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Letters to the Editor

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epithelium (adenoma and adenocarcinoma). Ophthalmology 1996; 103: 2007–16. Bae JH, Kwon JE, Yang WI, Lee SC. Adenoma of the nonpigmented ciliary epithelium presenting with recurrent iridocyclitis: unique expression of glial fibrillary acidic protein. Graefes Arch Clin Exp Ophthalmol 2011; 249: 1747–9. Pecorella I, Ciocci L, Modesti M, Appolloni R. Adenoma of the non-pigmented ciliary epithelium: a rare intraocular tumor with unusual immunohistochemical findings. Pathol Res Pract 2009; 205: 870–5. Santagata S, Maire CL, Idbaih A et al. CRX is a diagnostic marker of retinal and pineal lineage tumors. PLoS ONE 2009; 4: e7932. Terry J, Calicchio ML, Rodriguez-Galindo C, Perez-Atayde AR. Immunohistochemical expression of CRX in extracranial malignant small round cell tumors. Am J Surg Pathol 2012; 36: 1165–9. Jakobiec FA, Tso MO, Zimmerman LE, Danis P. Retinoblastoma and intracranial malignancy. Cancer 1977; 39: 2048–58. Mamalis N, Font RL, Anderson CW, Monson MC, Williams AT. Concurrent benign teratoid medulloepithelioma and pineoblastoma. Ophthalmic Surg 1992; 23: 403–8. Desai VN, Lieb WE, Donoso LA, Eagle RC Jr, Shields JA, Saunders R. Photoreceptor cell differentiation in intraocular medulloepithelioma: an immunohistopathologic study. Arch Ophthalmol 1990; 108: 481–2. Perentes E, Rubinstein LJ, Herman MM, Donoso LA. S-antigen immunoreactivity in human pineal glands and pineal parenchymal tumors. A monoclonal antibody study. Acta Neuropathol 1986; 71: 224–7. Mullen RJ, Buck CR, Smith AM. NeuN, a neuronal specific nuclear protein in vertebrates. Development 1992; 116: 201–11. Kim KK, Adelstein RS, Kawamoto S. Identification of neuronal nuclei (NeuN) as Fox-3, a new member of the Fox-1 gene family of splicing factors. J Biol Chem 2009; 284: 31052–61. Froen R, Johnsen EO, Nicolaissen B, Facsko A, Petrovski G, Moe MC. Does the adult human ciliary body epithelium contain ‘true’ retinal stem cells? Biomed Res Int 2013; doi: 10.1155/2013/531579. [Epub ahead of print.]

Ruptured canthal steatocystoma simplex presenting as a lacrimal sac mass Steatocystoma simplex (SS) is a benign nevoid malformation of the pilosebaceous duct junction zone.1 The entity of Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.

