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Initial surgical treatment

A B C

D E F

G H I

Figure 10.6 Combined trabeculotomy and trabeculectomy. The conjunctival incision may be either a fornix-based or limbal-based flap. A limbal-based flap is shown in these photographs. A partialthickness scleral flap is prepared (A). Some surgeons use intraoperative mitomycin-C after this step. Schlemm’s canal is identified (B), and the trabeculotome is passed into Schlemm’s canal (C). The trabeculotome is rotated into the anterior chamber (D), which is often accompanied by bleeding from Schlemm’s canal. The other trabeculotome is passed in the opposite direction and rotated into the anterior chamber (E). After completion of the trabeculectomy, a sclerostomy is prepared (F). A Descemet’s punch may be used to prepare the sclerostomy. A peripheral iridectomy is performed, and the scleral flap is closed with interrupted sutures (G). The conjunctival incision is closed with a running suture (H). In this eye, the corneal edema cleared, and the intraocular pressure remained

J

stable during the postoperative period. The appearance of the eye is shown at 3 years after the procedure (I, J).

capsule are then closed with a running suture of an absorbable material (e.g. 8–0 or 9–0 polyglactin).

Most surgeons perform a paracentesis opening with a beveled corneal incision at the beginning of the surgery. Through the paracentesis, the anterior chamber can be reformed with balanced salt solution and patency of the trabeculectomy can be tested at the conclusion of the surgery. Also, identification of Schlemm’s canal may be facilitated when the intraocular pressure is lowered after the paracentesis. Subconjunctival injection of an antibiotic–steroid preparation is performed, topical antibiotic–steroid medica-

tions are placed into the conjunctival fornix, and a patch and shield are applied to the eye.

The dressing is removed on the first postoperative day. An antibiotic–steroid combination is prescribed four times a day. A cycloplegic (e.g., cyclopentolate 1% BID) is used only if the eye has a shallow anterior chamber and the child is seen frequently in the office for monitoring. Examination of the patient under anesthesia is performed approximately 3 to 4 weeks after surgery. If all is stable, the patient is scheduled for another evaluation under anesthesia in approximately 3 months. The evaluations may be repeated at quarterly

70

Long-term follow-up and prognosis

intervals for the first year after surgery. After the first year, the examinations are biannual until the child is old enough to cooperate fully with an office examination. Office visits may reduce the need for examinations under anesthesia. These patients should be followed up for an indefinite time to determine whether or not adequate control of intraocular pressure has been achieved. The success rate is high for children with infantile glaucoma and surgery within the first year of life, whereas patients with Sturge–Weber syndrome have increased failure with longer follow-up.

Whether combined trabeculotomy–trabeculectomy is superior to trabeculotomy alone is debatable. Biedner and Rothkoff70 found no difference between trabeculotomy and combined trabeculotomy–trabeculectomy in a small series of 7 patients with congenital glaucoma. Dietlein et al71 investigated the outcome of trabeculotomy, trabeculectomy and a combined procedure as initial surgical treatment approaches in primary congenital glaucoma. Although the combined procedure seemed to have a favorable outcome, after 2 years the advantages of this procedure over trabeculotomy or trabeculectomy was not statistically significant according to the life table analysis. Elder72 compared primary trabeculectomy with combined trabeculotomy–trabeculectomy and found the combined procedure to be superior. The superior results of the combined procedure may be because of the dual outflow pathway as explained by Elder. Mullaney et al73 and Al-Hazmi et al74 used mitomycin-C in primary combined trabeculotomy–trabeculectomy and reported a higher success rate. The results reported by Mandal et al75–81 from India are comparable to that reported by Mullaney et al and Al-Hazmi et al from Saudi Arabia but Mandal et al did not use mitomycin-C in primary surgery.

The long-term results of trabeculectomy–trabeculotomy have been described by Mandal et al,81 who reported longterm outcome of 299 eyes of 157 consecutive patients who underwent primary combined trabeculotomy–trabeculectomy for developmental glaucoma by a single surgeon over a 12 year period. Kaplan–Meier survival analysis demonstrated the success probabilities of 94.4%, 92.0%, 86.7%, 79.4%, 72.9% and 63% at the 1st, 2nd, 3rd, 4th, 5th, and 6th year, respectively (Fig. 10.7). The success rate of 63.1% was maintained until 8 years of follow-up. Data on visual acuity was available in 49 patients. At the final follow-up visit, 20 patients (40.8%) had normal visual acuity (best-corrected visual acuity of better than or equal to 20/60 in the better eye).

