From the Department of Ophthalmology (AC, LG), Sunderland Eye Infirmary, Sunderland; and the Department of Ophthalmology (JI), Hull and East Yorkshire Eye Hospital, East Yorkshire, United Kingdom.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Lawrence Gnanaraj, FRCSEd, FRCS, Department of Ophthalmology, Sunderland Eye Infirmary, Sunderland, SR2 9HP, United Kingdom.
Congenital pupillary-iris-lens membrane is a vascularized membrane that extends onto the lens with attachment peripherally to Schwalbe’s line. First described in 1986 by Cibis et al.,1 it is thought to originate from ectopic neural crest cells.2 The membrane can result in visual loss due to stimulus deprivation amblyopia from occlusion of the visual axis or progressive angle-closure glaucoma. Surgical treatment may be necessary to clear the visual axis or manage the impending angle closure.2–5
We report a case of congenital pupillary-iris-lens membrane that showed previously unreported evidence of membrane regrowth after surgical removal of the membrane.
A 3-month-old healthy female infant born at term to non-consanguineous parents presented to the Ophthalmology Department when her mother noticed a white spot on the left iris. The child was born by elective caesarean section for a breech presentation. She was otherwise well and there was no family history of any ocular problems.
The eyes were central, steady, and maintained fixation. Visual acuity tested with Leah Gratings (Leah-Test, Helsinki, Finland) was 2 cycles per degree (cpd) in her right eye and 1 cpd in her left eye. Slit-lamp examination of her left eye showed a visible white membrane stretching from the anterior lens capsule to the pupillary margin to the anterior iris stroma and continuing on to the drainage angle (Fig. 1). The crystalline lens was clear and a good red reflex was present. Examination of the right eye was normal.
Figure 1. Intraoperative Photograph Showing the Pupillary-Iris-Lens Membrane.
She was initially treated with tropicamide 1% (Chauvin Pharmaceuticals Ltd., Surrey, United Kingdom) once a day in the left eye and patching of the right eye.
Examination under anesthesia was performed 6 weeks later. Horizontal corneal diameter was 11 mm in the right eye and 10.5 mm in the left eye. Retinoscopic findings were +4.25 diopters in both eyes. Intraocular pressure by Perkins applanation tonometry (Clement Clarke Ltd., London, United Kingdom) was 20 mm Hg in the right eye and 14 mm Hg in the left eye. The axial length measured using OcuScan (Alcon Laboratories, Inc., Fort Worth, TX) was 20.14 mm in her right eye and 20.4 mm in her left eye. Gonioscopy showed extension of the membrane as strands from the iris surface to the drainage angle. Membranectomy and iridectomy was performed because the membrane was noted to obscure the visual axis. A clear lens was found beneath the membrane. She recovered well with a clear visual axis and was observed regularly.
A repeat examination under anesthesia at 13 months of age confirmed regrowth of the membrane, but the visual axis was clear. The corneal diameter, intraocular pressure, and axial length were normal and similar in both eyes. Further review showed progression of membrane growth obscuring her visual axis (Fig. 2) and she underwent excision of the membrane at 15 months of age. The postoperative period was uneventful. She remains under regular follow-up. At the most recent visit 18 months after her second surgery, there was no evidence of further regrowth (Fig. 3).
Figure 2. Anterior Segment Photograph Showing Re-Growth of the Vascularized Membrane Extending over the Clear Lens.
Figure 3. Eighteen-Month Postoperative Anterior Segment Photograph Showing Absent Membrane and Clear Lens.
Cibis et al. first described pupil-iris-lens membrane in 1986.1 Although there are other case reports and series,2–6 relatively little is known about the natural history of this condition. The clinical picture is thought to be due to lack of proper migration, formation, and regression of iris neural crest cell–derived tissues.2,5 The pupil-iris-lens membrane stretches from the anterior lens capsule to the pigment layer of the pupillary margin to the anterior iris stoma and continues on to the drainage angle. Previous reports have described the prominence of tufts of vessels along the membrane in early years that disappeared later.1
The most common presentations were unilateral abnormalities of iris and pupil within the first few months of life.1–6 The membrane on presentation may be associated with an ectopic pupil, occlusion of the visual axis, or progressive growth to occlude the visual axis.1–6 Several cases have developed flattening of the anterior chamber, iris bombe, and angle-closure glaucoma, whereas others have remained dormant and have required no surgical intervention.1,6
The objective of the treatment is to reduce risk of amblyopia and glaucoma. Topical mydriatics may dilate the pupil sufficiently to keep the visual axis clear,1,6 but surgery may be required to remove the membrane or enlarge the pupillary opening.2–5 Surgery may also be required if growth or traction of the membrane induces angle-closure glaucoma.2–5 Avitabile et al.4 reported two cases needing surgical intervention: the first patient needed pupil enlargement with a vitreous cutter and the second patient underwent surgical iridectomy. There were no recurrences reported after a mean follow-up of 14 years. Visual outcomes remained poor in both patients due to the development of amblyopia.
Our case is unique in that it shows the ability of the membrane to regrow after excision. There have been no reported cases of recurrence after surgical excision. It was believed that the recurrence of the membrane in this case represents subtotal excision and regrowth from the peripheral remnants, which were impossible to excise at the time of the operation without damage to the angle structures.
This case report illustrates that not all membranes act in the same slowly growing manner. This aggressive regrowth highlights the requirement of close monitoring of these patients even after surgical excision.
- Cibis GW, Waelterman JM, Hurst E, Tripathi RC, Richardson W. Congenital pupillary-iris-lens membrane with goniodysgenesis (a new entity). Ophthalmology. 1986;93:847–852.
- Cibis GW, Tripathi RC, Tripathi BJ. Surgical removal of congenital pupillary-iris-lens membrane. Ophthalmic Surg. 1994;25:580–583.
- Robb RM. Fibrous congenital iris membranes with pupillary distortion. Trans Am Ophthalmol Soc. 2001;99:45–50.
- Avitabile T, Castiglione F, Marano F, Reibaldi M. Congenital pupillary-iris-lens membrane with goniodysgenesis: clinical history and ultra biomicroscopic findings. J Pediatr Ophthalmol Strabismus. 2002;39:248–250.
- Cibis GW, Walton DS. Congenital pupillary-iris-lens membrane with goniodysgenesis. J AAPOS. 2004;8:378–383. doi:10.1016/j.jaapos.2004.04.010 [CrossRef]
- Deshpande N, Shetty S, Krishndas SR. Pupillary-iris-lens membrane with goniodysgenesis: a case report. Indian J Ophthalmol. 2006;54:275–276. doi:10.4103/0301-4738.27957 [CrossRef]