Paediatric Head and Neck Hemangiomas: Scopes, Challenges and Outcome of Surgical Management

Abstract

Haemangiomas are benign vascular tumours frequently encountered in head and neck region. The different systems of nomenclature leading to terminological confusion and management protocols for vascular lesions have been contentious issues and have undergone substantial paradigm shift over the years. A wait and watch policy has been the mainstay of treatment owing to spontaneous regression of majority of paediatric hemangiomas along with sclerotherapy, laser therapy, drug therapy and radiotherapy. However, in carefully selected cases, surgical excision; alone or in a combined approach, should be adopted as treatment of choice. Cases of paediatric vascular lesions were diagnosed and appropriately classified as per the recent guidelines of The International Society for the Study of Vascular Anomalies (ISSVA). The cases of haemangiomas were identified and individualized treatment protocols were adopted for every case in a multispecialty team approach. Patients deemed as suitable candidates for surgery were undertaken for excision and the outcome was assessed on the indices of colour, size, aesthetics and patient opinion regarding the outcome. A total of 39 patients with multiple head and neck hemangioma lesions were included, with a female preponderance and mean age of the presentation at 8 years. The most common sites were the buccal mucosa, palate, tongue, and angle of mandible. Among the cases, 12.8% were observed without treatment, 12.8% were treated with sclerotherapy, 51.28% underwent surgery, and 23% received a combined treatment approach with regular follow up. The therapeutic response and patient satisfaction were significantly higher in group with surgical intervention. An individualised, comprehensive and holistic approach towards management of paediatric haemangioma based on appropriate classification system gives optimum treatment outcomes. Proper counselling, reassurance and timely intervention is necessary to preserve cosmetic aesthetics as well as fragile child psychology in this impressionable age group. We feel that surgical approach has been substantially underutilized as preferred treatment modality and may occupy a greater space in paediatric haemangioma treatment in future.

Introduction

Vascular anomalies consist of a heterogenous group of congenital lesions of abnormal vascular development. These vascular anomalies of the head and neck region constitute approximately 60% of vascular anomalies diagnosed in children [1]. The field of paediatric vascular anomalies has been complicated due to varied classification systems in prevalence in the past. However, with the adoption of recommendations of International Society for the Study of Vascular Anomalies (ISSVA) [2], these anomalies have now been classified into two broad categories; vascular tumours (including haemangiomas) and vascular malformations (Table 1). Haemangiomas are the commonest vascular tumours prevalent in paediatric population. The treatment protocols for haemangioma has undergone a significant paradigm shift over the years as a variety of management options are available. The change from the customary wait and watch policy [3] for spontaneous regression to more aggressive treatment methodology is quite evident. There are scopes for controversies and non standardized practice persists. In carefully selected patients, surgical excision; alone or in a combined approach, gives excellent long-term results. The effect of a hemangioma on the physical and psychological development of a child is immense. Hence, it is imperative that an individualized patient-based approach with proper counseling regarding treatment option available is adopted.

Table 1.

Classification of vascular anomalies

Vascular tumors	Vascular malformation
Infantile hemangioma	Slow flow
Congenital hemangioma	Capilliary malformations
Tufted angioma	Venous malformations
Kaposiform hemagioepithelioma	Lymphatic malformations
Spindle cell hemangioendothelioma	Fast flow
Dermatologically acquired vascular tumours	Aeteriovenous malformations
Other rare hemangioendothelioma	Complex combined vascular malformations

We attempt to discuss and critically scrutinize the management protocol of paediatric hemangiomas from a surgical perspective in the light of varied presentations, diagnostic profiles, treatment preferences and patients’ expectation in an evidence-based manner.

Material and Method

This prospective interventional study on hemangiomas of head and neck region was conducted at an urban tertiary health care centre in India over a period of 3 years. Paediatric population presenting with vascular lesions in head and neck region were clinically evaluated and suitable diagnostic tests were performed to segregate the vascular lesions as per the International Society for the Study of Vascular Anomalies (ISSVA) recommendations. The diagnostic test battery involved colour Doppler ultrasonography, contrast enhanced Computed Tomography (CT) / Magnetic Resonance Imaging (MRI) along with detailed hematological work up for features of thrombocytopenia. Non hemangiomatous vascular lesions such as vascular malformations were not included in the study. Detailed counseling regarding the nature of lesion, stages of hemangioma, treatment options and possible complications of each treatment modality was explained to parents and their expectations regarding outcome of treatment was gauged by the team of care providers. A meticulous note of any treatment previously adopted such as propranolol [4] or corticosteroids [5] drug therapy, laser therapy [6], cryotherapy was made in detailed form.

