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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 74-75

Unilateral isolated type 3 persistent pupillary membrane: A rare case report

Department of Glaucoma, JPM Rotary Club of Cuttack Eye Hospital and Research Institute, CDA, Cuttack, Odisha, India

Date of Submission05-May-2021
Date of Acceptance06-Sep-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Naresh Desinayak
JPM Rotary Club of Cuttack Eye Hospital and Research Institute, CDA, Sectoe - - 6, Cuttack, Odisha - 753 014
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1082_21

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We report a 65-year-old male with unilateral type 3 persistent pupillary membrane, which was an incidental finding and was not a contributing factor for his presented symptom of diminished vision. Persistent pupillary membrane is a frequently encountered congenital anomaly of iris in routine ophthalmological practice. Different clinical types of persistent pupillary membrane do exist. However, this clinical variety is a rare one to be reported so far in the existing literature.

Keywords: Axenfeld–Rieger syndrome, Duke–Elder classification, persistent pupillary membrane

How to cite this article:
Desinayak N, Panda A. Unilateral isolated type 3 persistent pupillary membrane: A rare case report. Indian J Ophthalmol Case Rep 2022;2:74-5

How to cite this URL:
Desinayak N, Panda A. Unilateral isolated type 3 persistent pupillary membrane: A rare case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 19];2:74-5. Available from: https://www.ijoreports.in/text.asp?2022/2/1/74/334853

Persistent pupillary membrane (PPM) is a congenital anomaly of iris commonly seen by ophthalmologists and sometimes by neonatologists in their routine clinical practice. It is the embryonic remnant of tunica vasculosa lentis that nourishes the lens in utero but its timely regression is crucial for the proper functional development of the eye. Morphologically, PPM varies from thin, sparse connective tissue strand to dense, thick iris tissue band. Usually, they are asymptomatic and require no intervention, but few cases deserve timely intervention for prevention of severe stimulus deprivation amblyopia.

  Case Report Top

A 65-year-old male presented to our outpatient department with the complaint of blurred vision in his right eye (OD) for 6 months. His past history was uneventful. He was a known diabetic and hypertensive for eight years, well-controlled with oral medications.

On examination, his best-corrected visual acuity was 20/30 OD and 20/40 in the left eye (OS). Anterior segment evaluation of OD revealed a thick, dense iris tissue strand extending from the collarette to corneal endothelium at 6 o'clock position not associated with corneal opacity or infiltration [Figure 1]. Anterior chamber was deep and quiet. Pupil was round and central in spite of the iridocorneal strand and reacting well to the direct and consensual light. Early cortical opacity was noted in the lens. OS was within normal limits except for early cortical cataract.
Figure 1: Slit-lamp photograph OD showing thick iris strand extending from the collarette to corneal endothelium at the 6-o'clock position

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Gonioscopy showed open angle in both eyes (OU). The iridocorneal strand in OD was away from the inferior angle and not associated with any sign of inflammation [Figure 2]. Dilated posterior segment examination revealed normal symmetrical fundus with a vertical cup disc ratio of 0.5:1 and healthy neuroretinal rim OU. Intraocular pressure was 18 mm Hg OD and 16 mm Hg OS on the Goldman applanation tonometer. Central corneal thickness measured 537 μm OD and 538 μm OS. Humphrey's visual field 24-2 threshold test revealed right homonymous hemianopia. On the basis of visual field defect, MRI brain was advised, which revealed lacunar infarct in left paramedian, pons, and frontal white matter.
Figure 2: Gonioscopy OD inferior angle showing the isolated iridocorneal strand, wide open angle, and not associated with any sign of inflammation

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A diagnosis of right homonymous hemianopia due to cerebrovascular accident and type 3 PPM OD was made. No medical or surgical intervention was done for type 3 PPM, knowing it is visually asymptomatic. The patient was referred to a neurologist for subsequent management.

