Abstract
To evaluate the clinical perspective of Veno lymphatic malformation and definitive management in respect to outcome. (1) To discuss clinical presentation, symptomatology of Veno lymphatic malformation. (2) Demonstration of radiological features, diagnosis and management of Veno lymphatic malformation with its complication. This prospective study was conducted on four patients attending ENT-OPD of R.G.Kar Medical College, Kolkata, India who had presented with suspected vascular malformation. The study was conducted from March 2021 to March 2023 for a period of 2 years. The patients were subjected to detailed history and examination. The diagnosis of the Veno lymphatic malformation was based on the results of Doppler ultrasonography, computed tomography and magnetic resonance imaging. In our study there was male predominance. Most of the patients belonged to the 2nd and 3rd decade of life. The main sites of involvement were lateral neck followed by parotid region. The lesion size ranged in between 3.5 × 3.5 cm and 7 × 5 cm. The patient with parotid lesion was found to have phlebolith. Since most of the lesions were small with well-defined margins, we were able to excise the lesions completely without leaving any residue. Out of four cases one patient developed temporary paresis of spinal accessory nerve which resolved eventually. Veno lymphatic malformations are rare and there is no definitive protocol for management and to be individualized. Our study will be helpful for furthering the existing knowledge regarding the management of Veno lymphatic lesion emphasizing the need of multimodality approach in surgical decision making.
Keywords: Veno lymphatic malformation, Doppler ultrasonography, Head and neck, MRI
Introduction
Congenital vascular malformations are a group of developmental defects involving one or more compartments of vascular system-arterial, lymphatic and venous.
They are present most commonly at birth arising at various stages of embryogenesis. Usually, they don't undergo spontaneous involution. Inciting factors such as trauma, puberty and pregnancy can cause accelerated growth. Vascular malformations are defined as the disorganized vascular development with dysplastic cell growth. They are subclassified on the basis of anatomy of blood vessels. They are as follows:
Type 1-Simple vascular malformation and
Type 2-Combined vascular malformations.
Combined vascular malformations is constituted by the malformation of more than one type of network of vessels within a single lesion (capillary, venous and venolymphatic). Vascular malformations again to be subdivided into high flow and low flow lesions. Venous, lymphatic and venolymphatic malformation are considered to be low flow lesions.
Lymphovascular malformations (LVM), also known as Hemangiolymphomas, are rare. Congenital slow flow combined vascular malformations affecting mainly Head and neck regions (50%) and account for 12% of all vascular anomalies affecting 1 out of 2000–4000 individuals. Although present at birth, they can be diagnosed antenatally by ultrasound (USG). LVM’s can be associated with Klippel-Trenaunay syndrome and Noonan syndrome. Lesions typically present as painless, soft, cystic compressible swelling and may be associated with the bluish discolouration of skin suggestive of skin changes. Investigations include USG with colour doppler, MRI, CT scan (required if a phlebolith is suspected). US-guided FNAC helps confirming the diagnosis. Management is mainly sclerotherapy for early and small lesions and surgical for advanced lesions. Lasers can be used as an adjuvant. Combinations of different modalities are required. Here we are going to discuss about the clinical presentation, radiographic pictures and other investigation modalities and treatment modalities of venolymphatic malformations (LVM).
Case 1
A 21-year-old female presented to our hospital with painless mass over the left side of neck for the past 4 years gradually increasing in size. Examination revealed mass was 2.5 cm × 3.5 cm, located in left supra-clavicular region, soft, cystic, compressible, mobile, non-tender, without any skin changes (Fig. 1).
Fig. 1.
Clinical Photograph of the patient showing a left sided neck mass
Patient underwent Doppler-USG(D-USG) and MRI. USG features are suggestive of combined vascular malformation with venous component and few “flow-void” cystic areas denoting lymphatic areas. In MRI the lesion was found to be a 3 cm × 3.5 cm hyperintense multilobulated lesion in T1 weighted images in left supraclavicular region with a clearly defined margin with a feasibility of achieving complete surgical resection (Figs. 2 and 3).
Fig. 2.
T1W MR in Axial view shows multilobulated hyperintense mass lesion posterior and deep to left Sternomastoid muscle (SCM)
Fig. 3.
T1WI Coronal view shows mutilobulated hyperintense mass lesion in left Supraclavicular region
As Imaging suggested, intraoperatively, the mass was delineated properly with a clear margin and complete resection was achieved without leaving a residual mass via lateral trans cervical approach (Figs. 4, 5 and 6).
Fig. 4.
Intraoperative photograph depicting the surface landmark of the left sided mass lesion and incision marking over the lesion
Fig. 5.
Intraoperative photograph showing the post triangle of neck with Spinal accessory nerve in view traversing through SCM to Trapezius muscle
Fig. 6.
Lymphovascular malformation deep in the posterior triangle
Sample: Macroscopically, the mass was 3 cm × 3.5 cm, multilobulated, soft, cystic, filled with yellowish fluid. Sample was sent for Histopathological examination (HPE). HPE was suggestive of combined vascular malformation with lymphatic and vascular components.
