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Glaucoma in Children
PEDIATRIC OPHTHALMOLOGY
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GLAUCOMA IN CHILDREN Rudolph S. Wagner, MD
Glaucoma that occurs during the first 3 years of life is broadly classified as infantile glaucoma. The more commonly used term congenital glaucoma implies that the disease is present at birth, which may be true pathologically in most cases. The clinical manifestations of the disease may not be recognizable until some time after birth, however, so the term infantile glaucoma seems more appropriateY The terms primary and secondary refer to the etiology of the glaucoma as being either isolated to a developmental anomaly of the iridocorneal angle or associated with other ocular or systemic disease, respectively. Juvenile glaucoma refers to an onset of disease after 3 years of age and is more commonly a secondary type of glaucoma. 3o As in adults, glaucoma in children is associated with elevated intraocular pressure and results in damage to the optic nerve head with subsequent loss of visual field and visual acuity.
INFANTILE GLAUCOMA
Primary infantile glaucoma occurs in the absence of any systemic disease or other ocular condition. The incidence of this disease is 1 out of 10,000 live births, but as many as 5% of individuals institutionalized in the past for visual disability in the United States had infantile glaucoma. 14 In recent years, earlier diagnosis and advances in treatment have resulted in cure rates approaching 90%.30 Even Supported in part by a grant from the New Jersey State Federation of Women's Clubs, Evening Membership Division to the Department of Pediatric Ophthalmology, and an unrestricted grant from Research to Prevent Blindness, Inc, New York to the Department of Ophthalmology of the University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, New Jersey.
From the Department of Pediatric Ophthalmology, Children's Hospital of New Jersey; and Department of Ophthalmology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, New Jersey
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Figure 1. A 3-month-old child with bilateral infantile glaucoma. Epiphora, corneal enlargement, and corneal haziness are seen in both eyes.
when the intraocular pressure has been controlled, however, many of these children are left with significant visual loss in one or both eyes. Clinical Signs
The presenting signs and symptoms of infantile glaucoma include epiphora (tearing), blepharospasm (voluntary eyelid closure), photophobia (light sensitivity), corneal enlargement and corneal haziness resulting from corneal edema, and, rarely, cupping of the optic nerve (Fig. 1). There may be injected conjunctival blood vessels associated with these other signs, which may mimic a conjunctivitis or other more common causes of a red eye (Table 1). Parents of children with primary infantile glaucoma most frequently bring their child to the pediatrician because of clinically evident corneal edema?O This may manifest as a diffuse haze of the cornea or as a partial or total corneal opacification. The classic triad of epiphora, blepharospasm, and photophobia may occaSionally precede the enlarged corneal diameter (megalocornea), enlarged globe (buphthalmos), or corneal edema. Light sensitivity and compensatory blepharospasm may be secondary to corneal epithelial breaks or secondary iritis or it may result from glare caused by the corneal edema. Reflex tearing follows the breakdown of the epithelium with resultant irritation.
Table 1. DIFFERENTIAL DIAGNOSIS OF SIGNS AND SYMPTOMS IN PEDIATRIC GLAUCOMA
Tearing Nasolacrimal duct obstruction Corneal abrasion or foreign body Conjunctivitis Ocular inflammation-iritis Corneal Haziness Corneal dystrophies (e.g., congenital hereditary endothelial dystrophy) Mucopolysaccharidosis or other metabolic diseases (e.g., Morquio syndrome, cystinosis) Interstitial keratitis (syphilis or rubella) Obstetric birth trauma (forceps injury) Sclerocornea Congenital infection (HSV, keratitis) Corneal or Ocular Enlargement Congenital megalocornea Congenital myopia \
I I
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Infantile glaucoma rarely presents with excess tearing in the absence of other signs and symptoms. The pediatrician must recognize and distinguish the much more common condition of nasal lacrimal duct obstruction as a cause of epiphora. Although epiphora, photophobia, and blepharospasm are considered the classic symptoms of infantile glaucoma, Seidman et aF6 found that these symptoms are not frequently present at the time of diagnosis. Epiphora and photophobia were each present in approximately half of their cases, and the combination was present in only one third of their cases at the time the disease was first recognized. They found that parents were as likely to notice signs of glaucoma (Le., corneal clouding and enlargement) as they were to notice the symptoms. More than 90% were found to have presented to their initial examining physician with either corneal edema, corneal enlargement, or both. Twenty-one percent of children presented with signs of infantile glaucoma without any history of the classic symptoms (Table 2).26 Ocular enlargement occurs because, unlike an adult, the neonatal globe is distensible. The immaturity of the scleral and corneal collagen permits the increased intraocular pressure to expand the globe. Corneal enlargement from increased intraocular pressure predominantly occurs before the age of 3, but the sclera may be deformable until approximately age 10 (Fig. 2).7 Measurement of the horizontal corneal diameter is helpful diagnostically. The mean corneal diameter is 10 mm at birth and increases to 11.8 mm at 1 year of age. A corneal diameter of more than 12 mm in an infant is suggestive of infantile glaucoma. The sclera also expands slowly secondary to the increased intraocular pressure and the resultant thinning causes an increased visibility of the choroid with a "blue sclera" appearance. As the overall size of the eye increases, a massively enlarged eye or buphthalmos (ox-eye) may result. The axial length of the globe also increases, resulting in myopia and astigmatism. 25 The anteroposterior diameter may be measured directly with A-scan ultrasonography or indirectly with refraction. Anisometropic amblyopia due to unequal refractive errors may result, leading to reduced vision in children whose glaucoma has been controlled. Younger children, espeCially those younger than 3 months of age, are more
Table 2. CLINICAL CHARACTERISTICS OF INFANTS AND CHILDREN WITH PRIMARY INFANTILE GLAUCOMA
Clinical Characteristics Problems noted by parents Tearing Photophobia Tearing and photophobia Corneal haze Corneal enlargement Signs noted by initial physician Corneal haze Corneal/global enlargement Corneal clouding and/or enlargement Bilateral involvement Male/female
Infants and Children (%)
55 41
32 41
32 83 58 92 58 54/46
From Seidman DJ, Nelson LB, Calhoun JH, et al: Signs and symptoms in the presentation of primary infantile glaucoma. Pediatrics 77:399-404, 1986; with permission.
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Figure 2. Infant with bilateral glaucoma and corneal enlargement.
likely to present with a hazy cornea from edema than older children who may have enlargement but no obvious corneal edema. Corneal edema can frequently be detected by gross inspection or by an irregular corneal reflex detected with a pen light or dulled red fundus reflex detected with the ophthalmoscope (Fig. 3). Corneal edema may be slight when the intraocular pressure is gradually elevated. Sudden ruptures of Descemet's membrane, caused by stretching of the cornea beyond 12.5 mm, may result in the acute onset of corneal edema. The breaks or tears in Descemet's membrane are called "Haab's striae" and are visible as horizontal lines crossing the central cornea or following the limbal curve around the central cornea. 15 Primary infantile glaucoma is reported to be bilateral in 58% to 80% of cases. 16,26 Signs of infantile glaucoma are more easily recognized in unilateral cases that allow comparison with the normal eye. In children with symmetric bilateral corneal enlargement, particularly in the absence of visible corneal edema, recognition may be delayed because parents believe the baby's big eyes to be an attractive feature (Fig. 4). The corneal edema may be reversible after reduction of intraocular pressure. Persistence and progression of glaucoma may lead to permanent sequelae such as stromal scarring, chronic stromal edema, and irregular corneal astigmatism. 15 Optic nerve damage defines glaucoma and appears as cupping of the optic nerve head. The normal horizontal cup-to-disc ratio of children is less than 0.2 to 0.3. In patients with infantile glaucoma whose optic nerves can be visualized, the
Figure 3. A 2-month-old child with acute corneal edema in the right eye secondary to infantile glaucoma.
