Popli G et al.: Cystic Hygroma Resembling Ludwig’s Angina CASE REPORT

A Rare Case of Cystic Hygroma Resembling Ludwig’s Angina 1 2 3 Gourav Popli , Raman Kansal , Gaurav Verma Correspondence to:

1- Senior Lecturer, Department of Oral and Maxillofacial Surgery, Subharti Dental College, Dr.Gourav Popli, H.No-18, Sant Nagar, Meerut. 2- Senior Lecturer, Department of Oral Medicine and Radiology, Guru Nanak Dev Karnal(132001), India. Dental College, Sunam. 3- MDS, Oral and Maxillofacial Surgery. Contact Us: www.ijohmr.com

ABSTRACT

Lymphatic malformation is an unorthodox pathology that mainly involves posterior triangles of neck in infants. The case presented here was of swelling in an adult male extending bilaterally in the neck region below inferior border of mandible (rare case). Above that, a history (acute) of 3 days and bilaterally grossly carious mandibular molars (odontogenic cause) and raised floor of mouth misindicated the diagnosis towards Ludwig’s Angina. Haemorrhagic fluid on aspiration indicated the need to further investigate the condition with the help of ultrasonogram, colour doppler and contrast enhanced computed tomography. Based on these investigations, low flow lesion was established leading to provisional diagnosis of lymphatic malformation. Surgical removal of the pathology was done under General Anaesthesia, and sample was sent for histopathological reporting, which confirmed the diagnosis being Cystic Hygroma. Sequential 3 year follow up is uneventfull. This reported casse has unusual variation in terms of age of onset, progression, anatomical location and pathological characters authors believe that this rare case report will add on to the academic literature. KEYWORDS: Cystic Hygroma, Ludwig’s Angina, Lymphatic Malformation AA

aaaasasasss INTRODUCTION breathing. No relevant history of trauma or respiratory Cystic hygroma is a congenital or acquired, benign infection was present. Extraorally, a diffuse swelling was proliferative developmental anomaly of lymphatic system seen below the inferior border of mandible, extending and consist of channels and cystic spaces of varying bilaterally upto the neck region with no signs of sizes.1 It was first described by Redenbacher in 1828.2 localization (Figure 1). Overlying colour and surface Lymphatic obstruction, trauma or infection usually leads to acquired forms. Etiologically, they originate from the sequestration of lymphatic tissue and its failure to divulge with the residual lymphato-venous system.1 Mostly cystic hygromas are seen in head and neck, and that too in posterior triangle region and about 90% of them arise at birth or till 2 year of age. They constitute less than 5% of all congenital neck masses in children and even less in adults.2 It is different from all other differentials of cervical swelling, in being brilliantly translucent. It neither posseses malignant potential nor has any familial tendency. Although benign, it often causes extensive invasion of tissue planes by tiny daughter cysts.3 This is a case report of cystic hygroma mimicking Ludwig's angina as per history and clinical examination. However, it had an unusual age of onset, progression, anatomical Figure 1: Extraoral swelling extending bilaterally. location and pathological characters. texture were normal. No obvious discharge from the swelling was observed. On palpation, temperature of CASE REPORT overlying skin was raised. It was firm, tender, non A 26 year old male patient visited the Department of Oral fluctuant in nature with no pus or blood discharge. and Maxillofacial Surgery with the chief complaint of Neither pulsation nor bruit was noted. Lymph nodes were massive swelling in the neck region for last 3 days. non-palpable because of swelling in the lower half of face History revealed that the patient noticed a small sized which extended upto the neck region. Mouth opening was swelling in the submandibular region which increased restricted (16 mm). Intraorally a diffuse, massive rapidly in size over a period of 3 days to its present size. erythematous swelling was appreciated in the floor of This was associated with dysphagia and difficult mouth causing it to raise the tongue (Figure 2). Swelling

How to cite this article: Popli G, Kansal R, Verma G. A Rare Case of Cystic Hygroma Resembling Ludwig’s Angina. Int J Oral Health Med Res 2016;2(5):84-87.

International Journal of Oral Health and Medical Research | ISSN 2395-7387 | JANUARY-FEBRUARY 2016 | VOL 2 | ISSUE 5 84

Popli G et al.: Cystic Hygroma Resembling Ludwig’s Angina CASE REPORT

cervical crease followed by dissection to free the pathology from the overlying platysma.Blunt dissection with thorough care was carried out to protect vital structures of the neck. Excised pathology measuring 4.5 x 3 x 1.2 cm was sent for histopathological evaluation which lead to the final diagnosis of Cystic Hygroma (Figure 4).

