From The Institute of Ophthalmology and Visual Science (SG, RW, AC), University of Medicine and Dentistry of New Jersey, Newark, New Jersey; the Ophthalmology Department (BF), Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA; and the Ophthalmology Department (BT), Mount Sinai School of Medicine, New York, New York.
The authors have no financial or proprietary interest in the materials presented herein.
Dr. Wagner did not participate in the editorial review of this manuscript.
Address correspondence to Suqin Guo, MD, The Institute of Ophthalmology and Visual Science, UMDNJ-New Jersey Medical School, 90 Bergen Street, DOC 6100, Newark, NJ 07101. E-mail: firstname.lastname@example.org
Cataract can occasionally be associated with infantile glaucoma. Zonular dehiscence in chronic primary infantile glaucoma is rarely reported1 as one of the potential complications of infantile glaucoma2 due to the stretch of lens zonules, a deepened anterior chamber, and an elongated axial length of the globe.3
We describe a rare case of a bilateral occult zonular dehiscence in a child who had been treated medically for bilateral infantile glaucoma. Zonular dehiscence was noted at the beginning stages of cataract surgery. A capsular tension ring was used for capsular support and an intraocular lens (IOL) was safely inserted in both eyes.
A 7-year-old boy was diagnosed as having bilateral infantile glaucoma and treated at 2½ years of age by a glaucoma specialist. He was referred to the Institute of Ophthalmology and Visual Science, University of Medicine and Dentistry of New Jersey for the management of bilateral cataracts.
His intraocular pressure (IOP) was 19 mm Hg in the right eye and 18 mm Hg in the left eye while he was prescribed Betimol (Vistakon Pharmaceuticals, LLC, Doylestown, PA) twice daily (he was taking two types of glaucoma medication during early years). His best-corrected visual acuity was 20/200 in the right eye and 20/400 in the left eye. Slit-lamp examination showed an enlarged cornea (15 mm), deep anterior chambers, and greater than 5-mm central posterior subcapsular and central nuclear cataracts in both eyes. His anterior chamber angle was noted to open with a high insertion of iris roots in both eyes examined by his glaucoma specialist. There was no apparent phacodonesis or iridodonesis in either eye. Cycloplegic refraction was −8.50 in the right eye and −9.00 in the left eye. The optic nerve had a 0.60 × 0.75 cup-to-disc ratio in the right eye and 0.60 × 0.70 in the left eye. A preoperative A-scan ultrasound showed elongated axial lengths of 27.52 mm in the right eye and 27.53 mm in the left eye with deep anterior chambers (4.21 mm in the right eye and 4.32 mm in the left eye) (Fig. 1).
Figure 1. (Top) A-Scan Ultrasound Showing Elongated Axial Length (AXL) (27.52 mm) and Deepened Anterior Chamber (4.21 mm). (Bottom) A-Scan Ultrasound Image of Axial Length (22.58 mm) and Anterior Chamber Depth (ACD) (3.64 mm) from a Normal 5-Year-Old Child.
The patient did not present with abnormally long or short limbs and had no cardiac, skeletal, joint, or neurological anomalies. His pediatrician completed a systemic work-up. There were no indications of Marfan’s syndrome, Well–Marchesani syndrome, or homocystinuria.
The child was to undergo cataract extraction with an IOL implantation in his right eye. As the anterior chamber was being filled with viscoelastic, three clock hours of zonular dehiscence and sub-luxated cataract were noted in the inferotemporal quadrant. The lens nucleus was prolapsed out of the capsular bag to reduce stress on the lens zonular fibers. Viscoelastic material was injected to keep the capsular bag open and prevent the vitreous from coming forward. The cataract was further removed using the vitrectomy device. A capsular tension ring was inserted into the capsular bag followed by IOL insertion. Zonular dehiscence in the temporal quadrant was similarly noted at the beginning stage of the cataract surgery in the left eye 4 weeks following the surgery in his right eye. The cataract was then removed and a capsular tension ring and IOL were inserted. The child’s best-corrected visual acuity improved to 20/30 in both eyes postoperatively. His IOP remained within normal limits while taking Betimol twice daily and the IOLs remained well centered (Fig. 2) in both eyes after a 2-year follow-up.
Figure 2. Capsular Tension Ring and Intraocular Lens in the Lens Capsular Bag 8 Months After Surgery.
