Tracheal hemangioma: a case report and literature review

Introduction and importance: Hemangiomas are the most common vascular tumors in children. Although hemangiomas are common, they are rarely seen in areas like the trachea and larynx.The most common clinical manifestations in tracheal hemangiomas are stridor and respiratory distress. The main diagnostic method is bronchoscopy. Other imaging techniques like computed tomography scans and MRIs are also helpful. Various treatment options are now used for treating the disease, including beta blockers like propranolol, local and systemic steroids, and surgical resection. Case presentation: An 8-year-old boy with the chief complaint of severe progressive dyspnea and a history of neonatal postbreastfeeding cyanosis was admitted. On physical examination, he had tachypnea, and stridor was heard upon auscultation. There was no history of fever, chest pain, or coughing. He underwent a rigid bronchoscopy followed by a neck computed tomography scan. The results indicated a soft tissue mass with a vascular nature. An MRI of the neck confirmed the diagnosis of tracheal hemangioma. The mass was not resectable during surgery; hence, angioembolization was carried out. Treatment was successful and there was no recurrence on the follow-up. Clinical discussion: Based on the findings in this literature review tracheal hemangiomas present with stridor, progressive respiratory distress, dyspnea, hemoptysis, and chronic coughs. Advanced tracheal hemangiomas commonly do not reduce in size by themselves and need treatment. A close follow-up ranging from 3 months to 1 year is recommended. Conclusion: Although tracheal hemangiomas are rare they should be considered in the differential diagnosis of severe dyspnea and stridor.


Introduction
Vascular diseases in children and infants include vascular tumors and malformations. Hemangiomas are the most common among vascular tumors and also the most common benign soft tissue tumor in infants [1,2] . Infantile hemangiomas occur in nearly 4-5% of infants, which may be present at birth or shown later in life [3] . Hemangiomas go through an involution stage once they have reached their complete growth near one year of age and it may take from months to years; however, a small percentage of hemangiomas never go through this stage and do not become smaller in size [3,4] . The most common areas of involvement are the head and neck with the subglottic region being the most involved [4] . Although hemangiomas usually do not require special intervention, factors such as hemangioma size, growth rate, and location can change this [5] .
Hemangiomas residing in the larynx or trachea, although rare, need close monitoring and intervention as they may lead to irreparable and life-threatening consequences. Tracheal hemangiomas present in various ways in children and adults. Dyspnea, stridor, chronic cough, and obstructive respiratory disease are the most common signs and symptoms of tracheal hemangiomas in children. The main diagnostic tool for tracheal hemangiomas is bronchoscopy [6] . Several different approaches have been HIGHLIGHTS • Hemangiomas are the most common benign soft tissue tumors in children. Even though their most common sites are the head and neck, they are rarely seen in the trachea and larynx. • Tracheal or laryngeal hemangiomas can significantly affect patients' lives, and even cause respiratory distress and arrest. Hence, proper diagnosis and treatment of these diseases are crucial. Tracheal hemangioma's most common signs and symptoms are stridor, respiratory distress, progressive dyspnea, chronic cough, and hemoptysis. • The main diagnostic tool for this disease is bronchoscopy.
• Treatment options vary from beta blockers like propranolol, local and systemic steroids, to surgical resection.  [5] .
Here, we present a case of an 8-year-old with intraluminal tracheal hemangioma presenting with respiratory distress to an academic tertiary center, which was managed with three sessions of angioembolization.
This manuscript was prepared following the Surgical CAse REport (SCARE) guidelines [7] .

Case presentation
An 8-year-old boy without any documented medical or surgical history was admitted to a tertiary academic center with the chief complaint of progressive dyspnea. His parents mentioned that he used to suffer from postbreastfeeding cyanosis in infancy and also after crying. In the years after infancy and before school age the patient's symptoms were described as exertional dyspnea becoming worse by exercising and walking accompanied by fast heartbeats and increased shallow breathes. As the patient had reached the school age, his dyspnea had become more severe with stridor heard while breathing. His dyspnea and stridor had progressed in the last 3 months. He had not experienced fever, chest pain, weight loss, and coughing in the course of his illness.
In general appearance, he was a child with a height of 125 cm and a weight of 27 kg, and physical examination revealed tachycardia (heart rate: 120), tachypnea (respiratory rate: 30), mild hypotension ( blood pressure: 90/70), and low oxygen saturation (Spo 2 : 85%). Laboratory results were normal and no anemia or leukocytosis was seen.
The patient underwent a rigid bronchoscopy. Bronchoscopy findings included normal tracheal mucosa and a soft tissue mass that could be compressed with the bronchoscope. Afterward, a neck computed tomography (CT) scan was performed revealing a vascular mass behind the larynx and cervical trachea (Figs. 1-3). Subsequently, surgery is performed for investigating the mass in the patient's neck. It was reported during the operation that the mass is of a vascular nature but resection could not be done. A tracheostomy was performed by the surgeon in the same session.
He was transferred to the radiology department for an MRI with contrast. At MRI, a multiloculated nodular lesion in the intraluminal part of the trachea and also partly in the infra-glottic area, measuring 12 × 8 mm and taking up half of the tracheal lumen, was found. The lesion was hypo-signal in the T1 sequence, hyper-signal in the T2 sequence, and heterogeneous after the contrast injection (Figs. 4 and 5). MRI findings confirmed the diagnosis of hemangioma.
Three sessions of angioembolization were carried out for the patient, and the patient's progress was controlled with MRI. Finally, after hemangioma atrophy tracheostomy was extracted.
He was given guaifenesin every 8 h and discharged from the hospital. At 3 month follow-up, the patient's symptoms were significantly improved and repeated bronchoscopy did not demonstrate any recurrence.