The compelling argument in favor of primary combined trabeculotomy–trabeculectomy in some ethnic populations is the higher incidence of successful intraocular pressure control with a single operative procedure, as has been reported from India and Saudi Arabia. O’Connor82 commented that combined trabeculotomy–trabeculectomy is potentially a very successful surgical procedure in the management of congenital glaucoma and may represent the next step in the search for the best surgical treatment of congenital glaucoma. Further prospective randomized studies are required to explore the surgical results of trabeculotomy, primary combined trabeculotomy and trabeculectomy, and 360° trabeculotomy. However such a study is difficult to conduct because most

 

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Survival

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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Figure 10.7

Long-term success after combined trabeculotomy and

 

trabeculectomy. Children with developmental glaucoma were operated within 6 months of birth, with 142 eyes included in the analysis. Surgery was considered a complete success when the intraocular pressure was less than 21 mmHg in patients examined under general anesthesia or less than 21 mmHg in patients who were old enough to be examined with the slit lamp, and when there was no progression of disc cupping or corneal diameter. The Kaplan–Meier curve shows the complete success probabilities. Modified with permission from Mandal AK, Bhatia PG, Bhaskar A, Nutheti R. Long-term surgical and visual outcomes in Indian children with developmental glaucoma operated on within 6 months of birth. Ophthalmology 2004; 111:283–290.

glaucoma specialists are better trained in and more comfortable with one angle procedure than the other.

Long-term follow-up and prognosis

Between 3 and 6 weeks after surgery, the postoperative control of the glaucoma must be judged. The degree of relief from photophobia, tearing, and blepharospasm usually reflect the effectiveness of surgery and may reasonably predict whether or not additional surgery will be required. Children with developmental glaucoma must be re-examined periodically and for an indefinite time to determine whether or not adequate control of intraocular pressure has been achieved. Most of the examination can be done in an office setting. Examination under anesthesia often allows more careful gonioscopy, in addition to other measurements. Each followup evaluation should include vision testing, external examination, refraction, ophthalmoscopy, corneal assessment, and tonometry. Gonioscopy, ultrasonographic biometry, and disc photography are performed as needed.

Vision testing techniques vary greatly with the age of the patient. In infants, good fixation and following as well as the absence of nystagmus are important indicators of good visual function. In children over 3 years of age, visual acuity and, eventually, visual fields can also be determined. The external examination is important in order to detect evidence of associated abnormalities, inflammation, or lacrimal duct obstruction. Subjective refraction is generally not possible, but retinoscopy of the eye can be compared to previous measurements of myopia and astigmatism. The optic disc can be examined by ophthalmoscopy to determine if the optic cup has remained the same, enlarged, or regressed.53 The cornea is assessed, the degree of corneal haze or edema is

71

Initial surgical treatment

Figure 10.8 Clinical appearance of child with unilateral corneal edema due to congenital glaucoma (A). The postoperative appearance of the child, showing resolution of corneal edema (B).

A B

noted (Fig. 10.8), and calipers are used to measure the corneal diameter. A problem with calipers in measuring corneal diameter is that it is difficult to distinguish the actual corneal diameter from a cord length. Accurate measurements of the corneal diameter are facilitated by the use of a plastic gauge with calibrated holes.83

Tonometry is best performed on the peaceful, awake infant. If an examination under anesthesia is required, tonometry is performed at an appropriate stage of anesthesia,84 but the significance of the intraocular pressure reading must be balanced carefully against the other clinical signs, if it is not in keeping with them. Many anesthetics alter intraocular pressure of patients with developmental glaucomas. Postoperatively, gonioscopy provides important anatomic information about the status of the anterior chamber angle treated with goniotomy or trabeculotomy ab externo. Ultrasonographic biometry may be performed, utilizing the A-scan ultrasound to measure axial length compared to presurgical readings. Sampaolesi85 and many other authors86–88 have stressed the clinical importance of echography in the diagnosis and follow up of the developmental glaucomas.

Disc photography may provide a record for future comparisons. A decrease in cupping can occur within hours or days after intraocular pressure control in the very young. This is especially marked in infants below 1 year of age. The prognosis of this disease is related to the time of its initial presentation, initial surgical intervention, degree of optic nerve damage, nature and quality of corneal enlargement, astigmatism, progressive refractive error, and anisometropic amblyopia.89 The inability to easily quantitate visual acuity and extent of visual loss in neonates and children makes these variables less helpful in following patients than measurements of corneal diameter and intraocular pressure. However, these data should not be relied upon exclusively to determine the success of treatment in developmental glaucoma.

Because of difficulties measuring intraocular pressure and visual fields in children, ophthalmoscopy often provides the most reliable information of elevated intraocular pressure as seen by cupping of the optic nerve. Continued enlargement

of the globe, as seen by retinoscopy and/or ultrasonography, signifies inadequately controlled pressure; while stability (and sometimes slight reduction90) of ocular size suggests adequate control of intraocular pressure during the long-term follow-up.

