Case Report

Report of rare bilateral nasolabial cysts

Marius P. MARCOVICEANU, DDS
1
, Marc C. METZGER, MD, DDS
1
, Herbert DEPPE, PhD, DDS
2
,
Nikolaus FREUDENBERG, PhD, MD
3
, Ahmad KASSEM, MD
3
, Christoph PAUTKE, MD, DDS
2
,
Bettina HOHLWEG-MAJERT, MD, DDS
2
1
Department of Oral and Maxillofacial Surgery, University Hospital Freiburg, Hugstetterstrasse 55, D-79106 Freiburg,
Germany;
2
Department of Oral and Maxillofacial Surgery, Technische Universitat Munchen, Klinikum rechts der Isar,
Ismaninger Strasse 22, D-81675 Munich, Germany;
3
Institute of Pathology, University Hospital Freiburg, Breisacher
Strasse 115a, D-79106 Freiburg, Germany
SUMMARY. Purpose: Nasolabial cysts are usually unilateral and are quite rare, while bilateral cysts are even
rarer. Patient and method: Our report concerns a 48-year-old female with bilateral nasolabial cysts. After
many years of misdiagnosis she was nally referred to our clinic with a subnasal swelling of unknown
origin. Result: Evaluation of the patients medical history, clinical examination and of a previous CT scan
led to the diagnosis of a nasolabial cyst, which was later conrmed by histological examination. Treatment
involved the surgical excision. Conclusion: A complete surgical excision is recommended using a sublabial
approach as the treatment of choice, although transnasal endoscopic marsupialization seems to be a simple
and effective alternative. It has been shown that after successful marsupialization, the nasolabial cyst is converted
to an air-containing paranasal sinus. 2008 European Association for Cranio-Maxillofacial Surgery
Keywords: cyst, oral surgical procedures, pathology
INTRODUCTION
The nasolabial cyst is a rare non-odontogenic soft tissue
cyst located in the upper lip. Histologically, it is lined
either with cylindrical epithelium with goblet cells or
with non-keratinized squamous epithelium. Zuckerkandl
rst described such a cyst in 1882. It was believed to be
a retention cyst or to originate from inammation of the
mucous glands. In 1913 Klestadt postulated for the rst
time that it was of an embryological origin. Since then,
several theories on the aetiology have been advanced.
Of two principal theories are under current discussion,
the rst assumes that the cyst could originate from
entrapment of embryonic epithelial cells in the mesen-
chyme after fusion of the medial and lateral nasal pro-
cesses and the maxillary prominence. This theory led to
the classication of the cyst as a ssural cyst. The other
theory postulates that it could be caused by the persistence
of misplaced epithelium from the nasolacrimal duct.
There have been a variety of names used to describe
this rare cyst. In the past, the most commonly used
term was nasoalveolar cyst, which derives from its
classication as a ssural cyst. Due to the fact that there
is never any bone involvement this term is inappropriate.
Nowadays the term nasolabial cyst is more commonly
used.
The patient will characteristically present with a pain-
less swelling, mostly left sided, of the upper lip adjacent
to the nasal alae of the nose. Sometimes, the cyst can
become infected, in which case the patient will complain
of pain, the most common reason for a medical consulta-
tion. The mean age of patients is in the fourth or fth
decade with a signicantly higher ratio of women (4:1).
The purpose of this paper is to present a rare case of
bilateral nasolabial cysts and to briey review the litera-
ture.
CASE REPORT
A 48-year-old woman was referred to our hospital with
bilateral swellings of unknown aetiology in the nasolabial
region. The variable signed swellings had already been
present for several years. She complained of localized dis-
comfort and, especially in winter, of nasal obstruction.
Her complaints were assumed to be of dental origin. She
had received root canal treatment on several upper incisors
and subgingival scaling had been repeatedly performed.
Due to severe bone loss, mainly in the upper jaw, her
last incisors were nally extracted 4 months before her
visit. Her complaints increased after the extraction so
that upper labial vestibular incision was necessary. She
reported discharge of a clear uid after the incision and
antibiotics were prescribed. After an initial reduction of
the swelling, it returned to its former size. As a conse-
quence, a CT scan was performed.
She was referred with slightly painful, soft, and uctu-
ant bilateral swellings at the nasal alae. The nasolabial
83
Journal of Cranio-Maxillofacial Surgery (2009) 37, 83e86
2008 European Association for Cranio-Maxillofacial Surgery
doi:10.1016/j.jcms.2008.11.006, available online at http://www.sciencedirect.com
fold was attened. The orthopantomograph (OPG)
showed an edentulous upper jaw. No denite pathologi-
cal features could be identied anteriorly. In the axial
view, the CT showed two pathological structures with
central enhancement of approx. 1.0 1.5 cm at both nos-
triall apertures. The nasal spine was unusually pointed
without any bony defect (Fig. 1).
