CASE REPORTS
Giant Bilateral Cysts of the extraocular muscle function. Orbital CT revealed a regular
Accessory Lacrimal Glands of
Wolfring in a Child
Eduardo Damous Feijó, M.D.*†,
George Alencastro Landim, M.D.*,
Mirna de Melo Dias, M.D.*,
Bruna Angelina Alves de Souza, M.D.*,
Roberto Murillo Limongi, Ph.D.†,
and Suzana Matayoshi, Ph.D.‡
Abstract: Ductal cysts of the accessory lacrimal glands of
Wolfring are rare clinical entities. They typically present as
mobile and translucent lesions on the superior or inferior
tarsal borders. Here, the authors describe a 7-year-old
male child presenting with giant bilateral cysts of Wolfring.
Management of such cysts is primarily surgical and the
preferred route for excision is transconjunctival. To the
best of the authors' knowledge, this is the first report of a
bilateral Wolfring dacryops affecting a child.
C ysts of the accessory lacrimal glands of Wolfring are rare
clinical entities. They typically present as mobile and
translucent lesions on the superior or inferior tarsal borders.
These cysts may cause mechanical blepharoptosis, proptosis,
or ocular dystopia.1,2 Their progression is chronic and insid-
ious, and they have been reported as being associated with
trachoma, conjunctival inflammation, ocular cicatricial pem-
phigoid, and prior ocular trauma.3,4 In the absence of under-
lying clinical conditions, the etiology of these cysts includes
congenital abnormalities of the lacrimal excretory ducts or
biochemical abnormalities in the secretions produced by the
gland.5-7 Due to the infrequency of this condition, the literature
on this topic largely consists of isolated case reports. To our
knowledge, this is the first report of a bilateral Wolfring dac-
ryops in a child. A written patient consent form for all clinical
photographs was obtained and is on file.
CASE REPORT
The study was IRB approved and adhered to the eth-
ical principles outlined in the Declaration of Helsinki. Seven-
year-old male patient presented with ptosis and tumors on the
upper lids of both eyes with chronic progression beginning at 6
months of age. There was no history of pregnancy or neonatal
complications. The external ocular examination revealed asym-
metrical bilateral ptosis (more pronounced on the left side) and
visible tumors in the central region of each of the upper eyelids
(Fig. 1A). The biomicroscopic examination revealed mobile,
translucent, and painless cystic conjunctival tumors located on
the superior tarsal border of each eye, showing whitish bands
suggesting conjunctival scarring (Fig. 1C,D). The patient did
not exhibit proptosis, ocular dystopia, or any issues involving
*Department of Oculoplastic Surgery, Ophthalmologic Hospital of
Anápolis (HOA), Anápolis; †Department of Ophthalmology, Federal
University of Goiás School of Medicine, Goiás; and ‡Department of
Ophthalmology, University of São Paulo School of Medicine, São Paulo,
Brazil.
Accepted for publication August 21, 2019.
The authors have no financial or conflicts of interest to disclose.
Address correspondence and reprint requests to Eduardo Damous Feijó,
M.D., Av Faiad Hanna, 235. Cidade Jardim, Anápolis-GO, 75080-410 Goiás,
Brazil. E-mail: eduardodff@yahoo.com.br
DOI: 10.1097/IOP.0000000000001494
Ophthalmic Plast Reconstr Surg, Vol. XX, No. XX, 2019
Copyright © 2019 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
cystic tumor with well-defined borders on each upper eyelid and
in close contact with the globe (Fig. 1B).
Surgical excision with a conjunctival approach was
