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1. The histamine analogue clobenpropit modulates IRF7 phosphorylation and interferon production by targeting CXCR4 in systemic lupus erythematosus models

Author

Nassima Bekaddour, Nikaïa Smith, Birgit Caspar, Severine Grinberg, Stephane Giorgiutti, Vincent Rodeschini, Stephanie Dupuy, Nicolas Leboulanger, Darragh Duffy, Pauline Soulas-Sprauel, Vincent Gies, Anne-Sophie Korganow, Sébastien Nisole, and Jean-Philippe Herbeuval

Subjects
interferons, pDc, SLE, CXCR4, pIRF7, Immunologic diseases. Allergy, RC581-607
Abstract

IntroductionSystemic lupus erythematosus (SLE) is an autoimmune disease characterized by an overactive immune response, particularly involving excessive production of type I interferons. This overproduction is driven by the phosphorylation of IRF7, a crucial factor in interferon gene activation. Current treatments for SLE are often not very effective and can have serious side effects.MethodsOur study introduces clobenpropit, a histamine analogue, as a potential new therapy targeting the CXCR4 receptor to reduce IRF7 phosphorylation and subsequent interferon production. We employed various laboratory techniques to investigate how clobenpropit interacts with CXCR4 and its effects on immune cells from healthy individuals and SLE patients.ResultsClobenpropit binds effectively to CXCR4, significantly inhibiting IRF7 phosphorylation and reducing interferon production. Additionally, clobenpropit lowered levels of pro-inflammatory cytokines in a mouse model of lupus, demonstrating efficacy comparable to the standard treatment, prednisolone.DiscussionThese results suggest that clobenpropit could be a promising new treatment for SLE, offering a targeted approach with potential advantages over current therapies.

Published
2024
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2. Juvenile onset recurrent respiratory papillomatosis: What do we know in 2024 ?

Author

Charles Lepine, Nicolas Leboulanger, and Cécile Badoual

Subjects
HPV, Papillomavirus, Papillomatosis, Airway, Children, Head and neck, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Juvenile onset recurrent respiratory papillomatosis is a lifelong benign squamous lesion associated with HPV infection, particularly HPV6 and HPV11 genotypes. These lesions are rare, but can lead to laryngeal obturations, which can cause disabling dyspnea, or transform into squamous cell carcinoma. The aim here is to provide an epidemiological, biological and clinical overview of this pathology, particularly in children, in order to understand the issues at stake in terms of research and the development of medical and therapeutic management tools.

Published
2024
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3. Craniofacial growth and function in achondroplasia: a multimodal 3D study on 15 patients

Author

Anne Morice, Maxime Taverne, Sophie Eché, Lucie Griffon, Brigitte Fauroux, Nicolas Leboulanger, Vincent Couloigner, Geneviève Baujat, Valérie Cormier-Daire, Arnaud Picard, Laurence Legeai-Mallet, Natacha Kadlub, and Roman Hossein Khonsari

Subjects
Achondroplasia, FGFR3, Sleep apnoea, Geometric morphometrics, Cephalometrics, Principal component analysis, Medicine
Abstract

Abstract Background Achondroplasia is the most frequent FGFR3-related chondrodysplasia, leading to rhizomelic dwarfism, craniofacial anomalies, stenosis of the foramen magnum, and sleep apnea. Craniofacial growth and its correlation with obstructive sleep apnea syndrome has not been assessed in achondroplasia. In this study, we provide a multimodal analysis of craniofacial growth and anatomo-functional correlations between craniofacial features and the severity of obstructive sleep apnea syndrome. Methods A multimodal study was performed based on a paediatric cohort of 15 achondroplasia patients (mean age, 7.8 ± 3.3 years), including clinical and sleep study data, 2D cephalometrics, and 3D geometric morphometry analyses, based on CT-scans (mean age at CT-scan: patients, 4.9 ± 4.9 years; controls, 3.7 ± 4.2 years). Results Craniofacial phenotype was characterized by maxillo-zygomatic retrusion, deep nasal root, and prominent forehead. 2D cephalometric studies showed constant maxillo-mandibular retrusion, with excessive vertical dimensions of the lower third of the face, and modifications of cranial base angles. All patients with available CT-scan had premature fusion of skull base synchondroses. 3D morphometric analyses showed more severe craniofacial phenotypes associated with increasing patient age, predominantly regarding the midface—with increased maxillary retrusion in older patients—and the skull base—with closure of the spheno-occipital angle. At the mandibular level, both the corpus and ramus showed shape modifications with age, with shortened anteroposterior mandibular length, as well as ramus and condylar region lengths. We report a significant correlation between the severity of maxillo-mandibular retrusion and obstructive sleep apnea syndrome (p

Published
2023
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4. French national diagnosis and care protocol (PNDS, protocole national de diagnostic et de soins): cystic lymphatic malformations

Author

Nicolas Leboulanger, Annouk Bisdorff, Olivia Boccara, Anne Dompmartin, Laurent Guibaud, Christine Labreze, Jacques Lagier, Bénédicte Lebrun-Vignes, Denis Herbreteau, Aline Joly, Julie Malloizel-Delaunay, Arnaud Martel, Stéphane Munck, Frédérique Saint-Aubin, and Annabel Maruani

