Fatal malignant cerebral vasospasm following pediatric craniopharyngioma resection: A case report and literature review

Abstract

Background:

Cerebral vasospasm is a rare but devastating complication following intracranial tumor surgery, particularly in pediatric suprasellar lesions such as craniopharyngiomas. Delayed cerebral ischemia secondary to postoperative vasospasm remains insufficiently recognized and may result in fatal neurological injury.

Case Description:

We report the case of a 5-year-old girl who underwent gross total resection of a cystic suprasellar craniopharyngioma through a subfrontal approach without intraoperative vascular injury. The immediate postoperative course was uneventful. On postoperative day 6, the patient developed sudden refractory status epilepticus associated with rapid neurological deterioration and bilateral fixed mydriasis. Brain computed tomography (CT) revealed extensive left hemispheric ischemia with severe cerebral edema. CT angiography demonstrated marked narrowing of the A1 + M1 segment of the left middle cerebral artery, consistent with malignant cerebral vasospasm. Despite intensive care management, the patient progressed to brain death and subsequently died.

Conclusion:

Delayed malignant cerebral vasospasm following pediatric craniopharyngioma resection is an unpredictable and potentially fatal complication that may occur even in the absence of intraoperative vascular injury. Prompt recognition of delayed neurological deterioration and early vascular imaging are crucial to improve diagnostic and therapeutic management.

INTRODUCTION

Craniopharyngiomas are benign epithelial tumors accounting for approximately 5–10% of intracranial tumors in children and are predominantly located in the sellar and suprasellar regions.[8] Owing to their close anatomical relationship with critical neurovascular structures, including the optic apparatus, hypothalamus, pituitary stalk, and vessels of the circle of Willis, surgical management remains particularly challenging despite advances in microsurgical techniques.[9]

Surgical resection remains the cornerstone of treatment for pediatric craniopharyngiomas; however, it may be associated with significant postoperative complications, including endocrine disturbances, hypothalamic injury, cerebrospinal fluid leakage, and vascular events.[9] Among these complications, cerebral vasospasm is an uncommon but potentially devastating entity that remains insufficiently recognized following intracranial tumor surgery.

Although cerebral vasospasm is classically associated with aneurysmal subarachnoid hemorrhage, increasing evidence suggests that it may also occur after resection of skull base tumors, particularly lesions located in the sellar and parasellar regions.[3,6] Proposed mechanisms include mechanical arterial manipulation, endothelial injury, inflammatory reactions, and occult subarachnoid blood products leading to delayed cerebral ischemia.[10,11]

Postoperative vasospasm following craniopharyngioma surgery appears to be particularly relevant in pediatric patients because of the intimate relationship between the tumor capsule and the vessels of the anterior circulation. Clinical presentation is often nonspecific and may include altered consciousness, focal neurological deficits, seizures, or rapid neurological deterioration, making early diagnosis difficult.[3,6]

We report a fatal case of delayed malignant cerebral vasospasm occurring after gross total resection of a pediatric craniopharyngioma, presenting with refractory status epilepticus and massive hemispheric ischemia. Through this observation and a review of the literature, we aim to highlight the diagnostic challenges, potential pathophysiological mechanisms, and importance of early vascular imaging in cases of delayed postoperative neurological deterioration.

CASE REPORT

A 5-year-old girl was admitted to our department for the management of a cystic suprasellar craniopharyngioma presenting with symptoms of intracranial hypertension and progressive visual impairment. Preoperative magnetic resonance imaging demonstrated a predominantly cystic suprasellar lesion compressing the optic chiasm and adjacent structures [Figure 1].

Figure 1:

(a-c) Preoperative contrast-enhanced magnetic resonance imaging in sagittal, coronal, and axial views demonstrating a suprasellar solid-cystic lesion consistent with craniopharyngioma. (d) Contrast-enhanced axial computed tomography scan showing the craniopharyngioma with intratumoral calcifications.