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Figure 4. A 6-month-old child with asymmetric corneal enlargement of the left eye secondary to infantile glaucoma.
cup-to-disc ratio is more than 0.3. Fewer than 2% of nonglaucomatous infants have cup-to-disc ratios larger than 0.3. 23 Less than 10% of normal infants have asymmetry of the cup-to-disc ratio between the eyes. 24 Asymmetry of the cup-todisc ratio therefore represents another important sign of pediatric glaucoma. Cupping develops rapidly in children but may be reversible with reduction of intraocular pressure. Reversal of cupping is usually noted in children less than 1 year of age. Recovery of up to 50% of the initial cup loss has been reported. 20 Visual field loss is characteristic of glaucoma in adults and occurs in pediatric glaucoma. Subjective tests of visual acuity and visual field are limited by the child's ability to report perceived stimuli. Perimetric visual field testing is useful only in children old enough and cooperative enough to test. When detected, optic nerve loss and secondary visual field changes in infantile glaucoma parallel those of adult glaucoma. 24 Visual acuity loss may occur early because of corneal edema and persist because of resultant corneal scarring or amblyopia secondary to induced refractive error. IS Pathogenesis
Primary infantile glaucoma is most likely caused by a developmental anomaly of the iridocorneal angle, so-called trabeculodysgenesis. There is no consensus of opinion regarding the basic pathologic defect. A diaphanous imperforate membrane occluding the chamber angle has been observed intraoperatively and may playa role in the etiology of this condition.5 Various other anomalies in and around the chamber angle have been described. In some cases the iris is inserted anteriorly in the iridocorneal angle. 3 Because the mechanism is not one of acute angle closure, as in narrow angle glaucoma seen in adults, increased intraocular pressure is not caused by systemic medications that dilate the pupil. Epidemiology and Genetics It is estimated that approximately 50% of infantile glaucoma is of the primary type. 14 Twenty-five percent of cases are diagnosed at birth and 80% are diagnosed before 1 year of age. It is noteworthy that the condition can occur in one or both eyes. Ninety percent of cases that present before 3 months of age are bilateral. In older infants, boys are affected twice as frequently as girls. 30 Congenital glaucoma is not inherited as a simple mendelian trait but appears to have a multifactorial inheritance pattern. Sporadic occurrence is observed in approXimately 90% of cases. The unequal distribution of boys being affected almost twice as frequently as girls supports multifactorial or polygenic inheritance pattern. In a family with an affected child, about a 5% chance exists that
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the next sibling will be affected. An affected parent has about a 5% chance of having an affected child.!O,28 Clinical Evaluation
Once a child with suspected glaucoma has been referred to an ophthalmologist, an attempt to measure the intraocular pressure should be made. The normal pressure usually ranges from 10 to 20 mm Hg. Measurement of the intraocular pressure may be difficult in the awake child. Sometimes measurement can be taken with a hand-held applanation tonometer. In most infants, an examination under anesthesia is performed, at which time the intraocular pressure is measured under light sedation because anesthesia-induced alteration of intraocular pressure can be large and cause inaccuracies. Sedation with barbiturates or opiates lowers intraocular pressure, whereas ketamine usually increases it. 34 General anesthetics such as halothane or enflurane are believed to lower intraocular pressure. 22 Chloral hydrate and benzodiazepines have less well-defined effects.34 Evaluation of the corneal clarity, measurement of the corneal diameter, and gonioscopic appearance aid in diagnosing glaucoma. Cupping of the optic nerve mayor may not be observed. Once the diagnosis of infantile glaucoma is made, surgery is usually performed during the same anesthesia. One or both eyes may require surgery. Some surgeons prefer to operate on one eye only and wait to perform surgery on the other eye for a week or two until the first eye heals. This approach may be modified pending the circumstances of the individual case. Management
Surgery is usually indicated for the definitive treatment of primary infantile glaucoma. The goal of surgery is to improve the aqueous outflow from the eye. Treatment should be instituted within hours to days to minimize irreversible damage to the optic nerve and cornea. Medical therapy may be used temporarily before surgery and may help clear the cornea, providing better intraoperative visualization of the iridocorneal angle. The procedures of choice are either a goniotomy or a trabeculotomy. A goniotomy is performed by passing a knife parallel to the iris across the anterior chamber, with visualization aided by a goniolens and operating microscope. A linear incision is made into the trabecular meshwork for approximately 100 deg of angle. 4 A single goniotomy incision effectively controls intraocular pressure in 80% of cases. Reoccurrence of elevated intraocular pressure occurs in approximately 20% to 30% of cases treated with goniotomy.<,9 One limitation of goniotomy is the requirement for a sufficiently clear cornea to provide adequate visualization of the anterior chamber of the eye. Some surgeons, because of personal preference or lack of adequate visualization of the angle because of a corneal haze, may choose to perform a trabeculotomy. In this procedure, a direct opening between Schlemm's canal and the anterior chamber is made using an external approach. 2 Success rates for this procedure are comparable to those for goniotomy.21 If the corneal diameter is more than 14 mm or the corneas are opaque at birth, the success rate of either procedure is lower. Parents must be advised that more than one surgical procedure may be required to control the intraocular pressure in many cases. Other procedures that have been used to treat infantile glaucoma include
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trabeculectomy, Nd:YAG laser treatment, and implantation of a Molteno implant.8 ,27 Resistant cases may require cyclodestructive techniques such as cyclocryotherapy or cyclodiathermy. End-stage glaucoma, in which a child may have a blind painful eye, may require retrobulbar injection of alcohol or even enucleation. Medical therapy may be necessary after surgery to maintain suffiCiently low intraocular pressure. Beta blockers, miotics, and carbonic anhydrase inhibitors are most frequently used. The dose for acetazolamide is 15 mg/kg/ d in three or four divided doses. Prognosis