performed with an intact wall in both eyes (Fig. 2A,B). The
histopathological analysis of the tissue revealed two layers of
nonciliated columnar epithelium with apical snouts typical of
glandular ducts (Fig. 2C) and did not mention adjacent conjunc-
tival scarring. There were no complications in the postoperative
period, and the patient experienced complete resolution of the
bilateral blepharoptosis (Fig. 2D).
DISCUSSION
Lacrimal gland cysts represent 4%–9% of all epithelial
tumors of the lacrimal glands and 5% of orbital cystic lesions.
The precise incidence of accessory gland involvement is not
well known due to the rarity of this condition.1,5 A case series on
128 cystic lesions of the ocular adnexa reported the involvement
of the accessory glands shown in 5 histological analyses, which
indicates that this pathology represents approximately 3.9% of
orbital cysts.8
In 1854, Krause described the accessory glands of the
upper and lower conjunctival fornices, with limited distribu-
tion in the nasal portion were described.9 In 1872, Wolfring10
described the anatomy of normal lacrimal tissue with acces-
sory glands embedded in the superior tarsal border, as well as
with limited distribution in the lower eyelid on the nasal side.
The intraglandular ducts pass through the upper portion of the
tarsi and drain into the conjunctival surface through short com-
mon excretory ducts.10 This anatomical description explains the
higher incidence of accessory cysts on the upper eyelids, as in
the case reported herein.7
Although various theories have been proposed, the etiopa-
thology of lacrimal cysts remains uncertain11,12. Studies hypoth-
esized that inflammation or trauma leads to the blockage of the
excretory ducts, which, in turn, results in mechanical expansion/
dilation due to the subsequent accumulation of secretion.13,14
In a histological study of 13 cases associated with trachoma,
Weatherhead13 suggests that an osmotic gradient formed by a
concentration of IgA could lead to the formation of cysts.
Accessory gland cysts have previously been associ-
ated with tracoma,13 dacryoadenitis,15 ocular cicatricial pem-
phigoid,16 and prior trauma or surgery.12 In a recent literature
review, trachoma was reported in 61% of cases. However, the
same literature review reported a series of 23 cases in which the
rate of association with trachomatous scarring was 39.1%; the
other cases were associated with other cicatricial diseases of the
conjunctiva or did not have any pathology described.3 In this re-
port, the cysts were not associated with any cicatricial diseases
of the conjunctiva.
In a case series described by Galindo-Ferreiro et al.,3
patients typically presented with a painless mass at the base of
the superior conjunctival cul-de-sac that presented clinically
as a translucent conjunctival cyst. The majority (73.9%) of ac-
cessory lacrimal gland cysts in their case series were found on
the upper eyelid (Wolfring dacryops). To our knowledge, this is
the first case report of a bilateral cyst of the accessory lacrimal
glands of Wolfring in a child.
Bullock et al.12 proposed a classification system for lac-
rimal cysts based on their location: palpebral lobe cysts; orbital
lobe cysts; cysts of the accessory lacrimal glands of Krause and
Wolfring; and cysts of ectopic lacrimal glands. Cysts are com-
monly associated with mechanical ptosis; and also, with prop-
tosis and dystopia as well, but less frequently.1,2,17,18
e1
 Case Reports Ophthalmic Plast Reconstr Surg, Vol. XX, No. XX, 2019

