- Email: [email protected]
Orbital dermoid masquerading as allergic lid swelling
Correspondence research question.12 More detailed algorithms based on the number of dependent variables and nature of the independent variables can be found at the University of California, Los Angeles, Institute for Digital Research and Education.13 Researchers are encouraged to consult with a biostatistician before planning research projects, as complex issues such as paired-eye data and repeated measures require statistical adjustments. Although Microsoft Excel (https://products.office. com/en-ca/excel) has some statistics functions and is available on most computers, Excel may lead to statistical errors14 especially with software versions before 2010. It may be wiser to reserve Excel as a spreadsheet for gathering data. A wealth of alternative free statistical analysis programs is available, including “R.”15 Power and sample size calculations can be done in many of the statistical packages or with free online resources.16 In general, statistics programs with menus, for example, SOFA (www.sofastatistics.com) are easier to learn, but the author does not have experience with nonproprietary software packages. The author uses the Stata program (www.stata.com/), but this is not free. The Framingham Heart Teaching Dataset17 is the classic freely available public-use dataset for biostatistics teaching. The Framingham dataset has over 4000 subjects and numerous covariates. Using random number generation and variable substitution, the Framingham Heart dataset can be modified to include ophthalmic variables. Provision of a large database with ophthalmic variables may be more motivational to eye residents and enhance the retention of statistics teaching in techniques such as logistic regression and survival analysis.1 Statistical literacy is a necessary skill for physicians to understand and implement research. Although a biostatistician should be consulted before and throughout the course of most research endeavours, it is imperative that the clinician conducting the research understand the principles of statistical analysis. A wealth of free online materials is available to help ophthalmologists acquire the statistical literacy needed for self-directed learning, critical evaluation of the medical literature, and research. Edsel Ing Michael Garron Hospital, University of Toronto, Toronto, Ont.
Orbital dermoid masquerading as allergic lid swelling CASE REPORT A 36-year-old woman of Afro-Caribbean descent was referred to our clinic with a history of painless swelling of
Correspondence to: Edsel Ing, MD, FRCS(C): [email protected] REFERENCES 1. Baldi B, Utts J. What your future doctor should know about statistics: must-include topics for introductory undergraduate biostatistics. Am Stat. 2015;69:231-9. 2. Windish DM, Huot SJ, Green ML. Medicine residents’ understanding of the biostatistics and results in the medical literature. JAMA. 2007;298:1010-22. 3. Anderson BL, Williams S, Schulkin J. Statistical literacy of obstetrics-gynecology residents. J Grad Med Educ. 2013;5:272-5. 4. Juzych MS, Shin DH, Sevesdsadr M, Siegner SW, Juzych LA. Statistical techniques in ophthalmic journals. Arch Ophthalmol. 1992;110:1225-9. 5. Lisboa R, Meira-Freitas D, Tatham AJ, Marvasti AH, Sharpsten L, Medeiros FA. Use of statistical analyses in the ophthalmic literature. Ophthalmology. 2014;121:1317-21. 6. Sensky T. 2016. Available from: 〈www.google.ca/webhp?sourceid= chrome-instant&ion=1&espv=2&ie=UTF-8q=tom%20sensky% 20basic%20statistics%20a%20survival%20guide〉. Accessed 15.01.16. 7. Weaver A, Goldberg S. Clinical Biostatistics and Epidemiology Made Ridiculously Simple. Miami: MedMaster; 2012. 8. American Journal of Ophthalmology. Series on Statistics. 2015. Available from: 〈www.ajo.com/content/statistics〉 Accessed 15.01.16. 9. NHS Ophthalmology Statistics Group. National Institute for Health Research Ophthalmology Statistics Group. 2015. Available from: 〈www. statistics-group.nihr.ac.uk/Group/ophthalmology〉 Accessed 15.01.16. 10. Study.com. List of free online biostatistics courses and classes. 2015. Available from: 〈http://study.com/articles/List_of_Free_Online_ Biostatistics_Courses_and_Classes.html〉. Accessed 15.01.16. 11. Petrie A, Sabin C. Medical Statistics at a Glance. 2009. Available from: 〈www.medstatsaag.com/mcqs3.asp〉 Accessed 15.01.16. 12. Nayak BK, Hazra A. How to choose the right statistical test? Indian J Ophthalmol. 2011;59:85-6. 13. Institute for Digital Research and Education UCLA What statistical analysis should I use? 2015. Available from: 〈www.ats.ucla.edu/stat/ mult_pkg/whatstat/〉 Accessed 15.01.16. 14. McCullough BD, Heiser DA. On the accuracy of statistical procedures in Microsoft Excel 2007 Comput Stat Data Anal. 2008;52:4570-8. 15. StatPages.net. Free statistical software. 2015. Available from: 〈http:// statpages.org/javasta2.html〉. Accessed 15.01.16. 16. Power and Sample Size.com. Overview of Power and Sample Size. com calculators. 2015. Available from: 〈http://powerandsamplesize. com/Calculators/〉. Accessed 15.01.16. 17. NIH National Heart, Lung and Blood Institute. BioLINCC Teaching Datasets. 2015. Available from: 〈https://biolincc.nhlbi. nih.gov/teaching/〉 Accessed 15.01.16. Can J Ophthalmol 2016;51:e142–e143 0008-4182/16/$-see front matter & 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2016.05.011
