Clinical features of intratarsal keratinous cysts

ABSTRACT PURPOSE Intratarsal keratinous cysts (IKCs) have been frequently misdiagnosed as chalazia or epidermal cysts. We reviewed a series of cases of IKCs to identify clinical features

that distinguish IKCs from other eyelid diseases. METHODS We retrospectively reviewed the medical records of 17 suspected IKC patients between January 2004 and September 2014. RESULTS

Seventeen patients who were clinically suspected to have IKC were enrolled. All patients presented with non-inflamed eyelid nodules fixed to the tarsus. Among them, 12 biopsy specimens were

available and 11 patients (91.7%) were diagnosed with IKC, with a pathological finding of stratified squamous cell lining with keratin material. The mean patient age was 55.1 years (31–71).

Six patients had a surgical history of incision or incomplete excision of the lesion, followed by recurrence. On eyelid eversion, five patients showed a white–yellow nodule, and three

patients had a bluish cystic lesion. The diameter of the nodules ranged from 4 to 10 mm. The intracystic material was a milky white fluid. Ten patients underwent a complete surgical excision

including partial tarsectomy and there was no recurrence. CONCLUSIONS IKC can be distinguished from other intratarsal lesions by a characteristic tarsal nodule fixed to the tarsus. To

prevent recurrence, complete excision with partial tarsectomy is needed. SIMILAR CONTENT BEING VIEWED BY OTHERS THE SURGICAL MANAGEMENT AND OUTCOMES OF KISSING NEVI OF THE EYELIDS Article 11

March 2023 A RETROSPECTIVE STUDY OF CONJUNCTIVAL LESIONS IN THE PAEDIATRIC EYE CLINIC OVER 12 YEARS Article 26 September 2023 COMPARISON OF THREE SURGICAL TECHNIQUES FOR INTERNAL ANGULAR

DERMOID CYSTS: A RANDOMIZED CONTROLLED TRIAL Article 20 November 2021 INTRODUCTION Most common primary intratarsal lesions are chalazia and sebaceous gland carcinomas.1 An intratarsal

keratinous cyst (IKC), recently described by Jakobiec _et al_,2 is an intratarsal mass lesion that originates from the meibomian glands or their ducts. Importantly, IKC has been frequently

misdiagnosed as chalazion or other benign eyelid cystic tumors because it is an intratarsal hard nodular mass. It is important to differentiate IKC from other eyelid intratarsal lesions

because its therapeutic approach and clinical outcomes are quite different; IKC has to be completely excised with partial tarsectomy. In contrast, a chalazion is easily treated with a simple

incision and curettage (I&C). Sebaceous gland carcinomas often metastasize and contribute to mortality despite radical surgical excision. Some reports3, 4 have highlighted the

histopathological features of IKC. However, there is no published report of the correlation between clinically suspected IKC and confirmed diagnosis of IKC by pathological examination. In

this study, we report on 11 cases of histologically confirmed IKC and their clinical features. MATERIALS AND METHODS Patients who underwent surgical treatment for eyelid masses at four

different medical centers from January 2004 to September 2014 were recruited, and their medical records and photographs were retrospectively reviewed. The records of patients who were

assumed to have IKC were collected and their pathologic slides were re-examined by an experienced pathologist (CL). The data included sex, age at the time of excision, location, color and

size of the lesions, related symptoms, duration of symptoms, findings on external examination and eyelid eversion, previous diagnosis, treatment, intraoperative findings, previous history of

treatment, follow-up duration, and recurrence. This study complied with the Declaration of Helsinki guidelines and was approved by the Institutional Review Board of Seoul Metropolitan

Government Seoul National University Boramae Medical Center, Seoul, Korea. RESULTS Seventeen patients who were clinically suspected to have IKC were enrolled. All patients presented with

non-inflamed firm nodules fixed to the tarsus. Among them, the biopsy specimens of 12 patients were available and 11 patients (91.7%) were given a confirmed diagnosis of IKC by pathological

examination (Table 1). The mean patient age was 55.1 years (range 31–71 years), and five patients were men and six were women. The overlying skin was freely movable without focal adhesion.