The patient underwent gross total tumor resection through a subfrontal approach. Intraoperatively, meticulous microsurgical dissection was performed around the vascular structures of the circle of Willis. No arterial laceration, direct vascular injury, or significant intraoperative bleeding was observed. No evidence of intraoperative vasospasm was noted during the procedure.

The immediate postoperative course was initially uneventful. The patient remained neurologically stable with a Glasgow Coma Scale score of 15 and no focal neurological deficit or visual worsening. No signs of diabetes insipidus or endocrine dysfunction were observed. A postoperative brain computed tomography (CT) scan obtained 48 h after surgery confirmed complete tumor resection without hemorrhagic complication [Figure 2]. Serum electrolytes, including sodium levels, as well as hormonal investigations, were within normal limits.

Figure 2:

Postoperative contrast-enhanced axial computed tomography scans demonstrating gross total resection of the suprasellar craniopharyngioma without immediate postoperative complication.

On postoperative day 6, the patient suddenly developed refractory status epilepticus associated with rapid neurological deterioration. Neurological examination revealed deep coma with bilateral fixed mydriasis. Laboratory investigations showed no metabolic disturbance or electrolyte abnormality that could explain the neurological worsening.

Emergency brain CT demonstrated extensive ischemia involving the left cerebral hemisphere within the middle cerebral artery territory, associated with marked cerebral edema. CT angiography revealed severe narrowing of the A1 + M1 segment of the left middle cerebral artery, producing a “string-like” appearance consistent with malignant cerebral vasospasm [Figure 3]. No postoperative hematoma or other compressive lesion was identified.

Figure 3:

(a and b) Axial computed tomography (CT) scans (red arrows) demonstrating extensive cerebral ischemia. (c) Axial CT angiography and (d) red arrows showing cerebral vasospasm with reduced caliber of the cerebral arteries.

The diagnosis of delayed cerebral ischemia secondary to postoperative cerebral vasospasm was retained. Despite intensive care management and supportive treatment, the neurological condition continued to deteriorate. The patient progressed to brain death and subsequently died.

DISCUSSION

Postoperative cerebral vasospasm following intracranial tumor surgery is a rare but potentially devastating complication that remains underrecognized in clinical practice. Its true incidence is likely underestimated because postoperative vascular imaging is not routinely performed, and early clinical manifestations are often nonspecific or absent. Recent studies suggest that tumors located in the sellar and parasellar regions, particularly pediatric craniopharyngiomas, represent a high-risk setting because of their intimate relationship with the vessels of the circle of Willis.[3,4]

In the present case, no intraoperative arterial injury or visible vasospasm was observed. Rather than excluding the diagnosis, this finding supports the current understanding that delayed cerebral vasospasm may occur despite the absence of overt vascular trauma, suggesting that mechanisms beyond direct arterial injury play a major role in its development.[4,6]

The pathophysiology is multifactorial, involving mechanical, inflammatory, and biochemical factors. Arterial manipulation may induce endothelial irritation, whereas microscopic subarachnoid hemorrhage may trigger a vasoconstrictive cascade. Endothelin-1 release, reduced nitric oxide bioavailability, and the local inflammatory response contribute to prolonged vasoconstriction and impaired cerebral autoregulation.[5,6] Recent evidence further suggests that tumor- and surgery-related inflammatory mediators may contribute to endothelial dysfunction and vascular hyperreactivity following craniopharyngioma resection. In addition, manipulation of the hypothalamic-pituitary region may contribute to autonomic dysregulation of cerebral vascular tone, particularly in pediatric patients, who may be more susceptible because of immature cerebrovascular autoregulation and the close anatomical relationship between suprasellar tumors and the vessels of the circle of Willis.[3,4,6]

The delay in onset, typically between postoperative days 3 and 10, resembles that observed after aneurysmal subarachnoid hemorrhage, suggesting shared pathogenic mechanisms. In our case, vasospasm occurred on postoperative day 6, which is consistent with this temporal profile.[3] Importantly, our patient remained neurologically intact until the abrupt onset of refractory status epilepticus. No prodromal symptoms, including headache, somnolence, fever, focal neurological deficits, electrolyte disturbances, or minor seizures, were observed before postoperative day 6, making earlier clinical suspicion particularly challenging despite the presence of anatomical risk factors.

