Management options for infantile subglottic hemangioma

Pediatric Otolaryngology Management Options for Infantile Subglottic Hemangioma Cynthia M. Gregg, MD, Brian J. Wiatrak, MD, and Charles F. Koopmann, Jr., MD

Subglottic hemangioma is a potentially lifethreatening lesion that was first reported in The first description of 1864 by MacKenzie.’ subglottic hemangioma in an infant was made by Phillips and Ruh in 1912.2 Subglottic hemangiomas have a 2:l female to male preponderance and 80% to 89% present within the first 6 months of life.3’4 Presenting symptoms representing various degrees of airway distress include stridor, cough, cyanosis, hoarseness, dysphagia, emesis, and hemoptysis. Typically, these symptoms fluctuate with periods of remission. Cutaneous hemangiomas occur in 44% to 51% of these patients, and are often found in the head and neck area. Histopathologically, subglottic hemangiomas may be categorized as capillary, cavernous, and mixed type. The majority of subglottic hemangiomas are of the capillary type. Although the natural history of this lesion is unpredictable, the majority begin to involute and regress after 18 to 24 months of age. Various treatment modalities have been de-

From the Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, University of Michigan Medical Center, Ann Arbor, MI and the Department of Pediatric Otolaryngology, The Children’s Hospital of Alabama, University of Alabama at Birmingham, Birmingham, AL. Presented at the American Society of Pediatric Otolaryngology Century Plaza Hotel and Tower, Los Angeles, CA, April 22, 1993. Address reprints requests to Brian J. Wiatrak, MD, Pediatric Otolaryngology, The Children’s Hospital of Alabama, 1600 Seventh Ave, South, ACC 320, Birmingham, AL 35233. Copyright 0 1995 by W.B. Saunders Company 0916-0709/95/1606-0007$5.00/O American

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scribed in the literature including: (1) tracheotomy alone or in combination with other treatments, (2) external radiation, (3) radioactive gold grain implantation, (4) cryotherapy, (5) sclerosing agents, (6) corticosteroids (systemic and intralesional), (7) laser ablation, and (8) open surgical excision. This is a presentation of three cases of infantile subglottic hemangiomas treated with different modalities at the University of Michigan Medical Center and discussion of the importance of patient selection in choosing an appropriate treatment modality. CASE REPORT 1 A s-month-old female infant, a product of an uncomplicated pregnancy, was diagnosed 3 weeks before admission with bronchiolitis. Her parents reported a history of “noisy breathing” since birth. She was transferred to the University of Michigan Medical Center (UMMC) after presenting to an outside emergency room with inspiratory stridor. There was no evidence of cyanosis or substernal retractions. Anterior-posterior and lateral cervical airway radiographs and fluoroscopy showed subglottic narrowing. Flexible laryngoscopy showed mobile vocal cords. Direct laryngoscopy and bronchoscopy showed a smooth, mucosal-covered, right-sided subglottic mass, obstructing greater than 50% of the airway. A tracheotomy was performed. At 9 months of age, repeat direct laryngoscopy showed an increase in the size of the hemangioma, resulting in a greater than 90% airway obstruction. Direct laryngoscopy performed at 14, 17, and 22 months of age noted progressive involution of the hemangioma. Involution of the subglottic hemangioma was confirmed by direct Iaryngoscopy at 23 months of age, and the patient was decannulated without incident.

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CASE REPORT 2 A l-month-old male infant presented with biphasic stridor. A diagnosis of subglottic hemangioma was made on flexible laryngoscopy, and a z-month course of oral steroids was initiated. The infant was then referred to the UMMC because of persistent stridor. Anterior-posterior and lateral cervical airway radiographs showed a left-sided subglottic mass (Fig 1). Direct laryngoscopy and bronchoscopy confirmed a left-sided subglottic hemangioma resulting in greater than 90% airway obstruction. Intralesional steroid injection with triamcinolone acetonide (2 mL of 40 mg/mL preparation) was performed, and the patient was intubated with a 3.5 mm endotracheal tube and monitored in the intensive care unit for 8 days. The patient was maintained on solumedrol (2 mgikglday IV] and required a midazolam hydrochloride drip with intermittent intravenous pancuronium bromide for sedation and muscle relaxation. The patient’s postoperative course was complicated by a nontension pneumothorax on postoperative day 5, which required a No. 12 French chest tube for re-expansion of the right lung. The patient also developed right upper lobe pneumonia requiring intravenous antibiotics.