the right upper eyelid for a few months. She had suffered intermittent right upper eyelid swelling for 10 years and had been seen in many hospitals. She had been given various diagnoses, most commonly allergic eyelid edema, but had also been treated for blepharitis and dry eyes. The swelling often resolved spontaneously or with the use of topical or oral steroids. A few months before review with us, she had developed a severe episode of persistent right
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Correspondence upper eyelid swelling that partially resolved with systemic steroids. She did not describe any blurring of vision, double vision, or ocular pain and there was no history of trauma, or systemic symptoms. A family history excluded neurofibromatosis, tumours, or autoimmune conditions. Examination of the right eye showed 4-mm proptosis, 2-mm hypoglobus, and moderate restriction of elevation (Fig. 1). There was no palpable mass present. Optic nerve function was normal and there were no choroidal folds. There was no pulsation on auscultation, and the Valsalva test was negative. Systemic examination was unremarkable. Her complete blood count, renal function tests, liver function tests, thyroid function tests, and inflammatory markers were all normal. An angiotensin-converting enzyme level, autoantibodies, and immune subtyping were also unremarkable. Magnetic resonance imaging (MRI) of the orbits showed a lobulated extraconal lesion within the right superior orbit, measuring 19 mm anteroposteriorly and 18 mm craniocaudally. The lesion was of high signal intensity on both T1 and T2, and had an area of stranding of intermediate signal intensity inferiorly within it on T1
(Fig. 2). An orbital computed tomography (CT) scan found the mass to be of fat density with some anteriorly placed calcifications. There was thinning of the right sphenoid wing, but no perforation, and the right superior oblique muscle showed some deviation (Fig. 2). An orbitotomy was performed through a lid crease incision under general anaesthesia. The cyst wall was extremely thin, suggesting a previous breach or leakage, and there were adhesions to the surrounding soft tissues, as well as extensions into craters within the frontal bone. The mass was carefully dissected free and was ruptured at the end of the procedure for ease of removal from the orbit. The dermoid contents were carefully aspirated, and the orbit was washed out with 20 mL normal saline mixed with 4 mg dexamethasone to minimize postoperative inflammation. Meticulous curettage of the adjacent sphenoid wing was performed to ensure complete removal of all cyst remnants. Histology of the excised specimen showed it to consist of fibrous and adnexal tissue, with both acute and chronic inflammation, including a giant cell reaction to likely keratinous debris, consistent with the diagnosis of a dermoid cyst. The patient showed a complete resolution of her symptoms, and at 6-month review she showed no
Fig. 1 — Clinical photograph showing proptosis and hypoglobus with limitation in elevation in right eye.
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Fig. 2 — (A) Computed tomography scan of orbit showing scalloping of the orbital roof and fissuring of the sphenoid wing with an extension of the dermoid into the defect. (B–D) T1-weighted gadolinium-enhanced magnetic resonance image of the orbit showing a lobulated extraconal mass lesion on the right.
proptosis, a full range of ocular movements, no ptosis, and a normal visual acuity.
DISCUSSION Approximately 3% of orbital dermoids occur inside the bony orbit and are classified as deep.1 Their rarity and subtle orbital signs mean that there is often a delay in the correct diagnosis being made. Instead, they often masquerade as other ophthalmic pathologies, frequently for several years. Typically, these masquerade syndromes relate to inflammatory changes secondary to leakage of sebaceous material from an expanding, thin-walled cyst or from limitations to ocular motility.2 Our patient had presented repeatedly to ophthalmologists with unilateral upper lid swelling, which was frequently self-limiting, and as a result she had been diagnosed as having allergic eyelid edema. Review of the literature relating to deep orbital dermoids does suggest that many present in an atypical manner, as highlighted by Table 1.3–15 In some cases, the only presenting feature may be limitation of extraocular movement, resulting in dermoids masquerading as Duane syndrome type II or monocular elevation deficiency.6,14 Alternatively, the dermoid may present with an extension into the periorbital tissues. This is particularly confusing if it occurs medially, for example, masquerading as a dacryocystocoele,12 or a meningocoele.10 In the former, however, the nasolacrimal duct remains patent, and in the