If left untreated, infantile glaucoma progresses steadily, eventually resulting in blindness. Spontaneous remission has been reported but is extremely rare. 24 The earlier the onset of the glaucoma, the poorer the visual prognosis. If glaucoma is present at birth, over 50% of the eyes will be legally blind, whereas with later onset only 20% of the eyes will be legally blind. Despite being able to control intraocular pressure in 85% of cases, only 35% have visual acuity better than 20/50. Diminished visual acuity may result from optic nerve damage, opacities of the cornea, cataracts, and amblyopia from visual deprivation or anisometropia. Amblyopia is frequently found because unequal refractive errors (anisometropia) occur. Axial myopia is associated with scleral stretching and increased axial length, and irregular astigmatism should be suspected if breaks in Descemet's membrane-so-called Haab's striae-are observed. Correction of refractive errors and patching of the better seeing eye are frequently necessary to avoid or treat amblyopia. Prolonged corneal edema may result in deprivation amblyopia. Significant decrease in visual acuity and peripheral visual field may result. Although the disease is uncommon, successful management to prevent blindness depends on early recognition by the pediatrician of the presenting signs and symptoms. SECONDARY GLAUCOMA IN CHILDREN
Secondary glaucoma in children (Table 3) is associated with structural, hamartomatous, metabolic, inflammatory, mitotic, or other congenital diseases of the eye. These conditions do not have the same underlying pathogenesis as primary infantile glaucoma. It is estimated that approximately 50% of infantile glaucomq is of the secondary type. Almost 78% of childhood glaucoma is secondary when older children are included. 6 ANTERIOR CLEAVAGE SYNDROMES
This group of disorders results from anomalous development of the mesodermal tissue in the eye under the influence of the neural crest cells.33 This results in recognizable structural defects in the anterior segment of the eye, such as a congenital central corneal opacity. Presenting signs include an anterior Schwalbe's line (posterior embryotoxin), iris atrophy, and anterior iris projections to the cornea, as in Axenfeld-Reiger syndrome. Peter's anomaly consists of a central defect in the corneal endothelium with central corneal opacification an~
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Table 3. SECONDARY GLAUCOMA IN CHILDREN Mesodermal Dysgenesis Peter's anomaly Axenfeld-Reiger syndrome Aniridia Connective Tissue Abnormality Marian syndrome Weill-Marchesani Phakomatosis Sturge-Weber syndrome Neurofibromatosis Congenital ectropian uveae Metabolic Abnormality Lowe syndrome Homocystinuria
Mitotic Abnormality Retinoblastoma Medulloepithelioma Juvenile xanthogranuloma Inflammation Rubella Uveitis Trauma-hyphema Congenital Disorders Oculo-dento-osseous dysplasia Rubenstein-Taybi syndrome Stickler syndrome (Pierre Robin sequence) Patau syndrome Down syndrome
iris-corneal adhesions. This condition is associated with glaucoma in approximately 70% of cases (Fig. 5). ANIRIDIA
Aniridia is a bilateral disorder characterized by absent or, more commonly, hypoplastic irides, macular hypoplasia, corneal and lenticular opacities, and glaucoma. Children with this condition are recognized at birth to have a pronounced "red reflex" as visualized with the ophthalmoscope (Fig. 6). They frequently develop nystagmus. An autosomal dominant familial pattern is found in two thirds of cases. An association with a deletion of the short arm of chromosome 11 also has been demonstrated. 19 Thirty percent of sporadic cases of aniridia are associated with Wilms' tumor. This renal tumor usually presents before 3 years of age. Glaucoma has been reported to occur in as many as 75% of cases. The onset of glaucoma is usually in later childhood, although it can occur at any time after birth. 19 Enlargement of the eye and other signs of glaucoma are usually not obvious in this condition because of the later onset of glaucoma. Once the diagnosis of aniridia is made, these children must be followed regularly by an ophthalmologist to monitor intraocular pressure. Glaucoma may be difficult to manage in these cases. OCULO-DENTO-OSSEOUS DYSPLASIA
Children with this condition have characteristic facial abnormalities that include telecanthus, epicanthal folds, and hypotelorism. Their long narrow faces
Figure 5. A 4-year-old child with Peter's anomaly of the right eye manifesting as a central corneal opacity. There is buphthalmos (severe enlargement) of the right eye secondary to glaucoma.
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Figure 6. Right eye of a child with aniridia. Irregular rudimentary iris and pupil are seen. There is also a central anterior cataract. The equator of the lens is easily recognized although the pupil has not been pharmacologically dilated.
and reduced amounts of coarse hair are suggestive of this syndrome. The globe may be small, and microcornea may be present. Anterior iris insertion is believed to underlie infantile onset glaucoma, which is the most common cause of visual loss in these children. 31 PHAKOMATOSES
The Sturge-Weber syndrome, trigeminal-encephalofacial hemangioma, is characterized by nevus flammeus (port wine stain, cavernous hemangioma) and intracranial calcified hemangiomas. Glaucoma has been reported to occur in 30% of cases. Sixty percent of children with glaucoma present within the first 2 years of life, and 40% present in later childhood.ll The glaucoma is usually unilateral and occurs ipsilateral to the skin lesion, particularly if the upper lid is involved (Fig. 7). The eye on the involved side may be larger than the contralateral eye. A
Figure 7. Infant with Sturge-Weber syndrome showing characteristic nevus flammeus on the right side of the face.
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choroidal hemangioma also may be present and is recognized with the ophthalmoscope as a darker red fundus on the involved side. Once this sporadic condition is recognized, ophthalmologic consultation should be sought to measure intraocular pressure. Glaucoma is particularly resistant to medical and surgical therapy in the condition.'2 Neurofibromatosis is characterized by cafe-au-lait spots, neurofibromas of the skin, and optic nerve gliomas. Glaucoma occasionally occurs in this autosomal-dominant disease. It may be secondary to direct tumor involvement of angle structures. Congenital ectropian uveae is an uncommon condition characterized by the presence of iris pigment epithelium on the anterior surface of the iris. This abnormality is usually unilateral and presents with a large irregularly shaped pupil on the involved side. The ectopic pigment on the iris surface simulates an enlarged pupil. This condition may occur in children with neurofibromatosis and it is usually associated with glaucomaY LOWE SYNDROME
Also called the oculo-cerebro-renal syndrome, this X-linked recessive disorder is characterized by aminoaciduria, hypotonia, and hyporeflexia. Sixty percent of these children have glaucoma, and 75% present with bilateral cataracts. 1 MARFAN SYNDROME (ECTOPIA LENTIS)
Marfan syndrome is a connective tissue disease associated with musculoskeletal, cardiovascular, and ocular disorders. Ocular findings in this autosomal dominant condition include ectopia lentis (subluxation of the lens), myopia, retinal detachment, and glaucoma (Fig. 8).'7 Children with homocystinuria may present with findings similar to Marfan disease. Homocystinuria is a receSSively inherited metabolic disease that is characterized by mental retardation, musculoskeletal disorders, elevated urinary homocystine, and bilateral dislocated lenses. Glaucoma is usually the result of dislocation of the lens. These children are prone to forming vascular thrombosis under general anesthesia.
Figure 8. Slit lamp photograph of an eye of a child with Marfan syndrome showing subluxation of the lens.