All mass lesions were located at the upper eyelid. On eyelid eversion, well-demarcated upraised intratarsal lesions were found through the conjunctiva, and the surrounding conjunctiva was

not inflamed. The nodules had a diameter of ~4–10 mm, with a mean diameter of 6.6 mm (Table 1). Five patients showed a white to yellow nodular bulge and three patients showed a bluish cystic

lesion (Figure 1). All IKCs were solitary except one in which multiple lesions distorted the eyelid margin. One clinically misdiagnosed patient was diagnosed as having an epidermal cyst.

Six patients had a history of incision or incomplete excision followed by recurrence. Previously, before recognition of IKC as an entity, patients were clinically diagnosed as having another

disease; two patients as having a chalazion, three patients as having an epidermal cyst, and four patients as having a tarsal mass. Pathological examination, which was performed on 12

patients prior to the discovery of IKC, indicated that 3 patients had an epidermal inclusion cyst, 3 patients had a steatocystoma, 2 patients had a keratinous cyst, 1 patient had a cyst of

Moll’s gland, 1 patient had a pilar cyst, and 1 patient had a benign cyst with fibrovascular tissue. After review by an experienced pathologist, all cases except one were re-diagnosed as IKC

(Figure 2). Ten patients underwent surgical excision including partial tarsectomy: four patients through a transcutaneous approach, four patients through a transconjunctival approach, and

two patients through an unrecorded approach. We did not suture the conjunctiva after using the transconjunctival approach and there were no corneal complications. Six cases were ruptured

during dissection and a milk-like fluid mixed with solid elements having the consistency of tofu residue, which is different from the ordinary content of chalazia, was extruded. None of the

patients experienced recurrence during the follow-up, which had a mean duration of 5 weeks (range, 1–24 weeks). Among the 12 patients who were clinically suspected as having IKC, only one

patient was ultimately pathologically diagnosed with an epidermal cyst. In this patient, an intratarsal gray–black mass was also observed on eyelid eversion. However, on pathological

examination, the cyst was lined by focal acanthotic squamous epithelium and had a granular layer in the cyst wall. Thus, these findings were indicative of an epidermal cyst, rather than IKC.

DISCUSSION IKCs are a recently described entity that arises from the Meibomian glands and are thought to be the third major cause of intratarsal swelling after chalazia and sebaceous cell

carcinomas.2 The clinical characteristics of IKCs are similar to those of chalazia and epidermal inclusion cysts, but there are differentiating features that enable physicians to distinguish

them. For example, lesions such as steatocystomas and pilomatrixomas are white to yellow, as are IKCs. In contrast, although chalazia are fixed to the tarsus similarly to IKC, they are

frequently accompanied by surrounding inflammation and the margin of the lesion is less clear than that of an IKC. These features are helpful for differentiation of chalazia from IKC.5 An

epidermal inclusion cyst is a non-inflammatory, palpable nodule that is not fixed to the tarsus but is freely movable, which is another differentiating characteristic with regard to IKC.6

The cyst content of a steatocystoma is a yellowish oily sebum containing hair shafts, and steatocystomas are not attached to the tarsus.7 Only two cases of pilomatrixoma observed at the

tarsal conjunctiva have been reported.8, 9 Usually, pilomatrixoma grows toward the skin and is freely movable from the tarsus because it is thought to originate from hair follicle cells.

Furthermore, patients with pilomatrixoma are usually young, and hyperemia is observed surrounding the lesion, in contrast to IKC. IKCs occasionally have a blue–gray color because of the

Tyndall effect, and a blue IKC is occasionally misdiagnosed as melanoma.2 As awareness of IKC increases, histopathological differences between this entity and other diseases are being

revealed. Even though IKC and steatocystoma share similar histological findings, such as trichilemmal keratinization, steatocystomas contain sebaceous glands in the cyst wall or adjoining

wall. However, for most cases, a careful clinical examination can provide sufficient evidence for a differential diagnosis. With the aforementioned clinical characteristics in mind, we

reviewed medical photographs and records to identify cases that were previously diagnosed as chalazia or epidermal cysts and were now clearly re-diagnosed as IKC. As a result, among 12

patients who were clinically suspected as having IKC, only one patient was ultimately pathologically diagnosed with an epidermal cyst. Previously, IKC was a commonly misdiagnosed lesion.

