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Nucci P (ed): Pediatric Cataract.

Dev Ophthalmol. Basel, Karger, 2016, vol 57, pp 85–106 (DOI: 10.1159/000442505)

Complex Cases in Pediatric Cataract Preeti Patil-Chhablani a Ramesh Kekunnaya a Kanwal Ken Nischal b a

L V Prasad Eye Institute, Hyderabad, India; b Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, Pa., USA

Abstract This chapter describes the techniques and pitfalls that the reader may come across when dealing with complex pediatric cataract cases. Each eye in these circumstances is unique, and the examples and general advice shared are intended to help the reader develop a plan for surgery and a road map to avoid potential problems. As in all types of surgery, careful planning is essential. The old saying ‘Fail to prepare, then you prepare to fail’ is no more true than when dealing with children who have complex cataract. In this chapter, the following circumstances where pediatric cataract may be seen are discussed: retinoblastoma, retinopathy of prematurity, lenticonus, congenital rubella syndrome, trauma, microcornea, pediatric uveitis, Hallermann-Streiff syndrome, Stickler syndrome, Lowe syndrome, subluxated lens, and after previous intraocular surgery (glaucoma, keratoplasty). © 2016 S. Karger AG, Basel

Children with retinoblastoma may develop cataracts as sequelae of the management of the primary disease by external beam radiotherapy. Cataracts may also be a manifestation of the primary disease processes itself [1, 2]. Surgical management is indicated not only for visual rehabilitation but also to improve visualization of the tumor and to aid further monitoring and treatment. Cataract surgery is challenging in patients with retinoblastoma due to several factors. Since tumor recurrence may be seen after intraocular surgery, tumor quiescence must be ensured before planning cataract surgery. The improvement in visual acuity is not dependent upon cataract surgery alone and is influenced by the extent of retinal/macular involvement and amblyopia.

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Retinoblastoma

Preoperative Considerations Although there is no consensus about the optimal length of quiescence of the tumor before intraocular surgery, the longer the period of quiescence, the lesser are the chances of tumor recurrence. A minimum period of 9–12 months is desirable prior to surgery [3]. A careful fundus examination is a must prior to surgery, not only to examine the tumor but also to note areas of local retinal detachment, vitreous adhesions, etc. If fundus examination is not possible due to lenticular opacity, an ultrasound B-scan is essential prior to planning surgery. A team approach is desirable, and it is best for the surgeon to discuss the need to perform a primary posterior capsulotomy (PPC), anterior vitrectomy (AV) and intraocular lens (IOL) implantation with the treating oncologist.

Parental counseling prior to surgery is of extreme importance. A realistic discussion about the need for cataract surgery, the possibility of a recurrence, need for further adjuvant chemotherapy/radiation, the visual prognosis and the rare possibility of enucleation is a must before undertaking the procedure.

Postoperative Care and Results Extensive use of lubricants in the postoperative period is mandatory, as there is a high chance of dry eye in these children. Most studies have shown good surgical results

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Dev Ophthalmol. Basel, Karger, 2016, vol 57, pp 85–106 (DOI: 10.1159/000442505)

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Surgical Procedure A higher rate of recurrence has been noted in eyes where a pars plana approach was taken. Hence, a clear corneal approach is preferable [4]. A clear corneal incision is also easily visible. The chances of visualization of subconjunctival seeding are higher when a scleral incision is avoided.

A large anterior capsulorrhexis is preferred in order to avoid capsular phimosis so that the view of the posterior segment is not hindered. However, it is still best to make the anterior opening smaller than the optic size to avoid later anterior displacement of the IOL. If phimosis is suspected within 8 weeks of primary surgery, radial cuts can be made using a YAG laser (in older children) or surgically back in the operating room in younger children. A PPC is best avoided in older children. In situations where it must be done (such as in young children or in the presence of a posterior capsular plaque), a manual capsulorrhexis is preferred (online suppl. video 1; for all online suppl. material, see www.karger.com/doi/10.1159/000442505).

The vitreous face should be left untouched wherever possible to decrease the possibility of dissemination of vitreous seeds. If a vitrectomy is performed, it is best to send a vitreous sample for histopathological analysis to look for tumor cells in the vitreous. This information must be communicated to the treating oncologist as early as possible. There are no specific contraindications for implanting an IOL under such conditions. In contrast, such patients are poor candidates for contact lens wear due to dry eye caused by radiation therapy, and hence, IOL implantation is preferred.

after cataract surgery in eyes with retinoblastoma. The recurrence rates reported in the literature range from 0 to 45% [3]. The ultimate visual outcome is dependent upon the location and size/stage of the retinoblastoma, visual rehabilitation, prompt refractive correction and amblyopia management.

Cataract in Retinopathy of Prematurity

Cataracts in eyes with retinopathy of prematurity (ROP) either are associated with the disease or are sequelae of treatment (laser photocoagulation and/or vitreoretinal surgery) for the ROP [5].

