Hyperplastic persistent pupillary membranes with congenital corneal anomalies

Hyperplastic persistent pupillary membranes with congenital corneal anomalies

CASE REPORT Hyperplastic persistent pupillary membranes with congenital corneal anomalies Deepa Viswanathan, MD, Prema Padmanabhan, MD, Aditi Johri, ...

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CASE REPORT

Hyperplastic persistent pupillary membranes with congenital corneal anomalies Deepa Viswanathan, MD, Prema Padmanabhan, MD, Aditi Johri, MD

We report a case of a 14-year-old girl who presented with impaired vision in the left eye since birth. Examination showed extensive bilateral persistent pupillary membranes associated with microcornea, cornea plana, and central localized posterior keratoconus in both eyes. The uncorrected visual acuity was 0.2 (logMAR) (20/32) in the right eye and 1.9 (logMAR) (20/1300) in the left eye, with no improvement with pinhole. Surgical excision of the membranes was done in both eyes using vitreous microscissors. At 6 months, the best corrected visual acuity was 0.1 (logMAR) (20/25) in the right eye and 1.3 (logMAR) (20/400) in the left eye. No significant intraoperative or postoperative complications were noted. J Cataract Refract Surg 2007; 33:1123–1126 Q 2007 ASCRS and ESCRS

Persistent pupillary membranes (PPMs) are usually nonpathogenic physical signs of normal intrauterine development.1 They are seen in up to 95% of neonates and typically regress within the first few weeks of life. Remnants that persist in adult life usually take the form of diaphanous arborizing or anastomosing strands attached along the iris collarette and are of no clinical or functional significance. Sometimes, however, they are extensive and hyperplastic, occluding the pupil and impairing vision. Hyperplastic PPMs can be managed conservatively2 with nonsurgical techniques such as neodymium:YAG laser membranectomy3 or may require surgical intervention.4–7 They may be seen in isolation or in association with other ocular or extraocular congenital defects.2 We report a case of bilateral hyperplastic PPMs associated with microcornea, cornea plana, localized posterior keratoconus, and unilateral macular hypoplasia. Surgical management of the pupillary membrane was indicated to create an optimum pupillary aperture, recognize and correct the

Accepted for publication February 5, 2007. From the Medical and Vision Research Foundation, Tamil Nadu, India. No author has a financial or proprietary interest in any material or method mentioned. Corresponding author: Dr. Prema Padmanabhan, Cornea Service, Sankara Nethralaya, 18 College Road, Chennai – 600 006, Tamil Nadu, India. E-mail: [email protected] Q 2007 ASCRS and ESCRS Published by Elsevier Inc.

existing refractive error and anisometropia, and perform a detailed examination of the eye.

CASE REPORT A 14-year-old girl presented with impaired vision in the left eye since birth. The child was born of a consanguineous marriage and a normal full-term pregnancy. Medical and family histories were noncontributory. On examination, the uncorrected visual acuity on the logMAR scale was 0.2 (20/32) in the right eye and 1.9 (20/1300) in the left eye. There was no improvement in vision with a pinhole. The absence of a red reflex precluded retinoscopy. There was mild left hypotropia. Ocular movements were normal. Slitlamp biomicroscopy showed a mild degree of microcornea in both eyes, the cornea measuring 9.0 mm and 9.5 mm in the vertical and horizontal axes. There was bilateral cornea plana, with localized midperipheral annulus of posterior keratoconus. Corneal haze at the level of the deep stroma and Descemet’s membrane affected the central and peripheral cornea; there was a relatively clear midperipheral zone. The anterior chamber appeared shallow, but gonioscopy revealed open angles in both eyes. There were bilateral thick, dark-brown membranes with radial folds occluding the pupil. The right eye showed a pinhead-sized fenestration in the membrane about 1.5 mm above and nasal to the corneal vertex. This did not widen with pharmacological dilation. The iris architecture appeared normal. There was no view of the crystalline lens or fundus (Figure 1). Corneal topography (TMS-4, Tomey Corp.) confirmed bilateral flat corneas (simulated K right eye, 32.21 @ 172/ 29.91 @ 82; simulated K left eye, 32.04 @ 13/31.22 @ 103) (Figure 2). Corneal pachymetry measured a mean corneal thickness of 429 mm in the right eye and 463 mm in the left eye. Specular microscopy showed healthy endothelia in both eyes, with 2747 cells/mm2 in the right eye and 2695 cells/mm2 in the left eye. Ultrasound biomicroscopy showed a normal crystalline lens with intact zonules. Ultrasound A-scan measured an axial length of 24.3 mm in the 0886-3350/07/$dsee front matter doi:10.1016/j.jcrs.2007.02.027