latter, the lesion is not compressible or pulsatile although it may transilluminate. There have even been case reports of orbital dermoids misdiagnosed as ectopic cilia or fistulae because of a sinus tract from the dermoid extending to the periorbital skin.3,11 The progressive and destructive nature of deep orbital dermoids means that surgical excision is often recommended as the best course of management.16,17 This can be complicated and requires detailed preoperative planning with orbital imaging, including a CT scan to assess bony destruction, fissuring, or tunnelling, as such bony changes can occur in up to 28% of cases.16 An MRI is also helpful to carefully assess for any extensions of the orbit through bone into the adjacent cavities, especially into the temporalis fossa. Meticulous cleaning of any cyst wall remnants from the adjacent bony surfaces is essential; otherwise, recurrence of the cyst will occur. This involves careful curettage and or burring of the bone. In addition, many such dermoids have deep extensions, and a detailed knowledge of orbital anatomy is essential to ensure their safe removal. To the best of our knowledge, this case is the first reported of a deep orbital dermoid masquerading as chronic allergic eye disease in this way. In hindsight, we believe that this was caused by recurrent leakage of sebaceous material from a very thin-walled cyst, which was located in the central-lateral superior orbit. We believe that our case highlights the need for a systematic orbital examination to
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Correspondence Table 1—Summary of atypical presentations of deep orbital dermoids in peer-reviewed journals Year of Age Reference Publication (yr)
Sex
Presenting Symptoms
Orbital Signs
Female Draining skin fistula
No
Male
No
Location in the Orbit
1. Pollard and Calhoun3 2. Hönig4
1975
6
1998
56
3. Parag et al.5
2001
1
Female Discharging sinuses and swelling of lower eyelid
Swelling of lower eyelid
Left temporal area
4. Gatzonis et al.6
2002
3
Female Limitation of adduction mimicking Duane syndrome
Exophoria, exophthalmos
Within lateral rectus muscle
5. Kudo et al.7
2008
85
Female Watering and blurred vision after head injury
6. Sari et al.8 2008
2
Extraocular movement disorder, slight exophthalmos Globe displacement
Superolateral compartment of anterior orbit Inferior extraconal
Superolateral globe displacement Cystic swelling in medial orbit
Intraorbital
Periorbital swelling
Superomedial nasal orbital region Superomedial
Recurrent discharging sinus in lateral edge of eyebrow
Female Lower eyelid swelling
7. Yeola et al.9
2009
18
Female Progressive, painless swelling in lower eyelid
8. Wani et al.10
2010
19
Female Painless swelling in the upper medial aspect mimicking menigocele
9. Wang et al.11
2010
19
Female Ectopic cilia, draining sinus
10. Lelli and Levy12
2011
2
11. Boyaci et al.13
2011
10
12. Srikanth et al.14
2012
22
13. Yu et al.15 2013
60
Superolateral extraconal space Greater wing of sphenoidal bone
Male
Medial canthal mass Medial canthal mimicking swelling dacryocystocele Female Sudden retro-orbital pain No
Female Ptosis, limitation of eye movements mimicking a monocular elevation deficiency Female Headache, ear pain
Proptosis, diplopia
None
Radiology Features
Management
Cystic lesion with Craniotomy and tract to skin excision biopsy Cystic lesion, with sinus tract to medial and lateral wall Mass with extension to the orbit Multiple, welloutlined heterogeneous cystic lesions Dermoid with intratumoural inflammatory hemorrhage Well-defined multilobulated cyst Well-defined lesion on orbital floor Unilocular, hypodense lesion in the superior medial orbit Well-defined hypodense lesion
Country USA
Excision biopsy with Germany removal of outer cortex of frontal bone Excision via cranial India and orbital approach, removal of discharging sinuses Conservative Greece
Orbitotomy and excision biopsy
Japan
Orbitotomy and excision biopsy
Turkey
Orbitotomy and excision biopsy
India
Orbitotomy and excision biopsy
India
Orbitotomy and excision biopsy
China
Well-defined cyst in medial orbit
Orbitotomy and excision biopsy
USA
Retro-orbital, intraconal, intramuscular Superior extraconal space
Small, encapsulated, solid lesion Well-defined hypodense lesion
Orbitotomy and excision biopsy
Turkey
Orbitotomy and excision biopsy
India
Intraconal
Well-defined intraconal lesion
Conservative
Hong Kong
Intraorbital
Please note that this list is not exhaustive and only includes selected atypical presentations.
be included in the assessment of patients with unilateral upper lid swelling. It also serves as a reminder of the many unusual ways in which deep orbital dermoids can present and that they should be included early on in the differential diagnosis of any unusual unilateral orbital presentations.
Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.
Vijay Kumar Wagh, MD, FRCOphth, Ki Crystal Lam, MBBS, A.M Susana Morley, BM BCh, FRCOphth, MD Department of Ophthalmology, Guy’s and St. Thomas’ NHS Foundation Trust, London, United Kingdom Presented at Trends in Ophthalmology meeting, London on Friday February 6, 2015. http://www.eyehope.co.uk.
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Correspondence to: A.M. Susana Morley, BM BCh, FRCOphth, MD: [email protected] REFERENCES 1. Shields JA, Kaden IH, Eagle RC Jr, Shields CL. Orbital dermoid cysts: clinicopathologic correlations, classification, and management. Ophthal Plast Reconstr Surg. 1997;13:265-76. 2. Bonavolontà G, Tranfa F, de Conciliis C, Strianese D. Dermoid cysts: 16-year survey. Ophthal Plast Reconstr Surg. 1995;11:187-92. 3. Pollard ZF, Calhoun J. Deep orbital dermoid with draining sinus. Am J Ophthalmol. 1975;79:310-3. 4. Hönig JF. A de novo discharging sinus of the fronto-orbital suture: a rare presentation of a dermoid cyst. J Craniofac Surg. 1998;9:536-8. 5. Parag P, Prakash PJ, Zachariah N. Temporal dermoid—an unusual presentation. Pediatr Surg Int. 2001;17:77-9. 6. Gatzonis S, Charakidas A, Papathanassiou M, Paikos P. Multiple orbital dermoid cysts located within the lateral rectus muscle, clinically mimicking Duane syndrome type II. J Pediatr Ophthalmol Strabismus. 2002;39:324.
