Ординатура / Офтальмология / Английские материалы / Pediatric Ophthalmology Current Thought and A Practical Guide_Wilson, Saunders, Trivedi_2008

to goniotomy, perhaps because of preexisting damage to the outflow system from chronically high IOP.

24.8.2.2Trabeculotomy ab Externo

This procedure does not rely on a gonioscopic view, but instead involves the identification of Schlemm’s canal in the bed of a partial-thickness scleral flap

(usually via careful radial incision); the canal is then cannulated and opened from the outside inward, tearing through the poorly functioning trabecular meshwork in that area [20, 108]. While standard trabeculotomy uses a stiff curved metal trabeculotome to tear through the inner wall of Schlemm’s canal, so-called suturetrabeculotomyutilizesa6-0Prolenesuturethat is pulled taut after being threaded into Schlemm’s canal for 180or 360-degrees, opening the respective angle. Both goniotomy and trabeculotomy produce excellent results in selected cases of infant-onset PCG, with neither proven superior in a randomized, controlled trial. Advantages to trabeculotomy include its similarity to trabeculectomy for surgeons comfortable with the prior procedure, and ability to perform the surgery in the absence of an angle view. Disadvantages include the need to incise conjunctiva and sclera, and the possibility of being unable to locate or cannulate Schlemm’s canal.

24.8.2.3Combined Trabeculotomy– Trabeculectomy

Combined trabeculotomy and trabeculectomy has been advocated in cases resistant to goniotomy, or in selected populations where birth presentation, severely opaque corneas, and poor prior success with primary trabeculotomy have been reported. Excellent success has been reported with this technique in selected cases [62, 63].

24.8.2.4Trabeculectomy (Filtering Surgery)

Trabeculectomy promotes filtration of aqueous humor by removing a segment of the angle tissue under a partial thickness scleral flap, creating a filtering

“bleb.” Usually reserved for cases where angle sur-

Fig. 24.14  Trabeculectomy in an eye with Axenfeld-

Rieger glaucoma (same eye shown in Fig. 24.8). This partly avascular bleb was achieved in a 16-year-old boy with intraand postoperative 5-fluorouracil injections, and has controlled glaucoma throughout 6 years of follow-up

gery has already failed or seems unlikely to succeed (e.g., many secondary glaucomas), simple trabeculectomy has a low success rate in children due to their healing response. Most pediatric glaucoma surgeons use antifibrotic therapy (usually intraoperative mitomycin C) and recently have advocated a fornix-based conjunctival flap superiorly, citing better bleb morphology and hopefully reduced risk of postoperative bleb infection [126]. Intraoperative 5-fluorouracil and beta irradiation have also been used with some success (Fig. 24.14) [67]. Trabeculectomy has a low success rate in infants younger than 2 years of age, and in aphakic eyes [12, 40, 112]. All children are at risk for bleb scarring and failure, and also for serious bleb-related infection, with the latter risk likely cumulative over time. Alternatives to mitomycin-enhanced trabeculectomy should be considered especially in infants, aphakic eyes, and children at high risk for inadequate infection precautions (see Sect. 24.8.2.5).

24.8.2.5Aqueous Drainage Device (Tube Shunt) Surgery

Initially reserved for end-stage cases, aqueous drainage device surgery has gained wider acceptance for pediatric glaucoma cases resistant to angle surgery, and having failed or not appropriate for trabeculectomy surgery. This surgery involves the placement of a flexible tube into the eye, which conducts aqueous

humor posteriorly to a reservoir (plate) sewn against the sclera, the latter becoming encapsulated to form a bleb from which trapped fluid exits into surrounding tissues. Several implant types have been widely implanted into eyes of children, including the Molteno, Baerveldt, and Ahmed glaucoma devices. Reported success and complications rates vary widely [25, 35, 39, 70, 71, 82, 83]. Less severe but more common problems include tube malposition or blockage, pupil abnormalities, cataract, motility disturbance, and encapsulation with elevation of IOP, while more severe complications such as retinal detachment (usually limited to aphakic eyes), epithelial downgrowth, and infection are fortunately fairly uncommon (Figs. 24.15,

24.16). The final IOP achieved after drainage implant surgery is not as low as after successful filtering surgery, and at least 50% of cases require continued adjunctive medication. Overall success after aqueous drainage device surgery in refractory PCG and aphakic glaucoma, approximates 75% at 2 years, but falls to ~50% after more than 5 years; success may vary with patient age and glaucoma diagnosis. In one reported series, drainage implant surgery appeared more successful at IOP control than did trabeculectomy, for children below the age of 2 years [11]. The specific devices and technique used to implant aqueous drainage devices in pediatric glaucoma cases should be adapted to the size of the eye, immediacy

of the need for IOP reduction, and glaucoma type.

For example, pars plana tube placement and complete vitrectomy may be needed for aqueous drainage device implantation in aphakic eyes.