Subjects
Cystic lymphatic malformations, Vascular anomalies, Complications, Treatment, Medicine
Abstract

Abstract Cystic lymphatic malformations (LMs) are rare chronic conditions which management differs according to the type (macrocystic LMs, microcystic LMs or both). Studies are lacking due to rarity of the pathology. We aimed to establish a French National Diagnosis and Care Protocol (PNDS: Protocole National de Diagnostic et de Soins), to provide health professionals with free open access synthesis on optimal management and care of patients with LMs ( https://www.has-sante.fr/upload/docs/application/pdf/2021-03/malformations_lymphatiques_kystiques_-_pnds.pdf ). The process included a critical review of the literature and multidisciplinary expert consensus. LMs are congenital but are not always discovered at birth. Nearly 75% of them are located in the head and neck because of the highly dense lymphatic system in this region. Physical examination (showing painless masses with normal skin color and depressible consistency, or cutaneous/mucosal lymphangiectasia) and color Doppler ultrasonography, usually allow for diagnosis. MRI (involving T2 sequences with fat saturation in at least two spatial planes) is the tool of choice for evaluating anatomical extension, characterizing lesions (microcystic and macrocystic), and before considering therapeutic management. A biopsy, coupled to a blood sample, can also be used for molecular biology analyses, to search for activating mutations of the PIK3CA gene, particularly with LM integrating in a syndromic form (CLOVES or Klippel-Trenaunay syndrome) but also in certain isolated (or common) LMs. The spontaneous evolution of LMs, in particular microcystic forms, is often toward progressive aggravation, with an increase in the number of vesicles, thickening, increased oozing and bleeding, while pure macrocystic LMs may regress due to “natural sclerosis”, i.e. fibrosis secondary to an inflammatory reorganization after common infantile infections. In case of voluminous LMs or syndromic forms, functional and psychological repercussions can be major, deteriorating the patient’s quality of life. LMs must be treated by physicians integrated in multidisciplinary teams, and be personalized. Management is a life-long process that involves one or several of these therapies: conservative management, physical therapy (compression), sclerotherapy, surgery, drugs such as mTOR inhibitors (sirolimus), that has shown efficacy in decreasing the volume of LMs, and, more recently, PI3K-inhibitors in syndromic forms. Psychological and social support is necessary, taking into account the patient and his family.

Published
2023
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5. Histological Severity Risk Factors Identification in Juvenile-Onset Recurrent Respiratory Papillomatosis: How Immunohistochemistry and AI Algorithms Can Help?

Author

Charles Lépine, Paul Klein, Thibault Voron, Marion Mandavit, Dominique Berrebi, Sophie Outh-Gauer, Hélène Péré, Louis Tournier, Franck Pagès, Eric Tartour, Thomas Le Meur, Sylvain Berlemont, Natacha Teissier, Mathilde Carlevan, Nicolas Leboulanger, Louise Galmiche, and Cécile Badoual

Subjects
juvenile onset recurrent respiratory papillomatosis, machine learning, deep learning, p53, p63, HPV, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is a condition characterized by the repeated growth of benign exophytic papilloma in the respiratory tract. The course of the disease remains unpredictable: some children experience minor symptoms, while others require multiple interventions due to florid growth. Our study aimed to identify histologic severity risk factors in patients with JoRRP. Forty-eight children from two French pediatric centers were included retrospectively. Criteria for a severe disease were: annual rate of surgical endoscopy ≥ 5, spread to the lung, carcinomatous transformation or death. We conducted a multi-stage study with image analysis. First, with Hematoxylin and eosin (HE) digital slides of papilloma, we searched for morphological patterns associated with a severe JoRRP using a deep-learning algorithm. Then, immunohistochemistry with antibody against p53 and p63 was performed on sections of FFPE samples of laryngeal papilloma obtained between 2008 and 2018. Immunostainings were quantified according to the staining intensity through two automated workflows: one using machine learning, the other using deep learning. Twenty-four patients had severe disease. For the HE analysis, no significative results were obtained with cross-validation. For immunostaining with anti-p63 antibody, we found similar results between the two image analysis methods. Using machine learning, we found 23.98% of stained nuclei for medium intensity for mild JoRRP vs. 36.1% for severe JoRRP (p = 0.041); and for medium and strong intensity together, 24.14% for mild JoRRP vs. 36.9% for severe JoRRP (p = 0.048). Using deep learning, we found 58.32% for mild JoRRP vs. 67.45% for severe JoRRP (p = 0.045) for medium and strong intensity together. Regarding p53, we did not find any significant difference in the number of nuclei stained between the two groups of patients. In conclusion, we highlighted that immunochemistry with the anti-p63 antibody is a potential biomarker to predict the severity of the JoRRP.

