Spheno-Orbital Aneurysmal Bone Cyst in children: a report of 3 cases and literature review.

Abstract

Background: Only 2% to 6% aneurysmal bone cyst (ABC) involve the cranial region, and even fewer show sphenoid and orbital involvement. The spheno-orbital ABC is prone misdiagnosis and can result in intraoperative bleeding and residual lesions. The study was to summarize the clinical and therapeutic characteristics of patients with spheno-orbital ABC in children.

Method: We retrospectively analyzed three childhood-onset spheno-orbital ABC cases at our hospital. A literature review was conducted and the spheno-orbital ABC cases were retrieved. The characteristics of clinical manifestations, treatment outcomes, and follow-ups were analyzed.

Result: All three cases involved the sphenoid and orbital bones, and had a history of surgery or biopsy before being transferred to our hospital. Two of them were terminated due to severe bleeding. The surgeries were performed through frontotemporal craniotomy, where the masses and the surrounding suspected diseased bones were removed. The pathological diagnosis was ABC. All the patient's eye symptoms improved. A review of the literature reveals that ABC typically presents as bone destruction, with some lesions containing fluid levels. Tumors that significantly enhance on contrast-enhanced MRI are often misdiagnosed. ABC is more prevalent in children. The ABC can lead to excessive intraoperative bleeding and residual lesions. Gross total resection is the treatment of choice.

Conclusion: Spheno-orbital ABC should avoid transorbital approach in favor of craniotomy. It is imperative to remove as much of the affected bone as possible and to intensify subsequent monitoring. It is essential to prepare blood prior to surgery to manage potential severe bleeding.