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Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Extensive lymphangioma presenting with upper airway obstruction
D M Hartl1, G Roger, F Denoyelle
1Department of Pediatric Otolaryngology-Head and Neck Surgery, Hopital d'Enfants Armand Trousseau, 26 avenue du Dr Arnold Netter, 75571 Paris Cedex 12, France.
Archives of Otolaryngology--Head & Neck Surgery
|November 14, 2000
Summary
Pediatric lymphangioma causing airway compression can be treated surgically. However, aggressive surgery may not improve outcomes for intrinsic airway involvement, suggesting less invasive options for these cases.
Area of Science:
- Pediatric Otolaryngology
- Surgical Oncology
- Respiratory Medicine
Background:
- Lymphangiomas are benign congenital tumors that can cause significant morbidity in children.
- Airway compromise due to lymphangioma is a critical concern, particularly when involving the tongue base, parapharyngeal space, or larynx.
- Management of pediatric lymphangioma with airway obstruction presents complex challenges.
Purpose of the Study:
- To evaluate the surgical outcomes of pediatric lymphangioma cases presenting with dyspnea due to airway compression.
- To identify prognostic factors influencing treatment success in these patients.
Main Methods:
- Retrospective review of 18 pediatric cases of lymphangioma with airway encroachment treated between 1983 and 1998.
- Surgical interventions included neck dissection, tracheotomy, V glossoplasty, cervicotomy, endoscopy, CO2 laser photocoagulation, and supraglottic laryngectomy.
- Outcome measures focused on residual disease, tracheotomy status, and persistent respiratory symptoms.
Main Results:
- A 50% rate of tracheotomy was required initially. Of those, 89% were decannulated after an average of 22 months.
- Residual lymphangioma was observed in 94% of patients postoperatively.
- Ten patients experienced persistent respiratory symptoms, while 6 remained asymptomatic.
Conclusions:
- External compression of the airway by cervical lymphangioma responds well to surgical intervention.
- Intrinsic involvement of the upper airway by lymphangioma appears to be a negative prognostic factor, with aggressive surgery offering limited benefit.
- Less aggressive, symptomatic management may be a viable alternative for patients with intrinsic airway involvement to avoid extensive surgical procedures.
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