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Correspondence 7. Kudo K, Tsutsumi S, Suga Y, et al. Orbital dermoid cyst with intratumoral inflammatory hemorrhage: case report. Neurol Med Chir (Tokyo). 2008;48:359-62. 8. Sari A, Yener T, Sari A, Basterzi Y. Atypical presentation of an intraorbital dermoid cyst. J Craniofac Surg. 2008;19:1715-6. 9. Yeola M, Joharapurkar SR, Bhole AM, Chawla M, Chopra S, Paliwal A. Orbital floor dermoid: an unusual presentation. Indian J Ophthalmol. 2009;57:51-2. 10. Wani BN, Jajoo SN, Bhole AM. Transilluminant naso-orbital dermoid cyst masquerading as meningocele. J Craniofac Surg. 2010;21:930-2. 11. Wang Y, Su F, Li Y, Xiao L. Orbital dermoid cyst with sinus tract mimicking ectopic cilia. J AAPOS. 2010;14:532-3. 12. Lelli GJ, Levy RL. Epidermoid cyst masquerading as dacryocystocele: case report and review. Orbit. 2011;30:114-5. 13. Boyaci S, Moray M, Aksoy K, Sav A. Intraocular neuromuscular choristoma: a case report and literature review. Neurosurgery. 2011;68:E551-5.
Case of Actinomyces lacrimal sac abscess mimicking malignancy Primary lacrimal sac malignancy is rare, but it is associated with significant morbidity and mortality.1,2 Classically, these tumours present as firm masses at or above the medial canthal tendon. However, this classic presentation may result from an unexpected outcome when complicated by comorbid patient factors, such as immunosuppression.
CASE PRESENTATION A 62-year-old Caucasian male presented to the local emergency department with a 1-week history of a left medial canthal mass without any associated epiphora or discharge. He was treated by the emergency room physician with intravenous cephazolin for 3 days with no improvement and was subsequently referred to the oculoplastics service for management. His medical history was significant for transitional cell carcinoma of the bladder with known pulmonary metastasis, for which he had just completed a 6-month course of chemotherapy consisting of cisplatin, carboplatin, and gemcitabine. On examination, there was a firm nonmobile nontender mass centred at the left canthal tendon with minimal associated erythema. Attempts to empty the sac did not produce any purulent discharge from the puncta. The upper and lower canaliculi were probed to 8 mm and were completely patent to irrigation. Slit-lamp and fundus examination findings were unremarkable. These nonspecific findings prompted further imaging studies. Computed tomography (CT) of the orbits revealed a circumscribed 16 mm 8 mm 16 mm rimenhancing lesion lateral to the left nasal bone at the level of the nasolacrimal sac (Fig. 1). No bone erosion, intralesional heterogeneity, or extension into surrounding tissue was seen.
14. Srikanth R, Meenakshi S, Chaterjee R, Mukherjee B. Orbital dermoid mimicking a monocular elevation deficiency. Oman J Ophthalmol. 2012;5:118-20. 15. Yu WL, Sitt CM, Siu YW. Intraconal orbital dermoid cyst in an adult patient: a rare location. Semin Ophthalmol. 2013;28:94-6. 16. Sathananthan N, Moseley IF, Rose GE, Wright JE. The frequency and clinical significance of bone involvement in outer canthus dermoid cysts. Br J Ophthalmol. 1993;77:789-94. 17. Sherman RP, Rootman J, Lapointe JS. Orbital dermoids: clinical presentation and Management. Br J Ophthalmol. 1984;68: 642-52. Can J Ophthalmol 2016;51:e143–e147 0008-4182/16/$-see front matter & 2016 Published by Elsevier Inc on behalf of the Canadian Ophthalmological Society. http://dx.doi.org/10.1016/j.jcjo.2016.04.026
This clinical presentation warranted a strong suspicion for malignancy, either a primary lacrimal sac tumour or a metastatic transitional cell carcinoma. An excisional biopsy was planned given the well-circumscribed nature of the mass on examination and imaging. Intraoperatively, there was adhesion between the medial wall of the sac and the surrounding bone with multiple feeder vessels to the mass; thus, an en bloc excision of the entire lacrimal sac and surrounding tissue, including proximal nasolacrimal duct, was performed. The excised sac measured 18 mm 18 mm 10 mm, with firm nodularity within its wall. Inspection of the sac lumen revealed discharge and abnormal-appearing mucosa but no dacryolith. Intraoperative frozen section of the nasolacrimal duct was negative for malignancy. Paraffinembedded histopathologic examination of the lacrimal sac revealed extensive subepithelial inflammation and an abscess within the sac wall (Fig. 2). Immunohistochemical stain for pooled cytokeratin did not identify any malignant cells. Gram stain revealed the presence of filamentous gram-positive bacteria (Fig. 2). Grocott’s silver stain demonstrated the branching pattern typical of Actinomyces. The patient had an uneventful recovery without recurrence of the medial canthal mass. Unfortunately, shortly after the surgery, the patient developed left upper limb weakness. Urgent CT of the brain demonstrated a hemorrhagic lesion in the right precentral gyrus. Although he was treated for possible intracranial actinomycosis with intravenous penicillin, repeat magnetic resonance imaging confirmed the intraparenchymal lesion to be a metastatic deposit, along with several other ring-enhancing lesions in the posterior fossa. The patient was offered palliative radiotherapy for these brain lesions.
