Management of head and neck hemangiomas in China

Hemangioma is a common benign vascular tumor, with an incidence of 2%-3% in the neonate and 10% in baby after 1 year.1 The incidence of hemangiomas is approximately 22%-30% of preterm infants with birthweight less than 1000 g. The development of hemangioma is therefore supposed to be related to various intra- and extra-uterine factors. Females are affected more often than males by a ratio of 3:1-5:1.1 Hemangioma can be found in all regions of the body, but 60% are localized on the head and neck, 25% on the trunk, and 15% on the extremities. Most of the lesions are focal and solitary, while 15%—20% are multiple with involvement of extracutaneous sites; including the liver, gastrointestinal tract, larynx, central nervous system, pancreas, gall bladder, thymus, spleen, lymph nodes, lung, urinary bladder, and adrenal glands. Although infantile hemangioma is usually benign, its growth is very unpredictable due to uncontrollable endothelial cell proliferation. Infantile hemangioma has a varied appearance depending on the location of the lesion, the depth of the hemangioma and the size. In the early phase white or pink spots are often noted. Sometimes, it appears as port wine stain-like lesion or fine telangiectasias surrounded by a faint halo of vascular blanching. The lesion grows rapidly and unproportionally during the first several weeks (usually 4 weeks) after birth into the proliferative phase, which usually lasts for 6 months.2,3 Capillary hemangioma and carvenous hemangioma were usually used to describe various types of hemangiomas in the past, but these terms are not exact, for many so-called carvenous hemangiomas are actually vascular malformations rather than true hemangiomas. Therefore, an important concept is that hemangioma is a true vessel cell tumor with distinct clinical and biological characteristics derived from vascular malformations. It can be divided into cutaneous, subcutaneous or mixed type depending on the depth of the lesions. The colour of the hemangioma is varied, mostly depended on the extent and depth of the lesion, the diameter of the involved vessels, the primary site, the skin temperature, as well as the status of the infancy such as crying or exciting. CLINICAL CHARACTERISTICS Hemangioma features a rapid proliferative phase followed by an involutive phase. At the end of 1 year the lesion begins to regress and arrests in growth. The colour changes from pink red to deep red. Involution usually lasts longer than proliferation and may persist for 1 to 10 years. In the involution phase, endothelial cells decrease in number and are substituted by fatty and fibrous tissues. Although most hemangiomas can resolve spontaneously, approximately 50%-60% of all hemangiomas resolve incompletely, leaving permanent changes in the skin. These changes include telangiectases, superficial dilated veins, stippled scarring, anetoderma or epidermal atrophy (particularly with superficial lesions), hypopigmentation and/or redundant skin with fibro-fatty residua (especially with subcutaneous lesions). Patients with ulcerations easily develop scars after healing. The residual lesions are related to the size and location of the primary hemangiomas, and usually need to be surgically corrected. The clinical and biological characteristics of hemangioma have a small but significant percentages of hemangiomas of infancy that develop complications; such as ulcer, secondary infection, bleeding, facial deformities and eye problems. Hemangiomas arising in the submandibular area are easily accompanied by respiratory problems; such as laryngeal stridor, hoarseness or even dyspnea. Laryngopharyngeal bronchoscopy is recommended for early detection and management in this circumstance. The diagnosis of hemangioma is normally made based on the history and clinical findings. In some cases, especially those with deep lesions, difficulties may exist in differentiating hemangiomas from vascular malformations. Patients can be observed for several days or weeks to see any changes of the lesions. If diagnosis is still uncertain, color Doppler ultrasonography and/or MRI may be used to aid in the diagnosis. Color Doppler ultrasonography is considered to be the most cost-effective imaging technique for the evaluation of hemangiomas. A proliferating hemangioma demonstrates nonspecific echogenicity with increased color flow and a high-flow pattern with an arterial and venous component. Numerous vessels are visualized (>5/cm2) with a high Doppler shift (>2 kHz) and low resistance. Using the criteria of high vessel density (>5/cm) and a high Doppler shift (>2 kHz), Dubois et al4 were able to achieve a sensitivity of 84% and a specificity of 98% in the diagnosis of hemangiomas. MRI is probably the single best modality to characterize vascular birthmarks. In MRI, proliferating hemangiomas demonstrate well defined, lobulated homogeneous soft-tissue masses. They are isotense-to-hypotense relative to muscle on T1 weighted images, and hypertense relative to muscle on T2 weighted images. Flow voids are seen within and around the lesions on spin echo images. The management of hemangiomas remains a subject of considerable controversy both in China and world wide. One frequently raised question is the necessity to treat this benign lesion that can resolve spontaneously. Indeed, the majority of hemangiomas require no intervention and in general the ultimate cosmetic outcome is usually better in hemangiomas that are allowed to involute spontaneously than those treated too