Klin Padiatr 2022; 234(05): 324-325
DOI: 10.1055/s-0042-1754462
Abstracts
Orals
Kasuistiken / Best-of-Abstracts

In severe juvenile-onset recurrent respiratory papillomatosis of a 10-year-old, systemic bevacizumab is highly effective and well tolerated

SS Dinges
1   Charité – Universitätsmedizin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany
2   Charité – Universitätsmedizin, Berlin Institute of Health, Berlin, Germany
3   Charité – Universitätsmedizin, Berlin-Brandenburg Center for Regenerative Therapies (BCRT), Berlin, Germany
,
A Coordes
4   Charité – Universitätsmedizin, Department of Otorhinolaryngology, Head and Neck Surgery, Berlin, Germany
,
SI Zabaneh
4   Charité – Universitätsmedizin, Department of Otorhinolaryngology, Head and Neck Surgery, Berlin, Germany
,
W Naumann
1   Charité – Universitätsmedizin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany
,
V Friedrich
2   Charité – Universitätsmedizin, Berlin Institute of Health, Berlin, Germany
5   Charité – Universitätsmedizin, Department of Neonatology, Berlin, Germany
,
LG Hanitsch
6   Charité – Universitätsmedizin, Institute of Medical Immunology, Berlin, Germany
,
D Grund
7   Charité – Universitätsmedizin, Department of Infectious Diseases and Respiratory Medicine, Berlin, Germany
,
MA Mall
1   Charité – Universitätsmedizin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany
2   Charité – Universitätsmedizin, Berlin Institute of Health, Berlin, Germany
8   German Center for Lung Research (DZL), Berlin, Germany
,
S Lau
1   Charité – Universitätsmedizin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany
,
S Dommerich
4   Charité – Universitätsmedizin, Department of Otorhinolaryngology, Head and Neck Surgery, Berlin, Germany
,
H von Bernuth
1   Charité – Universitätsmedizin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany
2   Charité – Universitätsmedizin, Berlin Institute of Health, Berlin, Germany
3   Charité – Universitätsmedizin, Berlin-Brandenburg Center for Regenerative Therapies (BCRT), Berlin, Germany
9   Labor Berlin GmbH, Department of Immunology, Berlin, Germany
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Introduction In juvenile-onset recurrent respiratory papillomatosis (JORRP), multiple benign tumors in the respiratory tract develop after an infection with human papillomavirus (HPV) 6 or 11. JORRP progresses from voice disturbance to airway obstruction, pulmonary lesions, and malignant transformation. Although being the most common laryngeal neoplasm, the prevalence is low (1.45 per 100,000). Diagnosis is often delayed.

The standard therapy is symptomatic repeated endoscopic ablation. Case reports suggest a therapeutic effect of systemic Bevacizumab.

Report We present a 10-year-old female patient with progressive aphonia since she began to talk, acute dyspnea, and a diagnosis of HPV 6-positive JORRP at the age of 2 years. After emergency intubations due to acute dyspnea, a tracheostomy tube was placed (3 years). Endoscopic sublaryngeal debulking and laryngeal laser ablation were conducted monthly (3–9 years), later every three weeks (9–10 years). At the end of each interval, severe dyspnea was relapsing.

Side effects from the ablations included laryngeal destruction (8 years), severe pneumonia (9 years), and a tracheal lesion with pneumomediastinum, tracheitis, and sinusbradycardia (9 years). The papilloma grew faster, at new locations, and per continuitatem deeper into the bronchial system. Bronchial obstruction resulted in bronchogenic cysts and parenchymal pulmonary destruction. Premalignant epithelial dysplasia developed.

Gardasil HPV vaccination, local Cidofovir, and local Bevacizumab and systemic Cidofovir, Nivolumab, and Interferon alpha had no effect. At 10 years of age, three weekly Bevacizumab infusions were started with success exceeding the expectations. For the first time, symptoms regressed and interoperative intervals were extended to more than six weeks (▶Fig. 1).

Discussion Awareness is needed to prevent delayed diagnosis. Symptomatic ablation is insufficient due to disease progression under therapy and side effects. Systemic Bevacizumab should be considered as adjuvant therapeutic providing high efficacy and good tolerance.

Zoom Image
Fig. 1 (a) Trachea prior to (b) and after second infusion of Bevacizumab.


Publication History

Article published online:
21 September 2022

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