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CASE REPORT: HYPERPLASTIC PPM AND CORNEAL ANOMALIES

Figure 1. Slitlamp photograph of the left eye showing central and peripheral corneal haze and a thick persistent pupillary membrane.

with the end-gripping vitreous forceps (Grieshaber no. 612.95). Healon was removed through a 20-gauge cannula through 1 paracentesis while balanced salt solution was irrigated through the other. There were no intraoperative or postoperative complications. A tapered dose of topical steroids and cycloplegic drops for 2 weeks was prescribed. Retinoscopy and a detailed fundus examination were performed 4 weeks after surgery. Postoperatively, the best spectacle-corrected visual acuity was 0.1 (logMAR) (20/25) in the right eye (C1.50 0.50  90) and 1.3 (logMAR) (20/400) in the left eye (C5.00 2.50  20). The pupil diameter in each eye was 2.5 mm (Figure 3). The pupils reacted briskly to light and could be pharmacologically dilated to about 5.0 mm, permitting examination of the fundus. While the fundus of the right eye was normal, the left eye showed a dull foveal reflex suggestive of macular hypoplasia. At the 6-month follow-up, the visual and ocular status was unchanged. There was no evidence of lenticular opacities in either eye.

DISCUSSION right eye and 23.8 mm in the left eye, and ultrasound B-scan revealed normal posterior segments in both eyes. A diagnosis of bilateral hyperplastic PPMs with microcornea, cornea plana, and localized posterior keratoconus was made. The nature of the membrane and the near-total obscuration of the pupil necessitated surgical excision of the membrane. Surgery was performed in both eyes 2 weeks apart. Two 1.5 mm limbal paracenteses were made at the 9 o’clock and 3 o’clock meridians with a microvitreoretinal blade, which was also used to initiate an opening in the pupillary membrane. Sodium hyaluronate 1% (Healon) was introduced to maintain the anterior chamber and was injected through the opening in the membrane to lift it away from the crystalline lens. An angled 20-gauge vitreous microscissors (Grieshaber no. 612.24) was inserted to carefully excise the membrane without injuring the crystalline lens. The membrane was then removed from the anterior chamber

During the first month of gestation, the developing eye of the fetus is characterized by the proliferation of blood vessels in the intraocular cavity to provide nourishment for the developing lens and to act as a morphological foundation for the developing structures of the eye. This vasculature, which enters the eye through the fetal fissure, traverses the vitreous compartment as the hyaloid artery and wraps the developing lens in a meshwork of capillaries known as the tunica vasculosa. This complex anastomosing vascular system reaches maximum evolution in the second to third month of gestation. By the start of the second trimester, they begin to involute and usually disappear completely by birth.

Figure 2. Topography image of the left eye depicting a flat cornea.

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CASE REPORT: HYPERPLASTIC PPM AND CORNEAL ANOMALIES

Figure 3. Postoperative slitlamp photograph of the eye in Figure 1 showing a round, 2.5 mm pupil.

Although the classic syndrome of persistent hyperplastic primary vitreous was described by Reese and Payne8 as early as 1946, the designation emphasized the features of the nonregression of the posterior tunica vasculosa. Goldberg1 recognized the varied manifestations of the postnatal persistence of some or all the intraocular fetal vessels affecting the anterior and posterior compartments of the eye and redesignated the entity with the more holistic nomenclature of ‘‘persistent fetal vasculature’’ (PFV). Persistent pupillary membranes thus represent one component of the PFV described by Goldberg; namely, the persistence of the anterior tunica vasculosa. Various grades of severity and various associated ocular malformations have been described.9–11 Our patient had not only a PPM but also other anterior segment anomaliesdmicrocornea, cornea plana, and posterior keratoconusdsuggesting some pathogenic event occurring around the fifth gestational month that resulted in an arrest of normal corneal development. Persistent pupillary membranes are most often seen as a fine network of strands and have no clinical significance. Cibis et al.12 and Robb10 report a few cases of a pupil–iris–lens membrane that showed a tendency to progress toward pupillary occlusion and, in a few cases, to angle-closure glaucoma. Spontaneous hyphema secondary to a vascularized fragment of PPM has been reported as a rare complication.13 Management depends on the severity of the manifestation. In our patient, the membranes were thick and had completely occluded the visual axis. Miller and Judisch14 report that a pupil of at least 1.5 mm is required for optimum vision. Although our patient

