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

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336

M. Edward Wilson and Rupal H. Trivedi

 

 

response to change of focus. It is unknown whether the fibrosis that often occurs throughout the pediatric lens capsule after surgery would influence the IOL movement. This IOL is not recommended when a primary posterior capsulectomy and anterior vitrectomy has been performed. The AcrySof ReSTOR IOL is based on the AcrySof design and material platform. The ReSTOR is a pseudo-accommodative, apodized diffractive IOL with a near add of +4.00 diopters built-in. The add results in +3.20 diopters in the spectacle plane. The ReZoom multifocal IOL is a secondgeneration refractive redesign (using acrylic) of the original silicone Array multifocal IOL. The newest multifocal is the Tecnis aspheric optic design multifocal IOL which is engineered to reduce the spherical aberration of the average corneas.

Each of the multifocal IOLs represents a compromise based on the simultaneous vision principle. Two or more images are formed on the retina at the same time, one image at near and the other at distance focus. The brain selects the image it wants to see. Some loss of contrast is inherent to simultaneous vision since the available light is split between the near focus and the distance focus. Uncorrected refractive error (cylinder of more than 1 diopter or the changes in sphere that occur with eye growth) may result in more significant blur because of the simultaneous vision concept. Alternating vision, which is provided by a monofocal IOL and bifocal glasses, results in only one object being in focus at a time and all incoming light is directed to this focus. While the increased use of multifocal and accommodative IOLs for implantation during the teenage years is predictable, we would caution surgeons that these lenses may not be advantageous in growing or amblyopic eyes. With residual refractive error, especially the myopia that develops after eye growth, multifocality may (ironically) result in more spectacle dependence compared to a monofocal IOL with residual myopia. This deserves further study.

23.4Postoperative Medications and Follow-up

are placed on the eye. A patch and Fox shield are placed over the eye. We prefer to secure the shield with two Tegaderm sheets instead of standard tape (Fig. 23.10). The patch and shield should remain on the eye until the morning after surgery. We remove it in the office, examine the eye, and show the parents how to apply the postoperative drops. There are some variances from the protocol in certain situations. With older children, the atropine may be deleted. Babies who are left aphakic do not receive the ointment. We use topical drops for these eyes, and rather than patching the eye, we apply a Silsoft contact lens (usually a 7.5 base-curve and +32 D or +29 D power) at the end of surgery. The parents can then begin the drops right away. For older children (above age 6–7 years) the parents are allowed to remove the patch and shield 4–5 h after the surgery and begin the postoperative drops. The eye is still examined on the first postoperative day. Topical atropine (0.5% in children less than 1 year of age, and 1% thereafter) is utilized once per day for 2–4 weeks in children up to age 6 years. Prednisolone acetate (1%) is used topically 6 times per day for 2 weeks and then 3–4 times per day for an additional 2 weeks. An antibiotic drop

(the same fluoroquinolone used preoperatively) is used for 1 week after surgery. Any residual refractive error is corrected after the wound stabilizes and the synthetic absorbable sutures dissolve. We rarely use oral steroids except in some uveitis patients or some trauma cases. We schedule postoperative examinations at 1 week, 4 weeks, 3 months, and 6 months postoperatively. We also consider a yearly EUA in order to measure intraocular pressure, examine the peripheral retina, monitor eye growth using A-scan ul-

Immediately at the end of surgery, a drop of dilute (5%) povidone iodine is placed on the operative eye. An antibiotic steroid ointment and atropine ointment

Fig. 23.10  A patch and Fox shield are placed over the eye. Note that we prefer to secure the shield with two Tegaderm sheets instead of standard tape

Chapter 23  Pediatric Cataract Surgery: Operative and Postoperative Issues

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trasound, examine the position of the IOL, and detect any secondary membrane or after-cataract formation. Once children become old enough and cooperative enough to undergo these examinations awake, the yearly EUA becomes unnecessary.