Risk factors include suprasellar tumor location, arterial manipulation, pediatric age, and, in some cases, electrolyte disturbances. However, our observation confirms that normal serum sodium levels do not exclude the diagnosis.

Clinical presentation is variable and often nonspecific. Delayed cerebral vasospasm may manifest with altered consciousness, focal neurological deficits, seizures, or signs of increased intracranial pressure. In our patient, refractory status epilepticus most likely represented the clinical consequence of extensive malignant cerebral ischemia rather than an isolated epileptic event, reflecting advanced cortical injury secondary to delayed vasospasm.

The diagnosis relies on vascular imaging. In our case, CT angiography demonstrated marked narrowing of the left A1 and M1 segments, confirming cerebral vasospasm. Although transcranial Doppler ultrasonography and near-infrared spectroscopy have been proposed as monitoring tools in selected high-risk patients, their routine use after pediatric craniopharyngioma surgery has not yet been validated because of the rarity of this complication and the lack of standardized surveillance protocols. Nevertheless, these modalities may facilitate earlier diagnosis in carefully selected patients.

Management is largely extrapolated from aneurysmal subarachnoid hemorrhage and includes nimodipine, hemodynamic optimization, and, in selected patients, endovascular therapies. In our patient, neurocritical care management, including nimodipine administration, was initiated immediately after diagnosis. However, by the time brain CT and CT angiography were performed, bilateral fixed mydriasis and extensive malignant cerebral infarction had already developed, suggesting that the therapeutic window for effective intervention had most likely been exceeded. Consequently, endovascular treatment was unlikely to provide meaningful neurological benefit at this advanced stage. Although prophylactic treatment cannot currently be recommended routinely, closer postoperative neurological surveillance and a lower threshold for vascular imaging may be justified in selected high-risk pediatric patients undergoing complex suprasellar surgery.

The prognosis is often poor, particularly when the diagnosis is delayed, as illustrated by our observation. This case highlights the importance of maintaining a high index of suspicion for delayed cerebral vasospasm following pediatric craniopharyngioma surgery. Prompt vascular imaging should be considered in any patient presenting with unexpected neurological deterioration, as early recognition remains the cornerstone for timely therapeutic intervention and may improve clinical outcomes.

A summary of previously reported cases is presented in Table 1.

Table 1:

Summary of reported cases of postoperative cerebral vasospasm following craniopharyngioma surgery.

Highlights

• Delayed cerebral vasospasm is a rare but potentially fatal complication following pediatric craniopharyngioma resection

• Malignant cerebral ischemia may occur despite the absence of intraoperative vascular injury

• Delayed neurological deterioration after skull base tumor surgery should raise suspicion for postoperative vasospasm

• Refractory status epilepticus may represent a presenting manifestation of extensive delayed cerebral ischemia

• Early vascular imaging is essential for prompt diagnosis and timely therapeutic management.

CONCLUSION

Delayed malignant cerebral vasospasm following pediatric craniopharyngioma resection is a rare but potentially fatal postoperative complication that may occur even in the absence of intraoperative vascular injury or early postoperative abnormalities. Sudden neurological deterioration, including seizures or altered consciousness, should promptly raise suspicion for delayed cerebral ischemia. Early vascular imaging is essential for timely diagnosis and therapeutic management. Greater awareness of this rare entity may contribute to earlier recognition and improved outcomes following pediatric skull base surgery.

Ethical approval:

Institutional Review Board approval is not required.

Declaration of patient consent:

Written informed consent for publication of the clinical data and radiological images was obtained from the patient’s legal guardian

Financial support and sponsorship:

Nil.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The author(s) confirms that they have used artificial intelligence (AI)-assisted technology for linguistic editing to improve the clarity and grammatical accuracy of the manuscript.

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