Surgical Considerations Scenario 1: Infant with Opaque Lens Secondary to Laser Complication or Anti-Vascular Endothelial Growth Factor Injection In these circumstances, the role of anesthesia is crucial. The infant should be paralyzed and have an appropriately low end-tidal CO2 to ensure that there is minimal positive pressure during the surgery. In these cases, there are often posterior synechiae present, so the viscoelastic is used to ensure that these synechiae are adequately broken. In these infants, the eyes are small, so if iris hooks are used, too many entry wounds for a small corneal circumference are needed; it is best to instead use an instrument like a Kuglen hook to retract or push the iris away from the vitrector or aspirating hand piece. To ensure as rapid a surgery as possible (these

Complex Cases in Pediatric Cataract

Nucci P (ed): Pediatric Cataract.

Dev Ophthalmol. Basel, Karger, 2016, vol 57, pp 85–106 (DOI: 10.1159/000442505)

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Preoperative Considerations If a cataract is the result of complications from laser therapy or inadvertent needle injury during anti-vascular endothelial growth factor injection, surgery may well need to be done early due to the development of an opaque lens. In this scenario, when the infant may be at term based on the postgestational age and yet 16 weeks postnatal, the need for rapid, efficient surgery is mandatory. However, if the cataract develops later, and even months later, the considerations for surgery change, and in such cases, the eyes tend to be myopic (axial and lenticular) and may have higher-than-average axial lengths. Careful biometry is a must in these eyes. The cataract must be examined meticulously; one must look for the presence of posterior capsular dehiscence (which may preexist due to intraoperative lens touching during previous vitreoretinal surgery), zonular weakness and the extent of pupillary dilatation. All these factors should be taken into consideration while formulating a surgical plan. The parents of the child must be given a realistic idea about the extent of visual acuity that the child is expected to gain after surgery. The presence of retinal scarring, macular drag, extreme myopia, amblyopia, etc. may compromise the final visual acuity after successful cataract surgery. Of course, the younger the infant is at surgery, the more visual rehabilitation will play a role in the final outcome.

infants often have other co-morbidities), a vitrector is used (150–200 mm Hg vacuum, 500 cpm cut rate) to create a large capsulotomy and aspirate the lens material. Attempting to place an IOL in these circumstances is not recommended; instead, capsule-sparing lensectomy needs to be performed to leave enough support for a secondary IOL later. If the iris is very inelastic because of the synechiae, it is worth considering mechanical enlargement of the pupil. Turning the vitrector cutter port face down, with the cutter ‘off’, breaches the posterior capsule. The capsule is aspirated into the port (it is seen to wrinkle), and at that moment, the cutter is turned ‘on’, thereby breaching the capsule. A posterior capsulotomy is performed, and an AV is also performed. The wounds are closed with 10/0 Vicryl, and it is worth placing either some intracameral preservative-free dexamethasone (2 mg) or some intracameral diluted preservative-free (10:1) triamcinolone (0.2 ml total volume).

Scenario 2: Older, Systemically Stable Child (>6 Months) Scleral rigidity may be low in this child’s eyes, and a high-molecular-weight viscoelastic material is desirable to maintain the anterior chamber while performing an anterior capsulorrhexis. In cases where previous retinal surgery has been done, silicon oil bubbles may be present in the anterior chamber. It is best to wash these bubbles out before beginning the surgery. A large anterior capsulorrhexis is preferred in order to facilitate a complete fundus evaluation in the future. Hydrodissection is best avoided since there may be weak areas in the posterior capsule that may not be evident preoperatively. The irrigating fluid flow rate/bottle height should be low. Primary capsulorrhexis, where required, must be larger. In cases where AV is required, a high-speed vitrector is desirable. It is best to limit vitrectomy to the anterior vitreous and avoid disturbing the core vitreous since these eyes often have dense peripheral vitreoretinal adhesions and are predisposed to developing retinal breaks. IOL implantation is preferred when there is adequate capsular support and in cases where the size of the eyeball (corneal diameter) allows IOL insertion [6].

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Dev Ophthalmol. Basel, Karger, 2016, vol 57, pp 85–106 (DOI: 10.1159/000442505)

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Postoperative Concerns The development of a retinal detachment postoperatively is one of the primary concerns in these children. Examination under anesthesia (EUA) is often needed in order to examine the fundus completely and with scleral indentation. Appropriate refractive correction is required as early as possible, in the form of either glasses or contact lenses, and amblyopia treatment, especially in unilateral cases, is the key to good visual acuity postoperatively. In the infant under 6 months of age, the risk of glaucoma must be kept in mind, and any signs of shallow anterior chambers must have the possibility of aqueous misdirection excluded.

Lenticonus

Lenticonus is a condition where there is an abnormal conical configuration of the lens capsule, and it is usually congenital but may be developmental, presenting later in early childhood. It may involve the anterior capsule (anterior lenticonus) or the posterior capsule (posterior lenticonus) or both. Anterior and posterior lenticonus should be considered as separate clinical entities. While anterior lenticonus often has systemic associations, posterior lenticonus is usually sporadic and is an isolated ocular finding.

Anterior Lenticonus

Preoperative Considerations Unless the anterior segment is examined carefully with a slit lamp, lenticonus may be difficult to detect and might be missed. In very young children, a handheld slit lamp will be needed to avoid missing the conical configuration of the lens and its capsule.

Anterior lenticonus is associated with Alport syndrome in more than 90% of cases [7]. Alport syndrome is a hereditary disease that primarily affects the kidneys and the auditory and visual systems. Alport syndrome may have a variable mode of inheritance, although it is predominantly X linked, and hence, the phenotype also differs.

