Large Conjunctival Inclusion Cyst after Anterior Approach Ptosis Surgery

Abstract

An 8-year-old male child who underwent an anterior approach left upper lid levator resection to correct congenital ptosis. After 6 months, he developed mechanical ptosis secondary to a painless cystic mass on his upper lid. Magnetic resonance proved a postseptal circumscribed cystic mass. The lesion was excised and a histopathology examination confirmed a conjunctival inclusion cyst (CIC). CIC is common benign lesions of conjunctiva but rarely detected as a complication of levator muscle surgery.

Introduction

Conjunctival inclusion cysts (CICs) are common benign lesions, representing 60%–80%[1-3] of the entire cystic lesions of conjunctiva. CIC may originate from the implantation of the conjunctival epithelial cells into aberrant locations or may arise from traumatic implantation of epithelium.[4] CIC can be detected after subconjunctival or conjunctival infections[3,5] or after strabismus, glaucoma, retina, and pterygium surgery. However, CIC is seldom detected as a complication of ptosis repair[6] with only seven previously described CIC cases after levator muscle surgery [Table 1]. We are reporting a child with a CIC in the eyelid, developed after congenital ptosis repair using the anterior approach.

Table 1.

Cases reported in the literature of conjunctival inclusion cyst postupper lid levator muscle surgery

Author, date	Number of cases	Type of eyelid alteration	Age of surgery (year)	Type of surgery	Relapse time from ptosis surgery (weeks)	Cysts size (mm)	Histopathology exam
Wuebbolt et al., 1993[6]	1	Thyroid retraction	38	Posterior approach	8	Oval Adherent 3–4	Layer of nonkeratinizing squamous epithelium No globet cells Loose fibrous connective tissue with chronic inflammatory cells formed the cyst wall
	1	Thyroid retraction	56	Posterior approach	5
	1	Ptosis overcorrection	39	Posterior approach	10
Gonnering and Carol 1986[4]	1	Congenital ptosis	34	Anterior approach	4	12 irregular cystic mass	Cyst lined by one to two layers of low cuboidal epithelium Cyst contained in a fibrous stroma composed of smooth muscle and duct-like structures
	1	Congenital ptosis	6	Posterior approach	Shortly after surgery	10 irregular cystic mass	Nonkeratinizing squamous epithelium Ductule-like structures Few mucin-secreting cells
	1	Congenital ptosis	5	Posterior approach	1	Most of the upper eyelid
Hussin et al., 2007[5]	1	Congenital ptosis	First surgery: 5 Second surgery: 25 Third surgery: 30	Posterior approach		26	Bilayered epithelium-lined cyst with a collagenous connective tissue wall No dermal adnexal structures
Sameshima and Beyer- Machule 1988[7]	1	Congenital ptosis	11 (two times) 20		240 months	22	Nonkeratinizing bilayered epithelium
Present case	1	Congenital ptosis	4	Anterior approach	6 months	10	Nonkeratinizing cuboidal epithelial cells with scattered goblet cells and collagenous connective tissue wall Apocrine snouts Periodic acid–Schiff-positive material in the apical cytoplasm Immunohistochemical: GCDFP-15 negative Smooth muscle actin was absent in the myoepithelial layer

GCDFP: Gross cystic disease fluid protein

Case Report

An 8-year-old male child presented with recurrent eyelid ptosis associated to a painless cystic mass on his left upper lid. He had an anterior approach levator resection on his left congenital ptosis at the age of 5. His lid remained in a good position for 6 months, then ptosis recurred. Upon examination, there was a bluish, round cystic lesion in the upper lid, above the tarsal plate. Marginal reflex distance 1 was 4 mm in the right eye OD and 0 mm in the left OS; levator function was 18 mm OD and 5 mm OS. A palpable, fluctuant, well-delimited rounded cystic lesion of 10 mm of diameter, with no inflammatory signs, was detected extending down beyond the lid crease and causing mechanical ptosis [Figure 1a-c]. The lesion was not visible in lid eversion. Magnetic resonance imaging (MRI) demonstrated a well-defined and sharply circumscribed cystic lesion located within the upper left anterior orbit, measuring approximately 10 mm × 5 mm × 10 mm, isointense to vitreous in T2-weighted images, and hypointense on fluid-attenuated inversion recovery in T1-weighted images, with increased diffusion and no evidence of enhancement on T2 images [Figure 1b].

Figure 1.