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STICKLER SYNDROME (PIERRE ROBIN SEQUENCE)
Glaucoma has been reported to occur in children with the Pierre Robin sequence, which includes micrognathia, glossoptosis, and cleft palate. Some of the reported cases were actually Stickler syndrome. In addition to the Pierre Robin sequence, these children have a flat facies, myopia, cataracts, retinal detachment, and spondyloepiphyseal dysplasia. The glaucoma may occur in infancy or any time in childhood. 29 CONGENITAL RUBELLA SYNDROME
Rubella is no longer as frequent a cause of congenital cataracts as it once was. Children with rubella cataracts may develop glaucoma due to abnormal angle development. This type of glaucoma is difficult to manage in many cases. CONGENITAL TUMORS
Iris granulomas may occur in juvenile xanthogranuloma, resulting in a spontaneous hyphema (nontraumatic) and secondary glaucoma. Retinoblastoma may cause glaucoma as tumor fills the anterior chamber. Medulloepithelioma, a ciliary body tumor, may cause glaucoma by direct growth into the iridotrabecular angle. TRAUMA
Glaucoma in children can occur after trauma to the eye. A traumatic hyphema (blood in the anterior chamber) can reduce aqueous humor outflow, resulting in a secondary glaucoma. The glaucoma is usually transient and can be controlled medically. Occasionally a total hyphema fills the entire anterior chamber with blood and requires surgical evacuation of the clot to reduce intraocular pressure. Glaucoma can occur following intraocular surgery. Children who have undergone cataract extraction must be followed at regular intervals for the possible development of glaucoma. UVEITIS
Children with uveitis as occurs in juvenile rheumatoid arthritis may develop glaucoma if the disease is advanced. Synechiae or adhesions between the iris and iridocomeal angle or posteriorly to the lens can result in glaucoma following chronic inflammation. RETINOPATHY OF PREMATURITY
Glaucoma may occur in children with severe retinopathy of prematurity in which there is contracture of the retrolental fibroproliferative tissue. The anterior chamber may be shallow and produce an angle closure glaucoma. Usually these children are blind and respond poorly to conventional medical and surgical therapy for glaucoma.
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SUMMARY
Glaucoma in children is a relatively rare but frequently debilitating disorder. Pediatricians must be aware of the association of glaucoma with certain systemic diseases and congenital ocular abnormalities. Recognition of the signs of infantile glaucoma allows the physician to refer patients for definitive care. Timely therapy is critical to the successful management of children affected with glaucoma. References 1. Abbassi v, Lowe CV, Colcagno PL: Oculo-cerebro-renal syndrome. Am J Dis Child 115:145-168,1986 2. Allen L, Burian HM: Trabeculectomy ab externo. A new glaucoma operation: Techniques and results of experimental surgery. Am J OphthalmoI53:19-26, 1962 3. Anderson DR: The development of the trabecular meshwork and its abnormality in primary infantile glaucoma. Trans Am Ophthalmol Soc 79:458-485,1981 4. Barkan 0: Surgery of congenital glaucoma: Review of 196 eyes operated by goniotomy. Am J OphthalmoI36:1523-1534, 1953 5. Barkan 0: Pathogenesis of congenital glaucoma: Gonioscopic and anatomic observation of the angle of the anterior chamber in the normal eye and in congenital glaucoma. Am J OphthalmoI40:1-11, 1955 6. Barsoum-Homsy M, ChevretteL: Incidence and prognosis of childhood glaucoma. A study of 63 cases. Ophthalmology 102:1331-1336,1984 7. Becker B, Shaffer RN: Diagnosis and Therapy of the Glaucomas. St. Louis, CV Mosby, 1965 8. Billson F, Thomas R, Aylward W: The use of two stage Molteno implants in developmental glaucoma. J Pediatr Ophthalmol Strabismus 26:3-8,1989 9. Broughton WL, Parks MM: An analysis of treatment of congenital glaucoma by goniotomy. Am J OphthalmoI91:566-572, 1981 10. Chew E, Morin JD: Glaucoma in children. Pediatr Clin North Am 30:1043-1060, 1983 11. Cibis GW, Tripathi RC, Tripathi BJ: Glaucoma in Sturge-Weber syndrome. Ophthalmology 91:1061-1069,1984 12. DeLuise VP, Anderson DR: Primary infantile glaucoma (congenital glaucoma). Surv OphthalmoI28:1-19,1983 13. Dowling JL, Albert DM, Nelson LB, et al: Ophthalmology 92:912-921, 1985 14. Duke-Elder S: System of Ophthalmology. Congenital Deformities. St. Louis, CV Mosby, 1969, pp 548-565 15. Hass J: Principles and problems of therapy in congenital glaucoma. Invest Ophthalmol 7:140-146,1968 16. Hoskins HD, Shaffer RN: Evaluation techniques for congenital glaucoma. J Pediatr Ophthalmol Strabismus 8:81-85, 1971 17. Maumenee IH: The eye in the Marfan syndrome. Trans Am Ophthalmol Soc 79:685733, 1981 18. Morin JD, Bryars JH: Causes of loss of vision in congenital glaucoma. Arch Ophthalmol 98:1575-1576,1980 19. Nelson LB, Spaeth GL, Nowinski TS, et al: Aniridia: A review. Surv Ophthalmol 28:621-642,1984 20. Quigley HA: The pathogenesis of reversible cupping in congenital glaucoma. Am J OphthalmoI84:358-370, 1977 21. Quigley HA: Childhood glaucoma: Results with trabeculotomy and study of reversible cupping. Ophthalmology 89:219-226,1982 22. Radtke ND, Cohan BE: Intraocular pressure measurements in the newborn. Am J OphthalmoI78:501-504,1974 23. Richardson KT, Shaffer TN: Optic nerve cupping in congenital glaucoma. Am J OphthalmoI62:507-509,1966 24. Robin AL, Quigley HA, Pollack IP, et al: An analysis of visual acuity, visual fields, and disc cupping in childhood glaucoma. Am J OphthalmoI88:847-858, 1979
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25. Sampaolesi R, Caruso R: Ocular echometry in the diagnosis of congenital glaucoma. Arch OphthalmollOO:574-577, 1982 26. Seidman OJ, Nelson LB, Calhoun JH, et al: Signs and symptoms in the presentation of primary infantile glaucoma. Pediatrics 77:399-404, 1986 27. Senft SH, Tomey KF, Traverso CE: Neodymium-YAG laser goniotomy versus surgical goniotomy: A preliminary study in paired eyes. Arch Ophthalmoll07:1773-1776, 1989 28. Shaffer RN: Genetics and congenital glaucomas. Am J OphthalmoI2:243-247, 1967 29. Smith JL, Cavanaugh JA, Stowe FC: Ocular manifestations of the Pierre Robin syndrome. Arch OphthalmoI63:110-118, 1960 30. Stern JH, Catalano RA: Current status of diagnostic and therapeutic measures in infantile glaucoma. Semin OphthalmoI5:166-175, 1990 31. Traboulisi EI, Parks MM: Glaucoma in oculo-dento-osseous dysplasia. Am J Ophthalmol 109:310-313, 1990 32. Wagner RS, Caputo AR, DelNegro RG, et al: Trabeculectomy with cyclocryotherapy for infantile glaucoma in the Sturge-Weber syndrome. Ann OphthalmoI20:289-295, 1988 33. Waring GO, Rodrigues MM, Laibson PR: Anterior chamber cleavage syndrome. A stepladder classification. Surv Ophthalmol 20:2-27, 1975 34. Whitacre MM, Ellis PP: Outpatient sedation for ocular exam. Surv Ophthalmol 28:643652, 1984
Address reprint requests to Rudolph S. Wagner, MD Department of Pediatric Ophthalmology Children's Hospital of New Jersey 15 South Ninth Street Newark, NJ 07107
0031-3955/93 $0.00 + .20
GLAUCOMA IN CHILDREN Rudolph S. Wagner, MD
Glaucoma that occurs during the first 3 years of life is broadly classified as infantile glaucoma. The more commonly used term congenital glaucoma implies that the disease is present at birth, which may be true pathologically in most cases. The clinical manifestations of the disease may not be recognizable until some time after birth, however, so the term infantile glaucoma seems more appropriateY The terms primary and secondary refer to the etiology of the glaucoma as being either isolated to a developmental anomaly of the iridocorneal angle or associated with other ocular or systemic disease, respectively. Juvenile glaucoma refers to an onset of disease after 3 years of age and is more commonly a secondary type of glaucoma. 3o As in adults, glaucoma in children is associated with elevated intraocular pressure and results in damage to the optic nerve head with subsequent loss of visual field and visual acuity.
