required. It is essential to choose antibiotics that have adequate blood-brain barrier penetration for intracranial coverage. Choices include penicillin or vancomycin, 3rd generation cephalosporin, and metronidazole. Once the culture has a final result, antibiotic therapy should be given for that specific pathogen. The length of treatment varies but is prolonged and includes 4-8 weeks of IV antibiotic therapy, including those patients who had surgery performed. Some small extradural collections are often treated with IV antibiotics, but aspiration/biopsy is highly recommended to obtain culture and guide antibiotic therapy.
Surgery: Surgical options include an open approach or a minimally invasive technique (endoscopic intranasal frontal sinusotomy). The goal of surgery is to drain the sinus and excise the infected bone; this is extremely important to obtain a successful treatment. Traditionally, an open approach was the standard of care due to better visualization of the frontal recess, but it can provide a cosmetic deformity. With recent advances in technique and experience, endoscopic intranasal frontal sinusotomy is frequently used. The endoscopic approach has significantly less morbidity and mortality, shorter convalescent period, and no external scarring. Patients with anatomical variations causing PPT require surgical intervention. Surgery options will depend on image findings, including the location and extent of disease. The surgical options include:
Differential diagnoses for pott puffy tumor include the following:
The morbidity, mortality, and prognosis of pott puffy tumor will be dependent on the early successful treatment of PPT. If severe complications are present (intracranial complications), the prognosis is worst. Also, the more time PPT is left untreated, the worst the outcome/prognosis.
Intracranial complications: These are the most common complications in the pott puffy tumor. It occurs in 60-85% of patients with PPT. The pathophysiology behind this complication is through septic thrombophlebitis or to the direct extension of infection. The complications are frequently related to the delay of treatment.
Medication complications: side effects associated with the use of antibiotics or analgesia.
Surgical complications: All procedures have associated risks related (infection, hemorrhage, anesthetic complications, coma, death), but one of the most notable complications following the external approach is cosmetic surgical scar or deformities.
Pott puffy tumor is a rare but critical diagnosis because if untreated, it can be lethal. Parents need to monitor patients' signs/symptoms when episodes of sinusitis are present. Patients presenting recurrent episodes of sinusitis with associated headache, febrile episodes, purulent rhinorrhea, or forehead swelling should be more than enough to take the patient to the emergency department for evaluation and management. Rapid recognition of PPT for emergent treatment is crucial for an optimal outcome.
The treatment after arrival to the emergency department encompasses a large team of multidisciplinary health care professionals, including nurses, administrative personnel, case manager, physicians, therapists, pharmacists, and technicians. Every member of this team contributes to the successful treatment of a patient with pott puffy tumor. An integrated approach is necessary for efficient inter-professional and multidisciplinary teamwork.
While the emergency department physician coordinates the management, prompt consultation with an interprofessional group of specialists and good collaboration of the rest of the team members will offer the best possible care of the patient. Respectful and efficient teamwork must be maintained to obtain the best possible outcome and prognosis.
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