In properly selected patients, namely those with isolated trabeculodysgenesis, surgical treatment (trabeculotomy ab externo or goniotomy) is often successful. It should be remembered, however, that increased intraocular pressure can occur at any time in the life of the patient and lifelong follow-up is necessary. The most important variables in the follow-up examinations are cupping of the optic disc visualized by ophthalmoscopy, axial length values measured by ultrasonographic biometry, intraocular pressure measured by applanation tonometry, and visual field evaluation (if possible).

Long-term vision outcomes have been described in patients

with developmental glaucomas.10,20,21,24,54,57,59,60,75,79,81,91–96

The vision outcome in studies of children with developmental glaucomas are summarized in Table 10.1. The prognosis for vision is variable, with some children achieving poor outcomes while many experience good vision outcome.

Conclusion

The responsibility of the surgeon does not end with surgery, and it is important not to be lulled into a false sense of security by surgical control of intraocular pressure. Visual rehabilitation is as important in the management of the disease as is intraocular pressure control. Visual rehabilitation involves correction of refractive errors, correction of opacities in the media, including corneal scarring and cataract, and orthoptic treatment to stimulate the development of binocular stereoscopic vision. Anisometropia and amblyopia must be aggressively managed to give these children the best chance for good vision in both eyes.

An attempt should be made to familiarize the parents with the protracted nature of the illness, the prognosis, the frequent necessity for repeat surgery, and the life-long necessity for

72

References

Table 10.1 Studies of visual outcome for developmental glaucoma

 

 

 

 

No. of subjects

 

 

Author(s)

Year

Type of glaucoma

or eyes

Best-corrected visual acuity

 

 

 

 

 

 

 

Scheie91

1959

Infantile glaucoma

53 eyes

20/50 (60% eyes)

 

Richardson et al20

1967

Infantile glaucoma

NA

20/50 (39% eyes)

 

 

 

 

 

20/200 (40% eyes)

 

 

 

 

 

 

Hass10

1968

Infantile glaucoma

NA

>20/50 (39%)

Biglan and Hiles92

1979

Infantile glaucoma

25 eyes

20/50 (64% eyes)

 

Robin et al94

1979

Infantile glaucoma

102 eyes

>20/50 (41%; 30 eyes)

 

 

 

 

 

<20/200 (41%; 30 eyes)

 

 

 

 

 

 

 

Broughton and Parks21

1981

Congenital glaucoma

29 eyes

20/40 (15 eyes)

 

 

 

 

 

20/60 (20 eyes)

 

 

 

 

 

 

 

Morgan et al93

1981

Congenital glaucoma

12 eyes

20/50 (58%; 7 eyes)

 

 

 

 

 

20/100 (9 eyes)

 

 

 

 

 

 

 

Shaffer24

1982

Developmental glaucoma

52 eyes

20/20–20/40 (28 eyes)

 

 

 

 

 

20/50–20/200 (11 eyes)

 

 

 

 

 

<20/200 (13 eyes)

 

 

 

 

 

 

 

Akimoto et al54

1994

Developmental glaucoma

111 eyes

>20/40 (68 eyes)

 

 

 

 

 

20/200–20/40 (23 eyes)

 

 

 

 

 

<20/200 (20 eyes)

 

 

 

 

 

 

 

Mandal et al75

1998

Juvenile-onset developmental

38 eyes

<20/200 (50%;19 eyes)

 

 

 

glaucoma

 

20/200 (26.3%; 10 eyes)

 

 

 

 

 

20/100–20/40 (7.8%; 3 eyes)

 

 

 

 

 

>20/40 (15.7%; 6 eyes)

 

 

 

 

 

 

 

Mendicino et al59

2000

Congenital glaucoma

24 eyes (trab.)

20/50 (79.2%; 19 eyes)

 

 

 

 

40 eyes (gonio.)

20/50 (52.5%; 21 eyes)

 

 

 

 

 

Meyer et al95

2000

Congenital glaucoma

35 eyes

Within normal nomogram range in 12 eyes

 

Mandal et al79

2002

Developmental glaucoma

28 eyes

<20/200 (28.6%; 8 eyes)

 

 

 

 

 

20/200–20/50 (28.6%; 8 eyes)

 

 

 

 

 

>20/50 (42.9%; 12 eyes)

 

 

 

 

 

 

 

Filous and Brunova57

2002

Congenital glaucoma

78 eyes

20/20–20/40 (64.1%)

 

 

 

 

 

<20/40 (35.9%)

 

 

 

 

 

