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Metastatic, secondary, and lacrimal gland tumors

Neuroblastoma and Ewing sarcoma are the tumors that most commonly metastasize to the child’s orbit. Wilms tumor, testicular embryonal sarcoma, ovarian sarcoma, and renal embryonal sarcoma occasionally do so. Rarely, rhabdomyosarcoma may also spread to the orbit from distant sites. It is important to differentiate between blood-borne deposits of a malignant tumor (metastatic disease) and direct extension of a tumor into the orbital tissues from an…

Other mesenchymal abnormalities

Introduction Orbital mesenchyme arises from the neural crest; mesenchymal stem cells develop into a wide variety of tissues, including smooth and skeletal muscle, fat, cartilage, and bone. Orbital mesenchymal lesions constitute about 5% of pediatric orbital disease. Bony lesions are the largest group. Every mesenchymal component of the orbit can give rise to benign (e.g. lipoma, rhabdomyoma, leiomyoma, fibroma, myxoma, angiomyxoma) or malignant tumors (e.g. alveolar…

Orbital rhabdomyosarcoma

General Points Rhabdomyosarcoma (RMS) is a highly malignant neoplasm that can develop in children or adults. This is the most common soft tissue sarcoma of the head and neck in children and represents 5% of childhood cancers. RMS can occur in any anatomic location of the body where there is skeletal muscle, as well as other sites without skeletal muscle, such as the soft tissues of…

Neurogenic tumors of the orbit

Introduction Primary neurogenic tumors of the orbit arise from the optic nerve, from peripheral sensory or motor nerves or from the autonomic ciliary ganglion. Secondary neurogenic tumors include retinoblastomas and intracranial meningiomas that invade directly from contiguous structures or neuroblastomas and peripheral neuroectodermal tumors (Ewing sarcoma) that spread hematogenously; these are discussed in other chapters. Fundamental Science Basic anatomy and origin of primary orbital neurogenic tumors…

Vascular diseases of the orbit

The vascular element of the orbit is an important source of disease in childhood with the potential to cause visual loss. For years, the names and nature of the various vascular anomalies which may affect the orbit were the subject of debate. The creation of the International Society for the Study of Vascular Anomalies (ISSVA), encompassing several relevant specialties, has helped to resolve these issues. Their…

The management of orbital disease in children

Orbital abnormalities in childhood may be congenital or acquired. Congenital developmental abnormalities can be confined to the orbit or part of a more widespread craniofacial malformation. For example, proptosis may be related to shallow or small orbits in the craniosynostoses ( Fig. 19.1 ). The relationship between the orbits may also be disturbed: in hypertelorism the orbits are widely separated; in hypotelorism, they are set close…

The lacrimal system

For video accompanying this chapter see ExpertConsult.com. See inside cover for access details. Introduction The lacrimal system has two components – the secretory and the drainage portions. The secretory component is composed of the lacrimal gland and accessory lacrimal glands of Wolfring and Krause. The lacrimal drainage system is composed of the puncta, canaliculi, lacrimal sac and the nasolacrimal duct. Tears secreted by the major and…

Lids: Congenital and acquired abnormalities − practical management

Introduction For video accompanying this chapter see ExpertConsult.com. See inside cover for access details. This chapter addresses the practical management of congenital and acquired eyelid conditions. The main indications for eyelid surgery in children are to optimize the potential for useful vision in severe congenital malformations, to prevent amblyopia, to control exposure and breakdown of the ocular surface, and to improve cosmesis. These aims are often…

Disorders of the eye as a whole

The ophthalmologist needs to make an exact ocular and systemic diagnosis in patients with eyes that are smaller than normal or those with malformations affecting anterior or posterior structures. A precise diagnosis allows good counseling of patients and families, an educated guess at visual outcomes and screening for possible ocular or systemic complications. Conditions that are associated with a small ocular size include microphthalmia, anophthalmia, nanophthalmos,…

External eye disease and the oculocutaneous disorders

This chapter focuses on the most common external eye conditions in children, which are summarized in Fig. 15.1 . Open full size image Fig. 15.1 Algorithm for classification of conjunctivitis. GVHD, graft-versus-host disease; HSV, herpes simplex virus; HZO, herpes zoster ophthalmicus; SJS/TEN, Stevens–Johnson syndrome/toxic epidermal necrolysis. Blepharokeratoconjunctivitis Blepharitis is a disorder of the lid margins that can be associated with ocular surface inflammation. Lid disease can…