INFANTILE GLAUCOMA
Primary infantile glaucoma occurs in the absence of any systemic disease or other ocular condition. The incidence of this disease is 1 out of 10,000 live births, but as many as 5% of individuals institutionalized in the past for visual disability in the United States had infantile glaucoma. 14 In recent years, earlier diagnosis and advances in treatment have resulted in cure rates approaching 90%.30 Even Supported in part by a grant from the New Jersey State Federation of Women's Clubs, Evening Membership Division to the Department of Pediatric Ophthalmology, and an unrestricted grant from Research to Prevent Blindness, Inc, New York to the Department of Ophthalmology of the University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, New Jersey.
From the Department of Pediatric Ophthalmology, Children's Hospital of New Jersey; and Department of Ophthalmology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, New Jersey
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Figure 1. A 3-month-old child with bilateral infantile glaucoma. Epiphora, corneal enlargement, and corneal haziness are seen in both eyes.
when the intraocular pressure has been controlled, however, many of these children are left with significant visual loss in one or both eyes. Clinical Signs
The presenting signs and symptoms of infantile glaucoma include epiphora (tearing), blepharospasm (voluntary eyelid closure), photophobia (light sensitivity), corneal enlargement and corneal haziness resulting from corneal edema, and, rarely, cupping of the optic nerve (Fig. 1). There may be injected conjunctival blood vessels associated with these other signs, which may mimic a conjunctivitis or other more common causes of a red eye (Table 1). Parents of children with primary infantile glaucoma most frequently bring their child to the pediatrician because of clinically evident corneal edema?O This may manifest as a diffuse haze of the cornea or as a partial or total corneal opacification. The classic triad of epiphora, blepharospasm, and photophobia may occaSionally precede the enlarged corneal diameter (megalocornea), enlarged globe (buphthalmos), or corneal edema. Light sensitivity and compensatory blepharospasm may be secondary to corneal epithelial breaks or secondary iritis or it may result from glare caused by the corneal edema. Reflex tearing follows the breakdown of the epithelium with resultant irritation.
Table 1. DIFFERENTIAL DIAGNOSIS OF SIGNS AND SYMPTOMS IN PEDIATRIC GLAUCOMA
Tearing Nasolacrimal duct obstruction Corneal abrasion or foreign body Conjunctivitis Ocular inflammation-iritis Corneal Haziness Corneal dystrophies (e.g., congenital hereditary endothelial dystrophy) Mucopolysaccharidosis or other metabolic diseases (e.g., Morquio syndrome, cystinosis) Interstitial keratitis (syphilis or rubella) Obstetric birth trauma (forceps injury) Sclerocornea Congenital infection (HSV, keratitis) Corneal or Ocular Enlargement Congenital megalocornea Congenital myopia \
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Infantile glaucoma rarely presents with excess tearing in the absence of other signs and symptoms. The pediatrician must recognize and distinguish the much more common condition of nasal lacrimal duct obstruction as a cause of epiphora. Although epiphora, photophobia, and blepharospasm are considered the classic symptoms of infantile glaucoma, Seidman et aF6 found that these symptoms are not frequently present at the time of diagnosis. Epiphora and photophobia were each present in approximately half of their cases, and the combination was present in only one third of their cases at the time the disease was first recognized. They found that parents were as likely to notice signs of glaucoma (Le., corneal clouding and enlargement) as they were to notice the symptoms. More than 90% were found to have presented to their initial examining physician with either corneal edema, corneal enlargement, or both. Twenty-one percent of children presented with signs of infantile glaucoma without any history of the classic symptoms (Table 2).26 Ocular enlargement occurs because, unlike an adult, the neonatal globe is distensible. The immaturity of the scleral and corneal collagen permits the increased intraocular pressure to expand the globe. Corneal enlargement from increased intraocular pressure predominantly occurs before the age of 3, but the sclera may be deformable until approximately age 10 (Fig. 2).7 Measurement of the horizontal corneal diameter is helpful diagnostically. The mean corneal diameter is 10 mm at birth and increases to 11.8 mm at 1 year of age. A corneal diameter of more than 12 mm in an infant is suggestive of infantile glaucoma. The sclera also expands slowly secondary to the increased intraocular pressure and the resultant thinning causes an increased visibility of the choroid with a "blue sclera" appearance. As the overall size of the eye increases, a massively enlarged eye or buphthalmos (ox-eye) may result. The axial length of the globe also increases, resulting in myopia and astigmatism. 25 The anteroposterior diameter may be measured directly with A-scan ultrasonography or indirectly with refraction. Anisometropic amblyopia due to unequal refractive errors may result, leading to reduced vision in children whose glaucoma has been controlled. Younger children, espeCially those younger than 3 months of age, are more
Table 2. CLINICAL CHARACTERISTICS OF INFANTS AND CHILDREN WITH PRIMARY INFANTILE GLAUCOMA
Clinical Characteristics Problems noted by parents Tearing Photophobia Tearing and photophobia Corneal haze Corneal enlargement Signs noted by initial physician Corneal haze Corneal/global enlargement Corneal clouding and/or enlargement Bilateral involvement Male/female
Infants and Children (%)
55 41
32 41
32 83 58 92 58 54/46
From Seidman DJ, Nelson LB, Calhoun JH, et al: Signs and symptoms in the presentation of primary infantile glaucoma. Pediatrics 77:399-404, 1986; with permission.
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Figure 2. Infant with bilateral glaucoma and corneal enlargement.
likely to present with a hazy cornea from edema than older children who may have enlargement but no obvious corneal edema. Corneal edema can frequently be detected by gross inspection or by an irregular corneal reflex detected with a pen light or dulled red fundus reflex detected with the ophthalmoscope (Fig. 3). Corneal edema may be slight when the intraocular pressure is gradually elevated. Sudden ruptures of Descemet's membrane, caused by stretching of the cornea beyond 12.5 mm, may result in the acute onset of corneal edema. The breaks or tears in Descemet's membrane are called "Haab's striae" and are visible as horizontal lines crossing the central cornea or following the limbal curve around the central cornea. 15 Primary infantile glaucoma is reported to be bilateral in 58% to 80% of cases. 16,26 Signs of infantile glaucoma are more easily recognized in unilateral cases that allow comparison with the normal eye. In children with symmetric bilateral corneal enlargement, particularly in the absence of visible corneal edema, recognition may be delayed because parents believe the baby's big eyes to be an attractive feature (Fig. 4). The corneal edema may be reversible after reduction of intraocular pressure. Persistence and progression of glaucoma may lead to permanent sequelae such as stromal scarring, chronic stromal edema, and irregular corneal astigmatism. 15 Optic nerve damage defines glaucoma and appears as cupping of the optic nerve head. The normal horizontal cup-to-disc ratio of children is less than 0.2 to 0.3. In patients with infantile glaucoma whose optic nerves can be visualized, the
Figure 3. A 2-month-old child with acute corneal edema in the right eye secondary to infantile glaucoma.