23.5 Special Considerations

23.5.1 Traumatic Cataract

Trauma is a common cause of unilateral cataract in children. At the time of presentation after the trauma to the eye, primary repair of a corneal or scleral wound may be needed along with a complete evaluation of damage to the intraocular structures (e.g., posterior capsule rupture, vitreous hemorrhage, and retinal detachment). The authors prefer to defer cataract surgery and IOL implantation in traumatic cataract patients, even when anterior lens capsule has been ruptured. A delay of 1–4 weeks may be helpful to allow corneal healing and to reduce the inflammatory response. Longer delays are avoided in children within the amblyopic ages. Implantation of an IOL is preferred in the cases of traumatic cataracts with corneal injuries, because contact lenses may be difficult to fit. On the other hand, a rigid gas-permeable contact lens may be needed to help with control of astigmatism and, if worn, can also provide aphakic correction. For this reason, some surgeons are less likely to place an IOL primarily in these cases.

Placement of the IOL in the capsular bag is preferred when capsular support is available. When stability of the capsular bag is compromised, a capsular tension ring (CTR) can be used (Fig. 23.11). Ciliary sulcus fixation of the IOL can also be done in the absence of adequate capsular support for in-the-bag

placement, but with a greater incidence of uveitis and pupillary capture [27].

23.5.2 Ectopia Lentis

Ectopia lentis is defined as displacement or malposition of the crystalline lens of the eye. The lens can be dislocated (luxated) or subluxated. Subluxation of the crystalline lens may occur as an ocular manifestation of systemic diseases including Marfan syndrome, homocystinuria, and Weil-Marchesani syndrome. It can also occur as an idiopathic isolated defect. Progressive subluxation of the lens induces large refractive errors and loss of accommodation. In addition, movement of the dislocated lens can cause the visual axis to be partly phakic and partly aphakic leading to marked visual disturbances. Surgical removal of the congenitally subluxated lens needs to be undertaken with caution. Although CTRs are useful with a moderate loss of zonular support (Figs. 23.12, 22.13), eyes with profound zonular compromise or marked lens subluxation are best treated with complete removal of the lens and capsular bag using vitrectomy instrumentation.

23.5.3Secondary Intraocular Lens Implantation

The vast majority of children undergoing secondary IOL implantation have had a primary posterior capsulotomy and anterior vitrectomy. If adequate peripheral capsular support is present, the IOL is placed into the ciliary sulcus or in the reopened capsular bag

Fig. 23.11a–d  Use of capsular tension ring in a child with traumatic cataract

338

M. Edward Wilson and Rupal H. Trivedi

 

 

Fig. 23.12  a Subluxation of lens. b Use of Cionni ring in eye shown in a

Fig. 23.13a–d  Ectopia lentis and use of Cionni ring

Fig. 23.14  Secondary in-the-bag: opening of capsular bag just completed with Sommerring ring removed

(Fig. 23.14) [33, 50]. Viscodissection and meticulous clearing of all posterior synechiae between the iris and the residual capsule is mandatory. An all-PMMA IOL is ideal for sulcus placement and should be considered, especially when the amount of capsule remnant is less than ideal. However, these IOLs require a larger incision for implantation. The most common IOL used in secondary implantation is the three-piece AcrySof IOL. It has a posterior angulation that helps make it suitable for the sulcus. However, the haptics are soft and decentrations can occur, especially in eyes with large anterior segments and axial length measurements greater than 23 mm. Prolapsing the IOL

optic through the fused anterior and posterior capsule remnants is very useful in preventing decentration and also eliminating the possibility of inadvertent pupillary capture. When inadequate capsular support is present for sulcus fixation in a child, implantation of an IOL is not recommended unless every contact lens and spectacle option has been explored fully. Anterior chamber IOLs and scleral or iris-fixated posterior chamber IOLs are used in children when other viable options are absent, but the long-term consequences of these placements are unknown. Anterior chamber