Based on this comprehensive evaluation, an individualized treatment plan was charted out for each patient in a multidisciplinary team approach. Patients with involuting, small, stable, inconspicuous lesions in a non vital site were kept in an observational phase with explanations regarding warning signs for future intervention. Paediatricians were involved for cases deemed suitable for propranolol therapy under observation at a dosage of 2 mg per kilogram per day for 6 to 8 months with discontinuation after tapering at 12 months unless early resolution occurs [7]. Corticosteroids or anti cancer drugs were not included in institutional protocols for drug therapy of hemangiomas. Dermatological intervention for laser therapy was undertaken for early, small and superficial hemangiomas. The progression of therapy for every hemangioma case was maintained in institutional register for future reference.

Surgical intervention was taken up for carefully selected cases of hemangioma by the department of Head and Neck Surgery. The criteria for surgical intervention followed in our study included the following presentations of paediatric hemangiomas (Table 2).

Table 2.

Inclusion criteriae for surgical management

a) Non involuting hemangiomas after 5 years of observational policy

b) Failure/ Contraindication of propranolol drug therapy

c) Involvement of vital facial structures, eg. Eyelids, nose, lips, auricle

c) Involvement of vital facial structures, eg. Eyelids, nose, lips, auricle

d) Functional problems such as dysfunction of vision, feeding, breathing, swallowing

e) Recurrent bleeding, ulceration, infection of hemangioma site

d) Involvement of vital organs and structures such as salivary glands, neck vessels, muscular hemangiomas

e) Residual lesions after spontaneous regression, laser or cryotherapy

f) Rapid growth in size of hemangioma

g) Deteoriating psychological staus of the child

h) Associated with Kasabach Merritt syndrome [8]

Based on the above-mentioned inclusion criteria for surgical intervention, cases were selected and documented consent was taken. The treatment included in our study comprised of the following interventions:

1. Observation (T1): Patients aged below 7 years were placed under observation for potential self-regression. They underwent monthly follow-up appointments and were advised to promptly report any significant increase in size.

2. Intralesional injection of sclerosing agent (T2) [9]: Patients presenting with mucosal or superficial small lesions (< 3 cm²) were selected and were injected with polidocanol (3%) using an insulin syringe after giving topical anaesthesia. Patient was followed after 15 days and according to the response, the next sitting was scheduled. The number of sessions depended on the site and response of the lesion.

3. Surgical excision of the lesion (T3): Well circumscribed lesions at accessible sites were posted for total surgical excision under general anaesthesia after detailed pre anaesthetic evaluation with arrangements for cross matched blood.

4. Combined approach (T4): Few patients presenting with non-circumscribed mass with extensive distribution or multifocal presentations were first given intralesional injection of polidocanol in multiple sessions which made the lesion discrete and comparatively avascular followed by surgical excision. Post surgical polidocanal sclerotherapy was also given in unresectable lesions or sporadic recurrence after surgery.

The entire details of the patients undergoing surgical treatment, presentation on arrival, associated morbidities, type of hemangioma, structures involved, surgical approach adopted, challenges and outcome of treatment were meticulously collated on pre structured forms and statistically tabulated using appropriate software. Continuous data was presented as mean/standard deviation while categorical data was evaluated using Chi-square test with a P value ≤ 0.05 regarded as statistically significant.

We adopted a scoring system to statistically interpret results based on the following criteria: a) Colour (b) Size (c) Patient’s/parents ‘opinion.

The grading of improvement was scored as:

• No change: 0.

• Partial improvement: 1.

• Complete improvement: 2.

Results

During the study period, 39 patients with a total of 54 hemangioma sites in the head and neck region were identified. All medical records were retrieved and subsequently followed up at our centre. The median age at the time of presentation was about 8 years (0–18 years), and the female/male ratio (28/11) was 2.54. Among these cases, 33.3% were under 5 years of age (Graph 1).

Graph 1.

Distribution of patients according to their age of presentation

All cases included in the study exclusively involved head and neck hemangiomas. The majority of it was observed in the buccal mucosa, followed by the palate, tongue, and angle of the mandible within the head and neck region (Graph 2).

Graph 2.

Distribution of patients by location of lesion

46% of the lesion were small (< 3cm²) of which few with small, stable lesions at a non-vital site were kept under observation and few were injected with polidocanol (3%). The average follow-up period for them was every 15 days initially. Figure 1 shows an intramuscular hemangioma of masseter muscle and Fig. 2 shows pre and post-op images of submandibular hemangioma of the submandibular gland.

Fig. 1.

Intramuscular Hemangioma in the Masseter Muscle

Fig. 2.