actively. One should keep in mind that hemangiomas can result in a variety of problems and impacts on children. The most serious situations are hemangiomas involving the upper aerodigestive tract and trachea. If laser therapy or tracheostomy is not applied adequately, the patients will have airway obstruction, asphyxia or even die.5 Hemangiomas in or around the eye requires early treatment to prevent amblyopia.6 Extensive cutaneous hemangiomas may lead to skin necrosis, ulceration and secondary infection,7 causing a series nursing care problems. The second category of problems caused by hemangiomas are not life-threatening or function-impairing sequelae, but various psychological problems due to disfigurement, such as negative self-image evaluation, lack of self-confidence and distress, etc. The third category of problem caused by hemangiomas is its unpredictability in growth several weeks or months after birth. There are now objective methods and variables to assess the extent of involution up to now. About 90% of infantile hemangiomas will involute eventually, but there are still 10% of them that will continue to grow with various complications. Therefore, it is imperative to control the growth of enlarging hemangiomas. Based on our clinical experiences on 165 cases and on literature reports, early and proper treatment of problematic hemangiomas is mandatory and effective. Many treatment modalities are currently available for management of head and neck hemangiomas, including careful observation, drug therapy, laser and operative therapy. Controversies still exist as to when and how to treat based on these treatment tools. One should understand the natural history of hemangioma and the indications of each method prior to giving treatment to the individual patient. In our opinion, treatment of hemangiomas should be dependent on the location, extent, growing phase of the lesions and techniques available. There is no gold-standard treatment applicable to all patients. The principles of management of hemangiomas are accelerating the resolution of the lesions, minimizing the psychosocial distress caused by the lesions, and minimizing any morbidity related to the treatment of the lesions.8 We would add that an attempt should be made to use the available methods in a reasonable order to normalize the child's appearance before entering school. “WAIT AND SEE” “Wait and see” used to be the widely accepted policy for management of hemangiomas. During the first several weeks after birth, the lesion is too small to be treated or early intervention may lead to severe complications. In the proliferative phase, the parents were instructed to observe closely in the hope of spontaneous regression. Usually, the lesion continues to enlarge and the parents visit the physician again, but there is no satisfactory treatment to be used, and the lesion has caused considerable cosmetic or functional problems. Nowadays, with the advent of various new techniques, especially the use of novel laser therapy, this old paradigm has been changed to benefit the patients. As it is impossible to precisely predict if the natural course of hemangioma is towards spontaneous resolution or continuous growth. If no intervention is delivered but only “wait and see”, most of the patients will not tolerate the long-term disfigurement. Therefore, early treatment in selected cases seems to be most important and imperative. The aim of intervention in this stage is to hinder enlargement of the lesion and accelerate regression. Facial hemangiomas are likely to develop ulceration and secondary infection, while hemangiomas arising from the upper aerodigestive tract may compress or obstruct the airway. Early treatment is therefore mandatory under these circumstances. “Wait and see” is indicated in the following situations: (1) hemangiomas in non-vital sites, small in size, without rapid growing, have no significant impacts on appearance and function; (2) hemangiomas in involuting or involuted phase. During the follow-up period, the growth of the lesion should be monitored using objective means such as digital camera or B-mode ultrasonography. In addition, close observation is suggested to replace the so-called “wait and see”, and to be conducted by experienced physicians not untrained persons. Photographs should be taken at periodic intervals to help the parents see the regression of the lesion over time. CRYOTHERAPY CO2 rime and liquid nitrogen used to be applied in management of cutaneous hemangiomas, but is seldom utilized today due to limited indications (<10 mm in diameter, <2 mm in depth), unreliable efficacies and possible side effects such as skin scar and pigmentation. LASER THERAPY Laser therapy is indictable for treatment of early, superficial hemangiomas. In our opinion, the small red skin spot or macule discovered after birth should be removed with a laser, to arrest its subsequent rapid growth into the proliferative phase and alleviate further damages to the children as well as the psychological strain from the parents. For proliferating hemangiomas, laser therapy can accelerate regression, shrink the lesions, benefit the subsequent treatments and aid the children to achieve satisfactory appearance before school ages (5–6 years). Drug therapy (corticosteroids or Interferon) is considered if the lesions continue to enlarge during laser therapy. There are several types of laser available for management of hemangiomas. Argon laser (514 nm in wavelength) can penetrate the skin to a depth of 0.5 mm, photocoagulate the hemoglobin