MacKinnon et al96

2004

Infantile glaucoma

83 eyes

20/40 or better (61.8%)

Mandal et al81

2004

Developmental glaucoma

49 patients

20/60 or better (40.8%)

 

Ikeda et al60

2004

Developmental glaucoma

131 eyes

20/40 (59.5%, 78 eyes)

 

 

 

 

 

20/40–20/200 (16.0%, 21 eyes)

 

 

 

 

 

<20/200 (24.4%, 32 eyes)

 

 

 

 

 

 

NA, not available.

continued examinations. The parents may be quite young, and may be emotionally and economically ill-equipped to cope with the problems that have suddenly and dramatically occurred. Their guilt problems must be assuaged, particularly if a family history of congenital glaucoma exists. The parents should also be familiarized with the various agencies that will afford financial assistance when necessary. Time and effort well-spent will reward the ophthalmologist many times over at a later date.

Fortunately, with early diagnosis and microsurgical techniques, the large majority of these eyes can be controlled if not completely cured. However, in a few patients who continue to show poor response to surgery, such operations may delay loss of vision and allow the child to develop visual images that will be valuable to him or her in later life. Eventually, the social aspects of the glaucomatous child will require repeated counseling. Poor vision will need explanation to the parents and schools, the child may require the help of visual aids,

and cosmetic blemishes will need correction where possible. Continued clinical monitoring combined with adequate social support should allow the best possible outcome for the child.

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73.Mullaney PB, Selleck, Al-Award A, et al. Combined trabeculotomy and trabeculectomy as initial procedure in uncomplicated congenital glaucoma. Arch Ophthalmol 1999; 117:457–460.

74.Al-Hazmi A, Zwaan J, Awad A, et al. Effectiveness and complications of mitomycin-C use during pediatric glaucoma surgery. Ophthalmology 1998; 105:1915–1920.

75.Mandal AK, Naduvilath TJ, Jayagandhan A. Surgical results of combined trabeculotomy-trabeculectomy for developmental glaucoma. Ophthalmology 1998; 105:974–982

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References

76.Mandal AK. Current concepts in the diagnosis and management of developmental glaucomas. Indian J Ophthalmol 1993;41:51–70.

77.Mandal AK. Microsurgical technique combines trabeculotomy and trabeculectomy to treat developmental glaucoma. Ocular Surgery News, International edition 1994; 5(8):38–43.

78.Mandal AK. Primary combined trabeculotomy-trabeculectomy for early onset glaucoma in Sturge-Weber syndrome. Ophthalmology 1999; 106:1621–1627.

79.Mandal AK, Bhatia PG, Gothwal VK, et al. Safety and efficacy of simultaneous bilateral primary combined trabeculotomy-trabeculectomy for developmental glaucoma in India. Indian J Ophthalmol 2002; 50:13–19.

80.Mandal AK, Gothwal VK, Bagga H, Nutheti R, Mansoori T. Outcome of surgery on infants younger than 1 month with congenital glaucoma. Ophthalmology 2003; 110:1909–1915.

81.Mandal AK, Bhatia PG, Bhaskar A, Nutheti R. Long-term surgical and visual outcomes in Indian children with developmental glaucoma operated on within 6 months of birth. Ophthalmology 2004; 111:283–290.

82.O’Connor G. Combined trabeculotomy-trabeculectomy for congenital glaucoma (Editorial). Br J Ophthalmol 1994; 78:735.

83.Kiskis AA, Markowitz SN, Mortin JD. Corneal diameter and axial length in congenital glaucoma. Can J Ophthalmology 1985; 20:93–97.

84.Dominguez J, Banos MS, Alvarez MT, et al. Intraocular pressure measurement in infants under general anesthesia. Am J Ophthalmol 1974; 78:110–116.

85.Sampaolesi R, Caruso R. Ocular echometry in the diagnosis of congenital glaucoma. Arch Ophthalmol 1982; 100:s574–577.

86.Reibaldi A. Biometric ultrasound in the diagnosis and follow-up of congenital glaucoma. Ann Ophthalmol 1982; 14:707–708.

87.Tarkkanen A, Vusitalo R, Mianowicz J. Ultrasonographic biometry in congenital glaucoma. Acta Ophthalmol 1983; 61:618–623.

88.Law SK, Bui D, Caprioli J. Serial axial length measurements in congenital glaucoma. Am J Ophthalmol 2001; 132:926–928.

89.deLuise VP, Anderson DR. Primary infantile glaucoma (congenital glaucoma). Surv Ophthalmol 1983; 28:1–19.

90.Morin JD, Merin S, Sheppard RW. Primary congenital glaucoma: A survey. Can J Ophthalmol 1974; 9:17–28.