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had a visual acuity of 0.2 (logMAR) (20/32) in the right eye, the pinhead-sized fenestration in the membrane was inadequate in size and eccentric in location, justifying surgical intervention. Even this small opening was enough to prevent amblyopia in the eye, and an enlargement of the aperture to an optimum size resulted in an improvement in vision. The left eye of our patient had no aperture in the pupillary membrane, probably rendering the eye amblyopic. Following surgery, the patient was found to have anisometropic hyperopia in this eye and macular hypoplasia. If the pupillary membrane had been surgically excised earlier in childhood, deprivation amblyopia could have been prevented and the anisometropic amblyopia corrected. Persistent fetal vasculature may be associated with various posterior segment anomalies; eg, congenital retinal detachment, retinal folds, and hypoplasia of the macula or optic nerve head. Surgical excision of the pupillary membrane facilitates examination of the posterior segment. Our patient had macular hypoplasia in the left eye, and this may have contributed to the visual impairment that persisted in the eye despite successful surgery.

REFERENCES 1. Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV); LIV Edward Jackson Memorial Lecture. Am J Ophthalmol 1997; 124:587–626 2. Thacker NM, Brit MT, Demer JL. Extensive persistent pupillary membranes: conservative management. J AAPOS 2005; 9: 495–496 3. Wang J-K, Wu C-Y, Lai P-C. Sequential argon-YAG laser membranectomy and phacoemulsification for treatment of persistent pupillary membrane and associated cataract. J Cataract Refract Surg 2005; 31:1661–1663 4. Tsai Y-Y, Chiang C-C, Tsai C-H. Surgical technique for removing an extensive persistent pupillary membrane. J Cataract Refract Surg 2004; 30:1622–1625 5. Lim KH, Yu YS. Surgical management for persistent pupillary membrane with vitreous scissors. Korean J Ophthalmol 1996; 10:124–126 6. Gupta R, Kumar S, Sonika, Sood S. Laser and surgical management of hyperplastic persistent pupillary membrane. Ophthalmic Surg Lasers Imaging 2003; 34:136–139 7. Pandey SK, Ram J, Jain A, et al. Surgical management of complete hyperplastic persistent pupillary membrane. J Pediatr Ophthalmol Strabismus 1999; 36:221–223 8. Reese AB, Payne F. Persistence and hyperplasia of the primary vitreous (tunica vasculosa lentis or retrolental fibroplasias). Am J Ophthalmol 1946; 29:1–24; also: Trans Am Ophthalmol Soc 1945; 43:163–192. Available at. http://www.pubmedcentral.nih. gov/picrender.fcgi?artidZ1315166&blobtypeZpdf; Accessed March 2, 2007 9. Kolin T, Murphee AL. Hyperplastic persistent pupillary membrane. Am J Ophthalmol 1997; 123:839–841

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10. Robb RM. Fibrous congenital iris membranes with pupillary distortion. Trans Am Ophthalmol Soc 2001; 99:45–50; discussion, 50–51. Available at. http://www.aosonline.org/xactions/ 1545-6110_v099_p045.pdf; Accessed March 2, 2007 11. Cibis GW, Walton DS. Congenital pupillary-iris-lens-membrane with goniodysgenesis. J AAPOS 2004; 8:378–383 12. Cibis GW, Waelterman JM, Hurst E, et al. Congenital pupillary iris-lens-membrane with goniodysgenesis (a new entity). Ophthalmology 1986; 93:847–852 13. Kotamarthi V, Sarodia O, Woodruff GH. Spontaneous hyphaema secondary to a vascularised fragment of persistent pupillary membrane. Eye 2001; 15:240–241

14. Miller SD, Judisch GF. Persistent pupillary membrane: successful medical management. Arch Ophthalmol 1979; 10: 1911–1913

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First author: Deepa Viswanathan, MD Medical and Vision Research Foundation, Tamil Nadu, India