Fig 2. IAl Case 2. Patent subglottic airway after steroid injection and 8 days of intubation. Arrows point to true vocal cords. (B) Case 2. Re-expansion of left-sided subglottic hemangioma (arrow) 5 minutes after extubation.

On postoperative day 7, direct laryngoscopy initially showed a patent subglottic airway [Fig 2). However, 5 minutes after extubation, re-expansion of the hemangioma resulted in greater than 50% airway obstruction, and a tracheotomy was performed. The patient was weaned off the corticosteroids and was discharged home on postoperative day 16. At 4 112 months of age, direct laryngoscopy revealed a greater than 75% obstruction of the subglottic airway, and a second intralesional injection of steroids was performed. At 6 months of age, 35% airway obstruction persisted, and a partial submucosal excision was accomplished with a CO, laser. Direct laryngoscopy at 9 months of age showed an increase in hemangioma size with a greater than 80% airway obstruction. The option of an open surgical excision was discussed with the parents. They preferred to wait for spontaneous involution of the lesion. Spontaneous involution was confirmed with direct laryngoscopy at 18 months of age, and the patient was decannulated without incident.

CASE REPORT 3

Fig 1.

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of left-sided

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A female infant, born by cesarean section secondary to breech presentation, was noted to have

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inspiratory stridor and cyanosis at birth. She was transferred to the University of Michigan Medical Center, where anterior-posterior and lateralcervical airway radiographs, chest radiograph, and a barium swallow were normal. Flexible laryngoscopy showed limitation of left vocal cord abduction. She was discharged home on an apnea monitor. At 6 weeks of age, she presented to the emergency room with substernal retractions and inspiratory stridor. Repeat airway radiographs and fluoroscopy were normal. Direct laryngoscopy and bronchoscopy showed a large, nonpulsatile, leftsided subglottic mass with near complete airway obstruction necessitating a tracheotomy (Fig 3). A computed tomography (CT) scan revealed a T-mm enhancing lesion located within the subglottis. No other neck masses were noted. Three partial submucosal laser excisions of the subglottic hemangioma were performed at 2 l/2, 5 112, and 10 l/2 months of age with a CO, laser. The hemangioma persisted with greater than 85% airway obstruction. The option of open surgical excision was discussed with the family. When the patient was year old, open surgical excision of the hemangioma was performed using a midline cricotracheotomy approach. The incision included the lower thyroid cartilage preserving the anterior commissure, cricoid cartilage, and first two tracheal rings. A submucosal excision of the hemangioma was accomplished with minimal blood loss. The lesion was sharply dissected free from the inferior margin of the left true vocal cord (Fig 4). The mucosal flap was replaced over the inner aspect of the cricoid lamina, and a finger cot stent was inserted. A threelayer closure was performed, and a Penrose drain

Fig 3. Case 3. Left-sided point to true vocal cords.

subglottic

hemangioma.

Arrows

and pressure dressing applied. Pathology confirmed that the specimen was a 1.2 x 0.6 x 0.4 cm capillary hemangioma [Fig 5). The patient was discharged home on postoperative day three. The finger cot stent was removed endoscopically on postoperative day twelve. Direct laryngoscopy at 15 months of age confirmed a patent airway of appropriate size, and the patient was decannulated. The patient remains asymptomatic at 30 months of age.

