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. 2024 Nov 11;15(3):419–427. doi: 10.4103/tjo.TJO-D-24-00034

Pediatric cataract surgery in congenital anterior segment dysmorphology: An overview

Goura Chattannavar 1, Adeline Shona Lasrado 1, Ramesh Kekunnaya 1,2,*
PMCID: PMC12456907  PMID: 40995308

Abstract

The purpose is to highlight the surgical modifications that are undertaken for a safe cataract surgery when associated with anterior segment dysmorphology. Surgical modifications that are undertaken in a case of congenital cataract complicated with congenital anterior segment dysmorphology such as microcornea, congenital corneal opacity, aniridia, anterior and posterior lenticonus of the lens, and persistent fetal vasculature are described. Careful preoperative assessment is crucial to anticipate and plan for intraoperative and postoperative challenges that may arise during cataract surgery in the presence of anterior segment dysmorphology. Surgical adjustments in cataract surgery when associated with anterior segment dysmorphology should be tailored to each individual case and the surgeon’s expertise.

Keywords: Anterior segment dysmorphology, cataract surgery, congenital cataract

Introduction

Cataract surgery in children poses challenges owing to the small size of the eye, less-rigid ocular coats, elastic anterior and posterior lens capsules. In addition, congenital cataracts can be associated with various anterior segment dysmorphology, such as microcornea, sclerocornea, cornea plana, anterior segment dysgenesis, aniridia, anterior and posterior lenticonus of the lens capsule, and persistent fetal vasculature (PFV) which makes the cataract surgery in children more challenging. The challenges faced during a cataract surgery in these eyes are small cornea, ocular surface problems, corneal opacity, poor view of anterior segment structures, iris and pupil abnormalities, shallow anterior chamber, anterior and posterior synechiae, etc., In this review, we highlight the surgical modifications that are undertaken for a safe cataract surgery when associated with anterior segment dysmorphology.

Pediatric Cataract Surgery in Corneal Abnormalities: Microcornea/Cornea Plana/Sclerocornea

Microcornea is a condition where the corneal diameter is ≤9 mm at birth and ≤10 mm in individuals aged two or older.[1,2] It can manifest as a cornea with normal thickness, curvature, and clarity, or it may involve thick, hazy, or flat corneas.[3,4,5] Microcornea can result in a shallow anterior chamber, leading to crowded angles and an increased risk of glaucoma.[6,7,8,9,10] Microcornea may or may not be linked to microphthalmos.[11] Congenital cataracts associated with microcornea can have various causes, either occurring in isolation or in conjunction with ocular or systemic abnormalities [Table 1].[12,13,14]

Table 1.

The various etiologies of congenital anterior segment dysmorphology

Cataract associated with Anterior segment dysmorphology Etiology
Cataract associated with microcornea
 Isolated Microcornea with cataract
 Ocular anomalies Peters anomaly
Axenfeld-Rieger syndrome
Sclerocornea
Aniridia
Persistant fetal vasculature
Uveal coloboma
 Systemic anomalies Lowe syndrome
Nance-Horan syndrome
TORCH infection
Warburg-Micro syndrome
Ehlers-Danlos syndrome
Myotonic dystrophy
Peters plus syndrome
Aniridia
 Isolated PAX6 associated
Other genes: PITX2, FOXC1, FOXD3, TRIM44, and CYP1B1
 Ocular associations Limbal stem cell deficiency
Nystagmus
Anterior segment dysgenesis
Corneal opacity
Cataract
Glaucoma
Foveal hypoplasia
 Systemic WAGR syndrome (Deletion involving PAX6 and WT1 gene) Wilms tumor, aniridia, genito-urinary abnormalities, range of developmental delay syndrome
Gillespie syndrome (ITPR1 gene): Aniridia, cerebellar ataxia and mental retardation
Anterior lenticonus
 Isolated
 Ocular associations Spherophakia
Anterior and posterior polar cataract
Posterior lenticonus
Retinal flecks
 Systemic association Alport syndrome
Posterior lenticonus
 Isolated
 Ocular associations Spherophakia
Anterior lenticonus
PFV
 Systemic Alport syndrome
Down syndrome
PFV
 Isolated Familial/nonfamilial
 Associated ocular anomalies Microphthalmia
Peters anomaly
Rieger anomaly
Posterior lenticonus
Morning glory syndrome
 Associated systemic anomalies (more common in bilateral PFV) Neurologic abnormalities
Cleft palate and lip
Polydactyly
Microcephaly
Trisomy 13
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PFV=Persistent fetal vasculature, AS=Anterior segment, TORCH=Toxoplasmosis, Rubella virus, Cytomegalovirus and Herpes Simplex Virus