The other ocular manifestations are predominantly retinal, with fleck retinopathy being the commonest retinal finding. A comprehensive ophthalmic evaluation is hence mandatory. In addition, where Alport syndrome is suspected, one must perform a urine analysis to look for hematuria and proteinuria. Auditory testing must also be carried out to detect sensorineural hearing deficits.

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Surgical Procedure The anterior capsule may be extremely elastic in these cases and has a high tendency to tear easily. Capsulorrhexis is challenging in such cases, and care must be taken to make small initial nicks in the thinned-out area of the capsule and then extend the capsulorrhexis. Another option is to use the two-incision push-pull rhexis technique, making the two initial stabs on either side of the cone, thereby avoiding the conic area altogether when removing it [see chapter by Nischal, this vol., pp. 15–28]. A highmolecular-weight viscoelastic is highly recommended in these cases [8]. Hydrodissection is best avoided since there are reports of anterior and posterior lenticonus being associated with Alport syndrome [9, 10], and this may result in posterior capsular rupture during hydrodissection. Spontaneous rupture of the anterior capsule in absence of trauma suggests anterior lenticonus (online suppl. video 2).

The posterior capsule may be fragile, and it is best to avoid capsule polishing in such cases [8]. Careful insertion of the IOL must be carried out to avoid unnecessary

stress on the capsulorrhexis margin. Descemet membrane detachment has also been reported with Alport syndrome [8], and hence, all intraocular maneuvers must be carried out with utmost care.

Postoperative Results It is important to remember that anterior lenticonus itself can cause a myopic shift, even in the absence of a cataract. These patients may have amblyopia, which may not be diagnosed preoperatively and can result in a lower visual acuity than expected. Patients must be counseled accordingly.

Posterior Lenticonus

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Surgical Technique Hydrodissection procedures must be avoided in order to prevent enlargement of the preexisting capsular defect or creation of an iatrogenic rupture in a thinned-out area of the capsule. Sometimes, the lens will be white and opaque. It is therefore necessary to always assume that there is a posterior defect in such cases and to both avoid hydrodissection and always aspirate the peripheral cortical lens matter first [see chapter by Nischal, this vol., pp. 15–28]. Use of an ultrasound B-scan can often diagnose the posterior lenticonus in such cases.

In some cases, posterior lenticonus may be diagnosed intraoperatively, especially in the presence of a total cataract. The presence of a ‘fish-tail sign’ is very specific to posterior lenticonus. Careful inspection of the posterior capsule during surgery is a must [11, 13]. The peripheral cortex is aspirated first, followed by the nucleus (online suppl. video 3). Vitrectomy must be performed before IOL implantation. IOL implantation may not be possible in all cases. In the presence of a small central defect with adequate posterior capsular strength, in-the-bag placement of the lens can be attempted with success [13]. In cases with large posterior capsular defects, sulcus placement of the IOL may be necessary, but often, the implant can still be placed in the bag. However, this

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Preoperative Considerations Posterior lenticonus is usually sporadic and is not associated with systemic diseases.

It is usually congenital, but a cataract may develop at a later date due to spontaneous rupture of the capsule or following trivial trauma.

A careful slit-lamp evaluation is necessary to note the size and extent of the lenticonus and the extent of the capsular defect, if any. Early signs of a lenticonus include an ‘oil droplet’ lenticular reflex (fig. 1) and a variable ‘scissoring’ retinoscopy reflex [11]. The cortex overlying the defect undergoes progressive opacification and may show chalk-like deposits, which eventually lead to cataractous changes in the entire lens [11, 12]. An ultrasound B-scan may help to detect the presence of a defect or posterior bulging of the posterior capsule.

Fig. 1. Two cases of posterior lenticonus. Both show an ‘oil droplet’ sign. The picture on the left shows some adjacent lens matter changes.

a

b

e

f

c

g

d

h

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Nucci P (ed): Pediatric Cataract.

Dev Ophthalmol. Basel, Karger, 2016, vol 57, pp 85–106 (DOI: 10.1159/000442505)

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Fig. 2. Collage showing how to place an intraocular lens when the posterior opening is larger than the anterior opening in a case of posterior lenticonus. a The white arrows show the edge of the posterior rhexis, while the black arrows show the anterior rhexis. The leading haptic is just being placed into the bag. b The leading haptic is in the bag, with the optic unfolded in the anterior chamber outside the bag. The trailing haptic is outside the eye. c, d Use of a Kuglen hook to nudge the optic into the bag, with the optic hugging the anterior capsule as it is nudged. Note the position of the leading haptic (black arrow); this haptic will not rotate during the whole manoeuver. e The optic is in the bag and the trailing haptic is still outside the eye. f Using a nontoothed angled forceps (here, a McPherson), the trailing haptic is grasped (black star). g Without rotating the implant (note the position of the leading haptic; black arrow) the trailing haptic is placed into the bag. h The intraocular lens is in the bag. The leading haptic has not rotated at all (black arrow).

necessitates first understanding that the after the leading haptic is placed in the bag, the optic must be opened in the anterior chamber, and not in the bag. The trailing haptic now sits outside the eye. The optic is nudged into the bag, hugging the anterior capsule opening as it is nudged, using a Sinskey hook or a Kuglen hook. The IOL cannot be rotated due to the posterior defect, so the trailing haptic is placed in the bag using a McPherson or other nontoothed angled forceps (fig. 2). The viscoelastic anterior to the IOL can be removed very carefully (the bottle height of the infusion must be very low), but the viscoelastic behind the IOL must remain in situ. To prevent an intraocular pressure (IOP) spike, acetazolamide 4–7 mg/kg is given stat intravenously at the end of the procedure and then three times daily orally for up to 2 days.