385 SS was first described as a solitary lesion in the dermatology literature in 1982.1 The more common autosomal dominantly inherited condition of multiple cutaneous cysts (often on the upper limbs and trunk) is known as steatocystoma multiplex (SM).2 Four questionable lesions of SS in the ocular adnexa have been reported: two in the caruncle and two in the eyelids.3,4 We report a case of a ruptured SS that simulated a lacrimal sac mass. A 21-year-old man presented with a five-year history of stable swelling over the left lacrimal sac that had begun to increase over a one-year period. On examination, a mass was noted in the lacrimal sac region below the medial canthal tendon (Fig. 1a, left panel). It was fluid filled but non-tender (with normal overlying skin). Computed tomography scan revealed a hypodense mass with fat density anterior to the lacrimal sac (Fig. 1b). Intraoperatively, during excision, a dense fibrous wall was found. The cyst appeared to have ruptured and contained residual yellow material similar to fat with adhesions to the underlying tissues (Fig. 1a, right panel). No other skin lesions were detected, and there was no family history of similar lesions. Histopathologically, a large cyst with an adjacent area of lipogranulomatous inflammation was observed (Fig. 1c). Foreign body giant cells replaced a portion of the cyst’s lining (Fig. 1d). Sebaceous lobules were plentiful near and within the cyst wall (Fig. 2a) with a direct connection demonstrated between the sebaceous lobules and the cyst’s lining (Fig. 2b). The latter was composed of stratified squamous epithelium lacking a keratohyalin layer. A prominent acellular, undulating, cuticle was present as the innermost layer (Fig. 2c). Abortive hair structures were also seen. Immunohistochemistry demonstrated that the cyst’s lining was cytokeratin (CK) 14+ in its full thickness (Fig. 2d, top panel) and CK17+ only in the suprabasilar cells (Fig. 2d, bottom panel). SS is extremely rare in the periocular skin and has not been reported in the lacrimal sac region. SS is the solitary counterpart to the histopathologically identical inherited condition of SM.1 Clinically, lesions are typically well circumscribed, soft, freely movable, non-tender and contain an oily yellow liquid.1 Several of the earlier reported cases in the eyelid are more likely to have been Meibomian gland keratinous duct cysts with secondary atrophy of the sebaceous lobules, leading to a histopathologic appearance mimicking SS.3 True SS is filled with sebum rather than keratin. The microscopic hallmarks of SS that distinguish it from a dermoid cyst are lack of a keratohyalin layer in the squamous lining, a crenulated eosinophilic inner membrane, and sebaceous lobules or individual sebocytes within or adjacent to the cyst wall,1,2 features also described in a keratinous sebaceous duct cyst of the caruncle.5 CK expression in SS is comparable to that of sebaceous ductal cells (CK17+ in the suprabasilar layers and CK14+ in the full-thickness epithelium).6 Epidermoid cysts are CK14+/ CK17−. Foreign-body giant cells and a lipogranulomatous response can be seen, as in this case, due to rupture.2 Unlike SS, dermoid cysts are usually present at birth, develop within embryologic/bony cleavage lines or

© 2014 Royal Australian and New Zealand College of Ophthalmologists

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Letters to the Editor

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Figure 1. Clinicopathologic features of steatocystoma simplex (SS). (a) Left panel: A 21-year-old man had a five-year history of a stable swelling below the medial canthal tendon with more recent enlargement (arrow). Note that the skin is uninflamed and non-crusted. Right panel: Yellow appearance of an encapsulated mass at the time of surgery. (b) An exquisitely well-circumscribed lesion is demonstrated by axial computed tomography (arrow). It also displays a centre of fat density. (c) Scanning photomicrograph of the lesion reveals an epithelial lining (crossed arrow) which has been partially replaced by a granulomatous lining (arrow) due to earlier rupture. The inset depicts the lipogranulomatous response in the adjacent stroma. (d) The granulomatous lining features mononucleated epithelioid cells and multinucleated giant cells, sometimes forming mounds (arrow and inset). (c and d, haematoxylin and eosin; ×12.5, inset ×100; ×40, inset ×100).

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Figure 2. Histopathology and immunohistochemistry of cystic epithelial lining of SS. (a) Squamous epithelium lines the cyst (arrows) and sheds little keratin into the lumen. Many sebaceous glands (S) are present in the wall of the cyst. (b) A sebaceous gland is partially incorporated (arrow) into the cyst’s epithelial lining. (c) The lining manifests undulation and a cuticular, refractile superficial layer (arrow). (d) Top panel: Cytokeratin (CK) 14 immunostains the full thickness of the epithelium. Bottom panel: CK17 reacts with the suprabasilar epithelial layers but not with the basilar and parabasilar layers (arrows). (a, b, c, haematoxylin and eosin; ×100, ×200, ×400; d top and bottom panel: immunoperoxidase reaction, diaminobenzidine chromogen, haematoxylin counterstain; ×200, ×200). © 2014 Royal Australian and New Zealand College of Ophthalmologists

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Letters to the Editor sutures, are located in the subcutaneous tissues (not the dermis) and exhibit features of an epidermis-like lining. Lacrimal sac enlargements or diverticula are lined by a non-squamous ciliated epithelium unless there has been squamous metaplasia, in which instance there is an absent cuticular inner layer. SS should therefore be included in the differential diagnosis of a cystic lesion in the lacrimal sac region. Simple excision of SS is curative.

Anna M Stagner MD,1,2 Frederick A Jakobiec MD DSc1,2 and Michael K Yoon MD2,3 1 David G. Cogan Laboratory of Ophthalmic Pathology, 3 Division of Ophthalmic Plastic Surgery, Massachusetts Eye and Ear Infirmary, and 2Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts, USA Received 16 September 2014; accepted 18 September 2014.