FIG. 1. Preoperative clinical presentation. A, Preoperative clinical presentation of bilateral cysts of the accessory lacrimal glands of
Wolfring. B, Orbital CT revealing bilateral upper eyelid lesions. C and D, Translucent cystic conjunctival tumors located on the superior
tarsal border of each eye.

FIG. 2. Transoperative and postoperative views. A and B, Immediate postoperative view. C, Histopathological analysis of the tissue
revealing 2 layers of columnar epithelial cells with apical snouts. D, Seven months postoperative.

e2 © 2019 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.

Copyright © 2019 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
 Ophthalmic Plast Reconstr Surg, Vol. XX, No. XX, 2019
Differential diagnoses of expansive palpebral lesions in-
clude tumors, granuloma resulting from a foreign body, tuber-
culosis, sarcoidosis, and dermoid or epidermoid cysts.7,19
Management of cysts of the accessory lacrimal glands of
Wolfring is primarily surgical and the preferred approach for ex-
cision is transconjunctival.13,20,21 Incomplete excision or simple
aspiration have been associated with recurrence; for this reason,
complete removal of the cystic lesion is important to avoid iat-
rogenic rupture of the cyst’s wall.20
REFERENCES
1. Ozgonul C, Uysal Y, Ayyildiz O, et al. Clinical features and man-
agement of dacryops. Orbit 2018;37:262–5.
2. Salam A, Barrett AW, Malhotra R, et al. Marsupialization for lac-
rimal ductular cysts (dacryops): a case series. Ophthalmic Plast
Reconstr Surg 2012;28:57–62.
3. Galindo-Ferreiro A, Alkatan HM, Muinos-Diaz Y, et al. Accessory
lacrimal gland duct cyst: 23 years of experience in the Saudi popu-
lation. Ann Saudi Med 2015;35:394–9.
4. Betharia SM, Pushker N, Sharma V, et al. Simple dacryops: a case se-
ries and review of the literature. Ophthalmologica 2002;216:372–6.
5. O’Duffy D, Watts P. Wolfring dacryops and needling. Acta
Ophthalmol Scand 1997;75:319.
6. Almeida AR, Feijó ED. Cisto ductal de glândula lacrimal em pa-
ciente com eritrodermia ictiosiforme congênita. Rev bras.oftalmol
2017;76:153–6.
7. Khoury NJ, Haddad MC, Tawil AN, et al. Ductal cysts of the ac-
cessory lacrimal glands: CT findings. AJNR Am J Neuroradiol
1999;20:1140–2.
© 2019 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2019 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Case Reports
8. Jakobiec FA, Bonanno PA, Sigelman J. Conjunctival adnexal cysts
and dermoids. Arch Ophthalmol 1978;96:1404–9.
9. Duke-Elder S, Wybar KC. System of ophthalmology. Vol. II: The
Anatomy of the Visual System. St. Louis: CV Mosby, 1961;559–68.
10. Wolfring. Untersuchungen tiber die Drusen der Bindehaut des
Auges. Zentralbl Med Wiss 1872;10:852–4.
11. Damato BE, Allan D, Murray SB, et al. Senile atrophy of the human
lacrimal gland: the contribution of chronic inflammatory disease.
Br J Ophthalmol 1984;68:674–80.
12. Bullock JD, Fleishman JA, Rosset JS. Lacrimal ductal cysts.
Ophthalmology 1986;93:1355–60.
13. Weatherhead RG. Wolfring dacryops. Ophthalmology
1992;99:1575–81.
14. Smith S, Rootman J. Lacrimal ductal cysts. Presentation and man-
agement. Surv Ophthalmol 1986;30:245–50.
15. Brownstein S, Belin MW, Krohel GB, et al. Orbital dacryops.
Ophthalmology 1984;91:1424–8.
16. Remulla HD, Rubin PA. Giant dacryops in a patient with ocular
cicatricial pemphigoid. Br J Ophthalmol 1995;79:1052–3.
17. Sen DK, Thomas A. Simple dacryops. Am J Ophthalmol
1967;63:161.
18. Tsiouris AJ, Deshmukh M, Sanelli PC, et al. Bilateral dacryops:
correlation of clinical, radiologic, and histopathologic features. AJR
Am J Roentgenol 2005;184:321–3.
19. Sacher M, Lanzieri CF, Sobel LI, et al. Computed tomography
of bilateral lacrimal gland sarcoidosis. J Comput Assist Tomogr
1984;8:213–5.
20. Tang SX, Lim RP, Al-Dahmash S, et al. Bilateral lacrimal gland di-
sease: clinical features of 97 cases. Ophthalmology 2014;121:2040–6.
21. Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the
lacrimal gland: an update. Curr Opin Ophthalmol 2008;19:409–13.
e3
DOI 10.5935/0034-7280.20190129 Case Report 199

Giant frontal lipoma mimicking

frontal mucocele
Lipoma frontal gigante mimetizando mucocele frontal

Rayssa Medieros Léda1 htttps://orcid.org/0000-0002-6555-6611

Mirna de Melo Dias1 htttps://orcid.org/0000-0003-0138-9428
Bruna Angelina Alves Souza1 htttps://orcid.org/0000-0002-6095-1532
Eduardo Damous Feijó2 htttps://orcid.org/0000-0001-7380-5684

Abstract
Lipoma is a usually painless benign adipose tumor. Nevertheless, it can cause compression of adjacent structures, depending on its location.
Mucoceles are benign cystic lesions in the frontal-ethmoidal region, caused by chronic obstruction of the paranasal sinus and consequent
inflammatory processes. The increase of mucocele diameter caused by inflammation often results in bony degradation and reabsorption,
increasing pressure on adjacent structures, including the orbit and the base of the skull, leading to possibly serious consequences. Here
we report a case of lipoma mimicking a frontal mucocele, managed surgically at the Ophthalmological Hospital of Anapolis.
Keywords: Lipoma; Mucocele; Orbital neoplasms; Ophthalmologic surgical procedures

