Asian J Oral Maxillofac Surg 2003;15:1xx-1xx.

Wada, Yamamoto,
Asian J Oral Maxillofac Ootsuki, et al
Surg 2004;16:63-66.
CASE REPORTS

Congenital Ranula

Takeshi Wada, Hirokazu Yamamoto, Reiko Ootsuki, Ken Nakatani, Shigeyuki Fujita
Department of Oral and Maxillofacial Surgery, Wakayama Medical University, Wakayama, Japan

Abstract
A patient with congenital ranula accompanied by feeding difficulties is reported and a treatment concept
presented. It is possible to predict the existence of epithelial lining inside the cyst wall based on the degree
of response to OK-432 injection therapy. Therefore, it might be possible to treat ranulas by simple procedures
such as OK-432 injection therapy for the mucous extravasation type, and by simple incision or
marsupialisation for the mucous retention type.

Key words: Congenital ranula, Epithelial lining, Injection therapy, Marsupialisation

Introduction penicillin G potassium, it is necessary to confirm a

Ranula is a clinical term given to a transparent cystic
swelling of the floor of the mouth associated with
the ducts from the sublingual or submandibular
glands. It may be caused by trauma to the sublingual
salivary duct or by atresia of the submandibular or
sublingual salivary duct, resulting in pooling of saliva
in the floor of the mouth. Although congenital ranula
is relatively rare, ascertaining the presence or absence
of a cyst wall is one of the important factors in
determining appropriate treatment.
negative reaction by skin testing beforehand.
Recently, we tried this therapy in the treatment
of mucous cysts in the oral cavity as a non-surgical
therapy. However, it seems that this therapy is not
effective in cases where the cystic lesions have a thick
epithelial lining such as stratified squamous epi-
thelium. It may be possible to predict the existence
of epithelial lining based on the degree of response
to OK-432 injection therapy.

Intra-lesional injection therapy of OK-432 for
cystic lymphangioma and ranula has been developed
in Japan with several reports showing the usefulness
of this therapy.1,2 OK-432 is a substance prepared by
incubating the culture of the low-virulence Su strain
of group A Streptococcus pyogenes of human origin,
treated with penicillin G potassium (picibanil, Chugai
Parmaceutical Co. Ltd, Tokyo, Japan). Local
inflammation produced by injection of OK-432 may
lead to absorption of cystic fluid and may cover the
extravasation site with fibrin and, finally, the cystic
wall may adhere to the floor of the mouth.3 Possible
side effects of OK-432 are shock, systemic fever, and
local inflammatory symptoms. As the shock due to
OK-432 is regarded as an allergic reaction to
Correspondence:
Takeshi Wada, Department of Oral and Maxillofacial Surgery,
Wakayama Medical College, 811-1 Kimiidera, Wakayama,
641-0012, Japan.
Tel/Fax: (81 73) 441 0643;
E-mail: twada@wakayama-med.ac.jp
This paper reports a large congenital ranula in a
neonate causing interference with feeding, reviews
the literature concerning the nature of the cyst wall
of congenital ranula, and discusses the treatment
modality.
Case Report
The patient was a Japanese boy born at 38 weeks
gestation to a healthy 27-year-old mother. The
birth weight was 3196 g. On the first day of life, the
obstetrician noted impairment of feeding and
an abnormal swelling of the floor of the mouth. The
patient was immediately referred to the Neonatal
Intensive Care Unit of Wakayama Medical University,
in Japan. Physical examination revealed no remark-
able abnormalities and arterial blood gas analysis
was within normal range.
Oral examination showed a 23 x 14 mm dark
blue swelling located in the floor of the mouth,

Asian J Oral Maxillofac Surg Vol 16, No 1, 2004 63

Congenital Ranula
Figure 1. Photograph just after birth showing swelling of the
floor of the mouth and raised tongue.
which fluctuated with pressure and the tongue was
significantly raised (Figure 1). Ultrasonography
showed a unicystic hypoechoic lesion in which
irregular hyperechoic granular materials were found
(Figure 2).
A tentative diagnosis of congenital ranula was
made. The differential diagnoses were cystic hygroma,
epidermoid cyst, or haemangioma. An aspiration
biopsy was performed, drawing approximately 7 ml
of whitish saliva-like fluid. Just after aspiration, the
swelling of the floor of the mouth was completely
decompressed and saliva flow from the orifices of
both submandibular ducts was confirmed. Laboratory
analysis of this fluid showed only S-type amylase
isoenzyme (Figure 3). The definitive diagnosis was
congenital simple ranula arising from the right floor
of the mouth.
In this study, OK-432 injection therapy was
performed 3 times using 0.8 KE, 1.0 KE and 2.5 KE
solutions after confirmation of negative reaction with
skin test for penicillin G potassium. Two percent
lidocaine containing epinephrine was used for local
infiltration anaesthesia to the ventral surface of the
tongue near the swelling. Saliva-like content in the
cavity of the swelling was aspirated by inserting an
18-gauge intravenous cannula into the cystic cavity
through the muscle layer of the tongue. Then, an
equivalent volume of 0.2 KE of OK-432 per 1 ml of
64
Figure 2. Ultrasonograph showing unicystic hypoechoic
lesion including granular hyperechoic substances (arrow).
1% lidocaine was injected into the cavity through the
same cannula. The final injection was used in a high
concentration of OK-432 solution. There were no
clinical effects, and there was a complete absence of
systemic fever as well as regional inflammation.
We surmised that there was an epithelial lining
inside the cyst wall, so a small diameter window
was created (marsupialisation) on the cyst in the right
floor of the mouth by removing the roof of the
swelling. The lining was carefully sutured to the oral
mucosa under local infiltration anaesthesia. The
histological report revealed that stratified squamous
epithelium lined a wall composed of loose connective
tissue which had a small number of lymphocytes
Fractions % IU/L
S-3
S-2
17.97
34.53
25.2
48.3
S-1 47.50 66.5
Figure 3. Laboratory analysis of the aspirated fluid
containing only S-type amylase isoenzyme.
Asian J Oral Maxillofac Surg Vol 16, No 1, 2004
 Wada, Yamamoto, Ootsuki, et al

