Spontaneous large subperiosteal orbital hematoma in a patient with neuroblastoma

Abstract

We present a case of spontaneous large subperiosteal orbital hematoma in a 2-year-old boy causing compressive optic neuropathy. On physical examination, he was found to have an abdominal mass. The patient underwent urgent drainage of the subperiosteal orbital hematoma along with a biopsy of the abdominal mass which confirmed the diagnosis of neuroblastoma. Following surgery, proptosis, optic disc swelling, and afferent pupillary defect (APD) improved, and after one month of receiving two doses of chemotherapy, his general condition improved.

INTRODUCTION

Neuroblastoma is the most common solid extracranial malignant tumor in childhood age with 20% of cases associated with orbital metastasis.[1,2,3] It originates from the adrenal gland medulla and para-spinally where there is a sympathetic nervous tissue. It may present with proptosis and periorbital ecchymosis (raccoon sign). Moreover, it may present with secondary Horner syndrome and secondary strabismus.[1] Bone marrow metastasis can cause pancytopenia which leads to subconjunctival hemorrhages. Here, we are presenting a patient with spontaneous large subperiosteal hematoma due to neuroblastoma metastasis.

CASE REPORT

A 2-year-old boy, not known to have prior medical illness, presented with his parents complaining of spontaneous left eye proptosis with mild eyelid redness and on-and-off fever for 1 month. On examination, visual acuity was fix-and-follow in each eye with relative afferent pupillary defect (APD) in the left eye. Right eye examination was within normal limits, but the left eye showed nonaxial proptosis and inferior nasal dystopia with supraduction limitation [Figure 1a]. Fundus examination showed grade II optic disc swelling in the left eye. On general physical examination, he was found to have an abdominal mass. Orbital computed tomography (CT) imaging revealed a large superotemporal subperiosteal collection with minimal lateral orbital wall bony changes [Figure 2a and b]. Abdominal CT showed the left/adrenal gland solid mass [Figure 3]. Complete blood count was normal. The patient underwent urgent drainage of the subperiosteal orbital hematoma due to the presence of compressive optic neuropathy along with a biopsy of the abdominal mass which confirmed the diagnosis of neuroblastoma. Chemotherapy was initiated, which was constituted of topotecan/cyclophosphamide by the oncology team. Following surgery, proptosis, optic disc swelling, and APD improved, and after one month of receiving two doses of chemotherapy, his general condition improved [Figure 1b]. The report adhered to the ethical principles outlined in the Declaration of Helsinki as amended in 2013.

Figure 1.

(a) External photographs revealing proptosis, periorbital erythema, and edema with inferior nasal displacement of the left eye at presentation. (b) Compete resolution of the left eye proptosis a few weeks following drainage of the subperiosteal orbital hematoma and starting of chemotherapy

Figure 2.

(a) Orbital coronal computed tomography imaging with soft-tissue window shows a left large superior temporal subperiosteal collection, (b) Orbital coronal computed tomography imaging with bone tissue shows left mild bony erosion of the lateral orbital wall with sphenoid bone marrow involvement (white arrow)

Figure 3.

Computed tomography abdomen shows the left adrenal gland solid mass (white arrow)

DISCUSSION

According to the International Neuroblastoma Staging System, once the eye/orbit is involved, the patient is considered to have stage-4 disease.[1] The orbital imaging of our patient showed minimal left lateral orbital wall bony changes with sphenoid bone marrow involvement which could be the cause of spontaneous progressive large subperiosteal hematoma over a few weeks. Frunză and Samoilă reported few years ago a case of a 2-year-old girl with an acute left eye proptosis following head trauma. Orbital imaging revealed a subperiosteal hematoma. On further investigation, the patient was found to have a gigantic abdominal-thoracic neuroblastoma.[4] To the best of our knowledge, our patient represents the first reported case in English literature of a spontaneous large subperiosteal hematoma gradually increasing over a few weeks from a metastatic neuroblastoma.

In conclusion, it is wise to include neuroblastoma metastasis to the orbit for patients presenting with large subperiosteal hematoma even with the absence of typically described clinical and radiological presentations.[1]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published, and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.