IOLs should be of an open-loop flexible design and sized appropriately for the anterior chamber. Scleralsutured IOLs are usually fixated with 10-0 Prolene suture but concerns of biodegradation have surfaced as more late (5–15 years after surgery) IOL decentrations have been documented. A new 10-0 polyester suture has now been tried. Some surgeons are also using 9-0 Prolene. Other suture materials are in design. Iris fixation is also an alternative in children when inadequate capsule is present for sulcus or bag fixation. A three-piece acrylic lens can be placed through a pharmacologically constricted pupil so as to purposefully pupil-capture the optic. The haptics are secured to the undersurface of the iris with one full-thickness iris suture each. The sutures are placed in the immobile peripheral iris. This technique has the advantage of a small incision since a foldable IOL is utilized. Iris fixation as in the “lobster-claw” style

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lenses (Verisyse) are utilized in some children as a phakic IOL for high myopia. The aphakic version of this IOL is available for compassionate use but must be requested through the FDA on a case-by-case basis. An abbreviated FDA approval process is being planned so that the aphakic IOL is easier to obtain.

23.6Postoperative Complications and Visual Outcome

23.6.1 Visual Axis Opacification

Secondary VAO is one of the most common complications of pediatric cataract surgery, especially when the posterior capsule is left intact. PCO is generally delayed in eyes with a hydrophobic acrylic IOL as compared with a PMMA IOL. VAO after acrylic implantation with an intact posterior capsule is more

“proliferative” compared to the “fibrous” reaction commonly seen in conjunction with the PMMA IOLs. After a primary posterior capsulotomy and an anterior vitrectomy, VAO is rare in older children when an acrylic IOL has been used. When VAO does occur, it is usually in a baby operated on in the first year of life. When infantile eyes are implanted with an IOL, VAO is common despite performing posterior capsulectomy and vitrectomy. Using hydrophobic acrylic IOLs, various articles have reported VAO averaging 44.0%, while ranging from 8.1% when all children under 2 years of age were reviewed to 80% when all children operated on below 6 months of age were included [18, 28, 35, 38]. Secondary VAO in eyes implanted in infancy tends to occur within the first 6 months after cataract surgery [35]. Thus, patients with longer follow-up will not likely change the incidence of VAO in infantile eyes. Eyes with associated ocular anomalies (e.g., anterior segment dysgenesis, iris hypoplasia, or persistent fetal vasculature) are at 9 times higher risk for developing VAO as compared to eyes without associated ocular anomalies [35]. In children older than 2 years of age at the time of cataract surgery, the secondary VAO rate after primary posterior capsulectomy and vitrectomy varies from 0% to 20.6% with an average of 5.1%. In older children, some authors prefer to do only posterior capsulorhexis (without vitrectomy). The average rate of secondary intervention in these eyes is 13.8%

(range 0–68%). With an intact posterior capsule, various articles have reported PCO ranging from 14.7% to 100% (average 25.1%, excluding eyes with 100%

PCO in children younger than 4 years of age). With longer follow-up, even for older children, the average Nd:YAG laser capsulotomy rate may be higher than the 25.1% noted here. Stager and colleagues reported that 70% maintained a clear visual axis after a single Nd:YAG procedure, 84% after two Nd:YAG procedures, and 88% after three Nd:YAG procedures

[30]. Surgical intervention to clear the visual axis was needed in 8%. The probability of maintaining a clear central visual axis after 24 months with a single

Nd:YAG laser procedure was 35% in children less than 24 months of age and 74% in older children.