Excision of the soft tumour was performed under
general anaesthesia using an intraoral approach. An inci-
sion was made on the upper alveolar ridge and following
elevation of a mucoperiosteal ap, the two lesions were
revealed (Fig. 2). After careful curettage they were com-
pletely removed. On the left side, the cyst measured
2.2 1.5 1.5 cm. Light microscopy of the cystic wall
showed a connective tissue with fewcells, lined with a at-
tened squamous epithelium (Fig. 3). The right cyst mea-
sured 2.2 1.4 1 cm. Microscopically, this cyst wall
showed foci of chronic inammatory cells and the wall
was lined with two layers of cylindrical epithelium, rich
in goblet cells (Fig. 4). Intraoperatively, small defects
were recognized on both sides of the nasal oor, which
were left open for secondary healing. After a 6 month
follow-up a good clinical result was evident with no recur-
rence. Only a light hypaesthesia was evident in the right
upper lip.
DISCUSSION
The aetiology of the nasolabial cyst remains controver-
sial. Originally there were three main theories on its
development. The embryological entrapment theory has
been discarded due to lack of evidence. The two remain-
ing theories are the ssural cyst or that of embryological
remnants of the nasolacrimal duct, which is currently
more widely accepted. The main argument for the latter
is the location of the nasolabial cyst, which is exclusively
at the oor of the nose. Due to the fact that embryologic
tissue still has pluripotential abilities, it is not possible to
draw a denitive conclusion on the origin solely from
their histological structure, especially since all respiratory
epithelium develops from ectoderm (Schmallenbach and
Austermann, 1976). However, a recently performed EM
study could not nd ciliated cells, which are typically
found in respiratory epithelium, also reported in light
microscopic studies by several authors (Su et al., 2006)
in their research cases. On the other hand, numerous
short globular microvilli were found, which may repre-
sent an embryonic or foetal status of respiratory epithe-
lium. Mucous-containing goblet and gland cells were
also found. Interestingly, in our specimens we found dif-
ferent type of epitheliums on each side: on the left side
a attened squamous epithelium and on the right side cy-
lindrical epithelium rich in goblet cells were seen, which
supports the theory of embryonal cell remnants with plu-
ripotent abilities. However there is still the problem of
how to explain the presence of nasal respiratory epithe-
lium, which is usually found within this cyst.
The nasolabial cyst is quite a rare entity and represents
approx. 0.7% of all maxillofacial cysts (Choi et al.,
2002). Since it was rst described by Zuckerkandl,
only 267 other cases have been reported in the English
literature (Patil et al., 2007). It is mostly one-sided,
only approx. 10% being bilateral (Vasconcelos et al.,
1999). Some authors suggest that it might be a more
common phenomenon (Yuen et al., 2007), especially
for certain ethnic groups (Holtmann et al., 1985).
Because of its rarity, it is often misdiagnosed and not
treated properly. This is also shown in our case where
an earlier misdiagnosis led to inappropriate treatment
for several years.
A preliminary diagnosis can be made by simply recog-
nizing the typical clinical features and by conducting an
occlusal radiograph (Chinellato and Damante, 1984). A
CT or cone beam computer tomography is not always
necessary, although in most cases, it will secure the diag-
nosis and detect the extension of the cyst (Talpos et al.,
2006). However, a conclusive diagnosis can only be
achieved through histological examination.
The most relevant differential diagnosis is a dental
abscess from the canine space, which can easily be
excluded by vitality testing of the affected tooth.
A thorough examination should be performed, because
dental abscess could lead to life-threatening infection
spreading intracranially (Zimmerer et al., 2006; Mylonas
et al., 2007).
Fig. 1 e Axial view of the computer tomography showing bilateral
tumours with central enhancement.
Fig. 2 e Intraoperative view of the two cysts after complete exposure.
84 Journal of Cranio-Maxillofacial Surgery
Other possible differential diagnoses include seba-
ceous cysts or implanted epidermal inclusion cysts which
clinically and histologically resemble the nasolabial cyst
(Bull et al., 1967). Benign or malignant salivary gland
tumours can usually be excluded on the strength of the
clinical and radiological features, such as their long-
standing nature and in very limited bone involvement
(Raudjarv and Soots, 2006). This case also showed an
alteration in the shape of the maxilla. Nevertheless, radic-
ular resorption has been described in three cases (Pereira
Filho et al., 2002).
The MelkersoneRosenthal syndrome is an important
differential diagnosis. It is rare, of unknown aetiology
and described as a triad of recurrent labial oedema,
relapsing facial paralysis and ssured tongue. The classic
triad is seldom seen. Labial swelling is often the rst
symptom and occurs mostly the upper lip (Kanerva
et al., 2008). However, the lip swelling is often unilateral,
rm, intermittent or persistent and, in the case of a cyst, it
is soft, movable, pale, round and localized (Zimmer et al.,
1992).
The clinical features of the nasolabial cyst are quite
specic. Its submucosal location at the anterior nasal
oor is characteristic and was described by Bull et al.
(1967) as virtually pathognomonic. Our case presented
typical clinical features: location at the ala of the nose
and with a long-standing history. Despite the fact that
it is usually asymptomatic it can sometimes produce
a feeling of pressure or discomfort and, when infected,
even pain (Holtmann et al., 1985). The variation in size
could be explained by the observation that it can sponta-
neously drain into the nose or mouth, especially when it
is infected, in approx. 50% of cases (Kuriloff, 1987;
Yuen et al., 2007).