Figure 2: Raised floor of mouth depicting an extension above the mylohyoid muscle. was painful on palpation and soft to firm in consistency, and no associated secondary changes were observed. Hard tissue examination revealed infected root stumps i.r.t 36, 46 and proximal caries i.r.t 37. Pathology was provisionally diagnosed as Ludwig’s Angina with the differential being lymphangioma, inflammatory lymphadenopathy and cystic swellings of neck. Figure 4: Sample measuring 4.5 x 3 x 1.2 cm which was sent for Orthopantomogram revealed proximal caries i.r.t 35, histopathological evaluation. infected root stumps w.r.t. 36, 46 and with diffuse periapical radiolucency i.r.t 46 suggestive of chronic DISCUSSION periapical abscess (Figure 3). Cystic Hygroma mostly involves posterior triangle of the neck in infants or newborns.2 To date, there have been fewer than 200 reports of adult cervicofacial cystic hygroma in the English literature.4 Etiology is uncertain, although trauma, bleeding and upper respiratory tract infection have been suggested as possible triggers for onset.4 In this case, there was no identifiable relevant history related to trauma or respiratory infection. Lymphatic malformations can be divided into capillary, cavernous, cystic hygromas and mixed variety as per endothelial characteristics which determine flow within the lesion. However, they actually represent a single type of defect in lymphatic development manifesting different Figure 3: Orthopantomogram reveals proximal caries i.r.t 35, infected root stumps w.r.t. 36, 46 and with diffuse periapical radiolucency i.r.t 46. degrees of severity. Most accepted theory for the origin is failure of lymphatics to divulge with the venous system, On aspiration, haemorrhagic fluid was aspirated. (USG) unorthodox budding of the lymphatic structures, and showed a multilocular cystic lesion with fluid levels. sequestration of lymphatic tissue. Recently, the vascular Amongst the differentials, ranula and dermoid cysts were endothelial growth factor C gene has been found to be a ruled out, based on fluid on aspirate and USG findings. critical factor for normal lymphatic development.1 On Doppler, high vascular flow was not noted, so possibility of low flow lesion was suspected. Contrast Based on anatomical location, they have been classified Enhanced CT scan showed non homogenous mass of soft into 5 stages by De Serres et al.5 (Table-1). McGill and tissue density in the lower sublingual region with no Mulliken have also classified them into two types based evidence of bone involvement, which gave an impression on relation with mylohyoid muscle.6 But in our case, of benign non enhancing lesion of soft tissue mass. Soft lesion extended bilaterally and was suprahyoid type, tissue mass was multilobulated with smooth septa of which has not been classified by De Serres. Secondarily, variable thickness which enhances uniformly following lesion extended both above as well as below the bolus injection of contrast media administration. mylohyoid muscle leading to the floor of mouth to be Aspiration of the lesion, USG, colour Doppler and raised, which also misindicated our diagnosis towards Contrast enhanced CT transformed our provisional Ludwig’s Angina. Other factors which lead to diagnosis from Ludwig’s angina to lymphatic misdiagnosis were presence of infected root stumps of malformation. Excision was planned under General lower first molar on both sides indicating the odontogenic Anaesthesia. A 5cm extraoral incision was given in the etiology.

International Journal of Oral Health and Medical Research | ISSN 2395-7387 | JANUARY-FEBRUARY 2016 | VOL 2 | ISSUE 5 85

Popli G et al.: Cystic Hygroma Resembling Ludwig’s Angina CASE REPORT

Mostly it presents as a non tender mass that enlarges slowly and progressively in initial stages, and over long period remains constant.1 In our case, onset was sudden, or there was a rapid enlargement of already existing Figure 5: Histopathologically, numerous large and small cystic spaces unidentified pathology. containing homogenous eosinophilic colloid material. Numerous red Haemorrhagic aspiration guided us to get the USG and blood cells filled blood vessels and areas of extravasated RBCs were also seen in the connective tissue. Doppler done, which indicated the multilocular cystic lesion with fluid level, without any association of high found. Age of onset, unusual anatomical location, sudden vascular flow. Contrast CT hardly gives additional onset and progression, presence of infected root stumps, diagnostic information over, Doppler but precise raised floor of mouth and haemorrhagic aspirate, all of extension of the lesion and relation with adjacent the enumerated factors caused a difficult diagnosis. structures and vasculature can be acquired. None of the Authors believe that this unusual case report will add on relevant vasculature was associated with the lesion. to the academic literature and will also help in Moreover, the association of cystic hygromas and establishing an exact diagnosis and better treatment plan. vascular malformations is extremely rare.7