91.Scheie, Harold G. The management of infantile glaucoma. Arch Ophthalmol 1959; 62:35–54.

92.Biglan AW, Hiles DA. The visual results following infantile glaucoma surgery. J Pediatr Ophthalmol Strabismus 1979; 16:377–381.

93.Morgan KS, Black B, Ellis FD, Helveston EM. Treatment of congenital glaucoma. Am J Ophthalmol 1981; 92:799–803.

94.Robin AL, Quigley HA, Pollack IP, Maumenee AE, Maumenee IH. An analysis of visual acuity, visual fields, and disc cupping in childhood glaucoma. Am J Ophthalmol 1979; 88:847–858.

95.Meyer G, Schenn O, Pfeiffer N, Grehn F. Trabeculotomy in congenital glaucoma. Graefes Arch Clin Exp Ophthalmol 2000; 238:207–213.

96.MacKinnon JR, Giubilato A, Elder JE, Craig JE, Macey DA. Primary infantile glaucoma in an Australian population. Clin Exp Ophthalmol 2004; 32:14–18.

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Chapter 11

Simultaneous surgery for bilateral congenital glaucoma

Introduction

Risks of surgery

Anesthetic risks

Simultaneous bilateral surgery

Conclusion

Introduction

The management of congenital glaucoma involves careful assessment, planning, and long-term care. In about two-thirds of the patients, congenital glaucoma is bilateral.1,2 The proposal of bilateral surgery for congenital glaucoma in one surgical session has been controversial. Many pediatric ophthalmologists strongly oppose this view because of the risks of bilateral sight-threatening complications such as endophthalmitis.

The risk of general anesthesia in infants, however, is still a major concern for congenital glaucoma surgery. If bilateral anti-glaucoma surgery is not performed in the same operative session, the afflicted children must undergo two separate general anesthetics for their bilateral congenital glaucoma.

The aim of this chapter is to compare the safety of simultaneous bilateral surgery in children with congenital glaucoma versus the risk of more than one general anesthesia within a short time period. The clinical appearance of a patient treated with simultaneous bilateral surgery for congenital glaucoma is shown in Fig. 11.1.

Risks of surgery

Because of the anesthetic risks in infants, bilateral simultaneous goniotomies for congenital glaucoma were performed by Litinsky et al in 1977.3 Their particular interest was to

evaluate the risks of operating on two eyes during the same anesthesia. All the goniotomies were performed by three surgeons (Shaffer, Hetherington, and Hoskins). A separate sterile setup was used for each eye. There were no infections in this series and no complications could be attributed to the performance of surgery on both eyes during a single anesthesia. The most serious complication was cardiopulmonary arrest which occurred in six cases, giving rise to an incidence rate of 1.8%. While apnea occurred quite often, all the patients could be successfully resuscitated. However, one patient suffered neurologic deficits which resolved in a period of 2 weeks. The surgical complications encountered were iridodialyses (four cases) and small cyclodialyses (two cases) with no serious sequelae. Based on their experience of 20 years, the authors believed that goniotomy on two eyes during the same anesthesia is the wisest choice when both eyes require surgery. The life-threatening risk of two anesthetics outweighs the danger of operating on both eyes at the same time.

The incidence of endophthalmitis following pediatric anterior segment surgery is currently unknown. The paucity of reports of endophthalmitis has led some observers to recommend simultaneous bilateral surgery for congenital cataracts or glaucoma. Wheeler et al4 surveyed over 500 pediatric ophthalmologists and glaucoma specialists concerning their knowledge on the occurrence of endophthalmitis following pediatric intraocular surgery. Seventeen cases of endophthalmitis were documented to occur out of 24 000 reported surgical cases. This results in an incidence estimate of 0.071% or seven cases per 10 000, which is similar to the reported incidence following extracapsular cataract extraction in adults. In six eyes, endophthalmitis followed goniotomy or trabeculotomy. One patient who underwent bilateral trabeculotomy for congenital glaucoma developed bilateral endophthalmitis leading to no light perception. All other cases

Figure 11.1 Clinical appearance of an infant preoperatively (A) and postoperatively (B) after simultaneous bilateral surgery for congenital glaucoma.

A

B

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Simultaneous surgery for bilateral congenital glaucoma

followed unilateral surgery. To our knowledge this is the only reported study on endophthalmitis following antiglaucoma surgery in children with congenital glaucoma.