23.6.2 Deposits and Synechiae

Increased inflammation is reported in eyes with a

PMMA IOL as compared with hydrophobic acrylic

IOLs [1, 17]. The incidence of deposits was reported as 6.4% [46], 25% [23], 35.9% [38], and 24.1% [35]. The incidence of deposits was significantly higher in younger age groups (age at surgery less than 2 years of age) than in the older groups (P <0.04) [38]. Younger age at the time of cataract surgery also increases the risk for synechiae formation. Vasavada and colleagues noted posterior synechiae in 14 eyes

(13.6%). All except one were operated on during the first 2 years of life [38]. The incidence of synechiae formation was significantly higher when children were operated on before 2 years of age compared to the older age groups (P <0.001).

23.6.3 Glaucoma

In a multicenter retrospective review, Asrani and coworkers reported a lower incidence (0.3%, or 1 in 377 cases) of open-angle glaucoma in eyes receiving a primary IOL implant compared with those that remained aphakic (11.3%, or 14 in 124 cases) after cataract surgery[2].Wereportedpostoperativeglaucomain3.8%

(10 of 266) of eyes with an IOL implant and 17% (8 of 47) of aphakic eyes [34]. However, for patients who underwent surgery during the first 4.5 months of

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their life, the glaucoma incidence was 24.4% (10/41) in eyes with an IOL implant and 19% (8/42) in aphakic eyes (P =0.55). The result of our study suggests that an IOL is not protective against the development of glaucoma [34]. Congenital cataracts that need to be operated on early in life are at higher risk for the development of glaucoma with or without an IOL. Childhood cataracts that develop after infancy are usually not associated with microphthalmia, are almost always implanted with an IOL, and are at very low risk for the development of glaucoma. Since the eyes at highest risk for glaucoma are also the eyes most likely to be left aphakic, IOL implantation may falsely appear to be protective against glaucoma.

23.6.4 Retinal Detachment

Rabiah and colleagues noted that aphakic retinal detachment (RD) is infrequent after pediatric cataract surgery [29].Although we have not analyzed our data for pseudophakic RD systematically, we do not recall seeing it in eyes with pediatric cataract in the absence of a predisposing etiology such as a history of RD, trauma, or retinopathy of prematurity.

23.6.5 Visual Acuity Outcome

We reported a median visual acuity of 20/30, with median visual acuity of unilateral and bilateral cases being 20/40 and 20/25, respectively [20]. Better visual acuity was associated with bilateral cataract, older age at surgery, and normal interocular axial length difference. Amblyopia was the major cause of residual visual deficit.

23.6.6 Choroidal Effusion

In eyes with Sturge-Weber syndrome, choroidal effusion can occur postoperatively (Fig. 23.15). The visual outcome of these eyes is generally good.

Acknowledgements. Supported in part by the Grady Lyman Fund of the MUSC Health Sciences Foundation, Charleston, S.C.

Fig. 23.15  Choroidal effusion in an eye with Sturge-Weber syndrome operated on for cataract

References

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2.Asrani S, Freedman S, Hasselblad V, Buckley EG, Egbert J, Dahan E, Gimbel H, Johnson D, McClatchey S, Parks M, Plager D, Maselli E (2000) Does primary intraocular lens implantation prevent “aphakic” glaucoma in children? J AAPOS 4:33–39

3.Basti S, Krishnamachary M, Gupta S (1996) Results of sutureless wound construction in children undergoing cataract extraction. J Pediatr Ophthalmol Strabismus

33:52–54

4.Bayramlar H, Totan Y, Borazan M (2004) Heparin in the intraocular irrigating solution in pediatric cataract surgery. J Cataract Refract Surg 30:2163–2169

5.Biglan AW, Cheng KP, Davis JS, Gerontis CC (1996) Results following secondary intraocular lens implantation in children. Trans Am Ophthalmol Soc 94:353–373

6.Buckley EG, Klombers LA, Seaber JH, Scalise-Gordy A,

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9.Gimbel HV (1997) Posterior continuous curvilinear capsulorhexis and optic capture of the intraocular lens to prevent secondary opacification in pediatric cataract surgery.

