Ear involvement in childhood Langerhans' cell histiocytosis

Abstract

Background Ear involvement (EI) in Langerhans' cell histiocytosis (LCH) occurs quite often. We reviewed the Italian pediatric population of 251 children with LCH diagnosed between 1982 and 1995, focusing on EI, to highlight the prevalence, the clinical presentation, the outcome during follow‐up, and the prognostic impact of otologic LCH. Methods EI was defined by chronic otorrhea and/or mastoid infiltration, external auditory meatus lesions, and middle/internal EI. The age at diagnosis, sex, system involved, organ dysfunction, treatment, disease control, and outcome were recorded. Results EI was noted at presentation in 34 children (13.5%). They had a younger age at diagnosis (p= .0013) and near totality of multisystem disease (93.8% of patients with EI). Among patients with multisystem disease, children with EI seemed to have a higher risk of poor response and a higher percentage of second line treatment (p= .003). Conclusions EI seems to identify patients with a particular disease behavior, which requires a more accurate evaluation at diagnosis, staging and treatment, and a strict follow‐up, considering the possibility of an unfavorable outcome. © 2000 John Wiley & Sons, Inc. Head Neck 22: 42–47, 2000.

Histiocitose das células de Langerhans: experiência de 16 anos

“…This manifestation is more common among children with multisystem disease; the presence of persistent otitis, refractory to common treatments, should raise the suspicion of LCH. 10,21 LCH often affects the hypothalamic-pituitary axis, and diabetes insipidus is the most common manifestation. Quite often, it manifests with a median of 10 to 12 months after the onset of the disease, but it may precede it or appear several years later.…”

Destruction of the vestibular organ by Langerhans’ cell histiocytosis

“…In the most detailed study available in the current literature, ear manifestation of LCH includes such heterogeneous symptoms as chronic otorrhea, otitis, mastoiditis, external auditory meatus lesions, preauricular swelling or Bezold's abscess [2].…”

Interdisziplinäre Therapie der Langerhans-Zell-Histiozytose im Kopf- und Halsbereich

“…des Mittel-bzw. Innenohrs nachgewiesen; 93,8% dieser 34 Patienten hatten eine "multiple system disease" mit schlechter Prognose [27]. Bei einer multizentrischen Auswertung aus England wurde bei 49 von 73 Kindern mit LCH eine Ohrbeteiligung mit Knocheninfiltration gefunden.…”

“…Dagegen haben verschiedene Multizenter-Analysen gezeigt, dass insbesondere bei Kindern mit "multi system disease" in 13,5-67,0% mit einer Beteiligung im HNO-Bereich gerechnet werden muss [1,2,20,27].…”