A thorough preoperative assessment is crucial, encompassing measurements of corneal diameter, corneal thickness, keratometry, gonioscopy, axial length, intraocular pressure (IOP), cataract morphology, and thorough systemic evaluation, including Toxoplasmosis, Rubella virus, Cytomegalovirus and Herpes Simplex Virus (TORCH) antibody serology and imaging of the brain to determine the underlying cause.

Microcornea eyes pose unique difficulties due to the limited surgical space, shallow anterior chamber, and associated corneal irregularities such as flat, thick corneas, or sclerocornea. Surgeons must exercise extreme care to avoid damaging the corneal endothelium. In our experience, creating two side port entries through stab incisions at a less obtuse angle is preferable to the conventional two-plane entry at a more obtuse angle [Figure 1]. This facilitates easier instrumentation within the eye and better removal of subincisional cortex. The use of a high molecular weight viscoelastic substance is recommended to maintain anterior chamber depth and aid instrumentation. Iris hooks, pupilloplasty, and Kuglen hooks should be used sparingly in cases with nondilating pupils.

Figure 1.

Figure 1

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Highlights of cataract surgery in Peters anomaly, microcornea, and underlying persistent fetal vasculature. (a) Stab incision for paracentesis, (b) The two paracenteses are closer to each other in a less obtuse angle, curved yellow arrow, (c) The view after pupilloplasty showing membranous cataract with elongated ciliary processes (d) Through the procedure of membranectomy using vitrector, showing the underlying fibrovascular stalk attaching to posterior lens capsule (yellow arrow)

In some instances, the entire surgery, including anterior capsulorhexis, lens aspiration, posterior capsulotomy, and vitrectomy, can be performed using an automated vitrector. This minimizes endothelial damage and reduces operating time, as it limits the number of instruments entering the eye. It is advisable to create large anterior and posterior capsulotomies. For younger children, a posterior capsulorhexis and vitrectomy should be performed under vigilance to prevent further endothelial damage and to ensure there is no remaining vitreous in the anterior chamber at the conclusion of the surgery. With the high risk of secondary glaucoma in these eyes, it is advisable to perform a peripheral iridectomy. All the wounds should be sutured. Postoperative inflammation is controlled by using frequent topical steroids and strong cycloplegics.

Typically, the anterior chamber lacks sufficient volume for safe intraocular lens (IOL) implantation, making lensectomy the preferred approach. IOL implantation is not recommended in such cases. However, in instances where the anterior chamber has a normal depth (≥2 mm) and volume, in-the-bag IOL implantation is the preferred method.

Congenital Corneal Opacity

Of the various causes of congenital corneal opacity, Peters anomaly is most commonly associated with congenital cataract. In Peters anomaly, alongside microcornea, there are typically features such as a flat cornea, a shallow anterior chamber, and a central corneal scar of varying density. This condition is associated with a defect in the posterior stroma of the cornea, including the descemet membrane and endothelium. It can be categorized into two types: Type I, characterized by synechiae between the iris and cornea, and type II, which involves keratolenticular adhesions (KLA).[15,16] Peters anomaly may also be associated with conditions such as PFV or morning glory disc anomaly in the Posterior segment of the eye.[17,18,19]

The management approach depends on the severity of corneal opacity. When localized KLA obscure the visual axis due to corneal opacity, an optical iridectomy can be considered. In cases where KLA is associated with an underlying cataract, lens extraction is crucial. In severe cases, conventionally penetrating keratoplasty was considered.[20,21] However, it is worth noting that when KLA is present, it necessitates both lensectomy and vitrectomy during penetrating keratoplasty (PK), which does increase the risk of corneal transplant rejection.