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Take Home Pearls

Modifications of the manual CCC in children over the age of 4 years and use of the vitrectorhexis in children under the age of 4 years can lead to successful anterior management in nearly every surgery.

The surgeon should strictly adhere to the principles of a closed-chamber technique. Proper wound construction and a tight

fit around the instruments helps assure chamber stability throughout irrigation and aspiration of cortex and nucleus. No phacoemulsification is needed and suturing the wounds in children is the norm.

Currently available single-piece acrylic IOLs have improved the intraoperative performance of pediatric cataract surgery and helped to assure the proper capsular placement of the implant.

In children beyond the infantile age group, combined posterior capsulectomy, vitrectomy, and hydrophobic acrylic IOL implantation avoids the need for a secondary intervention in most eyes.

In infant eyes, VAO is much more common when an IOL of any type is implanted compared with primary aphakia, even when a posterior capsulotomy and an anterior vitrectomy is performed.

In pediatric eyes with an intact posterior capsule, PCO is almost inevitable. Nd:YAG capsulotomy can treat this complication but requires more energy and may need to be repeated.

Patients undergoing cataract surgery during early infancy are at higher risk for the development of glaucoma with or without an IOL implant.

Amblyopia is major cause for poor visual outcome.

11.Gordon RA, Donzis PB (1985) Refractive development of the human eye. Arch Ophthalmol 103:785–789

12.Grieshaber MC, Pienaar A, Stegmann R (2005) Posterior vertical capsulotomy with optic entrapment of the intraocular lens in congenital cataracts: prevention of capsule opacification. J Cataract Refract Surg 31:886–894

13.Hollick EJ, Spalton DJ, Ursell PG, Pande MV, Barman SA, Boyce JF, Tilling K (1999) The effect of polymethylmethacrylate, silicone, and polyacrylic intraocular lenses on posterior capsular opacification 3 years after cataract surgery. Ophthalmology 106:49–54

14.Jacobs DS, Cox TA, Wagoner MD, Ariyasu RG, Karp

CL, American Academy of Ophthalmic Technology Assessment Committee Anterior Segment P (2006) Capsule staining as an adjunct to cataract surgery: a report from the American Academy of Ophthalmology. Ophthalmology 113:707–713

15.Kiel AW, Butler T, Gregson R (2003) A novel use for trypan blue to minimize epithelial cell proliferation in pe-

diatric cataract surgery. J Pediatr Ophthalmol Strabismus 40:96–97

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17.Kuchle M, Lausen B, Gusek-Schneider GC (2003) Results and complications of hydrophobic acrylic vs PMMA posterior chamber lenses in children under 17 years of age. Graefes Arch Clin Exp Ophthalmol 241:637–641

18.Kugelberg M, Kugelberg U, Bobrova N, Tronina S, Zetterstrom C (2006) Implantation of single-piece foldable acrylic IOLs in small children in the Ukraine. Acta Ophthalmol Scand 84:380–383

19.Kwok AK, Tham CC, Loo AV, Fan DS, Lam DS (2001) Ultrasound biomicroscopy of conventional and sutureless pars plana sclerotomies: a comparative and longitudinal study. Am J Ophthalmol 132:172–177

20.Ledoux DM, Trivedi RH, Wilson Jr ME, Payne JF (2007) Pediatric cataract extraction with intraocular lens implan-

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tation: visual acuity outcome when measured at age four years and older. J AAPOS 11:218–224

21.Lee HK, Kim CY, Kwon OW, Kim EK, Lee SC, Seong GJ, Kim SS (2004) Removal of dense posterior capsule opacification after congenital cataract extraction using the transconjunctival sutureless vitrectomy system. J Cataract Refract Surg 30:1626–1628

22.Mohammadpour M (2007) Four-incision capsulorhexis in pediatric cataract surgery. J Cataract Refract Surg 33:1155–1157

