The Journal of Emergency Medicine, Vol. 42, No. 1, pp. e19 – e21, 2012 Copyright © 2012 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$–see front matter
Visual Diagnosis in Emergency Medicine
METASTATIC NEUROBLASTOMA: THE MIMICKER OF BASILAR SKULL FRACTURE IN CHILDREN Antonio E. Muñiz,
MD, FACEP, FAAP, FAAEM
Department of Emergency Medicine and Pediatrics, The University of Texas Health Science Center at Houston, Houston, Texas Reprint Address: Antonio E. Muñiz, MD, FACEP, FAAP, FAAEM, Department of Emergency Medicine and Pediatrics, The University of Texas Health Science Center at Houston, 6431 Fannin Street, JJL-451, Houston, TX 77030
CASE REPORTS Patient 1 A 21-month-old Caucasian boy was seen at another hospital with a chief complaint of mild ecchymosis in the left infraorbital region. The parents denied any known trauma or fall. His examination did not reveal any obvious fracture and he was discharged with a presumed diagnosis of facial contusion from a fall. Three days later he presented with worsening ecchymosis and marked bilateral periorbital swelling. The child’s past medical history was unremarkable. He took no medications and had no allergies. Vital signs were normal. His facial examination showed marked swelling and periorbital ecchymosis with proptosis of both eyes. His ocular movements were decreased (Figure 1). His pupils were 4 mm and reactive. His nasal passages were obstructed with a small mass. He was lethargic but arousable. The remainder of his examination was unremarkable. The computed tomography (CT) scan of his head showed soft tissue mass in the orbital regions pushing the eyes forward and extensive bony erosions (Figures 2 and 3). He was admitted to the pediatric intensive care unit. A biopsy of the tissue revealed neuroblastoma. He was started on chemotherapy, but he developed neutropenic
RECEIVED: 28 February 2009; FINAL ACCEPTED: 23 August 2009
Figure 1. Periorbital ecchymosis and swelling caused by metastatic neuroblastoma.
fever and acute respiratory distress syndrome and after a few days, life support treatment was withdrawn and the patient died.
11 July 2009; e19
A. E. Muñiz
Figure 2. Computed tomography (CT) scan of the head showing extensive tumor invasion of the orbits and extensive bony erosions, as shown by the arrows.
Patient 2 A 30-month-old Caucasian boy presented with worsening periorbital edema and distinctive ecchymosis. The parents denied any known trauma. The child’s past medical history was unremarkable. He took no medications and had no allergies. Vital signs were normal. His facial examination showed marked swelling and very dark periorbital ecchymosis (Figure 4). His pupils were 3 mm and reactive. The remainder of his examination, including his neurologic examination, was unremarkable. He was admitted to the pediatric floor. He had elevated urinary levels of the catecholamine metabolites,
Figure 3. Computed tomography (CT) scan of the head showing extensive tumor invasion of the orbits and extensive bony erosions.
Figure 4. Periorbital ecchymosis and swelling cause by metastatic neuroblastoma.
vanillylmandelic acid (VMA) and homovanillic acid (HVA). An abdominopelvic CT scan revealed an abdominal mass and the biopsy confirmed neuroblastoma. He was started on chemotherapy, but he died of sepsis a few weeks later.
DISCUSSION Periorbital ecchymosis in children is usually caused by trauma to the anterior floor of the skull. Hemorrhage from the fracture infiltrates the periorbital tissues, causing the periorbital ecchymosis. This can either be inflicted or not. However, on occasion a metastatic neuroblastoma can mimic these physical findings (1). Neuroblastoma is the most common extracranial solid tumor in children under the age of 5 years. Metastatic neuroblastoma has a predilection to spread to the orbital spaces, causing periorbital edema and ecchymosis (“raccoon eyes”), which, on occasion, can be initially thought to occur from a basilar skull fracture (1–3). Ophthalmologic manifestations are a result of periorbital soft tissue infiltration of tumor, producing proptosis and periorbital hematoma or ecchymosis (4,5). There are a multitude of different diagnoses that could present with periorbital edema and ecchymosis in children, such as lymphoma, bleeding disorders (hemophilia, thrombocytopenia), druginduced coagulopathy (warfarin, heparin), amyloidosis, Kaposi’s sarcoma, infection of the soft tissue, or myxedema (2,3).
This case is presented to increase awareness of the broad differential diagnosis associated with periorbital ecchymosis and edema, as potential causes include both traumatic and medical etiologies. The clinical evaluation should include appropriate imaging as well as laboratory analysis based on clinical suspicion. Although both accidental and non-accidental trauma could be entertained, our two cases highlight the importance of considering medical causes as well.
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