Post operatively there was temporary spinal accessory nerve paresis was encountered which resolved over a period of 3 months.
Case 2
A 16-year-old male presented to our hospital with painless mass over the left side of the neck for the past 3 years, gradually increasing in size. Examination reveals mass to be 4.5 × 3.5 cm, located in the left supraclavicular region, soft compressible mobile, non-tender, smooth, well demarcated without any skin changes (Fig. 7).
Fig. 7.
Clinical photograph of Left supraclavicular lymphovascular malformation
Patient underwent D-USG & MRI of neck. D-USG features suggestive of combined vascular malformation with venous component and few “flow-void” lymphatic areas.
In MRI lesion was found to be 5 × 4.5 cm hyperintense lesion in T1 weighted image in left supraclavicular region with a clearly defined margin (Figs. 8 and 9).
Fig. 8.
T1W MRI coronal sections shows Hyperintense mass lesion in left Supraclavicular region
Fig. 9.
T1W MRI Axial section shows hypeintense lesion wedged in between SCM and Trapezius muscle
Intraoperatively, the mass was delineated properly with a clear margin and complete resection was achieved via Trans cervical approach.
Sample: Macroscopically, the mass was 5 × 4.5 cm soft, lobulated, cystic, filled with straw coloured fluid (Fig. 10). Sample was sent for HPE. HPE suggestive of venolymphatic malformation.
Fig. 10.
Photograph of Resected Lymphovascular lesion
Immediate Post op period was uneventful without any morbidity to the patient.
Case 3
A 17-year-old male presented to our hospital with mass over the right sub mandibular region for the past 2 years gradually increasing in size. Examination revealed mass was 6×4.5 cm, located in the left submandibular region, soft, cystic, compressible, mobile, non-tender, without any skin changes.
Patient underwent D-USG and MRI. D-USG features suggestive of combined vascular malformation. Non-contrast MRI of neck suggest the lesion to be 6*4.5cm hyperintense multilobulated lesion in T1 weighted images in the right submandibular region with a clearly defined margin and separate from the right submandibular gland. CT scan of Base of skull to root of neck showed well defined vascular lesion in right submandibular region with post-contrast enhancement with a hyper attenuated rounded mass suggestive of phlebolith.
Surgical resection via trans cervical approach was performed and mass was delivered in toto (Figs 11 and 12).
Fig. 11.
Intraoperative Photograph showing a blusih globular lesion just anterior to right SCM with Great Auricular nerve posterosuperior to the lesion
Fig. 12.
Intraoperative photograph of Delivering the lesion in to the Wound
Sample: Macroscopically, the mass was 6×4.5 cm, soft, cystic. Cut section shows a whitish “bony hard” phlebolith (Fig 13). Sample was sent for HPE. HPE was suggestive of combined vascular malformation with lymphatic and venous components (Figs 14 and 15).
Fig. 13.
Resected specimen shows Phlebolith inside the specimen
Fig. 14.
10X Photomicrograph; H & E stain
Fig. 15.
40X Photomicrograph; H & E Stain
Post operative period was uneventful.
Case 4
A 45-year-old male presented to our hospital with the mass over the right parotid region for the past 8 years gradually increasing in size. Examination revealed mass was 7 × 5 cm, located in the right parotid region with deep lobe involvement, soft, cystic, compressible, mobile, non-tender, without associated skin changes.
Patient underwent D-USG and MRI. D-USG features suggestive of combined vascular malformation with venous components and lymphatic areas.
In MRI the lesion was found to be a 7 × 5 cm hyperintense lobulated lesion in T1 weighted image mass arising from superficial lobe extending up to deep lobe (Figs. 16, 17 and 18).
Fig. 16.
T1WI axial section shows multilobulated lesion in the Right side of the neck deep to and posterior to SCM with a part of it extending deep to Trapezius
Fig. 17.
T1WI Parasagittal reformated images show multilobulated hyperintense lesion
Fig. 18.
T1W MR in sagittal reformatting shows hyperintense multilobulated mass in the parotid region
The mass was delineated properly with a clear margin and complete resection was then achieved via total conservative parotidectomy and all the branches of facial nerve were preserved.
Sample: macroscopically, the mass was 7 × 5 cm, multilobulated, soft, cystic, filled with yellowish straw coloured fluid (Figs. 19 and 20). Sample was sent for HPE. HPE suggestive of combined vascular malformation with venolymphatic malformation.
Fig. 19.
Intraoperative photograph following excision of specimen by Total Conservative Parotidectomy
Fig. 20.
Photograph shows multilobulated LVM
Post operatively there was temporary marginal mandibular nerve paresis was encountered which resolved over a period of 3 months with conservative treatment.
Result Analysis
Clinical Features
A total of 4 patients with venolymphatic malformation were included in our study. Among them M: F ratio was (3:1) respectively, showing male predominance. Most of the patients belongs to 2nd and 3rd decade of life.