23.Mullner-Eidenbock A, Amon M, Moser E, Kruger A, Abela C, Schlemmer Y, Zidek T (2003) Morphological and functional results of AcrySof intraocular lens implantation in children: prospective randomized study of age-related surgical management. J Cataract Refract Surg 29:285–293

24.Nanavaty MA, Johar K, Sivasankaran MA, Vasavada AR, Praveen MR, Zetterstrom C (2006) Effect of trypan blue staining on the density and viability of lens epithelial cells in white cataract. J Cataract Refract Surg 32:1483–1488

25.Nischal KK (2002) Two-incision push-pull capsulorhexis for pediatric cataract surgery. J Cataract Refract Surg 28:593–595

26.Nischal KK (2007) Who needs a reversibly adjustable intraocular lens? Arch Ophthalmol 125:961–962

27.Pandey SK, Ram J, Werner L, Brar GS, Jain AK, Gupta A, Apple DJ (1999) Visual results and postoperative complications of capsular bag and ciliary sulcus fixation of posterior chamber intraocular lenses in children with traumatic cataracts. J Cataract & Refract Surg 25:1576–1584

28.Plager DA, Yang S, Neely D, Sprunger D, Sondhi N

(2002) Complications in the first year following cataract surgery with and without IOL in infants and older children. J AAPOS 6:9–14

29.Rabiah PK, Du H, Hahn EA (2005) Frequency and predictors of retinal detachment after pediatric cataract surgery without primary intraocular lens implantation. J AAPOS 9:152–159

30.Stager DR Jr, Wang X, Weakley DR Jr, Felius J (2006) The effectiveness of Nd:YAG laser capsulotomy for the treatment of posterior capsule opacification in children with acrylic intraocular lenses. J AAPOS 10:159–163

31.Tassignon MJ, De Groot V, Vrensen GF (2002) Bag-in- the-lens implantation of intraocular lenses. J Cataract Refract Surg 28:1182–1188

32.Tassignon MJ, De Veuster I, Godts D, Kosec D, Van den Dooren K, Gobin L (2007) Bag-in-the-lens intraocular lens implantation in the pediatric eye. J Cataract Refract Surg 33:611–617

33.Trivedi RH, Wilson ME Jr, Facciani J (2005) Secondary intraocular lens implantation for pediatric aphakia. J AAPOS 9:346–352

34.Trivedi RH, Wilson ME Jr, Golub RL (2006) Incidence and risk factors for glaucoma after pediatric cataract surgery with and without intraocular lens implantation. J AAPOS 10:117–123

35.Trivedi RH, Wilson ME Jr, Bartholomew LR, Lal G, Peterseim MM (2004) Opacification of the visual axis after cataract surgery and single acrylic intraocular lens implantation in the first year-of-life. J AAPOS 8:156–164

36.Vasavada AR, Trivedi RH (2000) Role of optic capture in congenital cataract and intraocular lens surgery in children. J Cataract Refract Surg 26:824–831

37.Vasavada AR, Trivedi RH, Singh R (2001) Necessity of vitrectomy when optic capture is performed in children older than 5 years. J Cataract Refract Surg 27:1185–1193

38.Vasavada AR, Trivedi RH, Nath V (2004) Visual axis opacification after AcrySof intraocular lens implantation in children. J Cataract Refract Surg 30:1073–1081

39.Vasavada AR, Trivedi RH, Apple DJ, Ram J, Werner L

(2003) Randomized, clinical trial of multiquadrant hydrodissection in pediatric cataract surgery. Am J Ophthalmol 135:84–88

40.Werner L, Apple DJ, Crema AS, Izak AM, Pandey SK,

Trivedi RH, Ma L (2002) Permanent blue discoloration of a hydrogel intraocular lens by intraoperative trypan blue. J Cataract Refract Surg 28:1279–1286

41.Wilson ME (1999) Anterior capsule management for pediatric intraocular lens implantation. J Pediatr Ophthalmol Strabismus 36:314–319