  Discussion Top

PPM represents a vestige of anterior tunica vasculosa lentis. This common congenital ocular anomaly has a 95% prevalence among newborns and 20% among the adult population.[1]

These are commonly noticed by neonatologists as they are expected to involute completely by the 34th week of gestation and therefore they are more prevalent among premature babies. Thus, it can be used as an indicator suggestive of prematurity (gestational age 27th week to 34th week).[2] There are some evidence that intrauterine stress, such as chronic maternal hypertension, may accelerate the disappearance of these membranes.[3]

PPM can be unilateral or bilateral with a wide range of variations in appearance, size, configuration, and density. Three clinical varieties of PPM have been reported. According to Duke–Elder classification, membranes attached exclusively to the iris are type 1, whereas Type 2 PPM is characterized by iridolenticular adhesion. Subvariants are pigmented dendritic iris stromal melanocytes, singly or in clumps, situated aberrantly on the anterior lens capsule, called “Chicken tracks.” Iridocorneal adhesions are the type 3 variant and are commonly seen in Axenfeld–Rieger syndrome.[4]

The available literature reported unilateral or bilateral type 1 and type 2 PPM. However, the unilateral isolated type 3 variant has been rarely reported so far.

Our case can be clinically confused with adherent leucoma, where the iris is incarcerated in the back of the scarred cornea and is usually associated with old signs of inflammation. This case also needs to be differentiated from Axenfeld–Rieger syndrome, which is usually bilateral and is characterized by microcornea or megalocornea, anteriorly displaced Swalbe's line (posterior embryotoxon) with attached iris strands, ectropion uvea, and correctopia. The systemic associations are facial bone and dental defect, hypospadias, and redundant umbilical cord. This case can also be distinguished from Peter's anomaly, which is characterized by posterior corneal defect with stromal opacification (leucoma) to which the iris strands are attached.[5] Unilateral iridocorneal adhesion is also seen in iridocorneal endothelial (ICE) syndrome, which is associated with abnormal corneal endothelium, corneal edema, correctopia, and secondary glaucoma.[6] All these features reflect the uniqueness of our case unilateral and isolated type 3 PPM.

Mostly, the PPM are visually asymptomatic and often remain unnoticed. Minimum pupillary size of 1.5 mm is required for adequate retinal stimulation.[7] Smaller size may hamper the normal functional vision development due to poor retinal illumination and diffraction. In our case, the PPM was away from the central visual axis, making the patient unaware of it and hence left nonintervened.

  Conclusion Top

Type 3 PPM is extremely rare and exclusively asymptomatic and nonamblyogenic as it neither covers the visual axis nor is associated with corneal opacity. Differentiating it from other progressive vision-threatening anterior segment pathologies such as Axenfeld–Rieger syndrome, ICE syndrome, Peter's anomaly, adherent leucoma, and inflammatory synechiae is essential for their case based appropriate management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Tasman W, Jaeger E. Duane's Ophthalmology. Philadelphia: Lippincott Williams and Wilkins; 2007. p. 24-258.  Back to cited text no. 1
Hittner HM, Hirsch NJ, Rudolph AJ. Assessment of gestational age by examination of the anterior vascular capsule of the lens. J Pediatr 1977;91:455-8.  Back to cited text no. 2
Hadi HA, Hobbs CL. Effect of chronic intrauterine stress on the disappearance of tunica vasculosa lentis of the fetal eye: A neonatal observation. Am J Perinatol 1990;7:23-5.  Back to cited text no. 3
Duke-Elder S. System of Ophthalmology, Vol. 3. 1st ed. London: Henry Kimpton; 1964. p. 775.  Back to cited text no. 4
Gavriş M, Horge I, Avram E, Belicioiu R, Olteanu IA, Kedves H. Persistent pupillary membrane or accessory iris membrane? Rom J Ophthalmol 2015;59:184-7.  Back to cited text no. 5
Coviltir V, Valentin D. ICE syndrome--Case report. Rom J Ophthalmol 2015;59:119-22.  Back to cited text no. 6
Miller D, Johnson R. Quantification of the pinhole effect. Surv Ophthalmol 1977;21:347-50.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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