DISCUSSION This case demonstrates several unusual features that led to initial diagnostic uncertainty. Classical signs of dacryocystitis were absent in our patient: epiphora, pain, mass
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Orbital dermoid masquerading as allergic lid swelling CASE REPORT A 36-year-old woman of Afro-Caribbean descent was referred to our clinic with a history of painless swelling of
Correspondence to: Edsel Ing, MD, FRCS(C): [email protected] REFERENCES 1. Baldi B, Utts J. What your future doctor should know about statistics: must-include topics for introductory undergraduate biostatistics. Am Stat. 2015;69:231-9. 2. Windish DM, Huot SJ, Green ML. Medicine residents’ understanding of the biostatistics and results in the medical literature. JAMA. 2007;298:1010-22. 3. Anderson BL, Williams S, Schulkin J. Statistical literacy of obstetrics-gynecology residents. J Grad Med Educ. 2013;5:272-5. 4. Juzych MS, Shin DH, Sevesdsadr M, Siegner SW, Juzych LA. Statistical techniques in ophthalmic journals. Arch Ophthalmol. 1992;110:1225-9. 5. Lisboa R, Meira-Freitas D, Tatham AJ, Marvasti AH, Sharpsten L, Medeiros FA. Use of statistical analyses in the ophthalmic literature. Ophthalmology. 2014;121:1317-21. 6. Sensky T. 2016. Available from: 〈www.google.ca/webhp?sourceid= chrome-instant&ion=1&espv=2&ie=UTF-8q=tom%20sensky% 20basic%20statistics%20a%20survival%20guide〉. Accessed 15.01.16. 7. Weaver A, Goldberg S. Clinical Biostatistics and Epidemiology Made Ridiculously Simple. Miami: MedMaster; 2012. 8. American Journal of Ophthalmology. Series on Statistics. 2015. Available from: 〈www.ajo.com/content/statistics〉 Accessed 15.01.16. 9. NHS Ophthalmology Statistics Group. National Institute for Health Research Ophthalmology Statistics Group. 2015. Available from: 〈www. statistics-group.nihr.ac.uk/Group/ophthalmology〉 Accessed 15.01.16. 10. Study.com. List of free online biostatistics courses and classes. 2015. Available from: 〈http://study.com/articles/List_of_Free_Online_ Biostatistics_Courses_and_Classes.html〉. Accessed 15.01.16. 11. Petrie A, Sabin C. Medical Statistics at a Glance. 2009. Available from: 〈www.medstatsaag.com/mcqs3.asp〉 Accessed 15.01.16. 12. Nayak BK, Hazra A. How to choose the right statistical test? Indian J Ophthalmol. 2011;59:85-6. 13. Institute for Digital Research and Education UCLA What statistical analysis should I use? 2015. Available from: 〈www.ats.ucla.edu/stat/ mult_pkg/whatstat/〉 Accessed 15.01.16. 14. McCullough BD, Heiser DA. On the accuracy of statistical procedures in Microsoft Excel 2007 Comput Stat Data Anal. 2008;52:4570-8. 15. StatPages.net. Free statistical software. 2015. Available from: 〈http:// statpages.org/javasta2.html〉. Accessed 15.01.16. 16. Power and Sample Size.com. Overview of Power and Sample Size. com calculators. 2015. Available from: 〈http://powerandsamplesize. com/Calculators/〉. Accessed 15.01.16. 17. NIH National Heart, Lung and Blood Institute. BioLINCC Teaching Datasets. 2015. Available from: 〈https://biolincc.nhlbi. nih.gov/teaching/〉 Accessed 15.01.16. Can J Ophthalmol 2016;51:e142–e143 0008-4182/16/$-see front matter & 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2016.05.011
the right upper eyelid for a few months. She had suffered intermittent right upper eyelid swelling for 10 years and had been seen in many hospitals. She had been given various diagnoses, most commonly allergic eyelid edema, but had also been treated for blepharitis and dry eyes. The swelling often resolved spontaneously or with the use of topical or oral steroids. A few months before review with us, she had developed a severe episode of persistent right
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Correspondence upper eyelid swelling that partially resolved with systemic steroids. She did not describe any blurring of vision, double vision, or ocular pain and there was no history of trauma, or systemic symptoms. A family history excluded neurofibromatosis, tumours, or autoimmune conditions. Examination of the right eye showed 4-mm proptosis, 2-mm hypoglobus, and moderate restriction of elevation (Fig. 1). There was no palpable mass present. Optic nerve function was normal and there were no choroidal folds. There was no pulsation on auscultation, and the Valsalva test was negative. Systemic examination was unremarkable. Her complete blood count, renal function tests, liver function tests, thyroid function tests, and inflammatory markers were all normal. An angiotensin-converting enzyme level, autoantibodies, and immune subtyping were also unremarkable. Magnetic resonance imaging (MRI) of the orbits showed a lobulated extraconal lesion within the right superior orbit, measuring 19 mm anteroposteriorly and 18 mm craniocaudally. The lesion was of high signal intensity on both T1 and T2, and had an area of stranding of intermediate signal intensity inferiorly within it on T1