Figure 4. Photomicrograph of the cyst wall showing stratified

squamous epithelium and subepithelial loose connective
tissue infiltrated by a small number of the lymphocytes Figure 5. Photograph of the oral cavity 1 year after treatment
(hematoxylin and eosin stain, original magnification x 40). with no sign of recurrence.

(Figure 4). The patient made an uneventful recovery
and there was no recurrence in 1 year of follow-up
(Figure 5).
Discussion
It is well known that there are 2 types of ranula — a
mucous extravasation cyst lined with connective tissue
infiltrated with inflammatory cells, and a mucous
retention cyst formed by dilatation of a duct or acinus
lined with epithelium.4 Congenital atresia of any of the
multiple ducts of the sublingual gland may occur, as
may congenital atresia of the submandibular duct into
which the duct of the greater sublingual gland usually
drains.5,6 Persistent secretion from the sublingual gland
(a spontaneous secretor) into a duct that has failed
to canalise fully could therefore lead to rupture of the
gland and mucous extravasation into the tissue.7 If dila-
tation of the duct develops instead of rupture, a mucous
retention cyst with an epithelial lining may form.
Amin and Bailey have reported 2 cases of
congenital atresia of the orifice of the submandibular
salivary duct.8 The cystic lesion that arises from this
causes a special type of retention cyst and is lined by
ductal epithelium. In the patient discussed here, there
was a stratified squamous epithelium. It is estimated
that there is a high occurrence of epithelial lining in
congenital ranulas (Table 1).

Study Type Treatment Existence of epithelial Prognosis Others

lining
Matt and Plunging Enucleation + Stratified squamous 18 months, good
Crockett10 Removal of sublingual gland epithelium
Saheeb11 Simple Marsupialisation - 2 months recurrence
(removal of sublingual gland) (good)
Redpath4 Simple Marsupialisation + Columnar epithelium Unknown
Rees7 Simple Simple incision Unknown 1 year, good Congenital atresia of
submandibular duct
Steelman at al9 Simple Follow up Unknown 20 months, good
Kinirons12 Simple Simple incision Unknown Good Relation to
submandibular duct
Amin and Simple Simple incision Unknown 15 years, good Congenital atresia of
Bailey8 submandibular duct
Simple Simple incision Unknown 4 years, good Congenital atresia of
submandibular duct
Seko et al13 Simple Enucleation of the cyst - 13 months, good
Present case Simple Marsupialisation + Stratified squamous 1 year, good Congenital atresia of
epithelium submandibular duct
Table 1. Studies of congenital ranula.

Asian J Oral Maxillofac Surg Vol 16, No 1, 2004 65

Congenital Ranula
Congenital ranula Observation Spontaneous resolution
Congenital atresia of the orifice of the submandibular duct
Response (mucous extravasation type)
OK-432 injection
No response (mucous retention type)
Figure 6. Wakayama Medical University treatment modality for congenital ranula.
Steelman et al have reported a congenital ranula
with spontaneous resolution and they have mentioned
that suspected ranulas in neonates without feeding
problems and/or airway obstruction should be
observed initially for spontaneous resolution.9 Con-
genital ranulas without atresia of the orifice of the
submandibular salivary duct should be observed for
approximately 2 months until the patient is able to
tolerate OK-432 injection therapy. Patients with
congenital atresia of the orifice of the submandibular
salivary duct, confirmed by absence of saliva flow from
the orifice with massage of the submandibular gland,
should be treated with simple incision of the cyst wall
as early as possible to prevent complications such as
atrophy of the submandibular salivary gland. For the
treatment of other types of ranula, OK-432 injection
therapy is the first choice of treatment. In our clinical
experience with adult patients, injection of low-
concentration OK-432 and the use of lidocaine as a
solvent does not cause systemic fever and severe local
inflammatory symptoms. It is a conservative proce-
dure that avoids damage to the adjacent tissue. If this
therapy is not effective, the existence of an epithelial
lining should be assumed. In such patients, marsupiali-
sation (removing the roof of the cyst and suturing the
lining to the oral mucosa) is the best method of
intervention. In the present patient, a small window was
created in the cyst while the patient was under local
infiltrating anaesthesia. There was no recurrence of
the cyst for 1 year after the operation. Also there were
no complications. Therefore, it may be possible to treat
congenital ranulas by simple procedures such as simple
incision, OK-432 injection therapy, or marsupiali-
sation depending on the clinical findings (Figure 6).
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Marsupialisation
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