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Figure 4. A 6-month-old child with asymmetric corneal enlargement of the left eye secondary to infantile glaucoma.
cup-to-disc ratio is more than 0.3. Fewer than 2% of nonglaucomatous infants have cup-to-disc ratios larger than 0.3. 23 Less than 10% of normal infants have asymmetry of the cup-to-disc ratio between the eyes. 24 Asymmetry of the cup-todisc ratio therefore represents another important sign of pediatric glaucoma. Cupping develops rapidly in children but may be reversible with reduction of intraocular pressure. Reversal of cupping is usually noted in children less than 1 year of age. Recovery of up to 50% of the initial cup loss has been reported. 20 Visual field loss is characteristic of glaucoma in adults and occurs in pediatric glaucoma. Subjective tests of visual acuity and visual field are limited by the child's ability to report perceived stimuli. Perimetric visual field testing is useful only in children old enough and cooperative enough to test. When detected, optic nerve loss and secondary visual field changes in infantile glaucoma parallel those of adult glaucoma. 24 Visual acuity loss may occur early because of corneal edema and persist because of resultant corneal scarring or amblyopia secondary to induced refractive error. IS Pathogenesis
Primary infantile glaucoma is most likely caused by a developmental anomaly of the iridocorneal angle, so-called trabeculodysgenesis. There is no consensus of opinion regarding the basic pathologic defect. A diaphanous imperforate membrane occluding the chamber angle has been observed intraoperatively and may playa role in the etiology of this condition.5 Various other anomalies in and around the chamber angle have been described. In some cases the iris is inserted anteriorly in the iridocorneal angle. 3 Because the mechanism is not one of acute angle closure, as in narrow angle glaucoma seen in adults, increased intraocular pressure is not caused by systemic medications that dilate the pupil. Epidemiology and Genetics It is estimated that approximately 50% of infantile glaucoma is of the primary type. 14 Twenty-five percent of cases are diagnosed at birth and 80% are diagnosed before 1 year of age. It is noteworthy that the condition can occur in one or both eyes. Ninety percent of cases that present before 3 months of age are bilateral. In older infants, boys are affected twice as frequently as girls. 30 Congenital glaucoma is not inherited as a simple mendelian trait but appears to have a multifactorial inheritance pattern. Sporadic occurrence is observed in approXimately 90% of cases. The unequal distribution of boys being affected almost twice as frequently as girls supports multifactorial or polygenic inheritance pattern. In a family with an affected child, about a 5% chance exists that
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the next sibling will be affected. An affected parent has about a 5% chance of having an affected child.!O,28 Clinical Evaluation
Once a child with suspected glaucoma has been referred to an ophthalmologist, an attempt to measure the intraocular pressure should be made. The normal pressure usually ranges from 10 to 20 mm Hg. Measurement of the intraocular pressure may be difficult in the awake child. Sometimes measurement can be taken with a hand-held applanation tonometer. In most infants, an examination under anesthesia is performed, at which time the intraocular pressure is measured under light sedation because anesthesia-induced alteration of intraocular pressure can be large and cause inaccuracies. Sedation with barbiturates or opiates lowers intraocular pressure, whereas ketamine usually increases it. 34 General anesthetics such as halothane or enflurane are believed to lower intraocular pressure. 22 Chloral hydrate and benzodiazepines have less well-defined effects.34 Evaluation of the corneal clarity, measurement of the corneal diameter, and gonioscopic appearance aid in diagnosing glaucoma. Cupping of the optic nerve mayor may not be observed. Once the diagnosis of infantile glaucoma is made, surgery is usually performed during the same anesthesia. One or both eyes may require surgery. Some surgeons prefer to operate on one eye only and wait to perform surgery on the other eye for a week or two until the first eye heals. This approach may be modified pending the circumstances of the individual case. Management
Surgery is usually indicated for the definitive treatment of primary infantile glaucoma. The goal of surgery is to improve the aqueous outflow from the eye. Treatment should be instituted within hours to days to minimize irreversible damage to the optic nerve and cornea. Medical therapy may be used temporarily before surgery and may help clear the cornea, providing better intraoperative visualization of the iridocorneal angle. The procedures of choice are either a goniotomy or a trabeculotomy. A goniotomy is performed by passing a knife parallel to the iris across the anterior chamber, with visualization aided by a goniolens and operating microscope. A linear incision is made into the trabecular meshwork for approximately 100 deg of angle. 4 A single goniotomy incision effectively controls intraocular pressure in 80% of cases. Reoccurrence of elevated intraocular pressure occurs in approximately 20% to 30% of cases treated with goniotomy.<,9 One limitation of goniotomy is the requirement for a sufficiently clear cornea to provide adequate visualization of the anterior chamber of the eye. Some surgeons, because of personal preference or lack of adequate visualization of the angle because of a corneal haze, may choose to perform a trabeculotomy. In this procedure, a direct opening between Schlemm's canal and the anterior chamber is made using an external approach. 2 Success rates for this procedure are comparable to those for goniotomy.21 If the corneal diameter is more than 14 mm or the corneas are opaque at birth, the success rate of either procedure is lower. Parents must be advised that more than one surgical procedure may be required to control the intraocular pressure in many cases. Other procedures that have been used to treat infantile glaucoma include
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trabeculectomy, Nd:YAG laser treatment, and implantation of a Molteno implant.8 ,27 Resistant cases may require cyclodestructive techniques such as cyclocryotherapy or cyclodiathermy. End-stage glaucoma, in which a child may have a blind painful eye, may require retrobulbar injection of alcohol or even enucleation. Medical therapy may be necessary after surgery to maintain suffiCiently low intraocular pressure. Beta blockers, miotics, and carbonic anhydrase inhibitors are most frequently used. The dose for acetazolamide is 15 mg/kg/ d in three or four divided doses. Prognosis