Anesthetic risks

The risks of general anesthesia in infants is still a major concern for congenital glaucoma surgery. Despite major advances in anesthesia in the last few decades, pediatric morbidity and mortality related to anesthesia is still a perplexing problem. In this respect, infants differ more from children than children differ from adults.5

In a recent prospective study, Tiret et al6 reported that the overall complication rate was much higher in infants (43 per 10 000) than in older children (5 per 10 000). In infants, the complications were mostly due to respiratory problems, while circulatory problems were more frequent in older children. It has been pointed out, however, that most cardiac arrests are due to unrecognized hypoxemia.7

Respiratory failure during anesthesia is the major risk factor in infants, the age group in whom congenital glaucoma surgery is the most urgent. This risk increases if additional factors including a history of previous anesthesia,5 intubation,8 and an upper respiratory infection are present preoperatively.9 In addition, anesthesia increases the risk for an upper respiratory infection.10

Smith found a mortality rate of 1.4 per 10 000 in a study covering over 100 000 surgeries in one institution over a 24-year period.11 During the first 10 years, infants up to 1 year had an anesthesia-related mortality rate of 8 per 10 000, whereas a lower rate was found for children from 1 to 10 years (1 per 10 000).

There are many conditions associated with congenital or infantile cataracts and glaucoma in which anesthetic difficulties are higher than average.12 Although formal studies are lacking comparing the risks of more than one induction and extubation with those of prolonged anesthesia time, most anesthesiologists believe that the former is riskier than the latter.

Simultaneous bilateral surgery

Recently, there have been reports of simultaneous surgery for bilateral congenital cataracts.13,14 Guo et al13 reported on the results of 16 children (32 eyes) who had simultaneous surgery for bilateral congenital cataracts, and concluded that bilateral simultaneous surgery can be performed to avoid a second general anesthesia in infants with bilateral dense congenital cataracts. In 1996, Zwaan14 reported on a series of nine children (18 eyes) treated with bilateral simultaneous lensectomies. He concluded that simultaneous removal of bilateral infantile cataracts should probably be reserved for selected cases in whom the anesthetic risks are higher than average.

Most of the pediatric ophthalmologists have strongly opposed simultaneous bilateral surgeries because of the risk of bilateral endophthalmitis. Endophthalmitis after cataract surgery in adults and children is uncommon. The risk of

endophthalmitis in adults varies in several published reports, a summary of which places the overall incidence at 0.35%.15 The occurrence of endophthalmitis after intraocular surgery in infants has been reported. Good et al16 in 1990 documented endophthalmitis after cataract surgery in children, which argues against simultaneous bilateral surgery. A short time delay between operated eyes should not significantly affect visual outcome. Kushner17 also believes that it is safer to perform two separate surgical procedures in infants with bilateral cataracts.

There are several reports of simultaneous bilateral surgeries for senile cataracts.18–26 Hugkulstone et al27 published a respective review of 24 patients who had undergone simultaneous bilateral trabeculectomy over a 6-year period. They reported no striking advantages in performing simultaneous bilateral trabeculectomies in adults, although no patients suffered complications leading to bilateral blindness.

Clinical experience with simultaneous bilateral surgery for developmental glaucoma

One hundred and nine consecutive patients underwent planned simultaneous bilateral primary combined trabeculectomy and trabeculotomy were evaluated for intraoperative and postoperative ocular and anesthetic complications.28 The reasons for performing simultaneous bilateral surgeries were bilateral presentation of the disease and the anesthetic risks involved in two surgeries versus one. Success (intraocular pressure <16 mmHg) probabilities were 91%, 88%, and 69% at 1, 2, and 3 years, respectively. The success probability of 69% was maintained up to 6 years of follow-up. A patient with cleared corneas and preserved visual function is shown in Fig. 11.2.

There were no major intraoperative complications, and there was no incidence of endophthalmitis or any other sight-threatening complications. Eight eyes developed a shallow anterior chamber, which required reformation in one third of these eyes. Of the anesthetic complications, apnea occurred in 16% of patients, and all were successfully resuscitated. Two children had delayed recovery: one child recovered and one expired. Cardiopulmonary arrest occurred 5 hours postoperatively during feeding in one child who could not be resuscitated. In this series, simultaneous bilateral primary combined trabeculotomy and trabeculectomy was safe and effective for treatment of the ocular condition (developmental glaucoma), obviated the need for second anesthesia with its associated risks, and offered other benefits to the patients and families.28

Simultaneous bilateral surgeries for congenital glaucoma may be planned due to risks associated with repeat anesthesia and an urge to visually rehabilitate the afflicted children as early as possible to prevent stimulus deprivation amblyopia. Additionally, simultaneous surgery is cost effective because it reduces the hospital stay with greater satisfaction of the parents. The treating surgeon has to balance the risk of bilateral endophthalmitis against the reduced risk of one

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References

A

B

Figure 11.2 Preoperative (A) and postoperative (B) appearance of a child after simultaneous bilateral surgery for congenital glaucoma. Note resolution of corneal edema.

anesthesia versus two, reduced cost, and the advantages of simultaneous early visual rehabilitation. A clear understanding of these factors is mandatory for the treating physician as well as the parents.