In the authors’ personal experience, in certain cases, the opacification may extend into the vitreous and involve the anterior hyaloid face. The cortex may be adherent to the capsule and the adjacent vitreous. Careful aspiration of the lenticular material with adequate vitrectomy is desirable in such cases, taking care to remove all the opacified vitreous and to not extend the preexisting capsular defect.

Postoperative Results Most cases are associated with amblyopia. However, good results may be obtained with timely intervention and appropriate refractive correction postoperatively, followed by amblyopia therapy, especially in eyes with acute-onset total lenticular opacification [13].

Congenital Rubella Syndrome

Preoperative Considerations CRS remains one of the commonest causes of congenital cataracts in developing countries, and a high index of suspicion must be maintained to diagnose this condition. We advise serological testing for rubella virus (IgM and IgG antibodies) in all infants presenting with congenital cataracts in the absence of a definite familial history or any other ocular or systemic feature pointing to a different etiology. A detailed systemic examination by an experienced pediatrician and pediatric anesthetist is necessary prior to surgery, as children with CRS may have cardiac defects and are highrisk cases for general anesthesia. Many of these eyes have associated microcornea or microophthalmos. A meticulous anterior segment examination is a must. CRS may result in unilateral cataract, although the large majority of cases are bilateral. Most of these cataracts are nuclear in origin [14, 15]. Preoperative measurement of the corneal diameter, IOP and pachymetry is a must in these children.

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Congenital rubella syndrome (CRS) remains a major public health concern in developing countries even today, and concerns of outbreaks in certain developed countries have also raised alarm. CRS is associated with congenital cataract, congenital cardiac defects, sensorineural hearing loss and microcephaly.

Surgical Procedure Many of these eyes are associated with small pupils and may require the use of pupildilating hooks or pupilloplasty intraoperatively. A continuous curvilinear anterior capsulorrhexis should be performed where possible. In cases where a manual capsulorrhexis is not possible, a vitrectorrhexis may be performed (online suppl. video 4).

Complete and meticulous aspiration of the lenticular material is of utmost importance since the lens is known to harbor the live rubella virus. Persistence of lenticular matter not only may result in possible visual axis opacification but also may cause significant postoperative inflammation. A PPC and an AV are a must in young children. Implantation of an IOL is best avoided in eyes with microcornea or microophthalmos. In-the-bag IOL implantation is preferred where possible, but caution is advised if there are signs of intraocular inflammation.

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Postoperative Course Immediate postoperative inflammation remains a concern in these eyes. We recommend aggressive topical steroid therapy along with concurrent cycloplegics and antibiotics. Eyes with rubella cataracts commonly develop a transient corneal haze postoperatively, which usually clears in a period of 2–3 weeks [15]. This is probably due to rubella-virus-related endotheliopathy. The use of intracameral steroids as described above is recommended.

Secondary glaucoma has been reported and requires constant monitoring [15, 16].

Additionally, many of these eyes show the presence of salt-and-pepper retinopathy.

EUA is a must in the follow-up care of these children, and we recommend that EUA be performed at a frequency of every 3 months in the first year and every 6 months thereafter until the child reaches 5 years of age.

The final visual prognosis is determined not only by the cataract surgery but also by the subsequent refractive and amblyopia management and associated neurodevelopmental deficits and may remain suboptimal [15, 17].

Traumatic Cataracts

Preoperative Considerations A thorough ocular evaluation is necessary before planning surgery. In certain cases of penetrating trauma, the entry wound may be fairly obvious. One must, however, bear in mind that small corneal and scleral perforations may be hidden, and a careful ocular examination must be performed to detect such injuries. One must note the extent and depth of the entry wound; iris incarceration; pupillary tears; anterior synechiae; the status of the anterior and posterior lens capsule; the presence and degree

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Traumatic cataract can be a result of both blunt and penetrating trauma, and surgery can be extremely challenging due to heterogeneous presentation.

of anterior chamber inflammation; vitreous involvement; subluxation of the lens; and the presence of posterior segment pathology, such as vitreous hemorrhage or retinal detachment, if any. Subtle clues, such as the presence of a bullous subconjunctival hemorrhage, chemosis, an excessively deep anterior chamber, a peaked pupil, etc., may suggest an occult globe rupture in the absence of an obvious penetration [18]. Visual acuity estimation, pupillary examination to detect an afferent pupillary defect, IOP measurement (where possible) and fundus examination should be attempted. An ultrasound B-scan must be done in cases where the lenticular opacity precludes a view of the posterior segment. Ultrasound biomicroscopy of the anterior segment may be of use to detect posterior capsular rupture or subluxation of the lens [19]. Ocular inflammation and raised pressure must be managed medically before attempting surgery. Open globe injuries must be managed first, with repair of the corneoscleral tears taking priority over management of the cataract. It is also prudent to realistically discuss the goals of the surgery and the visual prognosis in detail with the patient.