In 2015, Medsinge and Nischal introduced a technique involving lens aspiration followed by the excision of the keratolenticular adhesion at the neck. They advised against peeling the adhesion to minimize the risk of additional corneal opacification.[22]

Nishimura et al. and Matalia et al. emphasized the importance of employing an endoilluminator in pediatric cataract cases with cloudy corneas. In situations where traditional microscopic visualization of intraocular structures was insufficient, they advocated using an endoilluminator. This device is positioned at the limbus with light directed obliquely, enhancing the visibility of intraocular structures by illuminating them against the cloudy cornea, thus facilitating a clearer view of structures hidden behind the hazy cornea.[23,24]

In 2022, Ramappa et al. introduced the selective endothelialectomy in Peters anomaly (SEPA) technique for cases with keratolenticular adhesion, provided that the opacity does not exceed 6 mm or at least half of the peripheral cornea remains clear. Their conclusion was that SEPA can serve as an effective surgical alternative to optical iridectomy and full-thickness penetrating keratoplasty (PK) for the benefit of numerous children worldwide suffering from blindness related to Peters anomaly.[22,25]

Topical antibiotics, topical steroids, and strong cycloplegics are recommended in the immediate postoperative period. Long-term care should involve regular examinations under anesthesia with measurements of corneal diameter, IOP, central corneal thickness (CCT), axial length, and refractive error to monitor the patient’s progress, and vigilance for the development of secondary glaucoma is essential.

Aniridia

Congenital aniridia is a rare and complex pan-ocular condition characterized by total or partial defects in the iris, affecting various ocular structures and often accompanied by systemic conditions [Table 1].[26,27,28,29] Cataracts in aniridia are noted in 40%–85%, ranging from discrete anterior polar opacity, posterior polar opacity, posterior subscapular flecks, mid-peripheral radial streaks to total cataracts.[30,31] Other associated lens abnormalities seen in aniridia patients are lens subluxation, coloboma, posterior lenticonus, and microspherophakia.[30]

The surgical management of cataracts in aniridia patients is challenging due to the pathological alterations in the eye. Careful selection of patients who would visually benefit from cataract surgery is highly essential due to the high complication rates. Preoperative evaluation should include assessing corneal clarity, presence, and degree of limbal stem cell deficiency, corneal diameter, gonioscopy, IOP, CCT, axial length, and keratometry. The aniridia associated with limbal stem cell deficiency and keratopathy should be carefully looked at, graded, and managed with intense preservative-free lubricants containing sodium hyaluronate in mild cases, autologous serum drops in moderate cases, and sometimes, an amniotic membrane graft is required in severe cases.[32,33,34,35] The management of aniridia-associated keratopathy in the preoperative period helps in maintaining the surface and better healing postsurgery.[32,36]

A modified corneal incision to spare deficient limbal stem cells, the use of high molecular weight viscoelastic agents, and smaller anterior capsulotomy are preferred. Minimal or no zonular stress during lens aspiration is essential. Avoid anterior capsular polish where the lens epithelial cells form a peripheral doughnut, forming a pseudo iris, which minimizes the effects of photophobia and glare. The use of single-/three-piece hydrophobic acrylic lenses is favored for their reduced incidence of posterior capsular opacification. In addition, yellow-tinted IOL further protects from photophobia.[37] Artificial iris segments and rings are known to have a higher incidence of postoperative inflammation, secondary glaucoma, and endothelial decompensation.[38] Primary posterior capsulotomy and minimal anterior vitrectomy prevent visual axis opacification [Figure 2].

Figure 2.

Figure 2

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Depicting series of surgical steps in a case of aniridia with cataract. (a) Co-axial reflex showing 360° partial aniridia with central nuclear cataract, (b) Injection of high molecular weight ophthalmic viscosurgical device to flatten the anterior lens capsule, (c) Nicking the anterior lens capsule with a microvitreoretinal blade, (d) Continuous curvilinear capsulotomy using rhexis forceps, (e) Initiating the lens aspiration using bimanual irrigation-aspiration canula, (f) Gentle aspiration of cortex and lens matter, (g) Visco-aspiration after in-the-bag single-piece intraocular lens implantation, (h) Primary posterior capsulotomy using an automated vitrector, (i) Minimal lens capsule polishing to aid formation of pseudo-pupil

Postoperative care with intensive topical steroids and cycloplegics will help to control inflammation. Complications, such as wound leak, hyphema, corneal edema, elevated IOP, progressive endothelial cell loss, potential cystoid macular edema, and severe anterior fibrosis syndrome, underscore the need for an extensive and attentive postoperative follow-up.[27,38,39] Severe anterior segment fibrosis syndrome is a rare complication of fibrous membrane overgrowth in the anterior segment seen in eyes with congenital aniridia undergoing intraocular surgeries and intraocular implants. This can lead to secondary angle closure glaucoma, capsular phimosis, and IOL tilt/subluxation.[40]