DISCUSSION Since infantile subglottic hemangioma was first described, various treatments have been recommended. Airway obstruction may be managed with a tracheotomy and/or an attempt to eliminate the obstructing lesion. External radiation was used before 1973, and implantation with a radioactive gold grain was introduced by Holborow and Mott.5P” However, the risk of developing a malignancy secondary to the treatment prevents current use of these treatments. Response of cutaneous hemangiomas to corticosteroid therapy suggest that this therapy might be beneficial in the treatment of subglottic hemangioma, but the potential side effects of systemic corticosteroids, including growth disturbance, endocrine abnormalities, and an increased risk of infection, must be considered.7 In 1990, Meeuwis et al described intralesional steroid injection and endotracheal intubation as an effective means toward early decannulation. The average length of intubation in their 6 patients was 19 days (range 7 to 36 days). The patients were reported to be asymptomatic at an average age of 7 months. Reported complications included 2 patients with pneumonia, and another patients with growth disturbance resulting from the administration of systemic corticosteroids.* The financial cost and emotional strain on the family caused by prolonged intensive care monitoring and hospitalization of their child must be considered when choosing this treatment modality. The sedation and paralysis required for an intubated pediatric patient often results in the need for total parenteral nutrition and intravenous access. As described in Case Report 2, the potential complications of long-term mechanical ventilation, including

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Fig 4. (A) Case 3. Midline cricotracheotomy incision including lower thyroid cartilage, cricoid cartilage, and first two tracheal rings. Arrow A indicates thyroid cartilage, arrow B points to the left-side subglottic hemangioma. (B) Case 3. The retracted mucosal flap with exposed cricoid cartilage (arrow A).

pneumonia and pneumothorax, may be encountered. Tracheotomy and observation for the involution of the hemangioma is considered by many to be the standard of care. Mortality resulting from accidental decannulation or tracheotomy tube plugging has been reported.3*4 Adequate family teaching, skilled nursing, and close follow-up with the physician are necessary to prevent these complications. The patient’s social situation and access to immediate medical care must be considered. As reported in Case Report 1, a patient treated with

tracheotomy and observation may require multiple laryngoscopic procedures to document the involution of the hemangioma and the formation of any tracheal granulation tissue. Prolonged tracheotomy may affect speech and language development. Kaslon and Stein9 provide evidence indicating that therapeutic intervention is necessary to avoid delays in receptive and expressive language development and deficits in voice production in children with a tracheotomy. A speech pathology program is needed to provide this therapy.

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Fig 5. Case 3. Capillary hemangioma spaces (arrows) and intervening stroma stain, original magnification x240).

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with small vascular Uiematoxylin-eosin

Healy et al” first described the successful use of the CO, laser to treat subglottic hemangioma. This experience was updated by Sie et all1 in 1994, reviewing 31 cases of subglottic hemangioma. Twenty percent of 20 cases undergoing CO, laser excision without a tracheotomy developed subglottic stenosis.ll Proponents of this treatment modality state that the use of the CO, laser reduces the duration of time that a tracheotomy is needed.” In their review of 20 patients, Wenig et all3 claim the CO, laser is the safest, most effective method for treating subglottic hemangiomas. Cotton et alI4 reported the occurrence of subglottic stenosis in three out of eight patients treated with CO, laser. They state that subglottic stenosis is more likely to occur when the subglottic hemangioma is large, has significant regrowth, or extends outside the tracheal lumen. Brodsky et al4 reported that the use of the CO, laser did not expedite decannulation in their experience with four patients. Both reports recommend the CO, laser over an &month period, without an appreciable decrease in the size of the subglottic hemangioma. In 1949, Sharp15 reported the first successful surgical excision of a subglottic hemangioma in a 5-month-old female who was decan-