If left untreated, infantile glaucoma progresses steadily, eventually resulting in blindness. Spontaneous remission has been reported but is extremely rare. 24 The earlier the onset of the glaucoma, the poorer the visual prognosis. If glaucoma is present at birth, over 50% of the eyes will be legally blind, whereas with later onset only 20% of the eyes will be legally blind. Despite being able to control intraocular pressure in 85% of cases, only 35% have visual acuity better than 20/50. Diminished visual acuity may result from optic nerve damage, opacities of the cornea, cataracts, and amblyopia from visual deprivation or anisometropia. Amblyopia is frequently found because unequal refractive errors (anisometropia) occur. Axial myopia is associated with scleral stretching and increased axial length, and irregular astigmatism should be suspected if breaks in Descemet's membrane-so-called Haab's striae-are observed. Correction of refractive errors and patching of the better seeing eye are frequently necessary to avoid or treat amblyopia. Prolonged corneal edema may result in deprivation amblyopia. Significant decrease in visual acuity and peripheral visual field may result. Although the disease is uncommon, successful management to prevent blindness depends on early recognition by the pediatrician of the presenting signs and symptoms. SECONDARY GLAUCOMA IN CHILDREN
Secondary glaucoma in children (Table 3) is associated with structural, hamartomatous, metabolic, inflammatory, mitotic, or other congenital diseases of the eye. These conditions do not have the same underlying pathogenesis as primary infantile glaucoma. It is estimated that approximately 50% of infantile glaucomq is of the secondary type. Almost 78% of childhood glaucoma is secondary when older children are included. 6 ANTERIOR CLEAVAGE SYNDROMES
This group of disorders results from anomalous development of the mesodermal tissue in the eye under the influence of the neural crest cells.33 This results in recognizable structural defects in the anterior segment of the eye, such as a congenital central corneal opacity. Presenting signs include an anterior Schwalbe's line (posterior embryotoxin), iris atrophy, and anterior iris projections to the cornea, as in Axenfeld-Reiger syndrome. Peter's anomaly consists of a central defect in the corneal endothelium with central corneal opacification an~
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Table 3. SECONDARY GLAUCOMA IN CHILDREN Mesodermal Dysgenesis Peter's anomaly Axenfeld-Reiger syndrome Aniridia Connective Tissue Abnormality Marian syndrome Weill-Marchesani Phakomatosis Sturge-Weber syndrome Neurofibromatosis Congenital ectropian uveae Metabolic Abnormality Lowe syndrome Homocystinuria
Mitotic Abnormality Retinoblastoma Medulloepithelioma Juvenile xanthogranuloma Inflammation Rubella Uveitis Trauma-hyphema Congenital Disorders Oculo-dento-osseous dysplasia Rubenstein-Taybi syndrome Stickler syndrome (Pierre Robin sequence) Patau syndrome Down syndrome
iris-corneal adhesions. This condition is associated with glaucoma in approximately 70% of cases (Fig. 5). ANIRIDIA
Aniridia is a bilateral disorder characterized by absent or, more commonly, hypoplastic irides, macular hypoplasia, corneal and lenticular opacities, and glaucoma. Children with this condition are recognized at birth to have a pronounced "red reflex" as visualized with the ophthalmoscope (Fig. 6). They frequently develop nystagmus. An autosomal dominant familial pattern is found in two thirds of cases. An association with a deletion of the short arm of chromosome 11 also has been demonstrated. 19 Thirty percent of sporadic cases of aniridia are associated with Wilms' tumor. This renal tumor usually presents before 3 years of age. Glaucoma has been reported to occur in as many as 75% of cases. The onset of glaucoma is usually in later childhood, although it can occur at any time after birth. 19 Enlargement of the eye and other signs of glaucoma are usually not obvious in this condition because of the later onset of glaucoma. Once the diagnosis of aniridia is made, these children must be followed regularly by an ophthalmologist to monitor intraocular pressure. Glaucoma may be difficult to manage in these cases. OCULO-DENTO-OSSEOUS DYSPLASIA
Children with this condition have characteristic facial abnormalities that include telecanthus, epicanthal folds, and hypotelorism. Their long narrow faces
Figure 5. A 4-year-old child with Peter's anomaly of the right eye manifesting as a central corneal opacity. There is buphthalmos (severe enlargement) of the right eye secondary to glaucoma.
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Figure 6. Right eye of a child with aniridia. Irregular rudimentary iris and pupil are seen. There is also a central anterior cataract. The equator of the lens is easily recognized although the pupil has not been pharmacologically dilated.
and reduced amounts of coarse hair are suggestive of this syndrome. The globe may be small, and microcornea may be present. Anterior iris insertion is believed to underlie infantile onset glaucoma, which is the most common cause of visual loss in these children. 31 PHAKOMATOSES
The Sturge-Weber syndrome, trigeminal-encephalofacial hemangioma, is characterized by nevus flammeus (port wine stain, cavernous hemangioma) and intracranial calcified hemangiomas. Glaucoma has been reported to occur in 30% of cases. Sixty percent of children with glaucoma present within the first 2 years of life, and 40% present in later childhood.ll The glaucoma is usually unilateral and occurs ipsilateral to the skin lesion, particularly if the upper lid is involved (Fig. 7). The eye on the involved side may be larger than the contralateral eye. A
Figure 7. Infant with Sturge-Weber syndrome showing characteristic nevus flammeus on the right side of the face.
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choroidal hemangioma also may be present and is recognized with the ophthalmoscope as a darker red fundus on the involved side. Once this sporadic condition is recognized, ophthalmologic consultation should be sought to measure intraocular pressure. Glaucoma is particularly resistant to medical and surgical therapy in the condition.'2 Neurofibromatosis is characterized by cafe-au-lait spots, neurofibromas of the skin, and optic nerve gliomas. Glaucoma occasionally occurs in this autosomal-dominant disease. It may be secondary to direct tumor involvement of angle structures. Congenital ectropian uveae is an uncommon condition characterized by the presence of iris pigment epithelium on the anterior surface of the iris. This abnormality is usually unilateral and presents with a large irregularly shaped pupil on the involved side. The ectopic pigment on the iris surface simulates an enlarged pupil. This condition may occur in children with neurofibromatosis and it is usually associated with glaucomaY LOWE SYNDROME
Also called the oculo-cerebro-renal syndrome, this X-linked recessive disorder is characterized by aminoaciduria, hypotonia, and hyporeflexia. Sixty percent of these children have glaucoma, and 75% present with bilateral cataracts. 1 MARFAN SYNDROME (ECTOPIA LENTIS)
Marfan syndrome is a connective tissue disease associated with musculoskeletal, cardiovascular, and ocular disorders. Ocular findings in this autosomal dominant condition include ectopia lentis (subluxation of the lens), myopia, retinal detachment, and glaucoma (Fig. 8).'7 Children with homocystinuria may present with findings similar to Marfan disease. Homocystinuria is a receSSively inherited metabolic disease that is characterized by mental retardation, musculoskeletal disorders, elevated urinary homocystine, and bilateral dislocated lenses. Glaucoma is usually the result of dislocation of the lens. These children are prone to forming vascular thrombosis under general anesthesia.
Figure 8. Slit lamp photograph of an eye of a child with Marfan syndrome showing subluxation of the lens.