Anterior Lenticonus

Anterior lenticonus is abnormal conical protrusion of the anterior lens surface of the crystalline lens.[41] Simultaneous anterior and posterior lenticonus are reported.[42,43] It can be isolated or associated with serious systemic conditions [Table 1]. Visual impairment is by myopia, irregular astigmatism, and spontaneous rupture of the thin capsule with the development of cataracts are frequently reported. Careful slit-lamp evaluation and anterior segment optical coherence tomography aids in identifying the disorder. Anterior lenticonus can be either an isolated or in association with Alport syndrome. The management rests on spectacles, contact lenses, lens extraction, and IOL implantation.[44] The lens extraction is indicated when spectacles and contact lenses fail to correct the refractive error and in the presence of a rupture anterior capsule and cataract formation.

Anterior capsulorhexis is challenging in these eyes owing to its highly elastic nature and high tendency to run away. A high molecular weight ophthalmic viscosurgical device (HMW-OVD) is recommended to maintain the anterior chamber and relatively flatten the anterior capsule [Figure 3]. Patil-Chhablani et al. advocates making small nicks in the thin part of the capsule and extend further.[45] A two-incision push–pull rhexis technique described by Nischal is another option.[46] Hydrodissection should be avoided in these eyes. Careful in-the-bag IOL implantation is preferred.

Figure 3.

Figure 3

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Surgical steps in a patient with anterior lenticonus and development of recent-onset cataract. (a) Preoperative slit-lamp picture showing bulging anterior lens capsule with total cataract, (b) Nicks made at the base of the cone using micro-scissors, (c) Anterior capsulorhexis being conducted using rhexis forceps, (d) Lens matter aspiration using bimanual irrigation-aspiration technique, (e) Excision of the thickened edge of the anterior capsulotomy, (f) In-the-bag implantation of intraocular lens

Posterior Lenticonus

Posterior lenticonus is a congenital condition characterized by the thinning of a portion of the posterior capsule of the lens, resulting in the posterior capsule bowing.[47] Sporadic cases are more common, followed by cases with autosomal dominant and X-linked inheritance. Unilateral occurrences are more frequent than bilateral ones, and central lenticonus is more common than eccentric forms.[48] The etiologies of posterior lenticonus are summarized in Table 1.

Due to the inherent weakness in the affected portion of the posterior lens capsule, the normal pressure within the lens causes the pliable cortex to bulge backward within the weakened area. This disrupts the lamellar arrangement of the lens fibers, leading to lens opacification.

The clinical presentation can vary, ranging from a dot-like opacity to a snail track along the posterior capsule or a total cataract. Posterior lenticonus is suspected when there is a recent onset of a white pupil (leukocoria) in the absence of posterior segment pathology or a history of trauma. Clinical signs that help identify a ruptured posterior capsule before surgery include a deep anterior chamber, atoll sign, and fishtail sign.[49] An ultrasound immersion B-scan can also assist in identifying the bulge and rupture in the posterior capsule.

Surgical intervention is indicated in cases of lenticular myopia, astigmatism leading to amblyopia, and the presence of a cataract. Performing anterior capsulotomy is relatively easy in cases of posterior lenticonus since the capsule is relatively flat. To avoid excessive pressure in the anterior chamber, the bottle height is reduced. Hydrodissection is avoided, and lens matter is aspirated starting from the periphery when the posterior capsule is intact, or from the center when a rupture is suspected.[50] Lens aspiration is preferably done with a vitrector, especially in cases of a ruptured posterior capsule, as it allows safe removal of vitreous tags that may be attached to the edge of the capsule [Figure 4]. The rehabilitation options include in-the-bag single-piece IOL implantation or sulcus implantation of a three-piece IOL with or without optic capture.

Figure 4.

