Posterior reversible encephalopathy syndrome (PRES) is a complex neurological disorder characterized by a range of signs and symptoms, along with distinct neuroimaging findings reflecting vasogenic edema involving the cortical grey matter and subcortical white matter of the bilateral parietal to occipital regions [1]. While the etiology of this syndrome is multifactorial, it has been most commonly associated with conditions such as hypertensive encephalopathy and eclampsia, wherein vasogenic edema is a prominent feature. Additionally, chemotherapy and immunosuppressive drugs have been proposed as potential triggers, particularly in cancer patients [2]. Despite these known associations, our report presents an unprecedented case of PRES occurring subsequent to surgery for adrenal neuroblastoma in a seven-year-old girl, a scenario not previously documented in literature.

PRES is an acute neurological condition initially described in 1996 by Hinchey et al. [4]. Although often associated with elevated systemic blood pressure, this is not always the case. PRES can present with symptoms such as headaches, visual disturbances, altered consciousness, and seizures, if not promptly recognized and treated, it can progress to coma and even result in death. In cases where early identification and aggressive intervention are implemented, clinical recovery tends to precede radiological improvement, which might take several weeks.

The brain's vasculature has a remarkable ability to maintain steady blood flow even when systemic blood pressure fluctuates, owing to various regulatory mechanisms. However, when the mean arterial pressure surpasses the range of 150–160 mmHg, or higher in chronic hypertensive individuals [5], this equilibrium is disrupted. According to the vasogenic theory [6], rapid and uncontrolled hypertension triggers dysregulation in cerebral blood flow, leading to the breakdown of the blood-brain barrier. This breakdown causes proteins and fluids to leak into the interstitial space, ultimately resulting in edema. The relatively diminished sympathetic innervation in the vertebrobasilar circulation could explain the selective involvement of the posterior regions, as commonly observed in PRES cases. This theory gains further support from the rapid recovery, both clinically and radiologically, achieved through the prompt normalization of blood pressure.

An alternative perspective suggests that a systemic inflammatory state causing endothelial dysfunction might underlie PRES [7]. This theory is particularly relevant as PRES is frequently associated with systemic inflammatory conditions such as eclampsia, chronic renal disease, transplantation, autoimmune disorders, cancer chemotherapy, and sepsis. Angiography studies have demonstrated reversible focal and diffuse abnormalities consistent with endothelial dysfunction. Acute hypertension induces cerebral vasoconstriction, potentially leading to hypoxia and subsequent vasogenic edema. Restoring normal systemic blood pressure alleviates vasoconstriction, facilitating recovery, as observed in PRES cases [7].

The clinical manifestations of PRES are diverse [2]. Visual disturbances can range from mild blurriness to more severe conditions like homonymous hemianopia or cortical blindness. Altered mental states encompass a spectrum from slight confusion and agitation to coma. Additional symptoms can include nausea, vomiting, and brainstem deficits, with seizures being a common occurrence, either focal or generalized. In a study by Fugate et al. [6], seizures (70%) and hypertension (60%) were the most prevalent symptoms, followed by encephalopathy, headache, and visual disturbances.

MRI reveals distinct features, primarily characterized by bilaterally symmetrical parieto-occipital white matter hyperintensities. Some cases may show atypical features such as hemorrhage and restricted diffusion [1]. While the parieto-occipital lobes are most frequently affected, other areas like the frontal lobes, cerebellum, and temporal lobes can also be involved. In some instances, atypical regions such as the basal ganglia, brainstem, and thalamus may be affected [1].

Effectively managing PRES necessitates early recognition and aggressive supportive care. The patients must be monitored in an intensive care setting, with appropriate measures based on the etiology [8]. Treatment strategies include,

In cancer patients, chemotherapy and immunosuppressive drugs have been proposed as potential etiological factors. Hematological malignancies, especially in children, have a higher incidence of PRES. Notably, the use of bevacizumab, either alone or in combination with other treatments, has been linked to PRES [2].

PRES has been reported in neuroblastoma following the administration of anti-GD2 Monoclonal antibody immunotherapy [9] and vascular endothelial growth factor receptor inhibitor lenvatinib [10]. In the context of surgery, PRES has been documented primarily in neurosurgical procedures, such as resection of posterior fossa tumors [11] and ventriculoperitoneal shunting for fourth ventricle ependymoma [12]. To the best of our knowledge, this report presents the first documented instance of PRES triggered by abdominal surgery for a solid organ malignancy, specifically in a pediatric patient with adrenal Neuroblastoma.

PRES can be triggered by a range of underlying conditions. Timely identification and proactive management involving intensive care, anti-hypertensive agents, anti-epileptic medications, and adequate hydration can mitigate severe consequences and facilitate a return to the patients’ baseline health. Although the prognosis is generally optimistic, some individuals with more severe presentations might experience lasting neurological deficits. Despite growing awareness and reporting, PRES remains enigmatic. Future research endeavors are imperative to unravel the complexities of this potentially devastating yet manageable disorder.