Ref 6 Herrera-2011-Unilateral Sub Mandibular Gland Aplasia

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Med Oral Patol Oral Cir Bucal. 2011 Jul 1;16 (4):e537-40. Submandi bular gland aplasia with subling ual gland hyper trophy

e537

Journal section: Oral Surgery

Publication Types: Review

Unilateral submandibular gland aplasia with ipsilateral sublingual

gland hypertrophy presenting as a neck mass

Gonzalo Herrera-Calvo 1, Belén García-Montesinos-Perea 2, Ramón Saiz-Bustillo 3, Jaime Gallo-Terán 4, Pe-

dro Lastra-García-Barón 5

1 Oral and Mailoacial Surgery epartment. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). esi-Oral and Mailoacial Surgery epartment. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). esi-

dent2 Oral and Mailoacial Surgery epartment. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). t-Oral and Mailoacial Surgery epartment. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). t-

tending Surgeon3 Oral and Mailoacial Surgery epartment. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). ead

o epartment4

eparment o adiodiology. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). esident5 eparment o adiodiology. ospital niersitario Marus de aldecilla� Santander (Cantabria� Spain). Sta radiologist

Correspondence:

Hospital Universitario Marqués de Valdecilla

Servicio de Cirugía Oral y Maxilofacial

Avenida de Valdecilla 25

39008 Santander (Cantabria) Spain

[email protected]

eceied: 27/02/2010

ccepted: 08/04/2010

AbstractThe congenital absence o the major saliary glands is a ery inreuent disorder� in which seeral glands are usu-

ally inoled at the same time. Sometimes this disorder can be associated with other deelopmental anomalies.

The unilateral aplasia of the submandibular gland is an extremely rare nding with only 14 cases reported in the

literature. Clinically, this kind of patients may complain of dryness of the mouth, difculties in chewing and swal-

lowing� seere periodontal disease or multiple caries� but usually they ollow an asymptomatic course. Saliary

gland aplasia can be diagnosed with a large ariety o imaging techniues� which include computer tomography(CT)� magnetic resonance imaging (M)� ultrasonography (S)� sialography� or scintigraphy. In this paper we

report a case o a patient reerred to our department with a long term and progressie growing neck mass� who has

an unilateral submandibular gland aplasia associated to an ipsilateral hypertrophy o the sublingual gland.

Key words: Submandibular gland aplasia, sublingual gland hypertrophy, neck mass.

errera-Calo G� García-Montesinos-Perea B� Saiz-Bustillo � Gallo-

Terán J� Lastra-García-Barón P. nilateral submandibular gland aplasia

with ipsilateral sublingual gland hypertrophy presenting as a neck mass.

Med Oral Patol Oral Cir Bucal . 2011 Jul 1;16 (4):e537-40.http://www.medicinaoral.com/medoralree01/16i4/medoral16i4p537.pd

Article Number: 16895 http://www.medicinaoral.com/

© Medicina Oral S. L. C.I.F. B 96689336 - pISSN 1698-4447 - eISSN: 1698-6946

eMail: [email protected]

Indexed in:Science Citation Inde Epanded

Journal Citation eports

Inde Medicus� MELINE� PubMed

Scopus� Embase and Emcare

Indice Mdico Español

doi:10.4317/medoral.16.e537

http://dx.doi.org/doi:10.4317/medoral.16.e537




e538

IntroductionMajor saliary gland agenesis is a ery uncommon

disorder. Since the rst case described in 1885, about

40 cases hae been reported in the literature. Most o

them were a congenital absence o all major saliary

glands. On the other hand� unilateral submandibular

gland aplasia is an etremely rare disorder. ccordingto our reiew� only 14 cases hae been described. Most

of them were incidental ndings because of the lack of

symptoms. In this study we report a case o an unilat-

eral submandibular gland aplasia with ipsilateral hyper-

trophy o the sublingual gland in a patient complaining

or a neck mass.

Case Report 26-year-old woman without any releant past medi-

cal or surgical history was reerred to our department

because o a long term submandibular swelling with

progressie growth. The patient didn’t complain about

any symptomatology, apart from a signicant anxiety

produced by the increasing size o the mass and the ig-

norance o her pathology.

