Child presenting with ocular ecchymosis.

A 10-month-old male child presented in our pediatric ophthalmology clinic with “raccoon eyes,” subconjunctival hemorrhage, proptosis [Figure 1a], and swelling around both ears [Figure 1b]. The mother informed that the infant had been very irritable for 1 week and had developed this symptom recently which has been rapidly progressive.

Figure 1

(a) Ecchymosis of right eye with subconjunctival hemorrhage and proptosis with swelling in front of right ear. (b) Swelling behind left ear

Questions

What is the differential diagnosis?

What are the investigations required?

Answers

Answer 1

Periorbital trauma

Surgery

Neuroblastoma

Rhabdomyosarcoma

Leukemia

Amyloidosis

Multiple myeloma

Vascular malformation (hemangiomas, orbital varices, and lymphangiomas).

Answer 2

Complete blood count, peripheral blood smears, serum electrolytes, coagulation studies, renal and liver function tests, and computed tomography of the orbits.

Investigations

Computed tomography of the head and orbit revealed lytic destruction of bones with hair on end-type periosteal new bone formation and also soft-tissue mass causing mass effect on globe [Figure 2a]. A mass lesion was also seen in left lateral ventricle [Figure 2b], suggesting metastasis from small round cell tumor (neuroblastoma). Abdominal ultrasound showed heteroechoeic soft-tissue mass lesion of size 4.5 cm × 4.13 cm in the left suprarenal region displacing left kidney inferolaterally. The infant had low hemoglobin count of 6 g/dl.

Figure 2

(a) Noncontrast computed tomography head showing large mass infronto-parietal and parieto-occipital region. (b) Noncontrast computed tomographyhead showing mass lesion in left lateral ventricle

Discussion

Ecchymosis presenting to an ophthalmologist could be commonly due to surgery or trauma. However, if these causes are ruled out, it could be due to some serious health conditions such as amyloidosis, multiple myeloma, or neuroblastoma. Neuroblastoma is a childhood malignancy with an incidence of about 9 per million children. Among them, around 10%–20% of children have orbital metastasis.[12] Ophthalmic manifestations of neuroblastoma are well documented and most commonly present as periorbital ecchymosis, subconjunctival hemorrhage, proptosis, mobility defect, ptosis, opsoclonus, and Horner's syndrome. However, ophthalmic manifestation according to the International Neuroblastoma Staging System signifies Stage 4 disease.[3] As ophthalmologists, we should be vigilant and keep all possible etiological factors in differential diagnosis so that there is no delay in diagnosis and management. Treatment involves aggressive chemotherapy followed by attempts to remove the primary tumor. After recovery, patients are started on oral 13-cis-retinoic acid for around 6 months.[4]

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.