Discussion
Hemangioma is a soft tissue tumor caused by hyperplasia of the endothelial cells. Hemangiomas are either congenital or infantile. Infantile hemangiomas appear in the first 8 weeks of birth and grow rapidly until 6-12 months of age, and then they usually shrink in a natural and physiological way and disappear completely. Congenital hemangiomas; however, are fully developed lesions at the time of birth [8] . Hemangiomas are classified by the extent of tissue involvement as focal, segmental, and indeterminate, and by the depth of the tissue affected as deep, superficial, and mixed [8] .   Tumors of the tracheobronchial tree are a rare concept in children and are divided in two groups of benign and malignant lesions. The most common benign tumors of the tracheobronchial tree in children are hemangiomas and squamous papilloma [9] . Various factors may lead to the formation of these hemangiomas consisting of hormonal imbalance in pregnancy, infections, arteriovenous malformations, and vascular growth factors alteration [10] .
The low prevalence of this disease causes a delay in its diagnosis, as seen in this case. The presence of a mass in the trachea can cause symptoms such as stridor, cough, shortness of breath exacerbated by exercise, and hemoptysis (in the case of hemangiomas) [11] . A tracheal hemangioma can present as intractable asthma and may lead to respiratory failure and even death in severe cases.
As the signs and symptoms of a tracheal hemangioma are not specific to the disease the initial diagnostic tool used for finding the etiology is a chest radiograph as in all the respiratory diseases. CXR is commonly not diagnostic for the disease but may exclude other differential diagnoses. Further diagnostic tools that have been proven to have appropriate sensitivity and specificity in diagnosing the lesion are the CT scan, MRI, and bronchoscopy [9] . Rigid bronchoscopy and flexible endoscopy are used to retrieve samples and histopathologic assessments confirm the diagnosis; however, some studies start the treatment with imaging findings and do not use pathologic samples for confirmation [9] .
The case presented in the current study is a display of a tracheal mass presenting itself with respiratory distress and severe dyspnea.
We used three databases of PubMed, Scopus, and Web of Science to conduct a literature review. Keywords used were 'Tracheal Hemangioma' and 'Hemangioma of Trachea' and all studies from 1 January 2013 to 5 March 2023 were included initially. The initial number of articles included was 368, which was then reduced to 216 after deleting the duplicates. After reviewing the remaining articles, irrelevant articles (articles discussing nontracheal hemangiomas, nonhemangioma tumors mimicking hemangiomas, subglottic hemangiomas not radiating to the trachea, cancer treatments, and articles not reporting cases of the issue) were excluded. The remaining articles were assessed for eligibility to be included in this study and 20 articles were excluded due to the age of the patients, as only pediatric case series or case repots are included in the current study. Although one study was conducted on patients with subglottic hemangiomas with reported tracheal stenosis, with the aim of investigating their perioperative airway managements, it did not specify the exact location of the hemangioma and the number of patients with tracheal hemangioma was not reported, hence the study was excluded from this review [12] . Finally, 15 studies were included in the review including 12 case reports and 3 case series. The summary of the studies are displayed in Table 1.
To  (1/19). It should be noted that one case suffered from severe prematurity and respiratory distress syndrome at birth, hyperbilirubinemia, and laryngomalacia at the same time. Two studies did not mention the past medical history of the patients and six patients did not have any comorbidities or prior medical conditions. Among 13 patients whose signs and symptoms were recorded, the most common signs and symptoms were stridor 69.23% ( 9/13), respiratory distress 38.46% (5/13), and hemoptysis 23.07% (3/13). Other complaints consisted of dyspnea, chronic coughing, recurrent respiratory infections, and sporadic fascial and cervical hemangiomas. The diagnostic tools used in all studies were mainly a CT scan, an MRI, bronchoscopy, and histopathologic assessments for confirmation. One study confirmed the accuracy of the 3d-CT/bronchoscopy diagnosis for the tumor.
Only one case series did not specify the treatment option used for the patient with tracheal hemangioma. The main treatment option observed in the studies was the use of Propranolol 61.11 (11/18), which in 10 patients led to improvement of the signs,    [19] Case report 12 months/ Male Respiratory distress

Dyspnea and stridor
Biphasic stridor increasing in supine position/ Posterior cervical hemangioma.
Flexible bronchoscopy showed a multilobulated subglottic hemangioma obstructing more than 70% of the tracheal lumen. MRI revealed an angiomatous malformation.
Not mentioned Systemic and local steroids Laser ablation at 5 years old.

