Isolated enophthalmos: an uncommon gateway to orbital tumors in pediatrics: 9 month-old female presenting with isolated enophthalmos as the unique sign of a metastatic orbital tumor: a case report

Abstract

Background: If extra-axial proptosis is by far the most common symptom of infantile malignant orbital tumors, enophthalmos is a rare and undocumented sign. We report the first case of a pediatric metastatic orbital tumor revealed by enophthalmos alone.

Case presentation: A 9-month-old girl was diagnosed with isolated right-sided enophthalmos. An orbital tumor was suspected and computed tomography undertaken showing osteolysis and periosteal reaction of orbital walls, malar bones and zygomatic arches. A Thoracic- abdominal CT scan confirmed a stage-4 neuroblastoma.

Conclusion: Enophthalmos can be the sole symptom of an orbital tumor and should lead to immediate imaging assessment. This association is not well known in pediatrics but is relevant to insure the best prognosis.

Figure 1

CT Scan showing enophthalmos of the right eye and the causal neoplastic process. a. CT cross-section showing a backwards displacement of the right eye comparatively to the left eye (Vertical white arrows). The baseline white bar does not sit on the lateral bony wall edge on the right side because of the osteolytic neoplastic process that compromises the integrity of the right zygomatic arch making it seem smaller but thicker and more heterogeneous (Black star, also in Figure  1b). The quality of the baseline bar’s horizontality was ensured by comparing it with the horizontal black double arrow that serves as point of reference. b. CT scan showing irregularly shaped osteolysis of both orbital walls, malar bones and zygomatic arches (Black star on the right side) with periosteal reaction. The orbital cavity seems to be increased on the right side as compared with the left side. The osteolytic process induces cracks and fractures on the inferior wall of the right orbit (White arrows), and the osteocondensation with bone neoformation at the superior wall of the left orbit (white arrow) associated with soft tissue inflammation, denotes less space available for the right globe as compared with the left globe, which could explain the enophthalmos of the right eye.

Figure 2

Thoracic- abdominal CT scan revealing a 83 mm*43 mm*42 mm retroperitoneal calcified heterogeneous mass (White Thick Bar) located on the left adrenal gland and perirenal region evocative of neuroblastoma (Black Arrows).