Postoperative Concerns Eyes with traumatic cataract usually present with severe postoperative inflammation.

Use of hourly topical steroids and cycloplegics is of extreme importance. Selected

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Surgical Procedure While most cases of traumatic cataract can be managed via the anterior approach, a posterior pars plana approach must be considered in the presence of extensive subluxation or posterior dislocation of the lens or large posterior capsular tears with prolapse of the lenticular matter into the vitreous cavity and in cases with associated vitreoretinal pathology. In cases with anterior synechiae, synechiolysis must be performed using intraocular microscissors. The presence of an underlying anterior capsular defect must be considered. If possible, an anterior capsulorrhexis must be attempted (online suppl. videos 5–7). In cases with capsular rupture, one must try to keep at least part of the capsular bag intact by performing an eccentric capsulorrhexis. In the case that vitreous is present in the anterior chamber, vitrectomy may be required prior to performing a capsulorrhexis. Hydrodissection should be avoided in cases where the integrity of the posterior capsule is in doubt. The lens must be aspirated carefully, avoiding any unnecessary stress on the zonules. Placement of the IOL is dependent upon the status of the posterior capsule and the zonules. A capsular tension ring may be used in cases with subluxation. Where this is not possible, a lensectomy with a scleral fixated or glued IOL may be required [20, 21]. In cases where the anterior capsular opening is much larger than the optic or where the integrity of the pupil sphincter is breached and it is clear that the IOL may not remain in the bag, it is worth performing posterior optic capture after a moderate AV. This keeps the lens centered and prevents forward displacement of the optic with subsequent iris lens capture.

cases may require a short course of oral steroids in addition to a subconjunctival steroid-antibiotic injection given at the end of surgery. If the cataract surgery is some weeks after the initial trauma and infection has been excluded, orbital-floor triamcinolone (1 mg/kg) should be considered in addition to subconjunctival steroids, which can obviate the use of systemic steroids. Visual prognosis is determined based on the time lapse between injury and treatment, the presence of posterior segment pathology and postoperative visual rehabilitation and amblyopia therapy.

Microcornea

Microcornea is defined as a corneal diameter less than 9 mm at birth. It may or may not be associated with microophthalmos. It may be associated with other ocular abnormalities, such as persistent fetal vasculature, aniridia, cornea plana and cataract. It is commonly seen as a part of CRS.

Surgical Procedure Intraocular surgery often becomes difficult in extremely small eyes (corneal diameter less than or equal to 8 mm). Careful handling is required in order to minimize any chance of damaging the corneal endothelium. Poor pupillary dilatation is often seen in these eyes, and pupilloplasty or iris hooks may be required. Use of high-molecularweight viscoelastic materials is helpful in maintaining the anterior chamber depth to aid manipulations. IOL implantation is best avoided in these eyes. In the authors’ experience, the entire surgery, including the anterior capsulorrhexis, lens aspiration, posterior capsulotomy and vitrectomy, may be completed with the help of an automated vitrector. This prevents the entry of multiple instruments into the eye, thus minimizing endothelial damage, and also reduces the operating time. A large anterior

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Preoperative Considerations Since microcornea may be associated with other ocular and systemic abnormalities, and especially CRS, appropriate testing must be carried out for these conditions. Preoperative EUA must include measurement of the IOP, central corneal thickness (CCT), corneal diameter and axial length. Eyes with microcornea are often associated with an increased corneal thickness [22]. The decision as to whether an implant can be placed or not is dependent on whether the capsular bag is large enough. Using a linear ultrasound probe (these are most often available from anesthetists, who use them to locate veins for difficult cannulation), the equator-to-equator length of the lens is measured, and 1 mm is added to this to give the total capsular bag diameter.

For an IOL with a 12.5 mm total diameter (from the end of the haptic to the end of the haptic), a minimum of an 8 mm capsular bag diameter is needed. If the IOL is a foldable one, this still holds true. For a rigid one-piece IOL, the capsular bag diameter needs to be 9 mm for an IOL with a 12.5 mm diameter.

Fig. 3. Postoperative aphakia in a child who had microcornea and cataract in both eyes.

and posterior capsulotomy is desirable (online suppl. videos 8, 9). In those cases where an IOL is placed, the axial length must be normal or longer than normal. A small cornea with a small axial length is a contraindication for IOL implantation.

Postoperative Concerns If only a lensectomy has been performed, the use of a strong cycloplegic, such as atropine 0.5 or 1%, depending on age, is recommended in the immediate postoperative period (fig. 3). Development of glaucoma remains a concern [23, 24], and regular EUA with measurement of the corneal diameter, IOP, CCT, axial length and refractive error is recommended.

Pediatric Uveitic Cataract

In children, juvenile rheumatoid arthritis is one of the most common causes of intraocular inflammation and is associated with secondary complications, such as cataract formation, glaucoma and band-shaped keratopathy [25].

Preoperative Considerations Management of the ocular inflammation preoperatively remains the single most important determinant of the ultimate surgical and visual outcomes. It is recommended that the disease should be quiescent for at least 3 months prior to intraocular surgery [25, 26], but even this may not be long enough. In addition to topical steroid therapy, oral steroids, oral immunosuppressive agents or periocular steroid injections can help to achieve preoperative control of inflammation [25]. However, one must consider the difficulty of giving periocular injections in children. The distinct possibility of repeated membrane formation and visual axis opacification if an IOL is placed must be clearly explained to the parents (fig. 4).