Conclusion

The data in the literature are inadequate for a formal comparison of the risks involved in multiple anesthetics and the possibility of bilateral endophthalmitis. It is therefore prudent to restrict simultaneous bilateral surgeries to infants with an increased vulnerability to anesthetic complications, mostly respiratory failure. However, bilateral simultaneous surgery may expedite treatment and minimize costs, while avoiding the risk of administration of two anesthetics when both eyes are treated with one general anesthesia session.

Parents should participate fully in the decision-making process and should understand the risks of possible bilateral endophthalmitis. Utmost care should be taken to eliminate the risks of infection. The afflicted children should be carefully checked for evidence of conjunctivitis, nasolacrimal duct obstruction, or upper respiratory tract infections. The second eye should be prepped and draped as if it were a new case. The surgeon and scrub nurse should re-scrub and use new gowns and gloves. An entirely different set of instruments must be used.

The key to successful management of the children afflicted with developmental glaucoma is accurate and early diagnosis coupled with prompt therapy. The earlier any glaucoma is diagnosed and brought under control, the better the

prognosis. In cases of bilateral affliction with congenital glaucoma, bilateral primary surgery offers good success rates and also avoids the risks of repeated anesthesia in these tiny patients.

References

1.deLuise VP, Anderson DR. Primary infantile glaucoma (congenital glaucoma). Surv Ophthalmol 1983; 28:1–19.

2.Hoskins HD, Shaffer RN. Evaluation techniques for congenital glaucoma. J Pediatr Ophthalmol Strabismus 1971; 8:81–87.

3.Litinsky SM, Shaffer RN, Hetherington J, Hoskins HD. Operative complications of goniotomy. Trans Am Ophthalmol Otolaryngol 1977; 83:78–79.

4.Wheeler DT, Stager DR, Weakley DR Jr. Endophthalmitis following pediatric intraocular surgery for congenital cataracts and congenital glaucoma.

J Pediatr Ophthalmol Strabismus 1992; 29:139–141.

5.SaintMaurice C. Paediatric anaesthesia. Curr Opin Anaesthesiol 1994; 7:249–250.

6.Tiret L, Nivoche Y, Hatton R, Desmonts JM, Vourch G. Complications related to anesthesia in infants and children: a prospective survey of 40 240 anaesthetics. Br J Anaesthesiol 1988; 61:263–269.

7.Keenan RL, Boyan CP. Cardiac arrest during anesthesia: a study of incidence and causes. JAMA 1985; 253:2373–2377.

8.Laycock GJ, McNicol LR. Hypoxaemia during recovery from anaesthesia: an audit of children after general anaethesia for routine elective surgery. Anesthesiology 1988; 43:985–987.

9.Cohen MM, Cameron CB. Should you cancel the operation when a child has an upper respiratory tract infection? Anesth Analg 1991; 72:282–288.

10.Tait AR, Knight PR. The effects of general anesthesia on upper respiratory tract infections in children. Anesthesiology 1987; 67:930–935.

11.Smith RM. Mortality in pediatric surgery and anesthesia. In: Smith RM, ed. Anesthesia for infants and children, 4th edn. Mosby: St Louis; 1980:653–661.

12.France NK. Ophthalmological diseases. In: Katz J, Steward DJ, eds. Anesthesia and uncommon pediatric disease. WB Saunders: Philadephia, PA; 1987:271–298.

13.Guo S, Nelson LB, Calhoun J, Levin A. Simultaneous surgery for bilateral congenital cataracts. J Pediatr Ophthalmol Strabismus 1990; 27:23–25.

14.Zwaan J. Simultaneous surgery for bilateral pediatric cataracts. Ophthalmic Surg Lasers 1996; 27:15–20.

15.Jaffe NJ. Cataract surgery and its complications. CV Mosby: St Louis; 1990:499.

16.Good WV, King S, Irvine AR, Hoyt CL, Taylor DSI. Postoperative endophthalmitis in children following cataract surgery. J Pediatr Ophthalmol Strabismus 1990; 27:283–285.

17.Kushner BJ. Simultaneous surgery for bilateral congenital cataracts (discussion). J Pediatr Ophthalmol Strabismus 1990; 27:26–27.

18.Duthie OM. Bilateral cataract extraction. Trans Ophthalmol Soc UK 1955; 75:25–31.

19.Akingbehin T, Sunderraj P. Simultaneous bilateral lens implantation: is the procedure justified? Eur J Implant Refract Surg 1992; 3:131–133.