(Fig. 2). An orbital computed tomography (CT) scan found the mass to be of fat density with some anteriorly placed calcifications. There was thinning of the right sphenoid wing, but no perforation, and the right superior oblique muscle showed some deviation (Fig. 2). An orbitotomy was performed through a lid crease incision under general anaesthesia. The cyst wall was extremely thin, suggesting a previous breach or leakage, and there were adhesions to the surrounding soft tissues, as well as extensions into craters within the frontal bone. The mass was carefully dissected free and was ruptured at the end of the procedure for ease of removal from the orbit. The dermoid contents were carefully aspirated, and the orbit was washed out with 20 mL normal saline mixed with 4 mg dexamethasone to minimize postoperative inflammation. Meticulous curettage of the adjacent sphenoid wing was performed to ensure complete removal of all cyst remnants. Histology of the excised specimen showed it to consist of fibrous and adnexal tissue, with both acute and chronic inflammation, including a giant cell reaction to likely keratinous debris, consistent with the diagnosis of a dermoid cyst. The patient showed a complete resolution of her symptoms, and at 6-month review she showed no
Fig. 1 — Clinical photograph showing proptosis and hypoglobus with limitation in elevation in right eye.
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Fig. 2 — (A) Computed tomography scan of orbit showing scalloping of the orbital roof and fissuring of the sphenoid wing with an extension of the dermoid into the defect. (B–D) T1-weighted gadolinium-enhanced magnetic resonance image of the orbit showing a lobulated extraconal mass lesion on the right.
proptosis, a full range of ocular movements, no ptosis, and a normal visual acuity.
DISCUSSION Approximately 3% of orbital dermoids occur inside the bony orbit and are classified as deep.1 Their rarity and subtle orbital signs mean that there is often a delay in the correct diagnosis being made. Instead, they often masquerade as other ophthalmic pathologies, frequently for several years. Typically, these masquerade syndromes relate to inflammatory changes secondary to leakage of sebaceous material from an expanding, thin-walled cyst or from limitations to ocular motility.2 Our patient had presented repeatedly to ophthalmologists with unilateral upper lid swelling, which was frequently self-limiting, and as a result she had been diagnosed as having allergic eyelid edema. Review of the literature relating to deep orbital dermoids does suggest that many present in an atypical manner, as highlighted by Table 1.3–15 In some cases, the only presenting feature may be limitation of extraocular movement, resulting in dermoids masquerading as Duane syndrome type II or monocular elevation deficiency.6,14 Alternatively, the dermoid may present with an extension into the periorbital tissues. This is particularly confusing if it occurs medially, for example, masquerading as a dacryocystocoele,12 or a meningocoele.10 In the former, however, the nasolacrimal duct remains patent, and in the
latter, the lesion is not compressible or pulsatile although it may transilluminate. There have even been case reports of orbital dermoids misdiagnosed as ectopic cilia or fistulae because of a sinus tract from the dermoid extending to the periorbital skin.3,11 The progressive and destructive nature of deep orbital dermoids means that surgical excision is often recommended as the best course of management.16,17 This can be complicated and requires detailed preoperative planning with orbital imaging, including a CT scan to assess bony destruction, fissuring, or tunnelling, as such bony changes can occur in up to 28% of cases.16 An MRI is also helpful to carefully assess for any extensions of the orbit through bone into the adjacent cavities, especially into the temporalis fossa. Meticulous cleaning of any cyst wall remnants from the adjacent bony surfaces is essential; otherwise, recurrence of the cyst will occur. This involves careful curettage and or burring of the bone. In addition, many such dermoids have deep extensions, and a detailed knowledge of orbital anatomy is essential to ensure their safe removal. To the best of our knowledge, this case is the first reported of a deep orbital dermoid masquerading as chronic allergic eye disease in this way. In hindsight, we believe that this was caused by recurrent leakage of sebaceous material from a very thin-walled cyst, which was located in the central-lateral superior orbit. We believe that our case highlights the need for a systematic orbital examination to
