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Benign Pediatric Orbital Tumors

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Pediatric Oculoplastic Surgery

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Abstract

Tumors of the orbit identified in the pediatric population are relatively rare lesions. Most are congenital hamartomas or choristomas. Of those that develop after birth, the majority are benign. Table 35.1 compiles the experience involving pediatric benign orbital tumors in recent retrospective studies. A total of 1820 lesions are reported in six separate series, representing the largest experience of pediatric orbital disease in print. Incidence varies among the series, but approximately 8–19% of these lesions were malignant (see Chap. 36). The largest group of benign pediatric orbital disorders among these series is infectious and inflammatory disorders, which are dealt with in separate chapters in this text. Structural, vascular, neurogenic, ossifying, and fibrous lesions arising in the pediatric orbit are reviewed in this chapter.

Many of the benign lesions have identifiable external characteristics that enable the examiner to diagnose the lesion based on historical and physical findings. One of the best examples is infantile hemangioma. The cutaneous features of this condition allow for immediate confirmation of its presence and are so rarely missing from the clinical features of the lesion that they are virtually required to confidently diagnose the condition (this lesion is also covered in Chap. 6). Similarly, plexiform neurofibromas have a tactile quality likened to a “bag of worms” that, when coupled with the cutaneous stigmata of neurofibromatosis, permits distinct clinical diagnosis without resorting to other ancillary diagnostic tests. Dermoid cysts have consistent anatomic locations and physical findings, allowing for their rapid identification with a high degree of accuracy. Although all cases are not classic and a differential diagnosis exists for all conditions, these particular entities account for nearly 60% of all pediatric orbital tumors, with the vast majority being diagnosed correctly based on clinical criteria alone. Any lesion of the orbit lacking the typical coloration of capillary hemangiomas, the cutaneous findings of neurofibromatosis, the consistent anatomic positioning of anterior orbital dermoids, or the external inflammation and physical findings of orbital cellulitis must be treated and evaluated in an expeditious manner because such a lesion has approximately a 50% chance of being malignant. In this chapter, we review the clinical findings, biological behavior, imaging features, and recommended treatment for benign tumors of the pediatric orbit.

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Authors and Affiliations

  1. The Children’s Hospital of Philadelphia and The Edwin and Fannie Gray Hall Center for Human Appearance, University of Pennsylvania, Philadelphia, PA, USA

    William R. Katowitz MD

  2. Eye Surgeons Associates, PC, Bettendorf, IA, USA

    Peter D. Fries MD

  3. Ophthalmology & Surgery, Columbia University, New York, NY, USA

    Michael Kazim

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  1. William R. Katowitz MD
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  2. Peter D. Fries MD
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  3. Michael Kazim
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Correspondence to William R. Katowitz MD .

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Editors and Affiliations

  1. Professor Emeritus of Ophthalmology and Director of Oculoplastic and Orbital Surgery, The Children’s Hospital of Philadelphia and The Edwin and Fannie Gray Hall Center for Human Appearance, Perelman School of Medicine at the University of Pennsylvania , Philadelphia, Pennsylvania, USA

    James A. Katowitz

  2. Associate Professor of Clinical Ophthalmology and Director of the Oculoplastic and Orbital Surgery Fellowship Program, The Children’s Hospital of Philadelphia and The Edwin and Fannie Gray Hall Center for Human Appearance, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA

    William R. Katowitz

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Katowitz, W.R., Fries, P.D., Kazim, M. (2018). Benign Pediatric Orbital Tumors. In: Katowitz, J., Katowitz, W. (eds) Pediatric Oculoplastic Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-60814-3_35

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