Pre-operative and post-operative image of a left submandibular hemangioma

Individualized treatment plans based on the characteristics of lesion were developed for all 39 patients. 5 patients (12.8%) were placed under observation(T1), while 5 (12.8%) underwent sclerotherapy(T2). 9 (23%) received treatment through a combined approach(T4). There was an indication for surgical(T3) treatment in 51.28% (20 patients) meeting the inclusion criteria as depicted in Table 2. Parents and professionals evaluated the improvement based on visual assessments. Additionally, a thorough physical examination, serial photo documentation, and measuring of superficial skin haemangiomas were used to conduct an assessment. If indicated, a radiological evaluation was conducted for both mixed and deep haemangiomas. The effectiveness of each treatment method was evaluated based on four criteria: resolution of colour, involution in size, cosmetic improvement and most significantly, the patient’s opinion. Each of these criteria was further graded on a scale of 0, 1, or 2, with the scores representing the level of improvement in each area (Graph 3). The surgical intervention consistently attained the highest mean score across all four outcome grading criteria, unequivocally indicating its superiority over alternative treatment strategies (Graph 4). Regular follow-ups were conducted for the patients and no complications have been documented to date.

Graph 3.

Mean scores obtained in 3 different criteria used for scoring the outcome in different treatment plans

Graph 4.

Mean of the total score obtained based on 3 criteria in different treatment plans

There was a statistically significant difference between the groups as demonstrated by The Kruskal- Wallis Test which does not make any assumptions about the data’s parameters, such as it’s mean or variance (The f ratio value is = 6.4823. The p value is 0.001321. The result is significant at p <.05) (Table 3). A Tukey post hoc test showed that the T3 (Surgery) group had a statistically significant better outcome score than the T1(Observation) group (p =.00690). (Table 4)

Table 3.

Descriptives and Kruskal Wallis test results

Descriptives
Treatment groups	N	∑X	Mean	∑X2	Std.Dev
1	5	10	2	26	1.2247
2	5	12	2.4	32	0.8994
3	20	83	4.15	373	1.2258
4	9	34	3.7778	138	1.0929
Total	39	139	3.564	569	1.3916
Kruskal Wallis test
Source	Sum of squares	Df	Mean square
Between treatment	26.2842	3	8.7614	F = 6.4823
Within treatment	47.3056	35	1.3516
Total	73.5897	38

Table 4.

Comparison between different treatment groups

Pairwise comparisons		HSD.05=1.6607 HSD.01=2.0636	Q.05=3.8140 Q.01=4.7393
T1:T2	M1 = 2.00 M2 = 2.40	0.40	Q = 0.92(p =.91499)
T1:T3	M1 = 2.00 M3 = 4.15	2.15	Q = 4.94(p =.00690)
T1:T4	M1 = 2.00 M4 = 3.78	1.78	Q = 4.08(p =.03205)
T2:T3	M2 = 2.40 M3 = 4.15	1.75	Q = 4.02(p =.03569)
T2:T4	M2 = 2.40 M4 = 3.78	1.38	Q = 3.16(p =.13305)
T3:T4	M3 = 4.15 M4 = 3.78	0.37	Q = 0.85(p =.92998)

Discussion

Hemangiomas are the commonest benign vascular tumours in the paediatric age group and most of them (60%) are encountered in the head and neck region [9]. They are histologically described as collection of proliferating immature endothelial cells and disorganized vascular channels. Hemangiomas belong to a group of lesions clubbed together as vascular anomalies which consist of a variety of lesion of distinct pathological characteristics. The terminology, nomenclature and classification system of vascular anomalies were a source of widespread uncertainty till Mulliken and Glowacki in 1982 developed a practical classification system based histological classification, histochemistry and clinical behaviour [10]. This classification was further modified and into two broad categories; vascular tumours and vascular malformations, by recommendation of International Society for the Study of Vascular Anomalies (ISSVA). Hemangiomas are the commonest type of vascular tumour. On the basis of presentation and natural progression, hemangiomas are further divided into two subtypes; (a) congenital hemangioma (CH): present since birth and categorized into rapidly involuting (RICH) and non involuting (NICH), and (b) infantile hemangioma (IH): commoner, appears after 2 months of birth. The natural progression of hemangioma influences the treatment decisions adopted for management. The general scenario is of the commoner infantile hemangioma to proliferate and grow rapidly, gradually stabilize and then involute. Congenital hemangioma has a variable progression and NICH subtype is known for non-regression and continuous growth proportionate to the growth of the child. Generally, involution of hemangiomas tends to begin at around one year of age and continues for 5–7 years [11] and about 70% of hemangiomas involute by 7 years of age.