Inspection showed an asymmetry in the submandibular

region� due to a sot tissue enlargement at the right side

(Fig. 1), as well as some ipsilateral oor of the mouth

eleation. Etraoral palpation was unremarkable� butintraorally there was a lobulated painless and mobile

soft mass of about 2 centimetres in the right oor of

the mouth. Oral mucosa had no alterations. The rest o

oropharyngeal and cerical eamination was normal.

Fig. 1. Sot tissue enlargement in the right submandibular area.

Fig. 2. ltrasonography o the right sublingual space (coronal cut).

Sublingual gland hypertrophy.

Fig. 3. T2-weightened images o magnetic resonance

(coronal cuts) � ight submandibular gland ab-

sence. B� ight sublingual gland hypertrophy.

B




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Neck S showed a slightly hyperechogenic homogene-

ous solid mass with smooth margins� located in the right

sublingual space (Fig. 2). No calcications or areas of

necrosis were noted. The right submandibular gland

could not be identied. No anomalies were observed in

the remaining major saliary glands� and cerical aden-

opathies were absent. These ndings were compatiblewith a hypertrophied right sublingual gland in associa-

tion with the absence o the ipsilateral submandibular

gland.

Our study was completed with M. ial and coronal

T1 and T2-weighted images showed absence o the right

submandibular gland and an enlarged right sublingual

gland� with the same signal characteristics as obsered

in the contralateral sublingual gland. ight sublingual

gland measured 22’38 12’47 millimetres� while the let

one measured 16’39 7’27 millimetres. Both parotid

glands and the let submandibular gland were normal�

as well as the rest o the study (Fig. 3).

DiscussionSaliary glands arise seuentially between the ourth

and the eighth week o etal deelopment as solid epi-

thelial buds rom primary oral caity� which grow and

etend into the underlying mesenchymal tissue as a sol-

id core o cells� which then undergoes etensie branch-

ing and eentually deelops lumina. Parotid glands ap-

pear during de ourth week o gestation� submandibular

glands during the sith� and sublingual glands during

the ninth (1).

The etiology o major saliary glands aplasia is still un-

known. This entity can accompany other deelopmental

anomalies� such as Treacher-Collins syndrome� hemia-

cial microsomia� clet palate or absence o the lacrimal

puncta (2�3). These abnormalities suggest that this en-3). These abnormalities suggest that this en-

tity may be associated with ectodermal deects o the

rst and second branchial arches and may be the result

of some disturbing inuence in early fetal development

(4). oweer congenital unilateral absence o the sub-

mandibular gland is not associated with other deelop-

ing anomalies� as well as our patient� which suggests

that this is an isolated phenomenon deried rom other

uniue deect o etal deelopment (5).

Clinical maniestations o the patients with saliarygland aplasia can ary depending on the number o

glands affected, their contribution in the salivary ow

production� and the presence o compensatory hypertro-

phy o the other glands. Some o them may be asympto-

matic� while others may present dryness o the mouth�

chewing and swallowing difculties or an increased in-

cidence o dental caries. This act is thought to be due

to reduction o the protectie eect o salia within the

oral caity (2).

Most o the patients with unilateral submandibular

gland aplasia reported in the literature had no symp-

toms related to the saliary gland absence� as well as

our case. Only two cases had dry lips and mouth� di-

culties with chewing solid oods and changes in taste

(6�7). A neck mass appearance as rst manifestation

has been only described three times� in two o them be-

cause o a compensatory gland hypertrophy (8)� and in

the other case because o a pleomorphic adenoma o thecontralateral submandibular gland (9). nilateral sub-

mandibular masses can be attributed to a high number

o pathologies with seeral origins. In the case described

by Shipchandler y Lorenz (8) the patient had a history o

suamous cell carcinoma o the tongue ipsilateral to the

mass, so the differential diagnosis was rst established

with a metastatic lymphadenopathy. Our patient had no

history o oral caity carcinoma. Other diagnostic pos-

sibilities considered were chronic sialadenitis, inam-

matory lymphadenopathy� saliary gland tumor and

lipoma.