There are a number of contemporary treatments for CONCLUSION lymphatic malformations, varying from simple trouble It can be concluded that various radiological investigative free non-invasive methods to complex surgical approaches like USG, Colour Doppler and Contrast procedures.1 Management to be adopted should be enhanced CT have their individualized role for obtaining individualized and based on several aspects such as the a precise diagnosis in neck pathologies. Thus, a hyoid level, age of onset, progression, size of the lesion, sequential diagnostic approach should be emphasised and and its relation with vital structures. Lesions with severe none of the investigative procedure should be overlooked. life threatening functional impairment should be treated early. Confined lesions can be treated with surgery, laser REFERENCES therapy or sclerotherapy, usually with excellent results. However, an individualized treatment method should be 1. Ramashankar, Chandan Prabhakar, Nishit Kumar Shah, Girish Giraddi. Lymphatic malformations: A dilemma in based on the extent of the lesion, its association with vital 8 diagnosis and management. Contemporary Clinical structures, and expertise. Surgery, however, is a more Dentistry. 2014 Jan-Mar ; 5(1):119-122 2 definitive treatment modality. In the present case, lesion [DOI:10.4103/0976-237X.128689] was not closely associated with vital structures or major 2. G. H. Shah, M. D. Deshpande. Lymphatic Malformation in vessels and was causing severe morbidity, so surgery was Adult Patient: A Rare Case. J. Maxillofac. Oral Surg. 2010 advised as being a definitive modality. While for larger July-Sept ; 9(3):284–288 [DOI 10.1007/s12663-010-0082- cases encroaching vital structures or one that could cause z] severe postsurgical morbidity, other methods should be 3. Naresh Kumar, Munish Kohli, Saumya Pandey, S. P. S. given a chance. Tulsi. Cystic hygroma. Natl J Maxillofac Surg. 2010 Jan- Jun ; 1(1): 81-85 [DOI: 10.4103/0975-5950.69152] Histopathologically, lesion was showing numerous large 4. L. Gow, R. Gulati, A. Khan and F. Mihaimeed. Adult- and small cystic spaces containing homogenous onset cystic hygroma: a case report and review of eosinophilic colloid material. The connective tissue management. Grand Rounds 2011; 11 :5–11 Specialities: General Surgery [ DOI: 10.1102/1470-5206.2011.0002] stroma showed collagen fibres with focal blisters of 5. De Serres LM, Sie KC, Richardson MA. Lymphatic lymphocytes. Numerous red blood cells filled blood malformations of the head and neck: A proposal for vessels and areas of extravasated RBCs were also seen in staging. Arch Otolaryngol Head Neck Surg 1995; the connective tissue. These findings were suggestive of 121:577-82. [DOI: 1995.01890050065012] Cystic Hygroma (Figure 5). 6. McGill T, Mulliken J (1993) Vascular anomalies of the head and neck otolaryngology head and neck surgery. Recurrence rate is 10–38% and is directly related to the 2 Mosby-Year Book, Baltimore, pg 333–346. excisional surgery. On sequential follow up for 3 years, 7. Erini Makariou, Andreas Pikis, Earl H. Harley. Cystic no postoperative complication or recurrence has been Hygroma of the Neck: Association with a Growing Venous

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Popli G et al.: Cystic Hygroma Resembling Ludwig’s Angina CASE REPORT

Aneurysm. AJNR Am J Neuroradiol 2003 47: 1105–1109 [ doi:10.1016/j.oraloncology.2011.08.001] November/December; 24:2102–2104. 8. Qin Zhou , Jia Wei Zheng , Hua Ming Mai , Quan Feng Luo et al. Treatment guidelines of lymphatic Source of Support: Nil Conflict of Interest: Nil malformations of the head and neck. Oral Oncology 2011;

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