VU Shanmugam1, Vidyachal Ravindra1*, RutaShanmugam1, RG Mariappan1, Balaji Swaminathan1, Prem Nivas1, C Dhanashekaran2 and SK Srinivasan2
1Department of ENT, RMMCH, Annamalai University, India
2Department of Anesthesia, RMMCH, Annamalai University, India
Submission: November 23, 2016; Published: December 09, 2016
How to cite this article: V Shanmugam, Vidyachal R, RutaShanmugam, R Mariappan, Balaji S et al. Sublingual Swelling - A Diagnostic Dilemma. GlobjOtolaryngol. 2016; 2(4): 555591. DOI: 10.19080/GJO.2016.02.555591
We report a rare case of sublingual swelling in an 11 month old child which was a diagnostic and surgical challenge. The child presented with a painless, bluish, and swelling in the sublingual and submental region with complaints of protrusion of tongue. A pre-operative CT scan was done, but a diagnosis was not truly established. Though it was a case of difficult intubation, surgical excision was done. During surgery Ranula was suspected but histopathological examination revealed it to be Vascular Hamartoma masquerading as a Ranula. Although rare, vascular malformation should be part of differential diagnosis in sublingual and submental swellings.
Hamartoma is derived from the Greek word hamartia meaning fault or defect and -oma denoting tumor. It was coined by Albrecht in 1904 to denote developmental tumor-like malformation . It is defined as a non neoplastic developmental malformation, comprising of normal mature cells which are native to the anatomic location . Histology shows disorganized architectural pattern with predominance of one of its components. They are common in lung, pancreas, spleen, liver and kidney but very rare in the head and neck region. It is commonly asymptomatic. But morbidity can arise due to obstruction, infection, infarction, hemorrhage, and rarely due to neoplastic transformation. Deeper masses such as the mass described in this present case, can cause Respiratory and swallowing disturbance. It may occasionally cause life threatening respiratory distress. Differential diagnostics of neck masses is a challenge. Initially the firm sublingual swelling was assumed to be Pleomorphic Adenoma. But intra-operatively the mass resembled a large Ranula. Post-operative histopathological examination revealed it to be Vascular Hamartoma.
We report a rare case of Vascular Hamartoma of the Sublingual Region masquerading as a Ranula. An 11 month old child presented to the ENT OPD with complaints of sublingual swelling of 2 months duration with protrusion of tongue since childhood which was ignored by the parents. Presently the obstructive nature of the swelling has led to the child to
develop difficulty in both respiration and swallowing, which has prompted the parents to come to the hospital. Bimanual palpation revealed a swelling in the floor of the mouth, 3 x 3 cm in size, ovoid in shape, bluish in colour, non-tender and firm in consistency. A provisional differential diagnosis of Ranula, Hemangioma, Lymphangioma, Pleomorphic Adenoma and Congenital Dermoid Cyst was made.
USG Neck: Multi-loculated cystic lesion in Submental Region. Differential Diagnosis - Lymph Cystic Lesion or Plunging Ranula.
CT - Neck: Hypodense lesion below the mandible in midline displacing the Genioglossus muscle. Extending into the sub-mandibular space. Mylohyoid muscle was pushed down.
The risk of life threatening respiratory distress made surgery necessary. Thus exploration on table with excision was planned under general anesthesia. It was a case of difficult intubation with standby Tracheostomy. Through an external neck incision, swelling was identified on retracting the mylohyoid muscle. Swelling was dissected from its surrounding tissue. Extension into the substance of the tongue was noted. The swelling was removed in toto. Corrugated drain was kept in site. Airway was maintained with Nasopharyngeal airway and Ryle’s tube was placed (Figures 1 & 2).
Histopathology: Ciliated vascular channels surrounded
by fibro-collagenous tissue and skeletal muscle tissue. Final
histopathological diagnosis was a Vascular Hamartoma.
Post-operative the child was uneventful and the child was
symptomatically better. Nasopharyngeal Airway was removed
on the third post-operative day. Ryle’s tube was removed on
the 6th post-operative day. Residual tongue edema remained.
Protrusion of tongue drastically reduced. The floor of mouth
swelling resolved and the cervical wound healed normally
(Figures 3 & 4).
Vascular Hamartomas are a non-neoplastic abnormality
that occurs due to errors in vascular morphogenesis. During
organogenesis, abnormalities in the regulating factors can lead to improper proportion and impairment in differentiation
. Thus defect in the regulatory pathway of vascular stem
cells leads to formation of hamartomatous lesion . They
may occur as primary lesion or in association with syndromes
such as Sturge-Weber, Klippel-Trenaunary, Proteus Syndrome,
Bannayan-Riley-Ruvalcaba Syndrome and Osler-Weber-Rendu
Syndrome. Lymphangioma is a congenital Hamartoma involving
the lymphatic system. They are common in the head and neck
region but very rare in the oral cavity. They were first described
for the first time by Redenbacher in 1828 .
In patients less than 20 years of age, they represent 6% of
all benign tumors of smooth tissue . Around 50% are noted
at birth and around 90% develop by the age 2 years. Clinically,
lymphangiomas of the oral cavity present with a plaque made
up of small vesicles with thin walls. These vesicles may be filled
with lymph or blood. In the case presented here, these plaques
were observed on the lingual surface of the tongue. Hamartoma
are usually exophytic but may rarely present as a flat pigmented
lesions. According to a study conducted by Kaplan, though
Hamartoma of the oral cavity is very rare, it may occur on the
tongue, labial mucosa, buccal mucosa, and median maxillary
alveolus. One of the differential diagnosis that we suspected
was a Plunging Ranula. Ranula is a extravasation cyst of the
sublingual gland which represents 6% of all oral sialocysts .
Plunging Ranula has deep extension beyond the
mylohyoid muscle. It can also have massive involvement of
the submandibular and parapharyngeal spaces. Its clinical
and radiological behavior can be misleadingly similar to other
cystic neck masses, particularly the cystic hygroma. But while
lymphoid malformations such as Lymphangiomas are present
at birth or early childhood, ranulas typically appear in young
adults. Both are centered in the submandibular space with a
possible continuous extension beneath the free edge of the
mylohyoid muscle. Whereas Plunging Ranulas involve only the
parapharyngeal and sublingual spaces, lymphangiomas are
far more infiltrative, extending further toward the para- and
retropharyngeal, carotid, posterior cervical and visceral spaces
and the mediastinum. In case of super-infection or previous
surgery the radiological image of the Plunging Ranula can be
deceptively similar to lymphangiomas.
In the past, the first line of management of vascular
malformations which did not spontaneously involute was radical
surgery. One of the disadvantages of radical surgery includes
injury to muscle and nerves. Furthermore, in toto excision was
only possible in about 40% of the cases and these anomalies have
a high incidence of recurrence. Alternative managements like
intra-cystic sclero-therapy can lead to disappointing cosmetic
and functional result . Presently OK-432 is the preferred
intra-lesional sclerosant. It is a lyophilized mixture of low
virulent Su Strain of type III group A Streptococcus pyogenes.
When administered intra-lesionally it causes inflammation and
infiltration with neutrophils and macrophages . In the present case, surgical removal was chosen because of the increasing
risk of life threatening respiratory distress and because the
possibility of the diagnosis of a Plunging Ranula could not be