Several treatment modalities have been described in the
management of the nasolabial cyst, for example surgical
excision, endoscopic marsupialization, simple aspiration,
incision and drainage, injection of sclerotic agents and
marsupialization. With the exception of complete surgical
excision and endoscopic marsupialization, all other treat-
ments are associated with a high recurrence rate (Yuen
et al., 2007). Therefore, we recommend complete surgical
excision using a sublabial approach as the treatment of
choice, although the transnasal endoscopic marsupializa-
tion seems to be a simple and effective alternative. It has
been shown that after successful marsupialization the na-
solabial cyst is converted to an air-containing paranasal
sinus (Su et al., 1999).
CONFLICT OF INTEREST
All authors state that there are no nancial interests or
commercial associations that may create a conict of in-
terest with information presented in this manuscript.
FUNDING SOURCE
There is no involvement of any study sponsors.
References
Bull TR, McNeill KA, Milner G, Murray SM: Naso-alveolar cysts.
J Laryngol Otol 81: 37e44, 1967
Chinellato LE, Damante JH: Contribution of radiographs to the
diagnosis of naso-alveolar cyst. Oral Surg Oral Med Oral Pathol
58: 729e735, 1984
Choi JH, Cho JH, Kang HJ, Chae SW, Lee SH, Hwang SJ, Lee HM:
Nasolabial cyst: a retrospective analysis of 18 cases. Ear Nose
Throat J 81: 94e96, 2002
Holtmann S, Martin F, Permanetter W: Dysontogenetic median
maxillary cysts. Laryngol Rhinol Otol 64: 331e334, 1985
Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkaranta A:
MelkersoneRosenthal syndrome. Otolaryngol Head Neck Surg
138: 246e251, 2008
Kuriloff DB: The nasolabial cyst-nasal hamartoma. Otolaryngol Head
Neck Surg 96: 268e272, 1987
Mylonas AI, Tzerbos FH, Mihalaki M, Rologis D, Boutsikakis I:
Cerebral abscess of odontogenic origin. J Craniomaxillofac Surg
35: 63e67, 2007
Fig. 3 eNasolabial cyst of the left side: the cystic lining shows attened
squamous epithelium without keratinization. Primary magnication:
100, inset: 400.
Fig. 4 e Nasolabial cyst of the right side: this cyst is lined with a two
layered cylindrical epithelium with goblet cells. Primary magnication:
100, inset: 400.
Report of rare bilateral nasolabial cysts 85
Patil K, Mahima VG, Divya A: Klestadts cyst: a rarity. Indian J Dent
Res 18: 23e26, 2007
Pereira Filho VA, Silva AC, Moraes M, Moreira RW, Villalba H:
Nasolabial cyst: case report. Braz Dent J 13: 212e214, 2002
Raudjarv I, Soots M: Diagnosis and treatment of salivary gland tumour.
J Craniomaxillofac Surg 34: 198, 2006
Schmallenbach HJ, Austermann KH: Current views on the etiology and
morphology of nasolabial cysts. Fortschr Kiefer Gesichtschir 21:
107e109, 1976
Su CY, Chien CY, Hwang CF: A new transnasal approach to
endoscopic marsupialization of the nasolabial cyst. Laryngoscope
109: 1116e1118, 1999
Su CY, Huang HT, Liu HY, Huang CC, Chien CY: Scanning electron
microscopic study of the nasolabial cyst: its clinical and
embryological implications. Laryngoscope 116: 307e311, 2006
Talpos S, Urtila F, Valeanu A: Giant maxillary sinus cyst with nasal
fosae, ethmoid, frontal sinus and orbital extension.
J Craniomaxillofac Surg 34: 121, 2006
Vasconcelos RF, Souza PE, Mesquita RA: Retrospective analysis of 15
cases of nasolabial cyst. Quintessence Int 30: 629e632, 1999
Yuen HW, Julian CY, Samuel CL: Nasolabial cysts: clinical features,
diagnosis, andtreatment. Br J Oral Maxillofac Surg45: 293e297, 2007
Zimmer WM, Rogers III RS, Reeve CM, Sheridan PJ, Rochester M:
Orofacial manifestations of MelkerssoneRosenthal syndrome.
Oral Surg Oral Med Oral Pathol 74: 610e619, 1992
Zimmerer S, Saldamli B, Muller A, Stu binger S, Schwenzer K:
Odontogenious cerebral abscess e report of 7 cases.
J Craniomaxillofac Surg 34: 46, 2006
Bettina HOHLWEG-MAJERT, MD, DDS
Department of Oral and Maxillofacial Surgery
Klinikum Rechts der Isar
Technische Universitat Munchen
Ismaninger Strasse 22
81675 Munich, Germany
Tel: +49 89 4140 5950
Fax: +49 89 4140 4493
E-mail: majert@mkg.med.tum.de
Paper received 13 June 2008
Accepted 7 November 2008
86 Journal of Cranio-Maxillofacial Surgery