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CASE REPORT

Conjunctival Lymphangiectasia Presenting as Pediatric


Pseudopterygium
Natalia Pastora, M.D., Jesus Peralta-Calvo, M.D, Ph.D., Laura Yebenes-Gregorio, M.D., Jose Abelairas, M.D, Ph.D.,
and Almudena Hierro-Zarzuelo, M.D.

begun to invade the cornea over the previous 3 months, and the
Objectives: The aim of this study is to report a case of conjunctival
lymphangiectasia simulating a pediatric pterygium.
patient reported continuous itching, resulting in ocular rubbing.
Methods: A 10-year-old girl with Turner syndrome and familial history Treatment included artificial tears, antiallergic eye drops, and
of pterygia presented because of a conjunctival growing lesion clinically consis- steroid and antibiotic combinations, all without relief. Visual acuity
tent with pterygium in her left eye. Visual acuity (VA) was 20/20 in the right eye (VA) was 20/20 in the right eye (oculus dexter [OD]) and 20/40 in
and 20/40 in the left eye. Cycloplegic refraction was +1.50sph 20.50cyl·93° the left eye (oculus sinister [OS]). There was an intermittent
and +9.00sph 29.00cyl·180° in the right and left eyes, respectively. exotropia of 25° with dominance of the OD. Slitlamp examination
Results: Resection of the lesion with amniotic membrane implantation and of the OD was unremarkable, but the OS showed a conjunctival
conjunctival autograft was performed. The histologic examination revealed triangular-shaped lesion from the conjunctiva extending onto the
conjunctival lymphangiectasia. Ten months later, the patient did not show limbus and cornea with Fuchs islets in the apex (Fig. 1). Intraocular
any signs of recurrence. Refraction in the left eye was +0.5sph 21.5cyl·70°,
pressure and funduscopy were within normal limits. Cycloplegic
and spontaneous VA was 20/20 in both eyes.
Conclusions: Pediatric pseudopterygium may be the clinical presentation
refraction was OD +1.50sph 20.50cyl·93°, OS +9.00sph
of several ocular surface disorders. Thus, pathologic analysis of this lesion 29.00cyl·180°. The clinical diagnosis was consistent with ptery-
should be considered to determine its exact nature. Surgery lessens the gium. The patient and her family were informed and approved the
refractive defect and hastens visual recovery. surgical procedure. Because of poor results for surgical resection of
familial pterygium in the pediatric population,1 we performed
Key Words: Conjunctival lymphangiectasia—Pediatric pterygium—
a combined approach. Under general anesthesia, the conjunctiva
Pseudopterygium—Turner syndrome.
was incised with Wescott scissors down to the bare sclera. The
(Eye & Contact Lens 2013;39: e12–e14) head of the lesion was gently peeled from the cornea. The scleral
portion of the lesion was excised with Wescott scissors, taking care
to identify the rectus muscle. The bare scleral defect measured

P terygium is rare in childhood and is generally related to xero-


derma pigmentosum or familial pterygium.1,2 Surgical resec-
tion should be advised in cases with growth of the pterygium into
11 mm·11 mm. Amniotic membrane (AM) was placed over the
entire excised area and fixed to the underlying sclera with fibrin
glue. A superior conjunctival autograft measuring 12 mm·12 mm
or close to the central visual axis, with induced amblyopia or with was carefully dissected away from the Tenon capsule and placed in
malignancy suspicion. Pathologic analysis of the resected lesion the recipient bed, maintaining the flap orientation. It was then fixed
provides a definite diagnosis not only in patients of pterygia but
also in patients with pseudopterygia. We present a case of con-
junctival lymphangiectasia simulating a pediatric pterygium, in
which histologic examination revealed the correct diagnosis.

MATERIALS AND METHODS


A 10-year-old Hispanic girl with Turner syndrome presented to
the office with a lesion in her left eye that had appeared and
progressively grown during the last year. Familial history was
significant for pterygia. The parents reported that the lesion had

From the Ophthalmology Department (N.P., J.P.C., J.A., A.H.Z.), Hospital


Universitario La Paz, Madrid, Spain; From the Pathology Department (L.Y.
G), Hospital Universitario La Paz, Madrid, Spain.
The authors have no funding or conflicts of interest to disclose.
Address correspondence and reprint requests to Natalia Pastora, M.D.,
Hospital Universitario La Paz, Paseo de la Castellana 261, 28046 Madrid,
Spain; e-mail: ntl.pastora@gmail.com
Accepted April 2, 2012. FIG. 1. Triangular-shaped, vascularized conjunctival lesion encroach-
DOI: 10.1097/ICL.0b013e31825878bd ing centripetally onto the cornea.

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Eye & Contact Lens  Volume 39, Number 3, May 2013 Conjunctival Lymphangiectasia Presenting

FIG. 4. Slitlamp view 10 months after surgery, no recurrence.

with fibrine glue to the AM, and because of the patient’s age, was
also secured with 5 interrupted 8-0 Vicryl sutures to the healthy
adjacent conjunctiva. A therapeutic soft contact lens was inserted,
and a pressure patch was applied for 24 hrs.

RESULTS
The day after the operation, the autograft remained in place
(Fig. 2), and the patient was given tobramycin and dexamethasone
eye drops 5 times daily for 1 month. Pathologic diagnosis was
conjunctival lymphangectasia (Fig. 3).
FIG. 2. Top (hematoxylin and eosin, ·10): dilated subepithelial
channels, without elastotic degeneration; bottom (D2-40 immunos-
One week after the operation, the superior donor conjunctiva
taining, ·10), positive staining in thin walled channels, reflecting was epithelized. Refraction was +0.5sph 21.5cyl·70°, and VA of
their lymphatic nature (lymphangiectasia). 20/30 in OS. Ten months after surgery, VA was 20/20 in both eyes,
and there was no recurrence (Fig. 4).