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Correspondence Table 1—Summary of atypical presentations of deep orbital dermoids in peer-reviewed journals Year of Age Reference Publication (yr)
Sex
Presenting Symptoms
Orbital Signs
Female Draining skin fistula
No
Male
No
Location in the Orbit
1. Pollard and Calhoun3 2. Hönig4
1975
6
1998
56
3. Parag et al.5
2001
1
Female Discharging sinuses and swelling of lower eyelid
Swelling of lower eyelid
Left temporal area
4. Gatzonis et al.6
2002
3
Female Limitation of adduction mimicking Duane syndrome
Exophoria, exophthalmos
Within lateral rectus muscle
5. Kudo et al.7
2008
85
Female Watering and blurred vision after head injury
6. Sari et al.8 2008
2
Extraocular movement disorder, slight exophthalmos Globe displacement
Superolateral compartment of anterior orbit Inferior extraconal
Superolateral globe displacement Cystic swelling in medial orbit
Intraorbital
Periorbital swelling
Superomedial nasal orbital region Superomedial
Recurrent discharging sinus in lateral edge of eyebrow
Female Lower eyelid swelling
7. Yeola et al.9
2009
18
Female Progressive, painless swelling in lower eyelid
8. Wani et al.10
2010
19
Female Painless swelling in the upper medial aspect mimicking menigocele
9. Wang et al.11
2010
19
Female Ectopic cilia, draining sinus
10. Lelli and Levy12
2011
2
11. Boyaci et al.13
2011
10
12. Srikanth et al.14
2012
22
13. Yu et al.15 2013
60
Superolateral extraconal space Greater wing of sphenoidal bone
Male
Medial canthal mass Medial canthal mimicking swelling dacryocystocele Female Sudden retro-orbital pain No
Female Ptosis, limitation of eye movements mimicking a monocular elevation deficiency Female Headache, ear pain
Proptosis, diplopia
None
Radiology Features
Management
Cystic lesion with Craniotomy and tract to skin excision biopsy Cystic lesion, with sinus tract to medial and lateral wall Mass with extension to the orbit Multiple, welloutlined heterogeneous cystic lesions Dermoid with intratumoural inflammatory hemorrhage Well-defined multilobulated cyst Well-defined lesion on orbital floor Unilocular, hypodense lesion in the superior medial orbit Well-defined hypodense lesion
Country USA
Excision biopsy with Germany removal of outer cortex of frontal bone Excision via cranial India and orbital approach, removal of discharging sinuses Conservative Greece
Orbitotomy and excision biopsy
Japan
Orbitotomy and excision biopsy
Turkey
Orbitotomy and excision biopsy
India
Orbitotomy and excision biopsy
India
Orbitotomy and excision biopsy
China
Well-defined cyst in medial orbit
Orbitotomy and excision biopsy
USA
Retro-orbital, intraconal, intramuscular Superior extraconal space
Small, encapsulated, solid lesion Well-defined hypodense lesion
Orbitotomy and excision biopsy
Turkey
Orbitotomy and excision biopsy
India
Intraconal
Well-defined intraconal lesion
Conservative
Hong Kong
Intraorbital
Please note that this list is not exhaustive and only includes selected atypical presentations.
be included in the assessment of patients with unilateral upper lid swelling. It also serves as a reminder of the many unusual ways in which deep orbital dermoids can present and that they should be included early on in the differential diagnosis of any unusual unilateral orbital presentations.
Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article.
Vijay Kumar Wagh, MD, FRCOphth, Ki Crystal Lam, MBBS, A.M Susana Morley, BM BCh, FRCOphth, MD Department of Ophthalmology, Guy’s and St. Thomas’ NHS Foundation Trust, London, United Kingdom Presented at Trends in Ophthalmology meeting, London on Friday February 6, 2015. http://www.eyehope.co.uk.
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Correspondence to: A.M. Susana Morley, BM BCh, FRCOphth, MD: [email protected] REFERENCES 1. Shields JA, Kaden IH, Eagle RC Jr, Shields CL. Orbital dermoid cysts: clinicopathologic correlations, classification, and management. Ophthal Plast Reconstr Surg. 1997;13:265-76. 2. Bonavolontà G, Tranfa F, de Conciliis C, Strianese D. Dermoid cysts: 16-year survey. Ophthal Plast Reconstr Surg. 1995;11:187-92. 3. Pollard ZF, Calhoun J. Deep orbital dermoid with draining sinus. Am J Ophthalmol. 1975;79:310-3. 4. Hönig JF. A de novo discharging sinus of the fronto-orbital suture: a rare presentation of a dermoid cyst. J Craniofac Surg. 1998;9:536-8. 5. Parag P, Prakash PJ, Zachariah N. Temporal dermoid—an unusual presentation. Pediatr Surg Int. 2001;17:77-9. 6. Gatzonis S, Charakidas A, Papathanassiou M, Paikos P. Multiple orbital dermoid cysts located within the lateral rectus muscle, clinically mimicking Duane syndrome type II. J Pediatr Ophthalmol Strabismus. 2002;39:324.
