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© Borgis - New Medicine 2/2002, s. 55-57
Mieczysław Chmielik, Beata Zając
Laryngeal haemangioma in children
Department of Paediatric Otorhinolaryngology, The Medical University of Warsaw, Poland
Head: prof. dr hab. med. Mieczysław Chmielik
Haemangiomas and vascular malformations located in the larynx are rare but potentially life-threatening conditions, because they compromise the airway. The authors present the clinical symptoms and method of diagnostic evaluation of these lesions. Various techniques for the treatment of laryngeal haemangioma are discussed, but no single approach has universal acceptance.
”Haemangioma” is a generic term for various vascular lesions used in medical literature. The classification of vascular anomalies based on histopathological studies and clinical behaviour was proposed by Mulliken and Glowacki (13, 20). The classification divides vascular anomalies into either haemangioma or malformation. Haemangiomas are not usually present at birth, but grow rapidly during the first 6 months of life. Histologically, specimens demonstrate proliferation of the endothelium. The growth phase is followed by slow involution. Vascular malformation is a collection of abnormal vessels forming a lesion that is present at birth. Malformation is a structural anomaly, an error in vascular morphogenesis. It presents a normal rate of endothelial cell turnover and grows commensurately with the child, without a tendency to spontaneous regression (20). The majority of haemangiomas and vascular malformations involve the skin. Visceral lesions may be located in the mediastinum, airways, abdominal cavity, on central nervous system. Haemangiomas and vascular malformations located in the larynx compromise the airway and produce the same symptoms. In most laryngological literature vascular anomalies are described using confusing terminology. The same term, ”laryngeal haemangioma”, is used to describe haemangioma or vascular malformations located in the larynx. ”Laryngeal haemangiomas” are most commonly included in the group of congenital anomalies of the larynx, but some authors classify these lesions as benign tumours of vascular origin.
Haemangioma located in the larynx are rare, accounting for 1.5% to 5% of all congenital laryngeal anomalies (15, 19). About half the patients also have cutaneous lesions (2, 3, 8, 14, 15, 17, 18). Laryngeal haemangioma may be associated with visceral lesions. Patients with cutaneous haemangioma located in a beard distribution (preauricular area, chin, anterior neck, lower lip) also have symptomatic involvement of the airway (14). Patients of this type represent 63% of cases of cutaneous haemangioma. Haemangioma is typically located in the subglottic region, but the supraglottic space, vocal cords or upper part of the trachea may also be involved. Haemangiomas exibit a characteristic history of rapid growth during the first 6 months of life, and then after 12 months a slow regression takes place. The main symptom of laryngeal haemangioma is obturation of the airway passage. At birth the child is usually asymptomatic, the first symptoms being seen between 4 and 12 weeks of life, progressing with the rapid growth of the haemangioma. Involution of the lesion generally occurs by 12 months of life and symptoms decreased in severity (1, 4, 7, 11, 14, 17, 19). Rarely, symptoms of airway obstruction occur at birth. These cases are probably vascular malformations and not haemangioma by Mulliken and Glowacki´s classification. Severity of symptoms may fluctuate in time, with exacerbation and remission. The intermittent nature of symptoms may be attributed to the changing haemodynamics of haemangioma. The symptoms aggravate with excitement and crying, and fluctuate due to the position of the body. Exacerbation of symptoms typically occurs during respiratory tract infection (2, 4, 5, 11, 15). The most common presenting symptom is biphasic stridor. The voice and cry usually remain clear (2, 15). Hoarseness may occur in cases with glottic involvement. The majority of patients present a characteristic croup-like cough. Respiratory distress progresses with enlargement of the haemangioma. The patient presents dyspnoea with suprasternal and intracostal retractions. Dysphagia is rarely seen (2). Difficulty in feeding occurs in cases of large haemangiomas involving the pharynx, oral cavity, or mediastinum. Haemoptysis is occasionaly noted in cases of haemangiomas extended in the superficial part of the mucosa (2, 4).
Diagnostic evaluation of laryngeal haemangiomas may bring many difficulties. Typical symptoms: stridor, dyspnoea, cough, and hoarseness are present in the clinical picture of other congenital anomalies of the larynx and laryngeal infections (5, 8, 17). The presence of associated cutaneous haemangiomas may aid in the diagnosis, especially if skin haemangiomas are located in the central region of the neck and face (14). If episodes of croup-like symptoms are presented in the first 4-6 months of life, diagnosis of haemangioma or subglottic stenosis is very likely. The possibility of haemangioma must be taken into consideration in children with reccurent episodes of ”croup” during the first year of life and in cases of severe symptoms of laryngeal infections which do not resolve after routine treatment. A definitive diagnosis of the subglottic haemangioma is based on endoscopic examination. Direct laryngoscopy reveals haemangioma as a soft, compressible, mucosally-covered lesion, bluish or red in colour. In some cases haemangioma may be covered with pale, unchanged mucosa. The subglottic region is most commonly involved. Extension of the haemangioma into the supraglottic space or upper part of the trachea is also reported in literature (3, 11). Large vascular lesions of the head and neck involving the prevertebral space or mediastinum may extend into the larynx and trachea or compress the airway (3, 13). Haemangiomas of the typical subglottic site are usually located unilaterally. In some cases bilateral, symetrical involvement of the subglottic space occurs. Localisation of a haemangioma limited to the anterior or posterior laryngeal wall and circumferential lesions are also reported in literature (2, 3, 4). Circumferential or bilateral haemangiomas are difficult to diagnose, because of the similarity of localisation with subglottic stenoses.