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Fig. 4. Juvenile idiopathic arthritis that had been quiescent for 2 years, with no uveitis, in a 7-yearold girl who was on methotrexate. She had a cataract extraction with a primary posterior capsulorrhexis and anterior vitrectomy, intracameral steroids and oral steroid taper postsurgery. She still developed recurrent anterior membranes (black arrow), 360-degree synechiae (white arrows) and iris bombe (white stars).

Surgical Procedure Posterior synechiae and poor pupillary dilatation, fibrinous membranes over the lens and unpredictable capsular elasticity can make an anterior capsulorrhexis difficult and adherent, precluding adequate exposure to the lens and thereby making surgery challenging (online suppl. video 10). A PPC and an AV are an absolute must in all children with this type of cataract, since the propensity of these eyes to develop inflammation results in a much higher chance and a faster rate of developing posterior capsular opacification. Implantation of an IOL in such eyes remains controversial.

Traditionally, IOL implantation was thought to increase the postoperative inflammation, resulting in poor visual outcomes, with a chance of phthisis. However, recent studies have shown encouraging results with IOL implantation in eyes with uveitis [27, 28]. It is, however, important to remember that intensive monitoring and appropriate control of perioperative inflammation are of paramount importance in these cases. Hydrophobic, acrylic IOLs are the preferred type of lenses in these cases [29]. Whether an IOL is placed or not, intraocular steroids must be given, with preservative-free dexamethasone given anteriorly and preservative-free triamcinolone given posteriorly.

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Postoperative Concerns The chances of postoperative complications are higher in these eyes, and monitoring is recommended. Some of the dreaded complications include intense postoperative inflammation, glaucoma, retrolenticular and anterior membranes, epiretinal membranes, cystoid macular edema, band-shaped keratopathy and phthisis bulbi. A multispecialty approach is desirable to successfully manage these cases and gain good visual outcomes.

Syndromic Cataracts

Preoperative Considerations While an ophthalmic examination is definitely a must, a complete physical evaluation of a child presenting with bilateral cataracts is desirable, with special emphasis on facial features, skeletal and dental abnormalities, dermatologic examination, mental growth and milestone assessment and genitourinary abnormalities. Appropriate genetic testing may aid in reaching a definite diagnosis.

Hallermann-Streiff syndrome is characterized by microcephaly, hypotrichosis, microphthalmia, cataracts, a beaked nose, micrognathia, and short stature [31]. In addition to cataract, other ophthalmic associations described include eyelid abnormalities, corneal opacities, sclerocornea and retinal detachment [32–34]. These children may pose significant difficulty during administration of general anesthesia, and intubation is difficult. We recommend preoperative evaluation by an experienced pediatric anesthesiologist.

Stickler syndrome is an inherited connective tissue disorder due to mutations in collagen genes. Ocular findings include myopia, cataract, retinal detachment, circumferential lattice degeneration, radial perivascular chorioretinal degeneration and glaucoma. Other features include hearing loss (both conductive and sensorineural), midfacial underdevelopment and cleft palate (either alone or as part of the Robin sequence) and mild spondyloepiphyseal dysplasia and/or precocious arthritis [35, 36].

A careful fundus examination (wherever possible), a detailed family history and examination of the family members are necessary. Again, difficult intubation during general anesthesia should be anticipated.

Lowe syndrome, also known as the oculo-cerebro-renal syndrome of Lowe, is a multisystem, X-linked disorder caused by a mutation in the gene OCRL1 localized at Xq26.1. This syndrome is characterized by anomalies affecting the eye, the nervous system and the kidney. The cataract is usually bilateral and is present at birth. There is associated severe hypotonia present at birth and proximal renal tubulopathy (Fanconi type) that becomes evident in the subsequent weeks or months. Psychomotor retardation is evident in childhood, while behavioral problems and renal complications arise in adolescence [37]. Infants presenting with bilateral congenital cataracts and severe hypotonia need to be evaluated for Lowe syndrome. Urinary testing to detect proteinuria and proximal renal tubular acidosis and neurological evaluation are recommended. Invariably, these children will have concomitant glaucoma, which can be difficult to manage.

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Many cataracts in children are not an isolated abnormality, but rather are a part of a larger multisystem affliction. Cataracts related to genetic syndromes are generally bilateral. While a large number of conditions are associated with congenital cataracts [30], some of the common and peculiar conditions that require special surgical attention include Hallermann-Streiff syndrome, Stickler syndrome, and Lowe syndrome.

Fig. 5. A Child with Hallermann-Streiff syndrome wearing aphakic glasses following cataract surgery in both eyes.

Surgical Procedure Hallermann-Streiff syndrome is usually associated with membranous cataracts that may get absorbed over time if untreated. Some older children may present with aphakia. Anterior and posterior capsules may be in close approximation with each other, and it may be difficult to perform an anterior capsulorrhexis. Additionally, it may be difficult to implant the IOL in the bag, and sulcus fixation may be required. These eyes may or may not be associated with microcornea and microophthalmos, thus precluding IOL implantation (fig. 5).

