Venolymphatic malformation of tongue: a prompt life saving intervention

Abstract

Venolymphatic malformations (VLMs) of the head and neck can have varied clinical presentation with associated complications. Timely diagnosis and treatment is warranted to curb the morbidity and obtain satisfactory outcomes. We present the case of a male patient in his early 20s with VLM of the tongue who received precise interventions which were executed with active contributions from a team of anaesthesiologists, interventional radiologists, oral and maxillofacial surgeons and intensivists. We highlight the importance of an immediate, comprehensive and multimodal treatment approach for VLMs of the maxillofacial region.

Background

Venolymphatic malformations (VLMs) have remained a diagnostic and therapeutic challenge since time immemorial. The aetiology pertains to errors of endothelial morphogenesis which are almost always present at birth, nevertheless may not become apparent until puberty. The reported incidence is over 40% in the cervicofacial region commonly involving the tongue, buccal mucosa and orbit, with a few other cases reported to occur in the truncal extremities and abdomen.¹ The rich vascularity of the head and neck region ascertains a heedful management due to the dreaded complication of massive bleeding. In particular, lesions affecting the tongue mandate precise diagnosis and timely intervention as it can be life threatening due to both bleeding and mechanical airway obstruction. Here, we report a case of a patient in his early 20s with VLM of tongue who received prompt and effective treatment.

Case presentation

A male patient in his early 20s presented to the emergency department with a 2-day history of intermittent, small volume, spontaneous oral bleed, with no history of trauma or self-inflicted injuries. Previous treatment included suturing of lesion over the dorsum of tongue under local anaesthesia. The medical history of the patient was unremarkable. After initial stabilisation, the oral and maxillofacial surgery team assessed the patient which revealed the presence of a nodular, irregular growth with a pebbly surface measuring approximately 4 cm × 2.5 cm × 1.5 cm on the dorsal aspect of anterior two-thirds of the tongue (figure 1A). The lesion was non-tender, non-pulsatile, non-indurated, but actively bled on digital pressure. Haemostasis was attained primarily with local pressure and topical tranexamic acid.

Figure 1.

(A) Preoperative status of the lesion over the dorsum of the tongue, (B, C) T2 fat suppressed sagittal and coronal images showing T2 hyperintense cystic spaces, (D) selective right lingual artery angiogram showing vascular blush, (E) postembolisation run showing disappearance of vascular blush with paucity of vessels around the lesion, (F) intraoperative photograph after excision of the lesion, (G) clinical appearance at 6 months follow-up, (H, I) T2 fat suppressed sagittal and coronal images revealing no evidence of residual lesion with a small postoperative scar (arrow) at 6 months follow-up.

Investigations

Initial ultrasound of the lesion revealed multiple compressible vascular spaces in an echogenic background. Colour doppler images showed slow flow within a few cystic spaces. Patient was subsequently taken up for contrast enhanced MRI which displayed a well-defined multiloculated cystic lesion on the dorsal surface of the tongue which was mildly hyperintense on T1WI (T1 weighted image) and heterogeneously hyperintense on T2WI (T2 weighted image). Few tiny blooming foci were evident on gradient echo images suggestive of phleboliths. The lesion depicted patchy progressive venous phase enhancement on time resolved angiography (TRICKS - Time resolved imaging of contrast kinetics) and heterogeneous enhancement on postcontrast images consistent with venous component of the lesion. Few cystic spaces were unenhanced even on delayed phase with septal enhancement indicating towards a lymphatic component of the lesion (figure 1B, C). The coagulation profile and the other routine haematological investigations were within the normal range.

Differential Diagnosis

The prime differential diagnosis was the occurrence of an arteriovenous malformation (AVM). Nonetheless, they generally present as a pulsating mass with evidence of arterial flow in the cystic spaces on colour doppler images, flow voids on spin echo images in MRI and venous shunting in arterial phase in TRICKS images. Based on clinical and radiological findings, AVM was ruled out as a possible diagnosis.

Treatment

The patient was taken to the operatory and awake fibreoptic nasal intubation was performed, thus establishing a definitive airway. The patient was then shifted to digital subtraction angiography suite, where a diagnostic angiography was performed. Right ECA (external carotid artery) angiogram showed a few small abnormal contrast blush with progressive filling in the venous phase, without any arteriovenous shunting (figure 1D). Right lingual artery was superselectively cannulated with microcatheter, and thin gelfoam slurry was slowly injected to stop oozing from the tongue lesion, and there was a disappearance of blush noted as depicted in figure 1E. Postembolisation, there was no further oozing and patient was taken for surgical resection for definitive cure. Emergency embolisation also helped during surgical resection by creating bloodless field. Surgical excision was then performed ensuring complete removal of the lesion (figure 1F). Additional haemostasis was achieved with topical haemostatic agents and primary closure was done. The patient received ICU care and mechanical ventilation for 24 hours postoperatively, to maintain a secured airway to manage further episodes of bleeding if any. Postoperative recovery was uneventful and the patient was discharged on day 4 after receiving a 3-day course of intravenous antibiotics. Histopathological examination was consistent with the presence of fibromuscular and fibrocollagenous tissue with multiple thick-walled vessels lined with a single endothelial layer accompanied by both acute and chronic inflammatory cells composed of neutrophils, lymphocytes and plasma cells, thus confirming the diagnosis of VLM.