The management protocols for hemangiomas have undergone significant changes over the years in the light of better understanding of the lesions, newer scientific rationale, changing patient aspirations and a multidisciplinary approach to treatment. Reassurance with regular observation, drug therapy, laser therapy, sclerotherapy, cryotherapy and surgical excision; have all been used in different clinical presentations and treatment setting around the globe. Historically, “wait and watch” policy of regular observation was based on the premise that most of these lesions will undergo spontaneous regression. It is widely known now, in the light of better understanding of hemangiomas that a considerable number of lesions will not disappear spontaneously or completely. Today, observation as a sole mode of treatment should be offered only for small, stable lesion at a non-vital site. The role of cryotherapy has been limited due to complications of cold urticaria, cryoprecipitate fibrinogen and cryoglobulinemia, proliferative or atrophic scars, hyperpigmentation or hypopigmentation, milia and tissue contracture [12]. Laser therapy has been used predominantly for early, cutaneous lesions and its role in deeper lesion is not established. The use of anticancer drugs [13], radiation and radioisotope therapy [14], immunomodulators, interferons etc. have never been the preferred modalities of hemangioma management.

The discussion about management of paediatric hemangioma would be incomplete without mentioning propranolol, a non-selective β1 and β2 antagonist which is widely accepted as current treatment of choice for infantile hemangiomas [15]. The mechanism of action has been attributed to vasoconstriction, angiogenesis inhibition, apoptosis and regulation of rennin angiotensin system [16]. Oral propranolol in a dosage of 2–3 mg per kilogram body weight along with or after feeding unless there are comorbidities or adverse effects has superseded all other treatment modalities as a first line of treatment across all parameters. However, there are conditions that must draw careful considerations and even potential exclusions from propranolol therapy in view of side effects of propranolol. Infants less than 5 weeks of age, potential evidence of cardiogenic shock or heart failure, sinus bradycardia, heart block greater than first degree, known or suspected PHACES syndrome (posterior fossa brain malformations, hemangiomas of the face, arterial cerebrovascular anomalies, cardiovascular anomalies, eye anomalies, and sternal defects or supraumbilical raphe) [17], including presence or risk of coarctation of the aorta, cerebrovascular anomalies, asthma or reactive airway disease and hypersensitivity to propranolol are some of the conditions which might necessitate alternative therapy to propranolol administration. Oral steroids used to be the preferred treatment modality before serendipitous discovery and universal clinical acceptance of propranolol, and maybe used if there are contraindications or an inadequate response to oral propranolol. However, a careful evaluation of risk benefit ratio must be undertaken before initiation of treatment [18].

Surgical intervention has been a rather underutilized treatment option over the years in the management of paediatric head and neck hemangiomas. While the shift from watchful observation to corticosteroid therapy to now widely accepted propranolol therapy has gradually occurred over past decades, surgical management has occupied a relatively peripheral role on the sidelines of head and neck hemangioma management protocols. A plausible reason for this could be attributed to the counseling offered to the parents at the time of presentation and it is not unusual to find parents who were relatively uninformed regarding the surgical option till much later in the course of treatment. However, with the advent of a multidisciplinary team approach, specialized hemangioma surgical units, growing patients’ expectation regarding final outcome aided by easier availability of information from various sources has evoked a greater interest in surgical options. The perils of surgical interventions in infancy like anaesthetic risks, blood lost from a vascular tumour, iatrogenic complications have become relatively insignificant due to modern technological advancements and improved dexterity in the domain of paediatric, plastic and vascular surgeries. The list of indications for surgical intervention in head and neck hemangiomas has become longer. In carefully selected cases, early surgical excision should be offered as first line of treatment.

There are a number of variables and indicators which are considered before deciding the appropriate treatment modality for any pathological lesion in the human body. There are multiple factors associated with paediatric head and neck hemangiomas too, that suggest the strategy of surgical treatment should be pursued aggressively. Probably the most vexing aspect of hemangiomas is to predict when the lesion will involute. The previously accepted adage that 50% of infantile hemangiomas will complete involution by five years of age, 70% by seven years of age, and 90% by nine years of age have been contradicted in multiple publications [19]. The rate of progression of a hemangioma cannot be predicted with certainty despite an extensive study of these lesions [20]. It is not uncommon for a small macule proliferate at an alarming rate causing distress to the caregivers and not being offered the option of surgical removal of a manageable lesion may be interpreted as a failure of appropriate counseling by the family members. It is also well known that permanent skin changes and cosmetic abnormalities are anticipated in a significant proportion of cases, even after complete involution [21] and may necessitate additional surgery and scar management. This may be perplexing to parents that they have waited many years for a lesion to proliferate and resolve only to have a surgery that they were not offered earlier when the lesion was easily manageable surgically. The effects of a hemagiomatous lesion on the psychological status of a growing child need to be assessed carefully, especially in a school going age. There is ample literature to suggest that such lesion can lead to considerable psychological grief to the children [22]. Performing surgery at an earlier age can be beneficial in minimizing stigma and impact on the self esteem of a growing child [23]. An effort is made in all congenital deformities to resolve the deformity; surgically or otherwise, prior to the child entering school. A similar approach to paediatric head and neck hemangiomas would ensure a holistic management protocol for these patients.