within the vessels, and remove the superficial hemangiomas effectively. The shortest exposure time for response is 100 milliseconds. Histopathlogically, hemoglobin within the vessels of the irradiated lesions absorbs abundant energy after argon laser radiation, causing coagulation necrosis of the dermis and vascular embolization of the papillary layer without damage to the cutaneous appendages. Argon laser is quite effective for superficial telangiectasia and small, flat hemangioma, but the response of larger hemangiomas and deeper lesions is varied and limited. Flashlamp-pumped pulsed dye laser (FPDL) (585 nm or 595 nm in wavelength) is the first laser specially designed to treat cutaneous vascular lesions and the first laser to eliminate these lesions predictably without producing a scar. It is characterized by a selective destruction of blood vessels by matching the wavelength of light absorbed by hemoglobin in the vessels and by using an exposure time less than the calculated thermal relaxation time (1–10 milliseconds) for the blood vessels. An exposure time of a millisecond or less was advocated to confine the heat to the vessel to decrease the risk of heat diffusion, which can cause skin scarring. In contrast to argon laser, FPDL is the only laser that can cause thrombosis within the vessels without damage to the dermis. When adjusted to 585 nm, the depth of penetration for 50% of the energy is calculated to be 0.8 mm.9 Because the dermis of facial skin is approximately 0.6 mm deep in children and approximately 0.9 mm deep in adults, FPDL has proved to be a safe and effective treatment modality for superficial hemangiomas in the face. Clinically, FPDL is often applied with the following settings: a spot size of 5–10 mm, an energy density of between 5 to 7 J/cm2, with a maximum spot overlap of 20% to 30%. The high pulse peak power of the FPDL disrupts the vessels. Immediately after radiation, the treated area turned blue-lilac (putpuric), with surrounding erythematous flare; this took 7 to 14 days to resolve. Some edema, especially in the periorbital area, was possible. If blanching or graying of the epidermis occurred during application, energy fluences were reduced to avoid blistering of the epidermis. After treatment, the treated areas were covered with panthenol ointment. In case of blistering or crusting, the patients' parents were instructed to cleanse the area with povidone-iodine the parents were instructed to keep or have them to avoid to the treated The patients were after to 4 depending on the of The treatment be after the first usually at a Hemangiomas with a deep or subcutaneous not benefit from FPDL treatment the of the FPDL is limited by its depth of vascular early intervention with FPDL may not prevent proliferative growth of the deeper or subcutaneous of the the deep of the hemangioma to after FPDL treatment, the superficial In this of to arrest the growing of the lesions, or further therapy with laser with continuous with to FPDL treatment, superficial is applied to the treated areas with During treatment, a can be used to the skin and the should be considered for children with hemangiomas over a and hemangiomas of periorbital area, especially if the were treated under the of the with an eye FPDL treatment is to cutaneous hemangiomas during the involution phase. Some may that superficial involuting hemangiomas need no treatment, in many of these lesions are to resolve and can be in a or area, and patients can benefit from laser treatment with a response and before school laser is a of laser a continuous and light with a wavelength of nm and a penetration depth of The laser energy can be absorbed mostly by blood while only a is absorbed by surrounding tissues. Because of the longer the laser is of deeper into the with a depth of approximately prevent thermal to the are used to the skin during In to laser is for a avoid of the and the laser power is limited to be to and the exposure time is less than For hemangiomas arising from the upper aerodigestive tract, laser can be applied an in diameter in the following settings: an power of to 5 an exposure time of 0.5 to The laser can penetrate into and within the deep to be an means for and It can be applied with and laser with a or technique is a for selected patients with larger or deeper in subcutaneous or mixed hemangiomas. The laser can be well on the lesion with a spot mm in size. light can photocoagulate and eliminate the lesion with to the tissues. The power of the laser is between to For deep red hemangiomas, to is with continuous with For subcutaneous hemangiomas, approximately is applied with continuous hemangiomas may need treatment at an of 4 to 6 These lesions often enlarge and laser treatment is to and to accelerate even if it is to the of the Hemangiomas in the can be treated with laser under of the laser treatment is very and should be under general Immediately after treatment the lesion and A or a may be but there is no necessity to the often 1 after treatment, and the lesion flat and to 4 weeks The response of laser therapy for hemangiomas is between and The size of hemangiomas at treatment is closely related to the the the size, the better Most hemangiomas can be treated and treatment is if the lesions continue to The of with the laser is with from the surrounding and laser therapy with power can result in skin scars in children. is and often is the result of spontaneous of hemangiomas. the side effects and laser therapy is a in the treatment of hemangiomas with rapid growth or in functional The of