42.Wilson ME, Trivedi RH, Mistr S (2007) Pediatric intraoperative floppy-iris syndrome. J Cataract Refract Surg

33:1325–1327

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47.Wilson ME, Trivedi RH, Buckley EG, Granet DB, Lambert SR, Plager DA, Sinskey RM, Vasavada AR (2007) ASCRS white paper: hydrophobic acrylic intraocular lenses for children. J Cataract Refract Surg 33:1966–1973

48.Wilson ME Jr (2004) Anterior lens capsule management in pediatric cataract surgery. Trans Am Ophthalmol Soc 102:391–422

49.Wilson ME Jr, Trivedi RH (2006) Low molecular-weight heparin in the intraocular irrigating solution in pediatric cataract and intraocular lens surgery. Am J Ophthalmol 141:537–538

50.Wilson ME Jr, Englert JA, Greenwald MJ (1999) In-the- bag secondary intraocular lens implantation in children. J AAPOS 3:350–355

51.Wilson ME Jr, Bartholomew LR, Trivedi RH (2003) Pediatric cataract surgery and intraocular lens implantation: practice styles and preferences of the 2001 ASCRS and AAPOS memberships. J Cataract Refract Surg 29:1811–1820

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52.Wilson ME Jr, Trivedi RH, Bartholomew LR, Pershing

S (2007) Comparison of anterior vitrectorhexis and continuous curvilinear capsulorhexis in pediatric cataract and intraocular lens implantation surgery: a 10-year analysis. J AAPOS 11:443–446

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Glaucoma in Infancy

24

and Early Childhood

Sharon F. Freedman and Suzanne C. Johnston

Contents

24.1

Introduction  . . . . . . . . . . . .   346

24.2Classification  . . . . . . . . . . . .   346

24.3Signs and Symptoms of Glaucoma

in Children  . . . . . . . . . . . . .   346

24.3.1Signs and Symptoms of Glaucoma

in Infancy and Early Childhood  . . . . .   348

24.3.2Signs and Symptoms of Glaucoma in Older

Children  . . . . . . . . . . . . . .   349

24.4Ocular Examination  . . . . . . . . .   350

24.4.1 Vision Testing (Acuity and Visual Fields)  .   350

24.4.2External Examination  . . . . . . . . .   350

24.4.3

Tonometry  . . . . . . . .

.

.

.

.

.   350

24.4.4

Anterior Segment Examination 

.

.

.

.

.   351

24.4.5Gonioscopy  . . . . . . . . . . . . . . . . . . . . . . . . .   351

24.4.6Optic Nerve and Fundus Examination  . .   352

24.4.7 Other Useful Diagnostic Tests  . . . . .   352

24.4.8Imaging Techniques: Fundus Photography, Optical Coherence Tomography  . . . . .   353

24.5

Differential Diagnosis  . . .

.

.

.

.

.

  353

24.6

Primary Childhood Glaucoma 

.

.

.

.

.

  353

24.6.1Primary Congenital/Infantile Open-angle Glaucoma  . . . . . . . . . . . . .   353

24.6.2Juvenile Open-angle Glaucoma  . . . . .   354

24.6.3Primary Pediatric Glaucoma Associated With Ocular Anomalies (Anterior Segment

Dysgenesis)  . . . . . . . . . . . .   355

24.6.4Primary Pediatric Glaucoma Associated

 

with Systemic Diseases  . . .

.

.

.

.

.   357

24.7

Secondary Childhood Glaucoma 

.

.

.

.

  359

24.7.1

Trauma  . .

.

.

.

.

.

.

.

.

.

.

.

.

  359

24.7.2

Neoplasm  .

.

.

.

.

.

.

.

.

.

.

.

.

  359

24.7.3Inflammation and Steroid-related Glaucoma    360

24.7.4Lens-induced Glaucoma  . . . . . . . .   360

24.7.5Aphakic (Pseudophakic) Glaucoma  . . .   360

24.7.6

Miscellaneous Causes 

.