In our study all of the patients seem to have a similar clinical feature like a painless swelling, mobile, non-tender, slowly increasing in size, but for one patient on palpation we found a hard substance not attached to the tissue suggestive of a phlebolith.
All the patients had first sought treatment in our institute and their average follow up was 2 years.
Type, Distribution and Location
Type, distribution and location of the treatment were evaluated through physical examination imaging tests in patients in whom venolymphatic malformation was suspected. The lesion size ranged in between 3.5 cm×3.5 cm to 7 cm×5 cm in most of the patients, the margins were well defined. Since the sample size was small the side distribution was equal with two patients had the mass in the left side and two patients in the right side. Altogether the lower neck region was most affected. The most common location was the neck followed by parotid region.
Based on depth and level of invasion, most commonly was skin, muscle layer and mucosa layer. Each patient diagnosis was confirmed through imaging test and biopsy mostly after a physical examination and history taking in patients suspected to have a vascular malformation. Imaging tests were performed with D-USG, CT and MRI. CT was performed only to corroborate the size, relationships with structures in vicinity for surgical accessibility and in presence of a phlebolith.
Treatment
Since sclerotherapy is not available in our institute all of our patients underwent surgery due to cosmetic issue. Complete resection was performed for small to moderate sized venolymphatic malformations that did not invade the vital structures. The surgery was performed with due consideration of post operative complications. There were some post operative complications which were treated conservatively and showed improvement with due course of time.
Discussion
A detailed history and clinical examination is important for diagnosis of venolymphatic malformation (LVM). An important examination finding of LVM is their sensitivity to compression from sources such as finger, which causes them to change size and they may also enlarge due to Valsalva maneuver or straining. LVMs can be associated with pain or without pain and can occur anywhere in the body especially in Head–Neck region. The pain or size will increase due to hormonal changes like puberty, menstruation and pregnancy [1–3].
On MRI these malformations are identified as high signal intensity lesions on T2 enhancement and low signal intensity on T1 enhancement. MRI is used to identify the depth of invasion, distribution, location of lesion, size and margin there by contributing to decision of treatment plans. CT can be used to observe phleboliths, which are characteristics of LVMs which we have seen in our case.
D-USG is a cost effective primary diagnostic tool that can observe low flow, low shunt vascular dynamics and they appear as hypo echoic mass on D- USG. It has disadvantage that it cannot precisely observe deep located lesions [4]. LVMs are classified as sporadic or inherited. Sporadic are mostly unifocal whereas inherited often manifest as multifocal [5].
Treatment modalities for LVMs laser therapy, sclerotherapy for smaller and surgically inaccessible lesions, and surgery. As difficulties are encountered in curing all LVMs in the same way, the treatment plan should be determined considering the extent and clinical symptoms of the LVM, the patient’s age and the availability in the institute
Sclerotherapy is a nonsurgical form of management. Injection sclerotherapy using STS foam has been reported to be a safe and effective treatment option, and percutaneous treatment with bleomycin foam has also been reported to be effective. However, after sclerotherapy, skin blistering, skin necrosis, ulceration, scarring, and airway obstruction due to swelling may occur. It also depends on availability in the institute [1, 6–9].
Laser treatment has been reported to remove the bluish discoloration and to reduce the size of the lesion after Nd:YAG laser treatment for superficial LVMs. Other studies have reported excellent treatment results for pain and bleeding control, as well as reductions in lesion size after long-pulse Nd:YAG laser therapy for LVMs in the lip and oral mucosa [10].
For larger lesions when clinic-pathological assessment reveals lesions resectability, surgical excision is the gold standard of treatment.
A unified treatment strategy has not been determined. Therefore, the treatment plan should be made after considering all the patients' symptoms, the location of the lesion, and its depth, size, type, and sequelae.
A multidisciplinary approach is needed for the diagnosis and treatment of LVMs.
Considering the high recurrence rate of LVMs and the difficulty of radical cure, the outcomes of this study were positive. However, the lack of family history and genetic studies means that the relationship of treatment results with a genetic diagnosis could not be determined.
Although there is no standard protocol form LVMs. In our study we have made a proper diagnosis with a help of pathologist and radiologist. All characteristic important radiological findings are important for subsequent appropriate treatment. Due to lack of sclerotherapy in our institute, with consent of all the patient we underwent surgery for complete removal and also for cosmetic purpose. Our study will be helpful for the management of LVMs in different location and different size through appropriate treatment. Regular follow up is very important to ensure that there is no recurrence.
Funding
This study received no specific grant from any funding agency, public or not for profit sectors.
Declarations
Conflict of interest
The authors declare that they have no conflict of interests.
Ethical Approval
The research was conducted in adherence to Declaration of Helsinski. Ethical approval for this study was obtained from Institutional Ethics Committee, R.G. Kar Medical College; Regn no – ECR/322/Inst/WB/2013, memo no – RKC/303.
Informed Consent
Informed consent was taken prior to inclusion of patients into the study, during data collection and before surgical procedure. Informed consent was also taken for publication of clinical photographs and radiographic and surgical images included herein.
Footnotes
Publisher's Note
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