laser therapy include scarring, and is often for skin scar or residual lesions. THERAPY or drug therapy is considered in patients with multiple hemangiomas, rapidly growing hemangiomas, life-threatening hemangiomas or hemangiomas involving Laser therapy can be applied during have been used to treat hemangiomas to or regression with various The use of to treat hemangiomas of infancy has been for over the first by and in It has the treatment for and hemangiomas as well as for patients with and should be during the proliferative phase have a on involuting lesions. The is over the location, size, and physician into the The of or in China is to 5 rather than as a single for The is to to at the 10 at the 5 at the and at the A second or third course of treatment may be at intervals of 4 to 6 weeks when in response from less than to than 84% on depending on of treatment and at which therapy is The was with a better response with a larger but there was no significant in of In addition, therapy should be within 1 year of the larger the at the of the treatment, the the treatment are well for the treatment of hemangiomas with no long-term side some side effects such as changes or appearance may but with of the therapy may be in patients with growth of the hemangioma without drug resistance. of or in is effective at the growth and the size of proliferating hemangiomas. treatment modality is to patients with response to or with of or laser therapy due to various The response of is have a response to but with It has been used for periorbital and localized facial lesions. use of a of of and 6 of that are into the tumor with a small diameter The are at intervals of 6 to weeks for a of to 5 of to treat hemangiomas was first by et in with more this treatment modality in management of head and neck hemangiomas and vascular malformations treatment modality has been to be easily and with the used and are and necrosis of skin or seldom Therefore, it is especially applicable for treatment of hemangiomas in the of and submandibular after in these areas is to prevent in the respiratory Based on our clinical resolution of small and hemangiomas than 4 in in the head and neck can be with treatment For larger and deeper hemangiomas, such as and are considered for limited need of secondary or used as an in was found to regression of to its use in life-threatening proliferating hemangiomas, that have to to and The of of on hemangiomas is include endothelial cell and proliferation and growth such as endothelial growth growth etc. It is usually used at a of for 7 to 10 The response is varied between and it not require that during the proliferation phase to be effective. The of is than that of usually several with has more side effects than with including and of If the is not observed after 1 should be the use of a was to be in the treatment of is and Drug for the treatment of and cell with and low It and a variety of including the of and the of a variety of including and which it may a case are currently available on the use of for the treatment of infantile hemangiomas. with cases that this treatment is especially for selected small and hemangiomas involving the regions such as the and the It is as it is to use for the parents at has side and can be used with in patients with multiple hemangiomas. It is applied in small usually and to affected area for to 5 the lesions are indicated in order to and of in the treatment of hemangiomas of both and have been used for the treatment of hemangiomas, including and The of in hemangiomas remains at this time and should be only for patients with hemangiomas using THERAPY of hemangiomas is no longer the treatment of but there are several indications for therapy of hemangiomas. Early therapy may be for or periorbital hemangiomas that may the to and for lesions the larynx, or the may be considered early for lesions of the or hemangiomas on the and the of the to as a result of damage to and cosmetic outcome may be by is for or hemangiomas that have not to more If the lesion is too and primary is laser therapy may be first followed by therapy is indicated in those hemangiomas that involute with residual scarring, cutaneous areas of on the or atrophy before school to the psychosocial distress from the of hemangiomas for both and laser is usually used as the treatment modality for hemangiomas with and in most but still 10% to 20% of patients require of the residual lesion or Laser therapy not in the treatment of infantile hemangiomas, but is with The between laser therapy and should not be too 1 year after laser therapy, the lesion will continue to The following principles are at 1 laser and/or drug at to 4 close at 5 to 6 In the treatment of hemangiomas should be to treat include size, location, growing and of the lesion and many such as the of or of of treatment of physician and Extensive or multiple hemangiomas usually require reasonable A should be widely to various types and of hemangiomas, of the currently available treatment modalities are to therapy. The following should be taken into in management of (1) Drug therapy and laser therapy for proliferating lesions, close observation for and involuting (2) dye laser for lesions less than mm in depth and larger than in size, laser for lesions in the with a depth more than mm, drug therapy for multiple Laser therapy and secondary for lesions in the and laser therapy for lesions in the and treatment for children less than 6 but selective treatment for children more than 6 drug therapy for life-threatening lesions or lesions involving