DISCUSSION
Pterygia and pseudopterygia are exceptional in childhood and
may be the clinical presentation of several, benign and malignant,
ocular surface disorders.1–3 Differential diagnosis of pseudoptery-
gium includes primary and secondary keloids,3,4 limbal dermoids,5
ulcerative and nonulcerative peripheral keratitis,6 focal limbal
stem-cell deficiency,7 Salzmann nodules,8 conjunctival intraepithe-
lial neoplasia,9 and conditions with chronic corneal epithelial dam-
age such as contact lens use or atopic keratoconjunctivitis.10
Squamous conjunctival cell carcinoma may also simulate a pseu-
dopterygium, especially in patients with xeroderma pigmentosum.9
The current case involved masked presentation as pseudoptery-
gium, of conjunctival lymphangiectasia in a patient with Turner
syndrome. Conjunctival lymphangiectasia is a rare condition in
which lymphatic vessels are dilated and prominent within the bulbar
conjunctiva.11 Most cases of conjunctival lymphagiectasia represent
secondary lymphedema after obstruction or disruption of the lym-
FIG. 3. Postoperative (24 hrs) after pseudopterygium resection, phatic system by inflammation, surgery, radiotherapy, scarring, or
amniotic membrane implantation, and conjunctival autografting. neoplastic disease. Infantile conjunctival lymphangectasia or

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N. Pastora et al. Eye & Contact Lens  Volume 39, Number 3, May 2013

lymphedema are usually primary, caused by hypodevelopment of 6. Lekhanont K, Patarakittam T, Mantachote K, et al. Progressive keratolysis
the lymphatic system, and included in disorders with significant with pseudopterygium associated with erythema elevatum diutinum. Oph-
thalmology 2011;118:927–933.
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nay–Weber syndrome,14 conjunctival and peripheral lymphedema Concept, aetiology, clinical presentation, diagnosis and management. Ind J
and/or distichiasis,15 and Nonne–Milroy–Meige disease.16 There- Ophthalmol 2000;48:83–92.
fore, a diagnosis of infantile conjunctival lymphangiectasia should 8. Das S, Link B, Seitz B. Salzmann’s nodular degeneration of the cornea:
A review and case series. Cornea 2005;24:772–777.
involve a general examination to eliminate other disorders.
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In the patient described in this report, atypical presentation of xeroderma pigmentosum: Clinical spectrum and outcome. Graefes Arch
conjunctival lymphangiectasia may have been caused by eye Clin Exp Ophthalmol 2011;249:1217–1221.
rubbing, which may have caused superficial defects in the corneal 10. Power WJ, Tugal-Tutkun I, Foster CS. Long-term follow-up of patients with
and conjunctival epithelium that favored the adhesion of the lesion atopic keratoconjunctivitis. Ophthalmology 1998;105:637–642.
11. Welch J, Srinivasan S, Lyall D, et al. Conjunctival lymphangiectasia: A
onto the limbus and cornea. The refractive defect could be the report of 11 cases and review of literature. Surv Ophthalmol 2012;57:
result of corneal flattening induced by contractile intralesional cells 136–148.
and lacrimal pooling in the apex of the lesion, similar to 12. Perry HD, Cossari AJ. Chronic lymphangiectasis in Turner’s syndrome.
pterygium.17,18 Br J Ophthalmol 1986;70:396–399.
13. Austin MW, Patterson A, Bates RA. Conjunctival lymphoedema in Turner’s
Although conjunctival lymphangectasia is not progressive, it can
syndrome. Eye (Lond) 1992;6:335–336.
produce irritation and discomfort.19 This case shows for the first 14. Belliveau MJ, Brownstein S, Jackson WB, et al. Bilateral conjunctival
time that it can also cause a significant refractive defect and lymphangiectasia in Klippel–Trénaunay–Weber syndrome. Arch Ophthal-
reduction of VA. mol 2009;127:1057–1058.
Several therapeutic strategies for conjunctival lymphangiectasia 15. Ramasubramanian A, Shields CL, Palamar M, et al. Conjunctival edema
and distichiasis in association with congenital lymphedema of the lower
have been described including surgical excision,20 liquid nitrogen legs. Ophthal Plast Reconstr Surg 2009;25:148–150.
cryotherapy,21,22 carbon dioxide laser ablation,23,24 transconjuncti- 16. Tabbara KF, Baghdassarian SA. Chronic hereditary lymphedema of the legs
val coagulation diathermy,25 and beta-irradiation.26 Surgical exci- with congenital conjunctival lymphedema. Am J Ophthalmol 1972;73:
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Mechanism of corneal topographic changes. Cornea 1990;9:200–204.
val lymphangiectasia.11 18. Touhami A, Di Pascuale MA, Kawatika T, et al. Characterisation of
In conclusion, this case reminds us that, in children, pseudop- myofibroblasts in fibrovascular tissues of primary and recurrent pterygia.
terygium may be the clinical presentation of different ocular Br J Ophthalmol 2005;89:269–274.
surface pathologic conditions. Thus, a histologic examination of 19. Shields JA, Mashayekhi A, Kligman BE, et al. Vascular tumors of the
conjunctiva in 140 cases. Ophthalmology 2011;118:1747–1753.
the lesion should be considered. In addition, this case report 20. Meisler DM, Eiferman RA, Ratliff NB, et al. Surgical management of
illustrates that pediatric pterygium or pseudopterygium can be conjunctival lymphangiectasis by conjunctival resection. Am J Ophthalmol
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21. Fraunfelder FW. Liquid nitrogen cryotherapy for conjunctival lymphangiec-
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