However, with further investigation, we found that most cases were correctly diagnosed as IKC. Regarding pathogenesis, Lucarelli _et al_10 suggested that eyelid surgery or trauma may cause a

blockage of the tarsal sebaceous ducts and squamous metaplasia, leading to the development of IKCs. In our study, there were eight cases of tarsal nodule excision or incision and one case

of a double eyelid operation, which occurred ~23 years ago. However, Jakobiec _et al_2 reported that IKC consists of a firm fibrous cyst and intracystic content, and that the fibrous wall of

the cyst is composed of tightly woven bundles of collagen. They also reported that the ducts and ductules of the Meibomian glands were composed of non-keratinizing squamous epithelium, and

there was no histological evidence of squamous metaplasia. Therefore, we did not exclude the patient who had a previous double eyelid operation from this study. In our study, there was one

case with multiple lesions. In 2012, Kim _et al_11 reported multiple IKCs of the eyelid for the first time. Subsequently, to our knowledge, there has been no report of multiple IKCs, and

thus, we assume that multiple IKCs are uncommon. Furthermore, we could not find a cause for the multiple cysts in our case, which is consistent with the case of Kim _et al_ Further

observation would be needed to understand the mechanism of multiple lesions. Therapeutically, it is important to distinguish IKC from other similar lesions because IKC requires complete

excision. There are many cases of multiple recurrences that were previously diagnosed as chalazia and subsequently operated on with an incomplete excision or I&C. The multiple

recurrences of chalazia suggest that the diagnosis was incorrect. To excise an IKC, a partial tarsectomy is mandatory because of its firm adhesion to the tarsus, and this procedure that will

remarkably reduce recurrence.2 Surgically, there are following two approaches: transcutaneous and transconjunctival. Patel _et al_3 reported that an anterior lid crease approach with full

excision of the intratarsal cyst and partial excision of the underlying anterior tarsus is effective for preventing local recurrence and avoiding more complex lid reconstruction. In

addition, Zhang _et al_4 suggested that a transconjunctival incision avoids an eyelid incision and disfiguration. They suggested that because the IKC is fixed to the tarsus and is

independent of the skin, the conjunctival approach with partial tarsectomy is a better way to excise the entire lesion and simultaneously avoid cosmetic problems. Comparisons of

transconjunctival _vs_ transcutaneous approaches for lower eyelid blepharoplasty or orbit wall fracture repair have been studied previously. However, no report has identified a better

approach for tarsal lesions. In our study, there were four cases with a transcutaneous approach and four cases with a transconjunctival approach, and no patients reported dissatisfaction

with the cosmetic outcomes. There was also no case of severe eyelid contour distortion or scar formation. In this study, we identified 11 cases of IKC and their clinical features. It is

important to distinguish IKC from other similar lesions because IKC requires complete excision. The results indicate that multiple recurrences after I&C or incision and drainage of a

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Grossniklaus HE . Multiple intratarsal keratinous cysts of the eyelid. _Ophthal Plast Reconstr Surg_ 2012; 28 (5): e116. Article  Google Scholar  Download references AUTHOR INFORMATION

AUTHORS AND AFFILIATIONS * Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea J A Kim & S I Khwarg * Department of Ophthalmology, Seoul National

University Bundang Hospital, Seongnam, Korea N Kim * Department of Ophthalmology, Seoul Metropolitan Government–Seoul National University Boramae Medical Center, Seoul, Korea H-K Choung *

Department of Ophthalmology, Hallym University Sacred Heart Hospital, Anyang, Korea M J Lee * Department of Pathology, Seoul National University College of Medicine, Seoul, Korea C Lee

Authors * J A Kim View author publications You can also search for this author inPubMed Google Scholar * N Kim View author publications You can also search for this author inPubMed Google

Scholar * H-K Choung View author publications You can also search for this author inPubMed Google Scholar * M J Lee View author publications You can also search for this author inPubMed 

Google Scholar * C Lee View author publications You can also search for this author inPubMed Google Scholar * S I Khwarg View author publications You can also search for this author inPubMed

 Google Scholar CORRESPONDING AUTHOR Correspondence to H-K Choung. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no conflict of interest. ADDITIONAL INFORMATION This paper has

not been presented in any academic conference. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Kim, J., Kim, N., Choung, HK. _et al._ Clinical features

of intratarsal keratinous cysts. _Eye_ 30, 59–63 (2016). https://doi.org/10.1038/eye.2015.184 Download citation * Received: 02 March 2015 * Accepted: 25 July 2015 * Published: 02 October

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