Historically, the role of surgery in head and neck hemangiomas have been limited to small localized lesions or remnants after primary therapy, anatomically favorable site specific lesions and bleeding or ulcerations at the site of lesion. The authors have endeavored to expand the scope of surgical treatment and have listed the indications as inclusion criteria for this study (Table 2). Hemangiomas in areas where a life threatening complication, significant cosmetic defect or functional defect might ensue should have surgical excision considered as first-line treatment. Subglottic hemangiomas may need surgical excision in cases where a severe obstruction is noted in airway which may require a lengthy intubation or remain symptomatic despite medical therapy. Periorbital hemangioma causing obstruction of the visual axis needs urgent multidisciplinary management as stimulus deprivation in early years of life may cause long lasting complications. This is a strong argument for intervening surgically when periorbital lesions are much smaller and more manageable. Obstruction to external auditory canal by hemangioma may occasionally result in conducting hearing loss with the secondary risk of speech delay [24] which demands active intervention, particularly for rare bilateral lesions. Nasal tip hemangiomas, commonly referred as “Cyrano Nose” occurs on the focal point of the face. These lesions are slow to regress, may cause contour deformities from the fibrofatty tissues and excess skin even after complete resolution resulting in deformation of the alar cartilages [25]. Early surgery to remove the affected tissue and preserve the anatomy is advocated in order to maintain aesthetic facial contours. Involvement of mouth and lips in paediatric hemangioma may result in permanent enlargement of upper and lower lips even after involution. Distortion of the vermillion boundary of lips is extremely difficult to correct at a delayed stage, leading to the loss of a critical aesthetic component of face [26]. In many of these cases, if a resection is likely to be required in future causing a resultant scar, an early surgical intervention in a smaller lesion can significantly contribute to preservation of cosmetics and function.

Surgical excision of head and neck hemangiomas should be meticulously planned and well executed, preferably by a specialised hemangioma unit, to accomplish acceptable aesthetic and functional result with minimum morbidity. An experienced team of anaesthetists must pre operatively appraise the patient taking into consideration the risks of general anaesthesia in paediatric age group. Surgical dexterity to prevent excessive and avoidable blood loss cannot be over emphasized. The availability of cross matched blood should be ensured beforehand to manage unforeseen emergencies. We encountered cases of extensive involvement of vascular structure of neck such as carotid sheath vessels in hemangiomas of the neck. Involvement of facial nerve was encountered in parotid hemangioma, temporal bone hemangiomas and masseter hemangiomas. Infraorbital nerve was involved in a case of large nasal tip hemangioma. The decision regarding the extent of resection has to be taken depending upon the surgical competence of operating team and its capability to deal with ensuing complications. In cases of intramuscular hemangiomas, effort should be made to avoid unnecessary resection of healthy tissue to preserve post operative function. The rationality of decision made and precision of execution go a long way to ensure good post operative quality of life in these paediatric patients. Basquino et al. in their study have observed that there are several clear indications for treating paediatric haemangiomas where surgery plays a crucial role [27]. This study also benefits from adopting a multidisciplinary approach at tertiary care institute thus strengthening the comparative value between different modalities and disciplines.

To summarize, surgical excision of paediatric head and neck hemangiomas, in the opinion of authors, is a reasonable and rational treatment option in carefully selected cases. It has the potential to ensure complete cure from the beginning in most of the cases, in contrast to other treatment modalities which require long period of treatment, follow up and thus, parental anxiety. The relative benefits of surgical excision should be considered carefully and must not be neglected during initial discussion and counseling regarding treatment options. The treatment protocols should be individualized according to each patient and merits of all interventions should be assessed in light of presentation of hemangioma. A multidisciplinary team effort towards management is the most appropriate way to approach these lesions. Maintaining long term quality of life should be used by all caregivers as the foremost and focal guide to prioritise treatment interventions in paediatric head and neck hemangioma patients.

Funding

None.

Declarations

Animal Participants Used

None.

Informed Consent From Human Participants

Taken.

Conflict of Interest

None.