Radiographic examination is very useful in a diagnostic evaluation of laryngeal haemangioma. In the past lateral and frontal radiographs have been most commonly performed (3, 11). Recently MR with intravenous administration of contrast is the most acceptable diagnostic procedure in the evaluation of subglottic hemangioma (1, 6, 9, 12, 13, 18).
The role of biopsy in diagnostic evaluation of haemangioma remains controversial. Some authors consider the clinical history, endoscopic and radiological examinations as sufficient for a definitive diagnosis of haemangioma. In their point of view, a biopsy is not necessary to confirm a diagnosis (3, 4, 5, 11,14). In our opinion, in cases when a diagnosis of subglottic haemangioma is in question, biopsy may resolve any doubt. The same view is presented in literature (9, 15). Most authors do not advocate biopsy because uncontrolled bleeding is possible. On the basis of our experience and review of the literature, biopsy seems to be a safe procedure, and accidental bleeding may be controlled without sequelae (15).
Over the years many different techniques have been advocated for the treatment of laryngeal haemangioma. At present the main therapeutic solutions in use are tracheotomy, systemic steroids, laser treatment, surgical excision, and interferon. The variety of therapeutic approaches suggest that there is no universally effective treatment.
Some authors consider laser therapy as a safe and effective form of management for subglottic haemangiomas (8, 18, 21). Others have reported complications such as granulation, scarring, and stenosis of the laryngeal lumen (3, 9, 15, 16, 19, 22). Reports of subglottic stenosis developing after aggressive laser ablation emphasise the need for caution when using this treatment for large lesions, especially the extending into the perichondrium (2, 22). Laser therapy can be effective, but it is probably only suitable for small haemangiomas (1, 2).
A positive response to systemic steroid treatment has been reported in the literature (11, 18). Steroid therapy may be used alone or be combined with intubation, tracheotomy or laser ablation. Although useful in some cases of subglottic haemangioma, steroids are not always effective. The predictive factors of response to steroid therapy are not known. Long-term treatment with systemic steroids has side effects such as a risk of infections, growth retardation, and cushingoid appearance (6, 15, 16, 22).
Some authors proposed intralesional steroid injection (1, 9). The treatment presented in literature (9) included intralesional injection of steroids, followed by 1 week´s intubation.
Recently, successful results with interferon alpha have been reported in the treatment of multiple or extensive lesions involved the trachea, mediastinum, hypopharynx, cervico-facial region or cervical spine (1, 3, 15, 22).
In the literature, surgical excision of haemangiomas has been described (6, 15, 16, 19, 22). Some authors reported complications after surgical excision of haemangiomas through laryngofissure. In their opinion the posibility of subglottic stenosis and impairment of future laryngeal growth militate against the use of surgical excision (5, 17). Recently, in a few papers, the authors have presented surgical excision of haemangioma as a succesful method of treatment (1, 6, 15, 16, 19, 22). Using this method a tracheotomy may be avoided or only needed for short period. They recommend the performance of open surgical resection combined with a cricoid split, to decompress and expand the subglottis. In some cases cricoid enlargement by cartilage grafts or stents are necessary for widening the subglottic area. In this way the problem of subglottic stenosis may be avoided (1, 19). However they feel that open surgical resection is warranted for large obstructive lesions.
Tracheotomy is considered by most authors to be the treatment of choice in cases of subglottic haemangioma with obstruction of the airway passage (2, 3, 5, 15-19, 21). But, used alone, without any other methods of treatment, this is reported to be associated with a mortality of 1-2% due to accidental decannulation and obturation of the tracheotomy tube, and a risk of delayed speech development (1, 6, 9, 15, 21, 22). In our experience tracheotomy is a safe procedure if skilled nursing and adequate teaching of parents are available. The same opinion is also presented in literature (2, 5, 17). In some papers tracheotomy and waiting for spontaneous involution is advocated only in selected cases of circumferential haemangiomas of the subglottic area and very large lesions which extend into the trachea (1).
Different therapeutic methods are difficult to compare, not only because haemangiomas regress spontaneously, but also several combinations of therapies are often used. It is difficult to establish the main reason for the involution of a haemangioma – treatment or spontaneous involution. The natural regressive character of the haemangioma and the enlargement of the subglottic lumen by normal growth have been the main factors in decannulation. In our opinion treatment shoul be conservative, since most haemangiomas will regress spontaneously. Open surgical excision or laser therapy shoul be reserved for those patients in whom a haemangioma in unchanged or continues to enlarge after around 2 years of age.
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New Medicine 2/2002
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