Resumo
O lipoma é um tumor do tecido adiposo de caráter benigno, usualmente indolor, porém que pode gerar compressão de estruturas
adjacentes, dependendo da sua localização. A mucocele consiste em uma lesão cística de caráter benigno, na região fronto-etmoidal,
devido à obstrução crônica dos óstios dos seios paranasais e consequente processo inflamatório. O aumento do diâmetro da mucocele
por inflamação muitas vezes causa degradação e reabsorção ósseas, o que pode elevar a pressão em estruturas adjacentes como órbita
e base de crânio, causando intercorrências possivelmente graves. Neste estudo relatamos um caso de lipoma simulando mucocele
frontal, com propedêutica cirúrgica.
Descritores: Lipoma; Mucocele; Neoplasias orbitárias; Procedimentos cirúrgicos oftalmológicos

1
Anápolis Ophthalmological Hospital, Anápolis, GO, Brazil.
2
Department of Ocular Plastic Surgery, Anápolis Ophthalmological Hospital, Anápolis, GO, Brazil.
The authors declare no conflicts of intersts.
Received for publication 27/09/2018 - Accepted for publication 09/12/2018.

Rev Bras Oftalmol. 2019; 78 (3): 199-201

200 Léda RM, Dias MM, Souza BAA, Feijó ED

Introduction The treatment chosen for this case was surgical, with excision of
the tumor (Figures 2A, B and C) and an anatomopathological study.

L
ipoma is a benign tumor of adipose tissue, originating from
mesenchymal cells. It is more frequent in females and has
a hereditary character. It is uncommon in children and,
despite being composed of fatty tissue, does not occur more often
in obese patients. True (encapsulated) lipomas are usually unique
and are located in the upper portion of the body. They are usually
painless, but can generate compression of adjacent structures
depending on their location. Lipomas in the frontal region are
extremely rare, and depending on the location, frontal sinus
mucocele may be the differential diagnosis. Frontal mucocele, unlike
lipoma, is a benign cystic lesion that usually occurs in the frontal-
ethmoid region, caused by obstruction of the paranasal sinuses. (1,2)
The course of mucocele is characterized by inflammation
following chronic obstruction of the paranasal sinuses. Migration
of monocytes and lymphocytes is accompanied by production of
cytokines by fibroblasts. This is followed by bony degradation and
resorption as the diameter of the mucocele increases; this may
increase pressure in adjacent structures including the orbit and
skull base as it expands, possibly causing ptosis, periorbital edema,
peripheral vision, proptosis, chemosis, restriction of extrinsic
ocular motility and consequent diplopia. (3,4)
In this report we present the case of giant fronto-orbito-nasal
lipoma simulating a frontal mucocele in a 72-year-old woman.

Case Report
Patient DRS is a 72-year-old woman who presented with a
4-year history of a tumor in the left fronto-orbito-nasal region. The
tumor grew painlessly and insidiously over the four years, achieving
considerable size by the time she was first seen in our service.
On palpation, the tumor had a fibroelastic consistency,
with substantial mobility. It was adhered to the deep planes at its
posterior portion. The patient had normal visual acuity, with absence
of exophthalmos, palpebral ptosis and ocular dystopia. She denied
previous history of chronic sinusitis or trauma in the region. Our
clinical diagnosis was frontal mucocele (Figure 1A).
We obtained a CT scan of the skull and orbits (Figure 1B),
which revealed an expansive, well-defined, regular, oval-shaped
lesion with fat density, measuring approximately 2.2 x 2.6 x 1.7 cm,
located in the subcutaneous tissue of the left fronto-orbito-nasal
region. There appeared to be no invasion of structures adjacent to
the lesion. The primary diagnostic hypothesis became mesenchymal
tumor of adipose lineage.
Figure 1: (A) the lesion preoperatively lesion, simulating a frontal
mucocele; (B) Computed tomography of the orbits with contrast,
revealing an expansive lesion of 2.2 x 2.6 x 1.7 cm, in the subcutaneous
tissue of the left fronto-orbito-nasal region.
Figure 2: (A) intraoperative excision of the tumor; (B) view after tumor
excision; (C) material sent for biopsy material; (D) tumor biopsy section
for anatomopathological analysis, consistent with lipoma, without signs
of malignancy.
The anatomopathological examination revealed a soft
nodular mass of yellowish tissue, measuring 3.0 x 2.0 x 2.0 cm,
consistent with a lipoma, without signs of malignancy (Figure 2D).
The patient progressed without complications.
Discussion
Lipomas are benign tumors of adipose tissue, usually
subcutaneous and slow-growing. They arise in any area of the body
where there is fat, including the frontal and periorbital region. It is
rare in the orbit, corresponding to less than 1% of all orbital tumors.
A review described by Shields et al. reported that, out of a total of
1264 patients with periorbital lesions, only two were lipomas.(5-7)
Because of their encapsulated and circumscribed character, clinical
appearance and similar location, lipomas can mimic mucoceles.
Khubchandani et al. reported that the highest incidence occured
in patients between the fifth and sixth decade of life.(8-10)
The etiology of lipomas and their variants remains uncertain.
Genetic aberrations, trauma and intermittent chronic compression,
are related to the appearance of lipomas. (11) Mucoceles more
frequently involve the frontal sinus (65% of cases), but may
also affect the anterior, maxillary, sphenoid and infrequently the
posterior ethmoid sinus. Usually, they are unilateral in nature
and affect patients in their 40s and 70s. The clinical appearance
is a palpable mass in the superonasal region of the face (as in the
present case), a region containing fatty tissue that may also be
the site of lipomas. The process develops when the mucosa of the
frontal and ethmoidal sinuses becomes inflamed, consequently
increasing the amount of mucus in the region. This accumulation of
mucus induces cells of the fibroblast lineage to produce cytokines,