Stickler syndrome is associated with extensive vitreous degeneration and a high risk of retinal detachment. Cataract surgery is challenging due to the lack of vitreous support. Careful lens aspiration or phacoemulsification with closed chamber fluidics is recommended (online suppl. video 11). Prophylactic cryotherapy may play a role in prevention of retinal detachment in these eyes [38], but the parents must be told of the possibility of a retinal detachment occurring during or soon after surgery.

Infants with Lowe syndrome require early cataract surgery. They usually have microcornea and microphakia. An iris hook or dilators may be required during cataract surgery. IOL implantation is safe in the absence of any other complication [39] (online suppl. video 12), but the development of glaucoma or the presence of glaucoma must be taken into consideration when making the decision to implant or not. It may be worth considering a secondary IOL after glaucoma is managed with a tube. In either case, the prognosis may be guarded.

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Postoperative Concerns Due to the varied systemic manifestations of these syndromes, a team approach is desirable in the management of such children. Careful monitoring for postoperative complications such as glaucoma and retinal detachment must be done.

Subluxated Lenses

The crystalline lens may be displaced from its position in the patellar fossa in the setting of various systemic and metabolic disorders. Acquired subluxation may be seen after trauma or intraocular surgery or in eyes with high myopia.

Surgical Procedure Extraction of the cataractous lens may be done via the anterior route or via the pars plana approach. If the anterior route is to be used, it is safer to remove the lens if at least one entry site is opposite the dislocation direction, e.g. if the lens is dislocated nasally, the wound should be temporal, so that when the lens material is being aspirated, the countertraction is against the strongest zonular area. If the lens is dislocated superiorly, two 8/0 nylon traction sutures are placed in the limbal cornea at the 3 and 9 o’clock positions, and the eye is rotated so that the lens is sitting toward the nose.

The wound made is temporally in that position, but when the eye is released from traction, the wound is actually at 6 o’clock (fig. 6).

In most of these cases, the degree of subluxation precludes in-the-bag fixation of an IOL. However, use of endocapsular rings with IOL implantation may be attempted in selected eyes with success [42]. Care must be taken to perform a complete vitrectomy with a high-speed cutter to ensure that there is no vitreous traction, which may cause retinal tears in these eyes. Scleral suture-fixated IOLs and, more recently, glued IOLs and iris-sutured IOLs have been implanted successfully in eyes with subluxated lenses in experienced hands [20, 21, 43], but the rates of late lens dislocations suggest that other means of visual rehabilitation should be considered. One such alternative is the iris clip lens [44–46], which has gained much favor in Europe and is now developing a following in the USA. Enclavation of the iris clip lens is fairly easy

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Preoperative Considerations Among the zonular diseases, the commonest is Marfan syndrome (MFS). The other associated diseases are Weill-Marchesani syndrome and homocysteinuria [40]. Less common conditions include sulfite oxidase deficiency, hyperlysinemia, molybdenum cofactor efficiency, aniridia, ectopia lentis et pupillae, Ehler-Danlos syndrome, and congenital glaucoma [41].

It is essential, therefore, to evaluate each child presenting with ectopia lentis in detail. A complete review of the family history and physical evaluation to look for signs of MFS, such as an increased arm span, arachnodactyly, and a high arched palate, or for those of Weill-Marchesani syndrome, such as short stature, brachydactyly and joint stiffness, must be performed. Cardiac evaluation to look for aortic dilatation or mitral valve prolapse, which are known to be associated with MFS, is also recommended. Serum and urine testing for homocysteine must be conducted. It is worth remembering that the anesthesia team especially needs to know about this because of a tendency toward thrombophilia.

s

s

Lens dislocated superiorly

n

n

a

b s

s

n

n

c

d

Fig. 6. Schematic showing how an eye with a dislocated/subluxated lens superiorly (a) can have 8/0 nylon traction sutures placed in the limbal cornea at 3 and 9 o’clock (b) and can then be rotated (c) so that the surgeon can approach the eye from a temporal incision (d; black line). s = Superior, n = nasal.

Postoperative Concerns Patients with MFS have a risk of developing glaucoma and retinal detachment and must be monitored. In cases where an IOL has been implanted, one must look out for decentration of the IOL. Other complications, such as cystoid macular edema and vitreous prolapse into the anterior chamber, have also been reported [42]. In eyes

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to learn (fig. 7), and it can be placed in a retropupillary fashion as well [45]. If the lens is extremely unstable, two 30 G needles can be used to impale the lens while it is removed (fig. 8). The overriding principle in operating on a dislocated lens is to ensure that no traction is being placed on the vitreous. In order to achieve this, all the lens material should be removed in an endocapsular fashion, with the port of the vitrector continually submerged in lens material inside the bag. This means that only a small opening is necessary to get into the bag and then remove the lens material. Once all the lens material is removed, the bag can be removed, but at this stage, a vitrectomy is also being done with the cutter ‘on’. A maximum vacuum of 150 mm Hg is advised, with a cut rate of at least 500 cpm. This technique is only viable if an IOL is not going to be placed in the bag.

Fig. 7. Picture showing an Artisan iris clip lens, even in a case with a large pupil. The patient had ectopia lentis et pupillae.

Fig. 8. In this case, the subluxated lens is very unstable, so two 30 G needles are being used to skewer the lens and keep it stable while it is removed using a vitrector.

where IOL implantation is not possible, appropriate aphakic correction with glasses or contact lenses must be prescribed along with amblyopia therapy, if required.