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STICKLER SYNDROME (PIERRE ROBIN SEQUENCE)
Glaucoma has been reported to occur in children with the Pierre Robin sequence, which includes micrognathia, glossoptosis, and cleft palate. Some of the reported cases were actually Stickler syndrome. In addition to the Pierre Robin sequence, these children have a flat facies, myopia, cataracts, retinal detachment, and spondyloepiphyseal dysplasia. The glaucoma may occur in infancy or any time in childhood. 29 CONGENITAL RUBELLA SYNDROME
Rubella is no longer as frequent a cause of congenital cataracts as it once was. Children with rubella cataracts may develop glaucoma due to abnormal angle development. This type of glaucoma is difficult to manage in many cases. CONGENITAL TUMORS
Iris granulomas may occur in juvenile xanthogranuloma, resulting in a spontaneous hyphema (nontraumatic) and secondary glaucoma. Retinoblastoma may cause glaucoma as tumor fills the anterior chamber. Medulloepithelioma, a ciliary body tumor, may cause glaucoma by direct growth into the iridotrabecular angle. TRAUMA
Glaucoma in children can occur after trauma to the eye. A traumatic hyphema (blood in the anterior chamber) can reduce aqueous humor outflow, resulting in a secondary glaucoma. The glaucoma is usually transient and can be controlled medically. Occasionally a total hyphema fills the entire anterior chamber with blood and requires surgical evacuation of the clot to reduce intraocular pressure. Glaucoma can occur following intraocular surgery. Children who have undergone cataract extraction must be followed at regular intervals for the possible development of glaucoma. UVEITIS
Children with uveitis as occurs in juvenile rheumatoid arthritis may develop glaucoma if the disease is advanced. Synechiae or adhesions between the iris and iridocomeal angle or posteriorly to the lens can result in glaucoma following chronic inflammation. RETINOPATHY OF PREMATURITY
Glaucoma may occur in children with severe retinopathy of prematurity in which there is contracture of the retrolental fibroproliferative tissue. The anterior chamber may be shallow and produce an angle closure glaucoma. Usually these children are blind and respond poorly to conventional medical and surgical therapy for glaucoma.
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SUMMARY
Glaucoma in children is a relatively rare but frequently debilitating disorder. Pediatricians must be aware of the association of glaucoma with certain systemic diseases and congenital ocular abnormalities. Recognition of the signs of infantile glaucoma allows the physician to refer patients for definitive care. Timely therapy is critical to the successful management of children affected with glaucoma. References 1. Abbassi v, Lowe CV, Colcagno PL: Oculo-cerebro-renal syndrome. Am J Dis Child 115:145-168,1986 2. Allen L, Burian HM: Trabeculectomy ab externo. A new glaucoma operation: Techniques and results of experimental surgery. Am J OphthalmoI53:19-26, 1962 3. Anderson DR: The development of the trabecular meshwork and its abnormality in primary infantile glaucoma. Trans Am Ophthalmol Soc 79:458-485,1981 4. Barkan 0: Surgery of congenital glaucoma: Review of 196 eyes operated by goniotomy. Am J OphthalmoI36:1523-1534, 1953 5. Barkan 0: Pathogenesis of congenital glaucoma: Gonioscopic and anatomic observation of the angle of the anterior chamber in the normal eye and in congenital glaucoma. Am J OphthalmoI40:1-11, 1955 6. Barsoum-Homsy M, ChevretteL: Incidence and prognosis of childhood glaucoma. A study of 63 cases. Ophthalmology 102:1331-1336,1984 7. Becker B, Shaffer RN: Diagnosis and Therapy of the Glaucomas. St. Louis, CV Mosby, 1965 8. Billson F, Thomas R, Aylward W: The use of two stage Molteno implants in developmental glaucoma. J Pediatr Ophthalmol Strabismus 26:3-8,1989 9. Broughton WL, Parks MM: An analysis of treatment of congenital glaucoma by goniotomy. Am J OphthalmoI91:566-572, 1981 10. Chew E, Morin JD: Glaucoma in children. Pediatr Clin North Am 30:1043-1060, 1983 11. Cibis GW, Tripathi RC, Tripathi BJ: Glaucoma in Sturge-Weber syndrome. Ophthalmology 91:1061-1069,1984 12. DeLuise VP, Anderson DR: Primary infantile glaucoma (congenital glaucoma). Surv OphthalmoI28:1-19,1983 13. Dowling JL, Albert DM, Nelson LB, et al: Ophthalmology 92:912-921, 1985 14. Duke-Elder S: System of Ophthalmology. Congenital Deformities. St. Louis, CV Mosby, 1969, pp 548-565 15. Hass J: Principles and problems of therapy in congenital glaucoma. Invest Ophthalmol 7:140-146,1968 16. Hoskins HD, Shaffer RN: Evaluation techniques for congenital glaucoma. J Pediatr Ophthalmol Strabismus 8:81-85, 1971 17. Maumenee IH: The eye in the Marfan syndrome. Trans Am Ophthalmol Soc 79:685733, 1981 18. Morin JD, Bryars JH: Causes of loss of vision in congenital glaucoma. Arch Ophthalmol 98:1575-1576,1980 19. Nelson LB, Spaeth GL, Nowinski TS, et al: Aniridia: A review. Surv Ophthalmol 28:621-642,1984 20. Quigley HA: The pathogenesis of reversible cupping in congenital glaucoma. Am J OphthalmoI84:358-370, 1977 21. Quigley HA: Childhood glaucoma: Results with trabeculotomy and study of reversible cupping. Ophthalmology 89:219-226,1982 22. Radtke ND, Cohan BE: Intraocular pressure measurements in the newborn. Am J OphthalmoI78:501-504,1974 23. Richardson KT, Shaffer TN: Optic nerve cupping in congenital glaucoma. Am J OphthalmoI62:507-509,1966 24. Robin AL, Quigley HA, Pollack IP, et al: An analysis of visual acuity, visual fields, and disc cupping in childhood glaucoma. Am J OphthalmoI88:847-858, 1979
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25. Sampaolesi R, Caruso R: Ocular echometry in the diagnosis of congenital glaucoma. Arch OphthalmollOO:574-577, 1982 26. Seidman OJ, Nelson LB, Calhoun JH, et al: Signs and symptoms in the presentation of primary infantile glaucoma. Pediatrics 77:399-404, 1986 27. Senft SH, Tomey KF, Traverso CE: Neodymium-YAG laser goniotomy versus surgical goniotomy: A preliminary study in paired eyes. Arch Ophthalmoll07:1773-1776, 1989 28. Shaffer RN: Genetics and congenital glaucomas. Am J OphthalmoI2:243-247, 1967 29. Smith JL, Cavanaugh JA, Stowe FC: Ocular manifestations of the Pierre Robin syndrome. Arch OphthalmoI63:110-118, 1960 30. Stern JH, Catalano RA: Current status of diagnostic and therapeutic measures in infantile glaucoma. Semin OphthalmoI5:166-175, 1990 31. Traboulisi EI, Parks MM: Glaucoma in oculo-dento-osseous dysplasia. Am J Ophthalmol 109:310-313, 1990 32. Wagner RS, Caputo AR, DelNegro RG, et al: Trabeculectomy with cyclocryotherapy for infantile glaucoma in the Sturge-Weber syndrome. Ann OphthalmoI20:289-295, 1988 33. Waring GO, Rodrigues MM, Laibson PR: Anterior chamber cleavage syndrome. A stepladder classification. Surv Ophthalmol 20:2-27, 1975 34. Whitacre MM, Ellis PP: Outpatient sedation for ocular exam. Surv Ophthalmol 28:643652, 1984
Address reprint requests to Rudolph S. Wagner, MD Department of Pediatric Ophthalmology Children's Hospital of New Jersey 15 South Ninth Street Newark, NJ 07107