None
The patient was followed for 14 years and was symptom free at that age. None M. A. Özgül, et al (Turkey) [20] Case report 12 years old/ Male None Recurrent hemoptysis since 2 years ago.

No significant findings
CT scan of thorax revealed a polypoid lesion on the left lateral wall of the proximal trachea Rigid bronchoscopy demonstrated a polypoid lesion with pedicle on the proximal trachea.

None
The patient was followed 6 months later and was doing well.

Tachypnea
Stridor more prominent on inspiratory phase bronchial wheezing.

Direct laryngoscopy and MRI
were carried out and both revealed a soft, subocclusive lesion obstructing almost all the laryngo-tracheal airway.
Subglottic hemangioma protruding and obstructing the trachea.

Not mentioned
The patient was initially treated with nebulized epinephrine and steroids With the aim of treating the respiratory distress and after the definite diagnosis she was started on propranolol 1 mg/kg/day (increased to2 mg/kg/day later).

None
The patient was fully recovered 1 year later; however, the treatment was continued for 2 years. None L. A.
Petrauskas, et al (USA) [22] Case report 6 weeks/ Not mentioned PHACE syndrome Stridor Severe biphasic stridor Retro-sternal retractions and respiratory distress Right sided facial hemangioma that extended to the right chest and arm.
Rigid bronchoscopy revealed a circumferential subglottic hemangioma, along the posterior tracheal wall to the carina.

None
The patient was followed for 8 months and was doing well until then.
None D. Y. Yang, et al (China) [23] Case report 9 months/ Male None Dyspnea Wheezing and severe respiratory distress.
High resolution computed tomography (HRCT) revealed a round lesion in the middle segment of trachea With filling defect of trachea Abdominal CT also showed the same lesion in the liver Bronchoscopy revealed a mass full of vessels in the middle of the trachea, originating from the right wall , which blocked almost all the airway. After several days of treatment with propranolol and seemingly reduction in the hemangioma, the patient developed dyspnea and fever.
On follow-up CT scan was done day 17 of the treatment which showed reduction size of the lesion, however later it was discovered that the lesion had increased in size again After surgical excision of the hemangioma bronchoscopy was done 1 weeks and 3 months later showing complete improvement.
None Y. Fujita, et al (Japan) [24] Case report 1 month/ Female None Severe dyspnea Stridor and severe respiratory distress.
Bronchofiberscopy revealed bulging in the subglottic tracheal membrane Primary neck MRI showed a hyper-signal (T2) compressed mass Hemangioma or Lymphangioma Exact diagnosis was not decided.
Not mentioned Intubation No specific treatment was done for the subglottic mass and the lesion was reduced in size naturally.
None Patient was followed for 5 months and did not show any symptoms. CT scan of the chest showed a polypoid lesion on the right anterolateral wall of the proximal trachea Bronchoscopy confirmed the CT scan findings The second bronchoscopy after 2 months of treatment with propranolol indicated the failure of the beta blocker treatment in reducing the size of the mass Histopathologic assessments confirmed the diagnosis of hemangioma(9 mm vascular proliferation).

Tracheal hemangioma 9 mm
Treatment with beta blockers was initially started which did not succeed The tumor was finally removed by electrical excision.

None
The patient was followed 24 months later with complete improvement and no hemoptysis Control bronchoscopy was not done.
None symptoms, and the size of the tumor. There was a failure of the propranolol regimen in one study, which led to electrical excision of the lesion. Laser ablation, steroids therapy, and surgical removal of the lesion were reported in 27.77% ( 5/18), 16.66 (3/18), and 16.66 (3/18), respectively. Natural shrinkage of the tumor was reported in one case. There was no report of a tracheal hemangioma from Iran in the past 10 years, therefore making this case report the only reported tracheal hemangioma in the past 10 years in Iran.
The study searching was conducted by two separate individuals; hence, improving the accuracy of the search results. All included studies were obtained in full-text form and were in English language; hence, no exclusion was needed in this part.
The limitations of this literature review include a publication bias as all studies are case reports and series. The results of this study depend on the quality of the literature search.

Conclusion
Tracheal hemangiomas are a rare concept, especially in children. These tumors present with stridor, progressive respiratory distress, dyspnea, hemoptysis, and chronic coughs. Propranolol has been reported to have great efficacy in improving the symptoms and reducing the size of the lesion. Systemic and localized steroids, surgical removal, and laser ablation are other successful methods for treatment. It is not common for a tracheal hemangioma presenting with severe symptoms to shrink naturally. Although there is not sufficient literature, based on this literature review, recurrence is not a common finding after the mentioned treatments as none of the cases reported showed recurrence. A close follow-up ranging from 3 months to 1 year is recommended.

Ethical approval
NA.

Consent
No personal information of patients have been revealed in the study.

Sources of funding
None.

Funding
None.