The risks with iris clip lenses include traumatic dislocation, but endothelial decompensation (unless after traumatic aphakia, in which case it is unclear whether the trauma or the implant is culpable) does not appear to be a significant issue, especially if the IOL is placed in a retropupillary fashion [44–46].

Cataracts may form in eyes that have undergone previous surgery and may be a direct result of prior intervention or may be related to the primary disease process itself. In either situation, performing a cataract surgery in these eyes is particularly challenging.

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Cataract Surgery after Previous Intraocular Surgery

Glaucoma Surgery Children with buphthalmos who have undergone previous trabeculotomy, trabeculectomy or valve implantation may require cataract surgery. Their eyes generally have larger axial lengths, and careful biometry preoperatively is recommended. We also recommend preoperative gonioscopy to ascertain the angle structure and measurement of the CCT and IOP. Intraoperatively, one can expect low scleral rigidity due to the thin sclera. A highmolecular-weight viscoelastic is recommended to maintain the anterior chamber prior to performing an anterior capsulorrhexis. Closed-chamber fluidics should be maintained during lens aspiration. Due to the axial elongation, zonular weakness may exist, and the stability of the bag must be ascertained before implanting the IOL. Decentration of the IOL has been reported after performing a PPC in the same sitting [47]; however, in our experience, a careful PPC after making sure that the zonules are intact can result in good outcomes (online suppl. video 13).

Postoperatively, regular IOP monitoring is a must. There is an increased risk of failure of the trabeculectomy after cataract surgery [48], and patients must be counseled accordingly. If a tube is present, it is essential that after the viscoelastic has been removed at the end of the procedure, the tube should be flushed with balanced salt solution (1 ml) to unblock any inadvertent viscoelastic tube blockage.

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Retinal Surgery

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Preoperative assessment of the cataract is important in these cases; one must look for preexisting zonular weakness, posterior capsular defects that may have occurred due to a lens touch during vitreoretinal surgery, and emulsified silicon oil in the anterior chamber.

Intraoperatively, the anterior capsule may be highly elastic in these cases, and there may be posterior synechiae or poor pupillary dilatation. It is best to wash out any silicon oil bubbles before using a viscoelastic. Excessive deepening of the anterior chamber is expected due to lack of vitreous support. Use of a high-molecularweight viscoelastic is recommended, and a cautious capsulorrhexis must be performed. The use of the two-incision push-pull rhexis technique can be very valuable in such circumstances, as the vector forces of the technique are always toward the center of the lens [see chapter by Nischal, this vol., pp. 15–28]. A lower infusion bottle height is preferred to guard against anterior chamber fluctuations, and a clear corneal incision is recommended to avoid difficulties associated with an unusually deep anterior chamber [50]. IOL implantation is not contraindicated, provided that there is adequate capsular support. A hydrophobic acrylic IOL is preferred. A PPC may be performed in younger children. A higher rate of intraocular complications, such as posterior capsular rupture, zonular dehiscence (online suppl. video 14) and nucleus drop, has been reported in such eyes [47].

Postoperative visual recovery may be limited by the underlying retinal pathology, and there remains a chance of retinal re-detachment [51].

Keratoplasty

References 1 Hasan SJ, Brooks M, Ambati J, Kielar R, Stevens JL: Retinoblastoma with cataract and ectopia lentis. J AAPOS 2003;7:425–427.

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Cataract formation is reported after penetrating and lamellar keratoplasty [50, 52].

Eyes that have undergone previous corneal transplantation need careful preoperative biometry due to the high astigmatism that is often associated with these cases. Additionally, preoperative pachymetry and endothelial cell counts are recommended to gauge the risk of subsequent graft failure [53].

During surgery, a scleral tunnel is recommended to avoid trauma to the cornea.

In the case that a corneal incision is used, a short incisional track must be made.

Use of a dispersive viscoelastic material or the ‘soft shell’ technique, which uses a combination of dispersive and cohesive viscoelastic, is preferred to coat the corneal endothelium and avoid trauma. A low infusion bottle height prevents excessive turbulence in the anterior chamber and thus avoids unnecessary endothelial trauma. Depending on the etiology necessitating keratoplasty, an IOL may or may not be indicated. In cases of developmental anomalies, such as Peters’ anomaly, in children under the age of 2 years, there is usually insufficient anterior chamber volume to allow safe implantation, so lensectomy usually suffices. If the anterior chamber is deep and of normal volume, in-the-bag implantation of the IOL is preferred. A PPC and vitrectomy may be performed in younger children, taking care to avoid additional endothelial trauma during vitrectomy and making sure that there is no vitreous persisting in the anterior chamber at the end of surgery (online suppl. video 15).

Control of postoperative inflammation is extremely important in order to minimize the chances of subsequent graft rejection. Intracameral preservative-free dexamethasone greatly improves immediate graft survival in the authors’ experience and should be considered. Hourly topical steroid eye drops along with cycloplegics must be prescribed; oral steroid therapy may be considered in high-risk cases.

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Kanwal Ken Nischal Children's Hospital of Pittsburgh of UPMC 4401 Penn Ave Pittsburg, PA 15224 (USA) nischalkk @ upmc.edu

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