Figure 4

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Surgical steps in a case of posterior lenticonus and cataract, (a) Clear peripheral cortex and (dashed yellow arrow) and central dense nuclear cataract, thickened edge of rupture capsule (solid white arrow). Fishtail sign was present in this case, (b) Anterior capsulorhexis utilizing the sheen of capsule (dashed curved line) using rhexis forceps, (c) Inside-out lens matter aspiration using bimanual irrigation and aspiration technique, (d) Shows anterior capsulotomy (dashed arrow) and preexisting posterior capsular dehiscence (solid white arrow), (e) Anterior vitrectomy to remove the vitreous tags at the posterior capsule edge, (f) In-the-bag intraocular lens implantation showing nudging of the trailing haptic in-the-bag

Persistent Fetal Vasculature

Persistent Fetal Vasculature (PFV) is a condition where certain components of the fetal intraocular vasculature persist after birth. The extent of this persistence can influence the specific characteristics observed in the eye, including the presence of features such as a persistent pupillary membrane, Mittendorf dot, membranous cataract, Bergmeister papillae, retrolental membrane, and a fibro-vascular fold extending from the posterior surface of the lens to the optic disc. This may or may not exert traction on the retina (as shown in the Figure 5). PFV is usually an isolated anomaly but sometimes can be associated with other ocular anomalies such as Peters anomaly, Rieger anomaly, and morning glory syndrome [Table 1].[19,51,52,53]

Figure 5.

Figure 5

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Stepwise management of persistent fetal vasculature cataract, (a) Thick membranous cataract with blood vessels traversing the membrane, 360° elongated ciliary processes, (b) Use of monopolar diathermy to cauterize the blood vessels, (c) A nick into the thick membrane using the microvitreoretinal blade, (d) Thick membrane excised using the micro-scissors, (e) Use of automated vitrector to remove the membrane in a spiral manner, (f) Central clear visual axis after completing the vitrectomy

The prognosis for individuals with PFV depends on the specific features they present. Prognosis tends to be better when the condition is primarily localized to the anterior portion of the eye, compared to cases involving the optic disc and macula.

In situations where a retrolental membrane is associated with plaque cataract, it is important to differentiate this condition from others such as Coats’ disease, Norrie disease, retinopathy of prematurity, or retinoblastoma. This differentiation is achieved through appropriate investigations such as ultrasound B-scan. The membrane or plaque may sometimes be associated with elongated ciliary processes, and, in some cases, blood vessels may be visible crossing it.

The approach to management of PFV-associated cataracts depends on the extent of the lesion. A posterior approach through the pars plana route is preferred in cases with fibrovascular bands exerting traction on the disc and macula, whereas an anterior limbal/corneal approach is favored in the absence of these findings. This review focuses on the steps involved in managing cataracts through the anterior approach. Careful case selection simplifies the surgical procedure.

After creating paracentesis incisions in the superonasal and superotemporal positions, the anterior chamber is maintained using an HMW-OVD. The anterior capsulotomy can be completed either with rhexis forceps or an automated vitrector. Following lens aspiration, any intervening blood vessels can be cauterized using a monopolar diathermy, the thick posterior capsule, and the retrolental membrane can be carefully nicked using a vitrector or a microvitreoretinal (MVR) blade. The posterior capsulotomy is initiated with a vitrector and completed in a spiral fashion. The thicker the membrane, the lower the cut-rate settings of the vitrector should be. Sometimes, the plaque can be very thick, necessitating the use of micro scissors. The traction on the ciliary processes is released. In selected cases, an IOL can be implanted in the sulcus [Figure 5].

Conclusions

  • Managing congenital cataracts presents unique surgical challenges in contrast to adult cataracts. When accompanied by anterior segment dysmorphology, these challenges become even more formidable

  • It is crucial to carefully assess and select cases before surgery, taking into account the potential visual benefits and surgical risks

  • Thorough preoperative evaluation is essential in all cases to anticipate and plan for the intraoperative and postoperative challenges that may arise

  • Surgical adjustments should be tailored to each individual case and the surgeon’s expertise

  • Immediate postoperative inflammation is controlled through the use of potent steroids and strong cycloplegics

  • Long-term monitoring for the development of glaucoma is imperative, along with appropriate visual rehabilitation, amblyopia therapy, and alignment correction.

Appropriate patient consent has been obtained to report/use the patient‘s data/figures/symptoms in the manuscript.

Data availability statement

Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.

Conflicts of interest

The authors declare that there are no conflicts of interests of this paper.

Funding Statement

The study was supported by Hyderabad Eye Research Foundation.

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Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.

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