Endophthalmitis

Infectious endophthalmitis occurs when bacteria or fungi enter the eye following a breach of the outer wall of the eye (exogenous endophthalmitis), or when microorganisms enter the eye from a source elsewhere in the body (endogenous endophthalmitis). Inflammation related to parasitic or viral innoculation of the eye is generally classified as uveitis. Exogenous endophthalmitis most frequently arises following surgery but may be a consequence of trauma.…

Preseptal and orbital cellulitis

The diagnosis of infective preseptal and orbital cellulitis is clinical. The goal is to prevent rapid deterioration and serious sequelae such as visual loss, cavernous sinus thrombosis, cerebral abscess, osteomyelitis, and septicemia. Children are at greater risk of these serious complications than adults; however, they are rare if early diagnosis is made and treatment is initiated. It must be managed promptly with appropriate antibiotics and medical…

Conjunctivitis of the newborn

Neonatal conjunctivitis or ophthalmia neonatorum is an inflammation or infection of the conjunctiva occurring within the first month of life. It is the most common infection in the first month of life, with an incidence from 1% to 24%, depending upon geographic region. The three etiologies of neonatal conjunctivitis are chemical, bacterial, and viral. Though some types of conjunctivitis are self-limited and not vision-threatening, others have…

Ocular manifestations of intrauterine infections

Introduction Intrauterine infection is defined as an infection transmitted to the fetus any time during pregnancy, except for the last week (this is a perinatal infection). Intrauterine infections contribute to adverse pregnancy outcomes, such as stillbirth, preterm birth, intrauterine growth restriction and congenital abnormalities. Approximately 2% of live-born infants have an intrauterine infection, which translates to approximately 2.5 million infants per year globally, with the low-…

Genetics and pediatric ophthalmology

Introduction The modern era of genetic ophthalmology began in 1985 with the discovery of the retinoblastoma gene. Exciting progress in deciphering the genetic basis of eye disease has been made since that time ( Fig. 10.1 gives a timeline of historic discoveries). Hundreds of genes associated with a wide variety of common and rare disorders have been discovered leading to clinically valuable diagnostic tests (e.g. for…

Orbit and visual pathway imaging

Introduction Imaging is often pivotal in making a diagnosis in pediatric ophthalmology. We hope to highlight the way the pediatric ophthalmologist and radiologist can form an effective team when dealing with children with orbital and/or visual pathway problems. Pre-imaging multidisciplinary collaboration Effective communication between ophthalmologist and radiologist is crucial to optimizing imaging. This could range from written request to personal conversation. This communication may be very…

Imaging the child’s eye

Introduction In 1850/1851, the German physiologist and physicist Hermann Helmholtz (1821–1894, ennobled 1883) designed the direct ophthalmoscope, enabling the founders of modern ophthalmology to view the living ocular fundus for the first time. New disorders were described, making this instrument essential in establishing the young specialty of ophthalmology. Until ophthalmic photography became available, ophthalmic images were recorded by artists who meticulously drew and painted detailed renditions…

Pediatric visual electrophysiology – objective measurement of visual function

For supplementary materials see . See inside cover for access details. Introduction Visual electrophysiology has an important role in modern pediatric ophthalmology. It offers unique functional measures of each part of the visual pathway, from the retina to the striate cortex. For children objective tests are critical for early clinical diagnosis, prognosis, and monitoring of neurologic and ocular sequelae. Visual electrophysiology findings also inform natural…

Managing refractive errors in children

Introduction Refractive errors cause blurred vision and potentially amblyopia through inability to form a perfectly focused image on the retina. Emmetropia is the state of a perfectly focused eye when the power of the cornea and lens are exactly appropriate for the axial length of the eye ( Fig. 6.1 ); this is uncommon in young children because their eyes have not completely matured, and a…

Examination, history, and special tests in pediatric ophthalmology

A child’s problem will likely be succesfully assessed with a little play, a few key tricks, and, mostly, a reasonable dose of spontaneity and patience. Cookie-cutter or hurried visit plans will rarely yield thorough assessments. Assent and Consent Bonhoeffer said, “The test of the morality of a society is what it does for its children.” As caregivers to children and their families, we have important ethical…