24.8.2.6Cycloablation

Cyclodestructive procedures aim to reduce IOP by damaging the ciliary processes and thereby the eye’s ability to produce aqueous humor; results are often unpredictable, and complications frequent. In cases refractory to medical and other surgical interventions, cycloablation constitutes a valid means of attempting control of otherwise vision-threatening glaucoma in children. This procedure may be especially helpful as an adjunct after aqueous drainage device placement with inadequate IOP reduction.

Cyclocryotherapy

Cyclocryotherapy(freezingtheciliaryprocessesfrom an external approach), the oldest cycloablative procedure, should be reserved for extremely severe cases of glaucoma where altered anatomy makes laser cycloablation unlikely to succeed. This procedure has limited success, requires repeat sessions, and has a

Fig. 24.15  This 8-year-old child had aqueous drainage device surgery in his eye at the age of 3 months for newborn-onset PCG. Vision is 20/60 with good IOP control off medications. Despite the fairly anterior tube position in the chamber, there is evident pupil distortion and focal cataract underlying the tube; these changes developed over several years after surgery

Fig. 24.16  This 11-year-old girl developed a huge, encapsulated bleb over the Ahmed aqueous drainage device which was placed in this eye with severe glaucoma after penetrating trauma and several prior related surgeries. Revision was needed due to the unacceptable resultant strabismus

significant risk of devastating complications (~15%); it should not be applied to more than 180 degrees in a single treatment (6–7 freezes of −80°C for 45–60 s) [4, 119].

Transscleral Laser Cyclophotocoagulation

Both the Nd:YAG sapphire probe and the diode laser G-probe have been used for transscleral cycloablation in children. Success has been reported at ~50% (including many retreatments), with less pain and inflammation than produced with cryotherapy, and a lower incidence of phthisis/severe complications. Problems include loss of effect over time, and inaccurate transscleral application of the laser energy to the ciliary processes [15, 55, 91].

Endoscopic Laser Cyclophotocoagulation

Endoscopic cyclophotocoagulation has the advantage of allowing direct application of laser energy to the ciliary processes with a 20-gauge probe (Microprobe; Endo Optiks, Little Silver, NJ, USA), and perhaps producing less inflammation than a transsclerally applied laser. This procedure has limited reported success, with retreatment often needed. Success of endoscopic diode cycloablation nonetheless is modest (43% in a reported series of 36 mostly aphakic eyes, after a mean cumulative arc of treatment of 260 degrees, with short mean follow-up time 19 months), and complications included retinal detachment, hypotony, and visual loss [79].

Take Home Pearls

•Infants with glaucoma often present for evaluation because the family or primary care provider has noticed signs (corneal opacity or enlargement, or tearing) or symptoms (photophobia) related to expansion of the eye under elevated pressure.

•Consider every child with myopia, optic nerve cupping, and conditions associated with glaucoma to have glaucoma until proven otherwise – check the pressure!

•Newer technologies can assist in the evaluation of the pediatric glaucoma suspect, but beware “adjusting” the measured intraocular pressure based on unusually high or low central corneal thickness. Use all available data, and if in doubt, diagnose “glaucoma suspect” and keep under surveillance.

•Most children with primary congenital glaucoma retain useful vision if diagnosed and treated early, but long-term follow-up is critical to maximizing vision and maintaining glaucoma control over time.

•All eyes are at risk for glaucoma after removal of childhood cataracts, and the risk increases over time, so life-long surveillance is needed.

•Surgery for childhood glaucoma, although sharing features with adult glaucoma surgery, is sufficiently different that it should be undertaken by a surgeon with expertise

and interest in pediatric glaucoma.

•It takes a village – optimal care of the child with glaucoma requires a team effort, with members often including at least one ophthalmologist, as well as the family and child, primary care physician, school, and community members. Much improvement remains to be made in the

diagnosis and care of these special children.

24.9Long-term Follow-up

of Children with Glaucoma

Every child with glaucoma must have periodic fol- low-up over his or her entire lifetime. Despite initial successful surgery or medical therapy, the glaucomatous eye may demonstrate asymptomatic loss of IOP control months or even decades later. Other progressive changes such as cataract or corneal decompensation may likewise occur many years after initial presentation of glaucoma, even in eyes with continued adequate IOP control. The target pressure must be continuously reevaluated, especially if progressive visual field defects or optic nerve changes occur despite previously accepted IOP levels. Eyes with functioning filtering blebs or aqueous drainage devices must be diligently followed for surgery-re- lated complications. Young children often lose vision from glaucoma despite adequate IOP control, from corneal scarring, anisometropia, and resultant amblyopia. Children with glaucoma that is controlled without medications should be followed at least every 6 months, and young children, or those whose IOP has been controlled for less than 2 years, should probably be evaluated at least every 3 or 4 months. In spite of timely diagnosis and optimal treatment, many children with childhood glaucomas suffer visual loss from their disease; there is much room for improvement in our current understanding and treatment of these special children.