nulated 1 month later.15 In 1974, Evans and Todd” described 3 cases of subglottic hemangioma treated with surgical excision after previous failed attempts with systemic steroid therapy. Each of their patients was decannulated within 2 months after surgical excisi0n.l’ In 1989, Mulder et al” reported three cases in which surgical excision using a midline cricotracheotomy incision resulted in decannulation in one patient and extubation in 2 patients several days after surgery. In 1991, Seid et all’ presented 2 cases treated with open surgical excision. They used a laryngofissure approach for exposure and excised the lesion from the inferior margin of the vocal cord in both cases. After being intubated for 5 days, both infants, ages 5 years and 24 months, were extubated in the intensive care unit. One of these patients required placement of a tracheotomy 6 weeks later. A complicating factor in this case was a history of prolonged intubation associated with repair of a coarctation of the aorta. The surgical approach in Case 3 used a midline cricotracheotomy incision reserving the anterior commissure. Our treatment plan was more conservative, in that a single stage procedure with endotracheal intubation was not used. CONCLUSION The most important issue to address in patients with a subglottic hemangioma is maintenance of the airway. The size of the lesion, available family support, patient age, and home care availability should all be considered when selecting the optimal treatment. Currently, accepted treatment options for small, circumscribed lesions include oral corticosteroids, CO, laser, or observation. A tracheotomy may occasionally be necessary to protect the airway. The literature suggests that CO, laser excision is successfully used in smaller lesions. However, results are less consistent with large obstructing lesions. Intralesional steroids and endotracheal intubation are treatment options in selected cases and are supported in medical literature. Problems may arise from prolonged intubation requiring paralysis and mechanical ventilation. Open surgical excision may be a viable treat-

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ment option for large or recurring hemangiomas that are not amenable to CO, laser ablation. More case reports and longer follow-up are needed to fully evaluate the indications for this treatment option. REFERENCE 1. MacKenzie M: Essay on growths of the larynx. Philadelphia, PA, Lindsay and Blakeston, 1987 2. Phillips J, Ruh HO: Angioma of the larynx: Especially its relationship to chronic laryngitis. Am J Dis Child 5:123-130, 1913 3. Shikbani AH, Jones MM, Marsh BR, Holliday MJ: Infantile subglottic hemangiomas, an update. Ann Otol Rhino1 Laryngol 95:336-347, 1986 4. Brodsky L, Yoshpe N, Ruben RJ: Clinicalpathological correlates of congenital subglottic hemangiomas. Ann Otol Rhino1 Laryngol 205:4-18, 1983 (suppl) 5. Holborow CA, Mott TJ: Subglottic hemangioma in infancy. J Laryngol Otol 87:1013-1017, 1973 6. Benjamin B: Treatment of infantile subglottic hemangioma with radioactive gold grain. Ann Otol Rhino1 Laryngol 87:18-21, 1978 7. Hawkins DB, Crockett DM, Kahlstrom EJ, MacLaughlin EF: Corticosteroid management of airway hemangiomas: Long term follow-up. Laryngoscope 94:633637, 1984 8. Meeuwis J, Box CE, Hoeve LJ, vanderVoort E: Subglottic hemangiomas in infants: Treatment with intrale-

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sional corticosteroid injection and intubation. Int J Pediatr Otorhinolaryngol 19:145-150, 1990 9. Kaslon KW, Stein RE: Chronic pediatric tracheotomy: Assessment and implications for habilitation of voice, speech and language in young children. Int J Pediatr Otorhinolaryngol 9:165-171, 1985 10. Healy GB, Fearon B, French R, et al: The treatment of subglottic hemangioma with the carbon dioxide laser. Laryngoscope 90:809-813, 1980 11. Sie KC, McGill T, Healy GB: Subglottic hemangioma: Ten years experience with the carbon dioxide laser. Ann Otol Rhino1 Laryngol 103:167-172, 1994 12. Mizono G, Dedo HH: Subglottic hemangiomas in infants: Treatment with CO, laser. Laryngoscope 94:638641, 1984 13. Wenig BL, Abramson AL: Congenital subglottic hemangiomas: A treatment update. Laryngoscopy 98:190192, 1988 14. Cotton RT, Tewfik TL: Laryngeal stenosis following carbon dioxide laser in subglottic hemangioma. Report of three cases. Ann Otol Rhino1 Laryngol 94:494-497, 1985 15. Sharp HS: Hemangioma of the trachea in an infant, successful removal J Laryngol Otol 94:494-497, 1949 16. Evans JNG, Todd GB: Laryngo-tracheoplasty. J Laryngol Otol 88:589-597, 1974 17. Mulder JJS, vandenBroek P: Surgical treatment of infantile subglottic hemangioma. Int J Pediatrl Otorhinolaryngol 17:57-63, 1989 18. Seid AB, Pransky SM, Kearns DB: The open surgical approach to subglottic hemangioma. Int J Pediatr Otorhinolaryngol 22:85-90, 1991