Document Detail


Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery.
MedLine Citation:
PMID:  19169133     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: To describe secondary acquired cholesteatoma in patients previously treated for Langerhans cell histiocytosis (LCH). To focus on misleading symptoms suggesting LCH relapse. PATIENTS: This study involved 3 patients aged 12, 20, and 58 months whose conditions were diagnosed with LCH. All 3 had involvement of the mastoid and of the skin of the external ear canal (EAC) at diagnosis. They were treated with steroids and vinblastine. INTERVENTIONS: Serial computed tomographic (CT) scans and clinical follow-up. MAIN OUTCOME MEASURE: Exploratory surgery of the mastoid. RESULTS: Otorrhea recurred in all 3 patients at 24, 17, and 26 months, respectively, with difficulties to clinically identify a hole in the posterior part of the EAC. The otorrhea was considered a sign of a new occurrence of LCH, leading to systemic chemotherapy in 2 cases. A CT scan showed a defect in the posterior wall of the EAC and suggested cholesteatomatous invasion of the mastoid (2 true cholesteatomas and 1 precholesteatomatous case).Surgery (canal wall up mastoidectomies) successfully removed the cholesteatoma (bilateral in 2 cases) and reconstructed the bony defect using cartilage. Biopsies ruled out LCH recurrence. CONCLUSION: Secondary acquired cholesteatoma (through a bony defect of the EAC) may occur in patients previously treated for LCH. Recurrence of symptoms and bone destruction on CT may wrongly suggest LCH recurrence. Surgery allows removal of the cholesteatoma as well as EAC reconstruction and control biopsy.
Authors:
Gilles Roger; Marie Dupré; Nicolas Leboulanger; Benoit Godey; Caroline Thomas; Virginie Gandemer; Philippe Bordure; Erea-Noel Garabedian; Jean Donadieu
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology     Volume:  30     ISSN:  1537-4505     ISO Abbreviation:  Otol. Neurotol.     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-01-26     Completed Date:  2009-04-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100961504     Medline TA:  Otol Neurotol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  190-3     Citation Subset:  IM    
Affiliation:
ENT Department, Armand Trousseau Children's Hospital, University Paris VI, AP-HP, Paris, France. gilles.roger@trs.aphp.fr
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MeSH Terms
Descriptor/Qualifier:
Antineoplastic Agents, Phytogenic / therapeutic use
Cerebrospinal Fluid Otorrhea / etiology
Child, Preschool
Cholesteatoma, Middle Ear / etiology*,  surgery*
Ear Canal / pathology
Female
Histiocytosis, Langerhans-Cell / complications*,  drug therapy,  pathology*
Humans
Infant
Male
Mastoid / pathology
Otologic Surgical Procedures*
Steroids / therapeutic use
Temporal Bone / pathology*
Tomography, X-Ray Computed
Treatment Outcome
Vinblastine / therapeutic use
Chemical
Reg. No./Substance:
0/Antineoplastic Agents, Phytogenic; 0/Steroids; 865-21-4/Vinblastine

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