Saliary gland aplasia can be diagnosed with a ariety

o imaging techniues� which include CT� M imaging�

S� sialography or scintigraphy (2�3�10). S is an ideal

tool for initial assessment of lesions in the supercial

parotid and submandibular gland. oweer� lesions in

the sublingual gland� located deeper and with a higher

percentage o malignant tumors� should be ealuated

by M imaging (11). CT cannot successully dieren-

tiate the sublingual gland rom the neighbouring mus-

cles. oweer� the ability o M imaging to dierenti-

ate the sublingual gland rom the surrounding tissues

acilitates the dierential diagnosis o the lesions in

this space. The sublingual gland has intermediate T1-

weighted signal intensity� lower than the at and higher than the muscle. On T2-weighted images the sublingual

gland remains hyperintense to muscle. Scintigraphy has

been described in some cases in the literature (2�3�12)�

but although this is a good method or determining the

unctional actiity o the saliary glands� it doesn’t de-

ne their anatomy (9). This technique may be useful for

discarding malignant origin in patients with personal

history of malignant tumors, as well as the ne needle

aspiration biopsy (FN) (8). Sialography by cannulat-

ing Wharton’s duct has also been used or submandibu-

lar gland agenesis conrmation (5). In our patient, US

was used as rst imaging technique showing the rightsubmandibular gland aplasia; this ecographyc ndings

were conrmed by MR imaging, which demonstrated

the ipsilateral sublingual gland hypertrophy.

The assessment o the patient and his amily is es-

sential� and ollow up seems to be worthwhile. Some

authors think that transoral sublingual gland ecision

should be considered in cases where the swelling were

aesthetically bothersome. In our case� the diagnosis and

the eclusion o other pathologies was enough or the

patient� who reused any therapy.

In conclusion� unilateral submandibular gland aplasia




e540

is an etremely rare disorder� and its real incidence is

probably unknown. Careul ealuation o the patients

who complain o erostomy� chewing and swallowing

difculties, severe periodontal disease and a neck mass,

may lead to the detection o new cases o this entity.

S and M imaging are adeuate techniues or this

patient’s study.

References1. Johns ME. The saliary glands: anatomy and embryology. Oto-

laryngol Clin North m. 1977;10:261-71.

2. Mconald FG� Mantas J� McEwen CG� Ferguson MM. Saliary

gland aplasia: an ectodermal disorder? J Oral Pathol. 1986;15:115-7.

3. igashino � orii T� Ohkusa Y� Ohkuma � Ino C� Nakazawa

M� et al. Congenital absence o lacrimal puncta and o all major sa-

liary glands: case report and literature reiew. Clin Pediatr (Phila).

1987;26:366-8.

4. Kubo S� be K� reshino T� Oka M. plasia o the submandibular

gland. case report. J Craniomailloac Surg. 1990;18:119-21.

5. oh JL. nilateral submandibular gland aplasia: an isolated phe-

nomenon o early etal deelopment. Otolaryngol ead Neck Surg.

2006;135:332-4.6. Yilmaz M� Karaman E� Isildak � Ener O� Kilic F. Symptomatic

unilateral submandibular gland aplasia associated with ipsilateral su-

blingual gland hypertrophy. ysphagia. 2010;25:70-2.

7. Gallego L� Junuera L� Cuesta P� osado P. Symptomatic uni-

lateral submandibular gland aplasia. Br J Oral Mailloac Surg.

2009;47:243.

8. Shipchandler TZ� Lorenz . nilateral submandibular gland

aplasia masuerading as cancer nodal metastasis. m J Otolaryngol.

2008;29:432-4.

9. García-Consuegra L� Gutirrez LJ� Castro JM� Granado JF. Con-

genital unilateral absence o the submandibular gland. J Oral Mai-

lloac Surg. 1999;57:344-6.

10. Yoshiura K� Yamada M� Yamada N. emonstration o congeni-

tal absence o then major saliary glands by computed tomography.

entomailloac adiol. 1990;2:77-8.11. Lee YY� Wong KT� King � huja T. Imaging o saliary

gland tumours. Eur J adiol. 2008;66:419-36.

12. Sucupira MS� Weinreb JW� Camargo EE� Wagner N Jr. Sali-

ary gland imaging and radionuclide dacryocystography in agenesis

o saliary glands. rch Otolaryngol. 1983;109:197-8.

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Ref 6 Herrera-2011-Unilateral Sub Mandibular Gland Aplasia

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