(a) Preseptal mass in the left upper lid. (b) MRI: circumscribed mass in the left anterior orbit, moulding the globe. Isointense to vitreous on T2-weighted images, and contained a few septations. (c) Surgical excision. (d) Microscopic exam showed unilocular cyst (H and E, ×10). (e) Cyst lesion with two layers of cuboidal epithelium (H and E, ×20). (f) Apical cytoplasm of the lining cells was PAS positive staining (PAS, ×40). (g) Immunohistochemical stain: Negative expression of the epithelial component (GCDFP-15, ×20). (h) Immunohistochemical stain: Absence of myoepithelial layer in the cyst wall (SMA, ×20). MRI: Magnetic resonance imaging, GCDFP: Gross cystic disease fluid protein, PAS: Periodic acid–Schiff, SMA: Smooth muscle actin

An excisional biopsy was performed through an upper lid crease incision. Intraoperatively, a large, encapsulated, thin-walled smooth, and transilluminated cystic lesion located on top of the levator muscle was detected. Two stitches of 5-0 polypropylene (PROLENE, Ethicon, USA), used in the previous surgery, were found in the superior border of the tarsus. Although the threads were still present, the levator muscle was disinserted [Figure 1]. The cyst was removed in toto and the levator muscle was reinserted. Histopathological examination showed an unilocular solitary cystic lesion lined by two layers of nonkeratinizing cuboidal epithelial cells with scattered goblet cells and collagenous connective tissue wall [Figure 1d]. The two cells lining the epithelium showed apocrine snouts [Figure 1e] and periodic acid–schiff-positive material in the apical cytoplasm [Figure 1f]. The lumen contained clear fluid. Dermal adnexal structures were not identified. Immunohistochemical findings were negative for gross cystic disease fluid protein-15 (GCDFP-15) [Figure 1g] and smooth muscle actin was absent in the myoepithelial layer [Figure 1h]. The features were consistent with CIC. At 2-year follow-up, upper lid stays simetric with contralateral lid.

Discussion

We present an acquired CIC in a child with anterior approach ptosis repair. There are only eight other CIC cases after levator surgery, reported in the literature [Table 1].[4-7]

In our case, 6 months postsurgery, he had ptosis recurrence due to CIC. It is reported that the CIC may evolve soon after the levator surgery[6] or later on, with a case reported 20 years after ptosis repair.[7]

Our patient presented with a large bluish palpable cystic mass in the upper lid. CIC caused by trauma or surgery has been known to attain a large size, as in our case.[4,7]

The globe was not displaced in our case, similar to the others.[4-6] The mechanical ptosis in our patient resulted in a mass effect caused by CIC. However, levator dehiscence was also observed during the surgical procedure, similar to other cases.[4,7]

The MRI demonstrated heterogeneous signal intensity mainly on T1 fat-suppressed imaging and the cyst was hyperintense in the deepest part of the lesion, suggesting differences in the cyst content. In addition, there is enhancement on T2.[4]

We performed an excisional biopsy using the previous incision located in the upper lid skin crease and the cyst was removed in toto, which is the preferable technique.[3] Simple aspiration of the cyst content or partial removal is usually temporary are useless.[6]

Our patient had the first ptosis repair through anterior approach. Of seven CIC cases already published [Table 1], only our case and another one[6] had an anterior approach surgery. All oculoplastic surgeons know that the conjunctiva should remain untouched during anterior approach levator surgery. However, we think that in our case, the CIC resulted from a small rupture of the conjunctiva during the dissection, resulting in conjunctival tissue entraptment.[4] Another hypothesis of CIC etioloy is that the nonabsorbable suture used during the levator reinsertion may worked as a “guide” for epithelial conjunctival cells to grow amidst the eyelid tissues.

CIC diagnosis must be confirmed by histopathology examination. It is lined by nonkeratinized cuboidal or columnar squamous epithelium, intermixed with goblet cells is very similar to the conjunctival epithelium. Possible differential diagnoses can be dermoid cyst, excluded due to the absence of adnexal structures in the cystic wall. Sudoriferous cyst must be also rule out, which is characterized by apocrine snouts, myoepithelial layer through smooth muscle actin, and GCFP-15 positive as a marker of apocrine differentiation. However, snouts may also appear in some CIC, as in our case. Nevertheless, we confirm the nonapocrine origin by the presence of globet cells, the absence of hyperplastic apocrine glands, the negative immunohistochemical features for GCDFP-15, and the absence of myoepithelial layer. Other cystic lesions should be included in the differential diagnosis such as cysts derived from accessory lacrimal glands, epidermoid cyst, epithelium cyst, teratogenous cyst, neural cyst, inflammatory cyst, mucoceles, lacrimal duct cyst, follicular cysts, epidermal inclusion cyst, hemangioma, lymphangioma, eccrine acrospiroma, and a hydatic or parasitic cyst.[1,3,5]

Our patient did not present a recurrence 24 months after the CIC removal but a long-term follow-up is required.

In conclusion, the authors recommend awareness of epithelium entrapped in the surgical sites during a levator aponeurosis surgery. Meticulous intraoperative care during conjunctiva edges manipulation is the best option to prevent lesions such as CIC.

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.