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Contents

25.4Pathogenesis  . . . . . . . . . . . .   376

25.7Setting Up an Organized Screening

25.7.2Timing of Examinations  . . . . . . . .   379

25.7.3Retinopathy of Prematurity Requiring

Treatment  . . . . . . . . . . . . .   379

25.7.4Cessation of Examinations  . . . . . . .   380

25.7.5Responsibility  . . . . . . . . . . . .   380

25.8Documentation and Communication  . . .   381

25.9Treatment of Acute Phase Disease  . . . .   381

25.10Involution and Monitoring of Infants

After Treatment  . . . . . . . . . . .   381

25.11Treatment of Chronic or Late-stage Disease    382

25.12Prognosis and Comorbidities  . . . . . .   383

25.13Future Screening and Treatment Options  .   383

25.14Medicolegal Considerations  . . . . . .   383 References  . . . . . . . . . . . . . . . . .   384

Core Messages

•Almost seven decades after it was first described, retinopathy of prematurity (ROP) remains an

important threat to pediatric vision.

•Cooperative, multicenter, prospective trials (such as CRYO-ROP and ETROP) have been useful in establishing screening and treatment guidelines for premature infants.

•An organized screening program is vital to timely detection and treatment of ROP to reduce the risk of vision loss.

•Aggressive posterior ROP is an uncommon, rapidly progressing, severe form of ROP that requires special attention.

•Documentation and communication among physicians, staff, and parents are important.

•Future advances in screening and treatment are forthcoming.

25.1 Introduction

The search for a cure for retinopathy of prematurity (ROP) has become a priority among researchers since the disease was first described by Theodore L. Terry in 1942 [68]. ROP is a common disorder, and the risk of visual impairment can be minimized in the vast majority of affected infants with careful management. Despite this, it is estimated that ROP accounts for 19% of cases of pediatric blindness worldwide [29] and is a leading cause of blindness in children attending schools for the blind [63, 66].

Continued interest in understanding and eradicating the disorder has brought about unprecedented collaboration among scientists, ophthalmologists, and public health activists in both developed and developing nations.

25.2 A Brief History

With the introduction of and liberal use of oxygen in the neonatal population in the 1930s, rates of “retrolental fibroplasia” (as ROP was originally called) in premature infants reached epidemic proportions, resulting in blindness or severe visual impairment in thousands of children worldwide [65]. Strict oxygen curtailment subsequently resulted in extensive morbidity and mortality [12]. Oxygen was thereafter liberalized, but monitored, carefully balancing the risk of ROP against other factors. Since ROP generally afflicts only the smallest, sickest infants, it is not surprising that after decades of decline, the incidence of disease again began to rise in the 1970s [31, 44].

This was concurrent with advances in obstetrics and neonatology that permitted the survival of low birth weight premature infants.

In the 1980s the Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) study demonstrated a significant reduction in the rate of unfavorable visual, developmental, and social [49] outcomes in infants randomized to cryotherapy of the avascular retina in eyes with threshold ROP compared to untreated controls [39, 49, 53, 60, 67]. Collaborative spirit fueled further clinical trials, including the Supplemental Therapeutic Oxygen for Prethreshold ROP (STOP-ROP) study [5, 25, 32, 45], which failed to demonstrate a significant benefit to the use of supplemental oxygen therapy when administered to infants

with prethreshold ROP; the Light Reduction in ROP study [4, 61], which showed no benefit to preterm infants from reduction in light exposure from birth to

32 weeks postconceptional age; and the Early Treatment for ROP (ETROP) study [36], which demonstrated that early treatment of high-risk prethreshold disease significantly reduced the risk of unfavorable outcomes and resulted in the modification of previous treatment guidelines. Currently, a cooperative study is being undertaken to evaluate the utility of biochemical cytokine modulators in ROP.

25.3 Risk Factors

Low birth weight and young gestational age are directly correlated with the most severe ROP [17, 41, 69]. Other risk factors that have inconsistently been linked to ROP severity include hypoxia [15, 75], oxygen administration [74, 75], intraventricular hemorrhage [42, 69], surfactant therapy [38], hypotension [64], fungemia [43, 47, 50], sepsis [42], and anemia [23, 28]. The overwhelming impact of both low birth weight and prematurity make establishing the full role of other risk factors difficult.

25.4 Pathogenesis

The relationship between premature birth, oxygen exposure, and ROP that eluded researchers for so long can now be explained on a molecular level, but a full appreciation of the pathogenesis of ROP still requires a basic understanding of ocular embryology and anatomy. Development of the retinal vasculature begins at the optic disc at approximately 16 weeks gestation, progresses relatively circumferentially and anteriorly, and is complete by around 40 weeks. Therefore, the proportion of mature retina at birth is determined by the degree of prematurity at birth. In full-term infants, the retina is generally fully vascularized and ROP cannot occur, but in preterm neonates, especially those born at or before 28 weeks gestation, retinal vascularization is incomplete and the normal process of development is at risk of being interrupted. Exposure to excessive concentrations of oxygen during this period can lead to vascular injury resulting in arrest of vascular development, oblitera-