Purpose
To describe a patient with 2 ipsilateral supernumerary caruncles and to determine if they are predictive of associated abnormalities.
Design
Retrospective and interventional clinicopathologic study with a critical review of the literature over the past 100 years.
Methods
Assessment of clinical features and histopathologic findings in paraffin-embedded tissue sections stained with hematoxylin and eosin, Masson trichrome, periodic acid–Schiff (PAS) reaction with and without diastase, and Ziehl-Neelsen method.
Results
Two placoid lesions causing ocular irritation after routine phacoemulsification were discovered in the inferomedial palpebral conjunctiva. They were totally separate from a normal caruncle. Their surface was studded with yellowish micronodules with projecting white vellus hairs. There were no associated local ophthalmic or systemic abnormalities. Microscopically, they were covered by a goblet cell–rich, nonkeratinizing squamous epithelium with subadjacent pilosebaceous units, the hairs of which were highlighted by the Masson trichrome and Ziehl-Neelsen stains, and small lobules of lacrimal gland tissue. The ocular irritation has not returned after surgery.
Conclusions
A literature review confirms that supernumerary (extra) caruncles coexisting with a normal caruncle are always unilateral and unassociated with any other ocular anomalies, as in the current case. They may, however, cause ocular irritation. They must be distinguished from ectopic (topographically displaced) or dysplastic caruncles that are generally bilateral and often associated with ocular adnexal abnormalities or Goldenhar syndrome. Ectopic or dysplastic caruncles, but not supernumerary ones, are characteristically accompanied by plical abnormalities or its absence. Well-documented supernumerary and ectopic caruncles have always been located in the inferior palpebral conjunctiva.
While tumors and other pathologic conditions of the caruncle have received substantial attention, very few articles over the past half-century or more have been devoted to malformations of this structure or the plica semilunaris. In particular, the distinction between a truly ectopic caruncle and a supernumerary caruncle has not always been clearly made. In this report we thoroughly document, clinically and histopathologically, a patient who had a normal caruncle with 2 additional, well-defined, neighboring supernumerary caruncles on the palpebral conjunctiva of the lower eyelid without any other local or syndromic associations. These lesions caused ocular irritation after cataract surgery. An ectopic caruncle, on the other hand, represents a displacement or dragging of a malformed (dysplastic) caruncle away from the position that a normal caruncle occupies. The principal reason to differentiate a supernumerary from an ectopic caruncle or a small dysplastic caruncle in its normal position is that the latter 2 can have other important associated ocular adnexal defects as well as involvement in Goldenhar syndrome.
Methods
A patient seen on referral in an academic institution–based corneal and external disease practice was evaluated for 2 palpebral conjunctival lesions. A thorough clinical history was taken and a complete ocular examination was performed including eversion of the eyelids and dilated fundus examination. Clinical photographs of the eyelid lesions were obtained with a slit-lamp camera. Histopathologic evaluation of the 2 specimens excised during the same surgery was performed on paraffin-embedded tissue and consisted of staining of 4- to 5-μm sections with hematoxylin and eosin, as well as with the periodic acid–Schiff (with and without pretreatment with diastase), Masson trichrome, and Ziehl-Neelsen acid-fast methods. The carefully assembled clinical and pathologic findings were closely compared with those from related and critically reviewed reports in the literature. A Pubmed search was conducted from 1960 to July 2009 using the keywords “caruncle,” “plica,” “malformation,” “ectopic,” “accessory,” “supernumerary,” and “dysplastic.” The bibliographies of relevant articles were scrutinized for any other studies bearing on the current subject. Only articles with adequate narrative descriptions of the clinical and pathologic findings, line diagrams, clinical photographs, and photomicrographs were cited in this article. Besides reports in English, others in German, French, Italian, Polish, and Russian were also reviewed with the assistance of translators fluent in the respective languages.
