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Review
. 2022 Nov;51(10):888-896.
doi: 10.1111/jop.13297. Epub 2022 Apr 6.

Pediatric vascular anomalies with airway compromise

François Gorostidi  1 , Nicolas Glasson  1 , Victoria Salati  1 , Kishore Sandu  1
Affiliations
Review

Pediatric vascular anomalies with airway compromise

François Gorostidi et al. J Oral Pathol Med. 2022 Nov.

Abstract

Vascular anomalies are rare lesions of diverse nature that may affect the head and neck region. Any mass in or around the upper airway has the potential to obstruct or compromise it. The absolute priority, before etiologic treatment, is the evaluation of the risk for the airway and its management. Prenatal diagnosis of an upper airway obstruction requires a planned delivery in a center having a specialized team experienced in managing a compromised feto-neonatal airway, and who could perform an ex-utero intrapartum treatment to secure the airway. Even after birth, the airway remains central in the patient's overall management. Signs and symptoms of airway compromise must be evaluated keeping in mind the specific requirements of infants and small children and being aware that rapid worsening may occur. The treatment is then tailored to the patient and his lesion with the goal of improving symptoms while avoiding treatment-related complications. Maintaining reasonable expectations by the patient and families are part of a successful management. Cure is achievable for small and localized lesions, but symptom relief and mitigation of functional, esthetic and psychological impairments is the goal for large and complex lesions. If a tracheotomy was required, decannulation is one of the primary management goals.

Keywords: airway management; lymphatic malformation; pediatric airway; subglottic hemangioma; tracheotomy; vascular anomaly; vascular malformation; vascular tumor.

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Conflict of interest statement

None.

Figures

FIGURE 1
FIGURE 1
(A) Fetal MRI showing a large lymphatic malformation with high suspicion of airway compromise (deviation of the airway, floor of mouth and base of tongue involvement). (B) newborn intubated after the EXIT procedure. (C) axial MRI view of the lymphatic malformation at 3 months of age with deviation of the airway to the right. Despite massive cervical involvement, the case was managed without the need for a tracheotomy
FIGURE 2
FIGURE 2
Three‐months‐old child which presented with a biphasic stridor, and signs of respiratory distress without cyanosis. (A) 0° rod lens telescope view showing a smooth submucosal subglottic mass with 90% obstruction of the lumen suggestive of subglottic hemangioma. Propanolol was started immediately, and non‐invasive ventilation could be weaned off after 48 h. (B) 0° rod lens telescope view showing almost complete response after 6 months of treatment. A remnant of the hemangioma obstructing only 10% of the lumen is still visible
FIGURE 3
FIGURE 3
Two‐years‐old child with a left cervical macro‐microcystic lymphatic malformation with mediastinal extension. The child suffered of severe obstructive sleep apnea syndrome necessitating non‐invasive ventilation and had a neck exploration prior to his referral to our clinic. (A) external view, (B) 0° rod telescope view showing extrinsic pharyngeal obstruction by the lymphatic malformation
FIGURE 4
FIGURE 4
Same child as in Figure 3. A (axial), B (sagittal) and C (coronal) preoperative MRI at 2 years of age. The mixed lymphatic malformation insinuates between cervical plans and causing moderate–severe degree of extrinsic airway obstruction and mediastinal extension. (D) Axial MRI at 7 years of age, following surgery and intralesional sclerotherapy with Bleomycin
FIGURE 5
FIGURE 5
Two years old child with a pharyngolaryngeal macro‐microcystic lymphatic malformation (LM) necessitating a tracheotomy at 3 weeks of life. (A and B) endoscopic status after 6 sessions of sclerotherapy with ethanol leading in a 70% decrease of size of the lesion. A large lesion of the base of the tongue extending to the supraglottis, especially the right side of the epiglottis and right aryepiglottic fold leading to severe airway obstruction. (C) result after CO2 laser supraglottoplasty. After several more endoscopic treatment including base of the tongue resection and epiglottopexy, the child was eventually decannulated
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