.

.

.

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24.8

Treatment  . . . . .

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24.8.1Medical Management  . . . . . . . . .   361

24.8.2Surgical Management  . . . . . . . . .   364

24.9Long-term Follow-up of Children

with Glaucoma  . . . . . . . . . . .   370

References  . . . . . . . . . . . . . . . . .   370

Core Messages

Early identification of glaucoma in infants and children by physicians and care providers maximizes the likelihood of preserving useful vision.

Glaucomas presenting in infancy share unique features related to the corneal and overall stretching of the eye under high intraocular pressure.

Glaucomas presenting in older children sometimes take the form of refractive error, and their timely diagnosis may rely on careful scrutiny of the optic nerve head configuration and tonometry.

M. E. Wilson et al. (eds.), Pediatric Ophthalmology,

DOI 10.1007/978-3-540-68632-3_1, © Springer-Verlag Berlin Heidelberg 2009

346

Sharon F. Freedman and Suzanne C. Johnston

 

 

All children with conditions predisposing to glaucoma should be regularly examined for glaucoma.

Medical therapy is appropriate first-line therapy for many secondary glaucomas and to help clear the cornea for angle surgery in primary congenital glaucoma.

Surgical intervention is essential for primary congenital glaucoma, and is often needed for other primary and secondary glaucomas inadequately controlled on medications.

Successful care of childhood glaucoma requires ophthalmologists experienced in treating these rare conditions,

but ultimately also requires a team approach including the family and child among other critical members.

24.1 Introduction

Childhood glaucomas are a rare, heterogeneous group of disorders which, like adult glaucoma, can have vision-threatening consequences. Diagnosis and management of the pediatric glaucomas present several unique challenges. It is often the parent, primary care or eye care provider who will first recognize glaucoma in this population and it is crucial that they are familiar with its clinical features and maintain a high index of suspicion when considering this diagnosis. In adults, glaucoma is often occult; however, in children, strong suggestive signs of glaucoma are often present. The presentation of infant and childhood glaucoma varies with the degree of pressure elevation as well as the age of onset. In addition, examination of young children can be challenging and treatment strategies less familiar than those in adult patients. Genetic, pharmacologic, and technologic advances in the diagnosis and treatment of glaucoma raise the hope that this disease will, in the near future, cease to rob children and adults of vision.

24.2 Classification

There have been several classification schemes proposed for the group of childhood glaucomas, each with its own merits and advantages [123]. One such system divides childhood glaucomas into those of primary and secondary origin, where a primary glaucoma results from an intrinsic disease of the aqueous outflow mechanism, often of genetic origin, while a secondary glaucoma results from another ocular disease, injury, drug, or systemic disease (Table 24.1). There is considerable overlap between these groups and their classification. In addition, there are some pediatric glaucomas which may have both primary and secondary etiologies (e.g., infantile-onset glaucoma in Sturge-Weber syndrome, neurofibromatosis, and even aniridia). As the genetics of conditions associated with childhood glaucoma becomes further clarified, one may expect that many of the current phenotypically driven diagnostic labels will be replaced or reorganized based upon their underlying genetic abnormalities.

Both primary and secondary pediatric glaucoma may be associated with significant systemic conditions. It is therefore important for the ophthalmologist to accurately interpret eye signs as clues for the diagnosis and classification of both the glaucoma and the associated systemic disease. It is also helpful to classify childhood glaucoma in terms of age of onset dividing glaucoma into infantile and juvenile glaucoma. Three years of age is often taken as the division between infantile and juvenile glaucoma, because it is at approximately this age that the eye no longer expands in response to elevated intraocular pressure (IOP) [29].

24.3Signs and Symptoms

of Glaucoma in Children

The signs and symptoms of glaucoma vary greatly among children, according to the age of the child and the degree of IOP elevation (see also Sect. 24.4).