20.Harfitt R, Moriarty A, Mastellone G. Is simultaneous bilateral cataract extraction with lens implantation justified? Eur J Implant Refract Surg 1992; 3:134–137.

21.Adhikary HP, Harrington L. Simultaneous bilateral intraocular lens implantation. Eur J Implant Refract Surg 1992; 3:138–139.

22.Nielsen PJ. Simultaneous bilateral cataract extraction: ECCE versus ICCE. Eur J Implant Refract Surg 1992; 3:140–144.

23.Joseph N, David R. Bilateral cataract in one session: report on five years experience. Br J Ophthalmol 1977; 61:619–621.

24.Fenton PJ, Gardner ID. Simultaneous bilateral intraocular surgery. Trans Ophthalmol Soc UK 1982; 102:298–301.

25.Jardine P. Simultaneous bilateral cataract extraction. Trans Ophthalmol Soc UK 1970; 70:719–724.

26.Shepard DD. Are there any indications for simultaneous bilateral cataract– intraocular lens (IOL) surgery? J Cataract Refract Surg 1988; 14:339–345.

27.Hugkulstone CE, Stevenson L, Vernon SA. Simultaneous bilateral trabeculectomy. Eye 1994; 8:398–401.

28.Mandal AK, Bhatia PG, Gothwal VK, et al. Safety and efficacy of simultaneous bilateral primary combined trabeculotomy-trabeculectomy for developmental glaucoma. Indian J Ophthalmol 2002; 50:13–19.

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Chapter 12

Management of refractory pediatric glaucoma

Introduction

Filtration surgery with antifibrosis drugs

Glaucoma drainage implants

Cyclodestructive procedures

Laser therapy

Conclusions

Introduction

Surgical treatment is usually required for developmental glaucoma, using goniotomy or trabeculotomy as primary surgical therapy. Both of these procedures have a high success rate, are equally effective as initial surgical treatment, and have been assessed in large numbers of patients over long periods of time.1,2 However, at least 10% to 20% of patients fail the initial surgical procedure for congenital glaucoma, and some patients have glaucomas with a poor prognosis for the success of initial goniotomy or trabeculotomy. When the intraocular pressure is not controlled after primary surgery, the next step varies according to individual patient factors and surgeon preferences. The surgical options available to treat these children include filtering surgery, glaucoma drainage implants, and cyclodestructive procedures.

Filtration surgery with antifibrosis drugs

Children or young adults undergoing filtering surgery do not enjoy the same success rate compared with older age groups. The barriers to success of filtering surgery in children include a thick and active Tenon’s capsule, rapid wound healing response, lower scleral rigidity, and a large buphthalmic eye

with thin sclera.3 Additionally, conjunctival scarring from previous ocular surgery may limit the success of repeat surgery in children with congenital glaucoma. Trabeculectomy without antifibrosis drugs in young patients has been unsuccessful in most,3–6 but not all,7 reports.

Antifibrosis drugs have been widely used in adult glaucoma filtering surgery to improve the success rate and produce lower mean postoperative intraocular pressures. The initial experience with antifibrosis drugs as adjunctive treatment for trabeculectomy was with postoperative subconjunctival 5-fluorouracil (5-FU) injections. Adjunctive use of 5-FU has been described in young patients, with some success in achieving intraocular pressure levels in the low teens.8,9 However, 5-FU has disadvantages, including the need for multiple subconjunctival injections (requiring multiple general anesthesias in children) and the possibility of ocular complications such as hypotony and recalcitrant corneal epithelial defects. Also, a small, prospective, randomized trial showed that 5-FU was less effective compared with mitomycin-C in achieving successful control of intraocular pressure in pediatric filtration surgery.10

Mitomycin-C has emerged as an effective antimetabolite for topical use during trabeculectomy (Fig. 12.1). It is an anti-

neoplastic antibiotic isolated from the fermentation filtrate of Streptomyces caespitosus, which has the ability to

significantly suppress fibrosis and vascular ingrowth after intraoperative application at the site of filtration surgery. Mitomycin-C is a more potent antifibrosis drug compared with 5-FU. In adults, eyes treated with mitomycin-C have lower mean intraocular pressure on fewer medications compared with eyes treated with 5-FU. Also, mitomycin-C is administered in a single intraoperative application, which is more convenient for the patient and surgeon compared with 5-FU.

Figure 12.1 Trabeculectomy with mitomycin-C. This child had been treated previously with trabeculectomy without adjunctive antifibrosis treatment, which had failed to control the intraocular pressure (A). The postoperative appearance shows the characteristic thin-walled, avascular bleb after trabeculectomy with mitomycin-C (B).

A B

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