Bilateral Cervical Cystic Lymphangioma and Bilateral Plunging Ranula dilemma in differential diagnosis: Or both?

Cystic lymphangioma is seen in 80-90% at birth, in 30% of infants within one year of birth in 50-60% within two years (6). The most sign or symptom of lymphangiomas is the presence of a mass. Most lesions are recognized early because of their size and associated symptoms of respiratory obstruction and problems with feeding, which are the another common symptoms (7). Cystic lymphangioma of neck usually enlarge rapidly because of an upper respiratory infection(8).

Imaging is very important for clinical diagnosis and for surgical planning of cystic lymphangioma.

Ultrasound is commonly used to investigate a neck mass. Ultrasound enables differentitiation between cystic and solid masses and identifies the anatomic location, association to other structures, and allows accurate needle aspiration or biopsy (9,10). Imaging findings of plunging ranula contain a unilocular large mass, lack of internal septations, a smooth capsuleand location extrinsic to the submandibular gland (11). Cervical US and Doppler US can distinguish between cystic and solid masses and show whether there is vascular circulation. So that the differential diagnosis of cystic lymphangioma from hemangioma can be made. Ultrasound, Doppler US, and ultrasound with fine-needle aspiration cytology, computed tomography or magnetic resonance imaging (MRI) may be used for diagnosis and differential diagnosis all cystic lesions (12,13); however, MRI is the ideal examination in neck cystic lymphangiomas to imaging its association with the adjacent organ and neck vascular nerve bundle (14).

In our case who was followed up for bilateral submandibular cystic swelling in the prenatal period, our first diagnosis was left cystic lymphangioma because of the presence of a left submandibular 47x22 mm lesion on the first postnatal US and the absence of a ranula. However, sporadic coexistence of ranula and left cystic lymphangioma was considered in the patient, since the left ranula developed first in the following months and remained uneventful until the age of two after ranula incision and marsupialization. Therefore, until the age of two, follow-up was done with US and Doplerr US.

First line treatment for cystic lymphangioma is complate cystectomy and injection of a sclerotic agent such as bleomycin and or radiotherapy (15,16,17,18). Local recurrence is possible if there component is incomplate. Large and complex lesions are usually related with vital structures such as cranial nerves and major vessels of the neck. Multiple-staged excisions and debulking procedures are often needed (8).

Many methods of treatment for ranula has been suggested and include aspiration of cystic fluid, sclerotherapy, marsupialisation, excision of the ranula only, excision of the ranula and the ipsilateral sublingual gland (19). The lingual nevre and submandibular duct will be at risk for injury particularly in infant patients because of near to the sublingual gland (20). In plunging ranula, the mucus collection is in the submandibular and submental area of the neck with or withouth an connected intraoral collection (20).

Cases which clinical evaluation and MRI can not differentiate a plunging ranula from other lymphatic lesions; fine needle aspiration and amylase measurement are important. Elevated levels of salivary amylase are characteristic to plungingranula fluid (21). Diagnosis is definitely established with fine-needle aspiration and demonstratetion of mucus and high amylase contenent (11). In our case, during the first bleomycin injection for cystic lymphangioma, no characteristic was detected in the intracystic aspiration fluid; however, since there was no ranula in the first application, amylase was not tested in the cyst fluid.

When the sublingual gland is complately excised, recurrence rate is 1% and if complate excision is not achievedit can be higher than 50% (5). Complate cystectomy is difficult in children when the cyst wall is thin and the lesion has infiltrated deeply into areas surrounding important cervical organs or has adhered them; this causes severe complications and recurrence (9,22,23).

In our case, the shrinkage and re-growth of the cyst after the first bleomycin injection suggested that there was a recurrence. When the two-year-old presented with bilateral prominent submandibular swelling and bilateral ranula, it was thought that our patient may have cystic lymphangioma with plunging ranula; this time not only US and Doppler US; at the same time, MRI was performed to show the borders of the cystic lesion, its neighborhoods, salivary glands and its relationship with the neck vascular nerve bundle. Indeed, in our case, it was detected that plunging ranula and cystic lymphangioma were bilateral, and she was operated with a multidisciplinary approach.

In conclusion; having plunging ranula with cystic lymphangioma and bilateral presence is a very rare condition. It should always be kept in mind that ranula or cervical cystic lymphangioma, which appears simple at first attempt, may recur or develop into plunging ranula. In all cystic lesions in close proximity to all cervical submandibular salivary glands; whether or not cystic lymphangioma or plunging ranula is considered; determination of amylase into the cystic aspiration fluid may be helpful in planning follow-up and treatment in cases with a complicated course.