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Correspondence 7. Kudo K, Tsutsumi S, Suga Y, et al. Orbital dermoid cyst with intratumoral inflammatory hemorrhage: case report. Neurol Med Chir (Tokyo). 2008;48:359-62. 8. Sari A, Yener T, Sari A, Basterzi Y. Atypical presentation of an intraorbital dermoid cyst. J Craniofac Surg. 2008;19:1715-6. 9. Yeola M, Joharapurkar SR, Bhole AM, Chawla M, Chopra S, Paliwal A. Orbital floor dermoid: an unusual presentation. Indian J Ophthalmol. 2009;57:51-2. 10. Wani BN, Jajoo SN, Bhole AM. Transilluminant naso-orbital dermoid cyst masquerading as meningocele. J Craniofac Surg. 2010;21:930-2. 11. Wang Y, Su F, Li Y, Xiao L. Orbital dermoid cyst with sinus tract mimicking ectopic cilia. J AAPOS. 2010;14:532-3. 12. Lelli GJ, Levy RL. Epidermoid cyst masquerading as dacryocystocele: case report and review. Orbit. 2011;30:114-5. 13. Boyaci S, Moray M, Aksoy K, Sav A. Intraocular neuromuscular choristoma: a case report and literature review. Neurosurgery. 2011;68:E551-5.
Case of Actinomyces lacrimal sac abscess mimicking malignancy Primary lacrimal sac malignancy is rare, but it is associated with significant morbidity and mortality.1,2 Classically, these tumours present as firm masses at or above the medial canthal tendon. However, this classic presentation may result from an unexpected outcome when complicated by comorbid patient factors, such as immunosuppression.
CASE PRESENTATION A 62-year-old Caucasian male presented to the local emergency department with a 1-week history of a left medial canthal mass without any associated epiphora or discharge. He was treated by the emergency room physician with intravenous cephazolin for 3 days with no improvement and was subsequently referred to the oculoplastics service for management. His medical history was significant for transitional cell carcinoma of the bladder with known pulmonary metastasis, for which he had just completed a 6-month course of chemotherapy consisting of cisplatin, carboplatin, and gemcitabine. On examination, there was a firm nonmobile nontender mass centred at the left canthal tendon with minimal associated erythema. Attempts to empty the sac did not produce any purulent discharge from the puncta. The upper and lower canaliculi were probed to 8 mm and were completely patent to irrigation. Slit-lamp and fundus examination findings were unremarkable. These nonspecific findings prompted further imaging studies. Computed tomography (CT) of the orbits revealed a circumscribed 16 mm 8 mm 16 mm rimenhancing lesion lateral to the left nasal bone at the level of the nasolacrimal sac (Fig. 1). No bone erosion, intralesional heterogeneity, or extension into surrounding tissue was seen.
14. Srikanth R, Meenakshi S, Chaterjee R, Mukherjee B. Orbital dermoid mimicking a monocular elevation deficiency. Oman J Ophthalmol. 2012;5:118-20. 15. Yu WL, Sitt CM, Siu YW. Intraconal orbital dermoid cyst in an adult patient: a rare location. Semin Ophthalmol. 2013;28:94-6. 16. Sathananthan N, Moseley IF, Rose GE, Wright JE. The frequency and clinical significance of bone involvement in outer canthus dermoid cysts. Br J Ophthalmol. 1993;77:789-94. 17. Sherman RP, Rootman J, Lapointe JS. Orbital dermoids: clinical presentation and Management. Br J Ophthalmol. 1984;68: 642-52. Can J Ophthalmol 2016;51:e143–e147 0008-4182/16/$-see front matter & 2016 Published by Elsevier Inc on behalf of the Canadian Ophthalmological Society. http://dx.doi.org/10.1016/j.jcjo.2016.04.026
This clinical presentation warranted a strong suspicion for malignancy, either a primary lacrimal sac tumour or a metastatic transitional cell carcinoma. An excisional biopsy was planned given the well-circumscribed nature of the mass on examination and imaging. Intraoperatively, there was adhesion between the medial wall of the sac and the surrounding bone with multiple feeder vessels to the mass; thus, an en bloc excision of the entire lacrimal sac and surrounding tissue, including proximal nasolacrimal duct, was performed. The excised sac measured 18 mm 18 mm 10 mm, with firm nodularity within its wall. Inspection of the sac lumen revealed discharge and abnormal-appearing mucosa but no dacryolith. Intraoperative frozen section of the nasolacrimal duct was negative for malignancy. Paraffinembedded histopathologic examination of the lacrimal sac revealed extensive subepithelial inflammation and an abscess within the sac wall (Fig. 2). Immunohistochemical stain for pooled cytokeratin did not identify any malignant cells. Gram stain revealed the presence of filamentous gram-positive bacteria (Fig. 2). Grocott’s silver stain demonstrated the branching pattern typical of Actinomyces. The patient had an uneventful recovery without recurrence of the medial canthal mass. Unfortunately, shortly after the surgery, the patient developed left upper limb weakness. Urgent CT of the brain demonstrated a hemorrhagic lesion in the right precentral gyrus. Although he was treated for possible intracranial actinomycosis with intravenous penicillin, repeat magnetic resonance imaging confirmed the intraparenchymal lesion to be a metastatic deposit, along with several other ring-enhancing lesions in the posterior fossa. The patient was offered palliative radiotherapy for these brain lesions.
DISCUSSION This case demonstrates several unusual features that led to initial diagnostic uncertainty. Classical signs of dacryocystitis were absent in our patient: epiphora, pain, mass
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