REFERENCES 1. Brownstein MH. Steatocystoma simplex. A solitary steatocystoma. Arch Dermatol 1982; 118: 409–11. 2. Cho S, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol 2002; 29: 152–6. 3. Jakobiec FA, Mehta M, Iwamoto M, Hatton MP, Thakker M, Fay A. Intratarsal keratinous cysts of the Meibomian gland: distinctive clinicopathologic and immunohistochemical features in 6 cases. Am J Ophthalmol 2010; 149: 82–94. 4. Tirakunwichcha S, Vaivanijkul J. Steatocystoma simplex of the eyelid. Ophthal Plast Reconstr Surg 2009; 25: 49–50. 5. Jakobiec FA, Mehta M, Greenstein SH, Colby K. The white caruncle: sign of a keratinous cyst arising from a sebaceous gland duct. Cornea 2010; 29: 453–5. 6. Kurokawa I, Nishijima S, Kusumoto K, Senzaki H, Shikata N, Tsubura A. Cytokeratin expression in steatocystoma multiplex. Br J Dermatol 2002; 146: 534–6.

387 While the demographic data and diagnostic features of patients presenting to acute eye services have been well studied,1,2 the diagnostic accuracy between various referral groups have not been so clearly addressed. Moreover, the characteristics of patients referred to smaller acute tertiary services, such as Hamilton in New Zealand is less clear. This study investigates the demographic features, referral patterns, clinical diagnosis and management plan of new patients presenting to the acute eye service of Waikato Hospital. The clinical and demographic details of patients who were assessed at the acute eye service of Waikato Hospital from 18 July 2011 to 2 December 2011 (138 consecutive days) were collected and analysed. Overall, 1625 patients were seen at the acute eye clinic in Waikato Hospital over a period of 99 days, an average of approximately 16 patients a day. Attending were 826 patients for follow-up appointments, and 799 were new patients. The average age of new patients assessed at the acute eye service was 48 years old. Patients aged between 51 and 75 years old represent the most frequent age group assessed in the clinic (39.4% of all new patients). The average visual acuity of new patients was 6/12, with a range of 6/3.8 to no perception of light. The majority of patients were referred by GPs (Fig. 1). The most common reasons for acute referrals were: ocular pain (26.8%), reduced vision (19.2%), visual disturbance (12.8%), ocular trauma (10.2%), red eye (9.2%) or ocular foreign bodies (7.3%). A high proportion of patients from ED were referred with traumatic ocular injuries, while most self-referred patients had ocular pain as their main presenting symptom. The majority of patients presented with anterior segment pathology (39.8%) and within this group, trauma was the main causative factor. The other main diagnostic groups were vitreo-retinal (20.3%), neuro-ophthalmic (8.7%), oculoplastic (8.5%), uveitis (7.0%), glaucoma (1.9%) and paediatric ophthalmology (1.0%). Table 1 demonstrates the accuracy of the referrals sent to the acute eye clinic, divided into the different ophthalmology subspecialties. Optometry referrals, which constitute

Characteristics and accuracy of referrals to an acute tertiary ophthalmic service in New Zealand Acute ocular pathologies may lead to permanent visual loss or morbidity if not managed appropriately. General practitioners (GP), optometrists or emergency department (ED) doctors are frequently involved in the primary assessment and care of acute ocular problems. Emergency ophthalmic services should provide secondary and tertiary care but are increasingly being involved in primary care at larger centres. Overcrowding, with a delay in patient care, is a common problem affecting all emergency services and can occur if there is a high proportion of self-referrals or inappropriate referrals. Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.

Self-referrals, 1.6%

Others, 2.6%

ED triage desk, 2.6% Ophthalmologists, 4.3% Phone enquiries, 5.0%

General practitioners, 50.5%

ED doctors, 8.4% Hospital-based doctors, 11.9%

Optometrists, 15.5%

Figure 1. Referral sources for new patients presenting to the acute eye clinic.

© 2014 Royal Australian and New Zealand College of Ophthalmologists

Ruptured canthal steatocystoma simplex presenting as a lacrimal sac mass.

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