Results
Clinical Findings
A 52-year-old Egyptian man was referred with decreased vision caused by cataracts. His past ocular history was significant for refractive amblyopia in the left eye. On examination, his best-corrected visual acuity in the right eye was 20/30 with a refraction of −7.50 −1.25 × 180. The best-corrected vision in his amblyopic left eye was 20/400 with a refraction of +6.75 −0.50 × 060. The slit-lamp examination was significant for a moderate posterior subcapsular cataract in the right eye with an opalescent central nucleus. The left eye displayed mild nuclear sclerotic changes. The remainder of his ocular examination was unremarkable. Review of other organ systems failed to uncover any significant symptoms, dysfunctions, or surgeries. There was no evidence of facial or auricular anomalies, nor was there any evidence of microsomia, skin tags, cutaneous fistulae, or obvious clinical or vertebral abnormalities.
The patient underwent uncomplicated phacoemulsification surgery in the right eye. Corrected postoperative vision 1 week after surgery was 20/20 with a refraction of −1.00 −0.75 × 004. He reported minimal ocular discomfort. His postoperative medication of prednisolone acetate 1% was tapered from 1 drop 4 times a day to 1 drop every 7 days. He returned for an emergency visit 1 month later, complaining of unilateral right eye pruritus, epiphora, photophobia, and hyperemia, which started 1 week after stopping his ocular medications. On examination, his vision with spectacle correction was 20/20 in the right eye; he had multiple inspissated meibomian gland orifices at the eyelid margin and moderate blepharitis in both eyes. There was trace conjunctival hyperemia in the right eye. The anterior chamber was quiet and dilated fundus examination was normal. He was diagnosed with blepharitis and meibomitis and was started on a regimen of eyelid hygiene consisting of vasocidin ointment at night, doxycycline 100 mg twice daily, and prednisolone acetate 0.12% twice daily in the right eye.
He returned for follow-up 2 weeks later with worsening symptoms and a new foreign body sensation in the right eye. On examination, the conjunctiva of the right eye displayed moderately severe hyperemia with increased tearing. On eversion of the right lower eyelid, a mildly elevated, oval mass was discovered in the inferomedial palpebral conjunctiva beneath the punctum. It did not involve the eyelid margin and measured 6 × 5 mm ( Figure 1 , Left and Right). It was separated from a normal-appearing caruncle by a strip of uninvolved conjunctiva and contained numerous yellow micronodules sprouting fine white vellus hairs ( Figure 1 , Right). Another, smaller mass in the middle third of the palpebral conjunctiva with similar characteristics was located lateral to the larger mass, from which it was separated by smooth, glistening intervening conjunctiva ( Figure 1 , Left).
After further consultation and discussion with the patient, he elected to undergo excision of both lesions for diagnosis and relief of symptoms. Intraoperatively, the margins of these lesions were noted to be ill-defined and they did not extend into the tarsus. After excision of each lesion, the tarsal conjunctival defect was covered with an amniotic membrane graft (Bio-Tissue, Miami, Florida, USA) secured with Tisseel (Baxter, Deerfield, Illinois, USA) fibrin sealant in an overlay fashion. By the first postoperative week, the patient had complete resolution of symptoms, which has continued over 10 months of follow-up.