Subluxation of the lens may be: (1) primary and develop from an inherited zonular degeneration; (2) secondary and develop as a consequence of zonular degeneration from chronic glaucoma and buphthalmos, or (3) congenital and develop secondary to multiple ocular anomalies, including zonular aplasia, microphakia, and congenital glaucoma.2 Congenital glaucoma/buphthalmos is considered one of the primary ocular disorders associated with ectopia lentis, although it is rare.4 Primary infantile glaucoma has been recognized as being associated with lens subluxation.5–7 A deep anterior chamber and elongated axial length can be responsible for zonular weakness in infantile glaucoma. The average axial length is approximately 24.15 mm in boys and approximately 0.5 mm shorter in girls.3 The mean anterior chamber depth is approximately 3 mm, which can vary with the measuring method and the age and sex of the patient,8 although the anterior chamber depth in infantile glaucoma can be deepened.9 A subluxated lens may develop in patients with infantile glaucoma because of an elongated globe and an enlargement of the anterior chamber.3 For patients with no clinical signs of zonular weakness, an elongated axial length of the globe and deep anterior chambers can be indications of lens zonular dehiscence or occult subluxation of the lens.7
Capsular tension rings have been successfully used to treat ectopia lentis in children.10–12 Vasavada et al.11 studied the Cionni modified capsular tension ring (Morcher GmbH, Stuttgart, Germany) and IOL implantation in the capsular bag in 35 eyes (22 children) with ectopia lentis with good outcome. The authors concluded that implantation of the Cionni capsular tension ring and a single piece of AcrySof IOL (Alcon, Fort Worth, TX) in the capsular bag in pediatric eyes with subluxated lenses was safe and effective and led to a stable IOL placement without significant complications.11 Konradsen et al.12 reported their experience treating 37 eyes of 22 children with lens dislocation. A Cionni modified capsular tension ring, a conventional capsular tension ring, and an AcrySof IOL were implanted in the capsular bag and the posterior capsule was left intact. Although secondary surgery for visual axis opacification was needed in some children, the capsular tension ring and an implanted IOL remained in stable position.12
Potential complications associated with capsular tension ring application have been reported, including tearing of the capsular bag during surgery and subluxation of the entire bag/capsular tension ring/IOL complex.13,14 Long-term and close postoperative follow-up should be indicated in patients with long axial length or Marfan’s syndrome, Well–Marchesani syndrome, or homocystinuria.
Occult subluxated lens or cataract can be associated with childhood glaucoma, although the mechanism remains unclear. A possibility includes the stretch of the lens zonules caused by a deepened anterior chamber and the elongated axial length of the globe. This case report shows that for patients who have infantile glaucoma and have no clinical signs of zonular weakness, elongated axial length of the globe and deep anterior chambers could be indications of possible occult subluxation of the lens. Both infantile glaucoma and axial myopia may have contributed to the elongated axial length and weakened lens zonules in our patient. Early recognition and proper management of occult lens zonular dehiscence and subluxated lens in childhood glaucoma can obviate severe surgical complications, improving surgical outcomes. We report that capsular tension rings can be used safely in the management of lens zonular dehiscence associated with infantile glaucoma.
- Walton DS. Chronic newborn primary congenital glaucoma with secondary lens subluxation. J Pediatr Ophthalmol Strabismus. 2009;46:200, 231. doi:10.3928/01913913-20090706-02 [CrossRef]
- Davidson MG, Nelms SR. Diseases of the lens and cataract formation. In: Gelatt KN, ed. Veterinary Ophthalmology, 3rd ed. Philadelphia: Lippincott, Williams & Wilkins; 1999:797–825.
- Choudhary AK, Jha B. Imaging findings of congenital glaucoma in Opitz syndrome. AJNR Am J Neuroradiol. 2008;29:1003–1005. doi:10.3174/ajnr.A1020 [CrossRef]
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- Gelatt KN. Color Atlas of Veterinary Ophthalmology. Hoboken: Wiley; 2001:261.
- Lavanya R, Teo L, Friedman DS, et al. Comparison of anterior chamber depth measurements using the IOLMaster, scanning peripheral anterior chamber depth analyser, and anterior segment optical coherence tomography. Br J Ophthalmol. 2007;91:1023–1026. doi:10.1136/bjo.2006.113761 [CrossRef]
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- Cionni RJ, Osher RH, Marques DM, Marques FF, Snyder ME, Shapiro S. Modified capsular tension ring for patients with congenital loss of zonular support. J Cataract Refract Surg. 2003;29:1668–1673. doi:10.1016/S0886-3350(03)00238-4 [CrossRef]
- Vasavada V, Vasavada VA, Hoffman RO, Spencer TS, Kumar RV, Crandall AS. Intraoperative performance and postoperative outcomes of endocapsular ring implantation in pediatric eyes. J Cataract Refract Surg. 2008;34:1499–1508. doi:10.1016/j.jcrs.2008.04.044 [CrossRef]
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