Outcome and Follow-up

There was no recurrence at 8 months follow-up clinically (figure 1G). MRI done at 6 months follow-up showed no evidence of residual lesion as shown in figure 1H, I.

Discussion

VLMs are rare, benign, congenital lesions which have an indistinct pattern of inheritance. However, somatic mutations of vascular growth factors have been ascribed to the development of these benign entities.^{2 3} They primarily comprise of low-flow lesions with a varied clinical presentation at different sites, either localised or diffuse, single or multiple. Patients present with either functional disability or pain secondary to mass effect on vital structures and rarely thrombosis.⁴ Specifically, its high preponderance in the maxillofacial region is associated with difficulty in speech, swallowing, complication of massive bleeding, airway compromise and aesthetic abnormalities.^{1 4} Ultrasound is the initial choice of imaging, to identify the type of vascular channels and also to exclude tumours. Contrast enhanced MRI with time resolved angiography is the prime imaging modality to confirm the type of vascular malformation.⁵ A variety of therapeutic options have been proposed in the literature ranging from lasers, percutaneous sclerosant therapy, transarterial superselective embolisation of vascular supply or surgical excision; with similar success rates of 70%–80%.^{6 7} Lasers are selectively absorbed by the intravascular proteins leading to coagulation and subsequent vascular damage and occlusion.⁸ Sclerotherapy comprises injecting an agent that obliterates the vascular channels by causing endothelial damage producing inflammation and fibrosis. However, such a modality of treatment remains confined to either small lesions or those which are inoperable due to aesthetic considerations or proximity to vital structures.⁸ Besides, VLMs of the tongue, floor of mouth or oropharynx entail interventions to prevent and manage upper airway obstruction. There has been much emphasis on securing a definitive airway prior to any intervention.⁹ Although sclerotherapy is an option, it is contraindicated in the acute condition involving the airway, as it has potential to cause temporary increase in the size of the lesion in postsclerotherapy period further aggravating the airway obstruction. On the other hand, embolisation effectively blocks the vascularity to the local site more instantaneously, thus curbing the possibility of lesion enlargement along with surgical haemostasis. More recently, pharmacotherapy with sirolimus which is an mTOR (mammalian/mechanistic target of rapamycin) pathway inhibitor has been proposed in the management of VLMs. However, long-term clinical trials evaluating its efficacy are still under progress.^{8 10}

Meagre studies have been reported in the literature with no consensus regarding the selection of a particular treatment modality. Singal and Bhatt¹¹ have reported successful resolution of an asymptomatic VLM of the tongue using two injections of 3% sodium tetradecyl sulfate sclerotherapy. De Souza et al¹² performed surgical excision of the lesion in the lateral border of the tongue with no reported bleeding complications. Likewise, Jian¹³ performed surgical excision to treat lymphangiomatous or lymphangiohaemangiomatous tongue lesions which resulted in improved cosmesis and function with no recurrences. Another technique of management of haemangiolymphangioma of tongue was reported by Vilalta and Mascaro¹⁴ which comprises transfixion suturing with resorbable polyglycolic sutures producing a superficial necrotic scar which eventually cicatrised.

In the present case, the increasing size of the lesion hinting towards a potential airway obstruction urged us to choose embolisation with surgery over the less invasive sclerotherapy. Moreover, lesions treated with sclerosants have a tendency to recanalise and present with enormous bleeding at a later date.¹⁵ We resorted to embolisation of the lingual artery with gelfoam, which aided during surgical excision of the actively bleeding lesion. More so, gelfoam was selected due to its resorbable nature with a recanalisation time of 2 weeks, thereby reducing the risk of establishment of collateral circulation.¹⁶ In a nutshell, the chosen treatment modality must be tailor-made according to the site of the lesion and the presenting problems be it life threatening, functional or aesthetic. A clinical follow-up of 1–3 months and a radiological follow-up with MRI at 6 months are recommended to assess for regression or recurrence.¹⁷

Patient’s perspective.

I was very worried with a growth over my tongue as it was hindering with my speech. Once it started bleeding profusely and increasing in size, it became a serious matter of concern. After I underwent surgery, I felt much improvement and comfort with my day-to-day activities. I would like to express my gratitude towards the entire healthcare team for their swift actions.

Learning points.

• Venolymphatic malformations (VLMs) of the head and neck region particularly the tongue, floor of mouth and oropharynx can cause airway obstruction and present with active bleeding.

• A wide range of treatment modalities including sclerotherapy, laser excision, surgery and more recently pharmacotherapy have been reported.

• Such cases presenting with active bleeding and impending airway compromise warrant prompt action.

• A multimodality approach with appropriate planning including anaesthesiologists, interventional radiologists, maxillofacial surgeons and intensivists can improve the outcomes.

Ethics statements

Patient consent for publication

Consent obtained directly from patient(s).