Histopathologic Findings
The 2 excised specimens each measured approximately 1 × 0.3 cm and consisted of soft tan-brown tissue with a pinkish glistening mucosa. The separately labeled medial and more laterally located lesions exhibited virtually identical histopathologic features. In both lesions, the epithelium was 4 to 6 cells thick and composed of nonkeratinizing squamous epithelium with a rich admixture of goblet cells ( Figure 2 , Top left). The goblet cells stained deep magenta with the periodic acid–Schiff (PAS) method ( Figure 2 , Top right and inset). Immediately beneath the epithelium was a light diaspora of lymphocytes and plasma cells set in a loose collagenous stroma. Numerous lobules of sebaceous glands were located in the substantia propria. Small collections of lymphocytes were occasionally found in intimate relationship to the sebaceous lobules and hairs ( Figure 2 , Top right). The Masson trichrome stain revealed an adventitial collagen investing the pilosebaceous units in a circumferential pattern ( Figure 1 , Middle left). Small lacrimal gland lobules were discovered in each specimen and were composed of acini containing PAS-positive, diastase-resistant zymogen granules ( Figure 2 , Middle right); exiting ductules had more columnar epithelial cells devoid of granules. Adipose tissue was distributed throughout the lower half of the 2 specimens. In the more medial lesion, widely separated myofibers of orbicularis muscle were identified ( Figure 2 , Bottom left), whereas in the more lateral one small fascicles of Mueller smooth muscle were observed ( Figure 2 , Bottom left insets), both of which were Masson trichrome positive. The hairs in the stroma were highlighted by the trichrome stain ( Figure 2 , Bottom left). The medullary cores of the delicate vellus hairs embedded in the stroma and emanating through the orifices of the pilosebaceous units were vividly demonstrated with the Ziehl-Neelsen acid-fast stain ( Figure 2 , Bottom right and insets). Compact tarsal collagen at the base, nerve twigs, and foci of hyalin cartilage were not identified in either lesion.
Results
Clinical Findings
A 52-year-old Egyptian man was referred with decreased vision caused by cataracts. His past ocular history was significant for refractive amblyopia in the left eye. On examination, his best-corrected visual acuity in the right eye was 20/30 with a refraction of −7.50 −1.25 × 180. The best-corrected vision in his amblyopic left eye was 20/400 with a refraction of +6.75 −0.50 × 060. The slit-lamp examination was significant for a moderate posterior subcapsular cataract in the right eye with an opalescent central nucleus. The left eye displayed mild nuclear sclerotic changes. The remainder of his ocular examination was unremarkable. Review of other organ systems failed to uncover any significant symptoms, dysfunctions, or surgeries. There was no evidence of facial or auricular anomalies, nor was there any evidence of microsomia, skin tags, cutaneous fistulae, or obvious clinical or vertebral abnormalities.
The patient underwent uncomplicated phacoemulsification surgery in the right eye. Corrected postoperative vision 1 week after surgery was 20/20 with a refraction of −1.00 −0.75 × 004. He reported minimal ocular discomfort. His postoperative medication of prednisolone acetate 1% was tapered from 1 drop 4 times a day to 1 drop every 7 days. He returned for an emergency visit 1 month later, complaining of unilateral right eye pruritus, epiphora, photophobia, and hyperemia, which started 1 week after stopping his ocular medications. On examination, his vision with spectacle correction was 20/20 in the right eye; he had multiple inspissated meibomian gland orifices at the eyelid margin and moderate blepharitis in both eyes. There was trace conjunctival hyperemia in the right eye. The anterior chamber was quiet and dilated fundus examination was normal. He was diagnosed with blepharitis and meibomitis and was started on a regimen of eyelid hygiene consisting of vasocidin ointment at night, doxycycline 100 mg twice daily, and prednisolone acetate 0.12% twice daily in the right eye.
He returned for follow-up 2 weeks later with worsening symptoms and a new foreign body sensation in the right eye. On examination, the conjunctiva of the right eye displayed moderately severe hyperemia with increased tearing. On eversion of the right lower eyelid, a mildly elevated, oval mass was discovered in the inferomedial palpebral conjunctiva beneath the punctum. It did not involve the eyelid margin and measured 6 × 5 mm ( Figure 1 , Left and Right). It was separated from a normal-appearing caruncle by a strip of uninvolved conjunctiva and contained numerous yellow micronodules sprouting fine white vellus hairs ( Figure 1 , Right). Another, smaller mass in the middle third of the palpebral conjunctiva with similar characteristics was located lateral to the larger mass, from which it was separated by smooth, glistening intervening conjunctiva ( Figure 1 , Left).