Rev Bras Oftalmol. 2019; 78 (3): 199-201

Giant frontal lipoma mimicking frontal mucocele
recruiting other cells of the immune system, including monocytes
and lymphocytes. (3,12,13)
Reports by Bijith et al. and Nikakhlagh et al. presented
patients with mucoceles, but with clinical aspects very similar to
those of the present case. Both patients presented with tumors in
the frontonasal region with more than three years of evolution
and characterized by insidious, painless, non-pulsatile and
mobile growth. These patients also had normal visual acuity and
negative histories of trauma and chronic sinusitis. As opposed to
the present case, these patients had restricted ocular movement
of the ipsilateral eye in terms of elevation and adduction; there
were also differences in the appearance of the lesions on optical
coherence tomography of the skull and orbits. (3,4)
Although mucoceles are primarily benign, their recurrent
nature causes them to dilate the paranasal sinuses due to the
accumulation of mucoid secretions, generating increased size
and pressure on adjacent structures. The upper wall of the orbit is
the most susceptible to bony erosion; in these cases, compression
exerted by the expanding mucocele can displace the globe
inferiorly. (4,14,15)
The clinical differentiation between lipoma and mucocele
can be difficult, particularly in the periorbital region, as this
is a preferential site for frontal mucoceles. In cases where the
diagnosis is in doubt, computed tomography of the skull and
orbits is recommended. Tomographic findings of a homogeneous
mass with fat density indicate a tumor of lipomatous lineage, and
may represent a lipoma or liposarcoma. To differentiate these two
entities, it is necessary to correlate clinical aspects, radiological
appearance and histology.(8)
Differentiated liposarcomas can simulate lipomas
because of their well-defined contours and mobility. Computed
tomography can reveal a mass with fat density interspersed with
areas of higher density that may resemble lipomas, in which case
the definitive diagnosis is made through biopsy. (16)
As described in the literature, the treatment of lipoma is
surgical excision of the tumor, especially if there is diagnostic
doubt, and complete removal is required to avoid recurrence. (5,8)
Paranasal sinus pathologies and those with lipomatous
lineage should be included in the differential diagnoses of orbital
and periorbital disorders, including in patients presenting with
proptosis or other ocular symptoms. Lipomas and mucoceles
are benign, potentially curable lesions that can mimic one
another. Therefore, early diagnosis with well-performed clinical
examination, differentiation and appropriate treatment are very
important for appropriate treatment.
201
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Corresponding author:
Eduardo Damous Feijó
Ocular Plastic Surgery Sector of the Anápolis Ophthalmological
Hospital, Anápolis, Goiás, Brazil.
Address: Av. Faiad Hanna, 235. Cidade Jardim Anápolis, GO,
Zip code: 75080-410
E-mail: eduardodff@yahoo.com.br
Rev Bras Oftalmol. 2019; 78 (3): 199-201