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Cavernous sinus thrombosis is a rare yet potentially fatal and debilitating condition in children which is important to diagnose early. It requires a high index of suspicion and confirmation by imaging.
A previously healthy, 6-year-old boy (Figure 1) presented to accident and emergency with a 1-week history of being unwell. He complained of a 1-week headache, which radiated from the occipital to the frontal region. It was not relieved by analgesia and he remained lethargic. He had a 5-day history of pyrexia not resolving. He also had a 1-day history of redness and swelling of the left periorbital area.
Examination revealed him to be irritable but with a Glasgow coma score of 15/15. He had puffiness around his left periorbital region with erythema. He had a full range of left eye movements, and pupils were equal and reactive to light. There was no involvement of his cranial nerves with normal external ocular eye movements. He otherwise had an unremarkable systemic and neurological examination.
He was commenced on intravenous antibiotics (cefuroxime and metronidazole). An initial diagnosis of preseptal cellulitis secondary to sinusitis was made. Thirty-six hours after admission it was felt that there was no improvement and he persisted to be lethargic and pyrexial so a computerized tomography (CT) scan was performed. The initial CT scan revealed bilateral cavernous sinus and left orbital vein thrombosis, sphenoid and ethmoidal sinusitis and a left prespetal cellulites (Figure 2).
He was therefore transferred to a tertiary centre for further neurological assessment and management. Anticoagulation with low molecular weight heparin was commenced. This was then changed to warfarin with the aim that his international normalized ratio should be maintained at three. His temperature continued to spike and his blood cultures on admission grew Streptococcus milleri. Magnetic resonance scan (MRI) of his sinuses was performed and the cavernous sinuses remained bulky. In view of this, an endoscopic sphenoidectomy and posterior ethmoidectomy was performed and a substantial amount of pus was drained from the sphenoid ostium.
A repeat MRI three months later revealed resolution of the thrombosis and anticoagulation was stopped. He made an uneventful recovery and had no neurological sequelae.
The advent of antibiotics, while apparently not lessening the frequency of sinusitis, has reduced the severity and complications. Children are at greater risk of developing serious life-threatening complications from oropharyngeal infections. Early recognition and treatment are essential to reduce any subsequent morbidity or mortality. The most feared complication is intracranial suppuration, which may manifest as cavernous sinus thrombosis. In a recent review, only one child out of 104 admitted with complications of acute sinusitis had cavernous sinus thrombosis.
Cavernous sinus thrombosis may result from any infection of the tissue drained by the cavernous sinus. This includes the midface, orbit and sinonasal cavity. The mortality rate remains high at 30%, and significant morbidity includes residual cranial nerve palsies and blindness.
Thrombophlebitis of the ophthalmic veins attributable to orbital inflammation secondary to ethmoiditis may extend posteriorly, causing cavernous sinus thrombosis. The sphenoid sinus, like the frontal, develops in diploic bone and has venous communications with the adjacent cavernous sinus. Therefore sphenoiditis can result in cavernous sinus thrombosis via this route.
Clinical features vary. The onset is usually abrupt, with unilateral periorbital edema, headache, photophobia, and proptosis. In our patient, preseptal cellulitis resulted from untreated sinusitis and represented local spread of inflammatory elements to the lid. Headache and lethargy were the marked features, which were persistent after 36 h from admission. Even though there were no neurological signs, his persistent symptoms raised the index of suspicion of an intracranial complication being present and in particular cavernous sinus thrombosis. Despite its intimate connection with the cranial nerves III, IV, V, and VII, neurological dysfunction might not be typical in the early stage of the illness. This highlights the difficulties of making a diagnosis based on clinical examination alone.
The principal bacterial agents causing sinusitis are Streptococcus pneumoniae, other streptococci, Staphylococcus aureus, and non-spore-forming anaerobes. Our patient grew S. milleri from his cultures, which is a common pathogen with complications of sinusitis in children. Other aerobic and gram-negative organisms have also been isolated from orbital infections. Polymicrobial infections are common. The incidence of orbital and preseptal cellulitis due to Haemophilus influenzae has decreased dramatically over the last 10 years, owing to widespread immunization.
Most cases of paediatric paranasal sinusitis do not require radiographic confirmation and are treated medically. If an intracranial complication is suspected, imaging is required. Emergent cross-sectional imaging should include contrast-enhanced axial CT (with coronal reconstructions) to evaluate the sinuses and orbits. MRI is required in addition to this if intracranial extension is suspected. There is no role for plain radiography in the evaluation of the complications of acute sinusitis and preseptal cellulitis in a child.
Treatment for cavernous sinus thrombosis includes high-dose intravenous antibiotics directed at the most common causative organisms, coupled with surgical drainage of the primary source of infection.
Our case highlights that the diagnosis of cavernous sinus thrombosis complicating sinusitis and periorbital cellulitis requires a high index of suspicion and confirmation by imaging. Examination may normal early on in this rare disease, and early multi-disciplinary team (ENT, ophthalmology, and radiology) working is vital. Preseptal cellulitis is a common presentation in children, and the possibility of underlying sinusitis and its complications should be thought of.
Competing interests None declared.