Acute otitis media is among the most common infectious conditions in childhood, with approximately 60% of children experiencing at least one episode by age three (1). Although uncommon, it may progress to otogenic cerebral venous sinus thrombosis (CVST), a serious intracranial complication with a reported prevalence between 0.2% and 2.7% (2). Thrombosis of the transverse or sigmoid sinus impairs venous outflow and cerebrospinal fluid (CSF) absorption, leading to elevated intracranial pressure (ICP), papilledema, and risk of permanent visual loss (3, 4).

While medical therapy with antibiotics, anticoagulation, and ICP-lowering agents remains first-line treatment, guidance regarding surgical escalation for vision preservation is limited (5, 6).Optic nerve sheath fenestration (ONSF) and CSF diversion procedures have been reported individually (7, 8), but their sequential use in pediatric otogenic CVST is not well defined (9, 10).

We report a pediatric case of otogenic cerebral venous sinus thrombosis with severe intracranial hypertension and vision-threatening papilledema, illustrating a stepwise neuro-ophthalmic management approach using optic nerve sheath fenestration followed by temporary lumbar drainage. This case emphasizes the role of timely, multidisciplinary intervention in preserving visual function when medical therapy alone is inadequate.

Lumbar puncture demonstrated an opening pressure of 42 cm H₂O with normal CSF cell counts and sterile cultures. Contrast-enhanced CT venography and MR venography confirmed complete thrombosis of the left transverse and sigmoid sinuses extending into the jugular bulb. MRI showed optic nerve sheath distension and posterior scleral flattening, consistent with elevated ICP (Figure 1).

Otogenic cerebral venous sinus thrombosis (CVST) is an uncommon but potentially life-threatening complication of acute otitis media in children, with reported mortality reaching 5–10% and significant risk for neurological and visual morbidity when diagnosis or treatment is delayed (4). Thrombosis most often involves the transverse and sigmoid sinuses due to their proximity to the mastoid air cells, where local inflammation can promote endothelial injury, platelet activation, and fibrin deposition, resulting in mural thrombus formation and possible propagation into the jugular bulb and petrosal sinuses (5). Impaired venous outflow disrupts cerebrospinal fluid (CSF) absorption, leading to otitic intracranial hypertension characterized by elevated intracranial pressure (ICP) and papilledema.

Clinical symptoms frequently begin with otologic features such as fever, otalgia, and otorrhea, followed by neurological or ophthalmic manifestations related to raised ICP. In pediatric cohorts, papilledema, abducens nerve palsy, diplopia, facial nerve palsy, headache, nausea, lethargy, and seizures are among the most commonly reported findings (11). These features were evident in our patient, whose bilateral severe papilledema and cranial nerve VI palsy reflected substantial intracranial hypertension.

Initial management universally includes broad-spectrum intravenous antibiotics and anticoagulation to prevent thrombus propagation and support restoration of venous drainage (6). Surgical interventions such as mastoidectomy or abscess drainage are generally reserved for cases with persistent infection or progressive intracranial complications despite adequate medical therapy (9). However, management becomes more challenging when visual function is threatened by refractory intracranial hypertension, and consensus regarding the optimal escalation strategy remains limited (12).

In this patient, papilledema persisted at Frisén grade 3–4 despite maximal medical therapy, placing the optic nerves at imminent risk of irreversible injury. Management was therefore guided by previously reported strategies for vision-threatening papilledema in cerebral venous sinus thrombosis (CVST). Optic nerve sheath fenestration (ONSF) was selected as the initial surgical intervention because progressive papilledema and visual risk were the dominant clinical concerns, consistent with published evidence demonstrating that ONSF can stabilize or improve visual outcomes in CVST when medical therapy alone is insufficient (13, 14).

Although ONSF resulted in improvement of papilledema in the operated eye, persistent edema in the contralateral eye indicated ongoing global intracranial pressure (ICP) elevation, a limitation that has been well described in prior studies (13). This incomplete bilateral response was further supported by neuroimaging findings, including optic nerve sheath distension and posterior globe flattening, which are recognized radiologic markers of sustained intracranial hypertension. These findings suggested that, while ONSF effectively protected the optic nerve locally, it did not adequately address the underlying global ICP burden related to persistent venous outflow obstruction.

Consequently, escalation to temporary lumbar drainage was undertaken to achieve systemic CSF diversion, an approach supported in refractory intracranial hypertension as a temporizing measure when permanent CSF diversion is undesirable, particularly in the setting of recent infection (15, 16). In our patient, lumbar drainage resulted in rapid bilateral improvement in papilledema, preventing the need for permanent CSF shunting. This clinical course supports a complementary, stepwise management strategy, in which ONSF serves to protect optic nerve function while lumbar drainage addresses global ICP elevation, in alignment with previously published experience.

This case contributes to the limited pediatric literature by providing detailed sequential ophthalmologic and radiologic documentation, illustrating how targeted interventions can preserve visual function in otogenic CVST with severe ICP elevation. Strengths of the report include high-quality imaging correlation and detailed examination timelines, while limitations include the inability to obtain pre-treatment OCT or formal visual acuity due to young age and symptom severity.

The patient’s family expressed concern about potential permanent vision loss but reported feeling reassured by frequent updates and clear explanations of the treatment plan. They noted significant rapid improvement after lumbar drainage and expressed satisfaction with the final visual outcome.