Published
2021
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6. Severity of Retrognathia and Glossoptosis Does Not Predict Respiratory and Feeding Disorders in Pierre Robin Sequence

Author

Anne Morice, Véronique Soupre, Delphine Mitanchez, Francis Renault, Brigitte Fauroux, Sandrine Marlin, Nicolas Leboulanger, Natacha Kadlub, Marie-Paule Vazquez, Arnaud Picard, and Véronique Abadie

Subjects
Pierre Robin sequence, retrognathia, glossoptosis, respiratory disorders, feeding disorders, Pediatrics, RJ1-570
Abstract

Pierre Robin sequence (PRS) may lead to life-threatening respiratory and feeding disorders. With the aim to analyse the association of the severities of retrognathia and glossoptosis with those of respiratory and feeding disorders, we retrospectively studied a series of 50 infants with retrognathia, glossoptosis, cleft palate, and airway obstruction. The patients were managed from birth to at least 6 years of age by a single pediatric team at the Armand Trousseau Hospital in Paris within a 12 years period (2000–2012). Retrognathia and glossoptosis were graded in the neonatal period according to a specific clinical examination. Ventilation assistance was required for 32/50 (64%) patients, and enteral feeding for 41/50 (82%). The grades of retrognathia and glossoptosis and the severity of respiratory disorders did not differ between patients with isolated PRS and syndromic PRS. Severe respiratory disorders were more common and long-lasting feeding (>12 months) was more frequently required in patients with syndromic PRS compared with isolated PRS (42 vs. 13%, p = 0.04 and 42 vs. 4%, p < 0.01 respectively). Using univariate analysis, neurological impairments and laryngomalacia were associated with severe respiratory disorders [Odds ratio (OR) 5.0, 95% CI 1.3–19.6; and OR 14.6, 95% CI 1.3–161.4; p < 0.05] as well as with long-lasting feeding (>12 months) disorders (OR 18.6, 95% CI 3.9–89.2 and OR 20.4, 95% CI 3,4–122.8; p < 10−2). Syndromic SPR status was also associated with severe respiratory disorders (OR 4.9, 95% CI 1–32.5; p < 0.05). Using multivariate analysis, only syndromic PRS status was predictive for severe respiratory disorders (adjusted OR 8, 95% CI 1.47–44.57; p < 0.05); and only neurological impairments remained a significant risk for long lasting feeding disorders (>12 months) (adjusted OR 21.72, 95% CI 3.4–138.63; p < 10−2). The grades of retrognathia and glossoptosis were not predictive factors for the severity of respiratory and feeding disorders.Conclusion: In children with PRS, the severity of clinical conditions may not correlate with anatomic variables but rather with laryngeal abnormalities, neurological impairement and syndromic PRS status.

Published
2018
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7. Unusual presentation of a first Branchial cleft cyst associated with an abnormal bony canal -a case report

Author

Amanda Fanous, E-N Garabedian, P. Gorphe, Louise Galmiche, Nicolas Leboulanger, Vincent Couloigner, Françoise Denoyelle, M. Alexandru, L. Coffinet, and Thomas Blanc

Subjects
Male, Hearing Loss, Conductive, lcsh:Surgery, Case Report, Parapharyngeal Space, Robotic Surgical Procedures, First branchial, Pharyngeal groove, medicine, Humans, Cyst, Parapharyngeal, Branchial cleft cyst, Child, Cholesteatoma, Cholesteatoma, Middle Ear, Cysts, business.industry, Branchial Cyst, Pharyngeal Diseases, Parotidectomy, Anatomy, lcsh:RD1-811, medicine.disease, Magnetic Resonance Imaging, Facial nerve, Conductive hearing loss, Robotic, Facial Nerve, Otorhinolaryngology, Pharynx, Surgery, Branchioma, Tomography, X-Ray Computed, business, Ear Canal
Abstract

Background First branchial cleft anomalies are rare, accounting for only 10% of all branchial cleft anomalies. We report an even more rare and unique case of a branchial cleft cyst with features of both first and second arch derivatives. Case presentation A 6-year-old boy presented to us with a left conductive hearing loss associated with pre-tympanic keratin debris and an ipsilateral painful cervical mass. He had a past medical history of left ear surgery for presumed cholesteatoma 2 years prior and left neck abscess drainage 6 months prior. CT and MRI revealed a lesion originating in the external auditory canal and extending cervically through a bony canal located medial to the facial nerve and terminating as a parapharyngeal cyst. The complete removal was accomplished in one surgical stage consisting of three distinct steps: robotic assisted transoral resection of the pharyngeal cyst, an endaural approach and a parotidectomy approach. Conclusion We believe that our detailed description of this rare first branchial cleft cyst with pharyngeal extension, possibly a hybrid case between a first and second branchial cyst, can serve as a valuable tool to Otolaryngologists – Head and Neck Surgeons who come across a similar unusual presentations.

Published
2020
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9. How Imaging Can Help Surgeons Prepare for Second-Look Cholesteatoma Surgery in Children

Author

Robin Baudouin, François Simon, Raphaël Levy, Nicolas Leboulanger, Vincent Couloigner, Erea-Noël Garabedian, and Françoise Denoyelle

Subjects
Surgeons, Diffusion Magnetic Resonance Imaging, Otorhinolaryngology, Cholesteatoma, Middle Ear, Recurrence, Second-Look Surgery, Research Letter, Humans, Surgery, Child
Abstract

This research letter compares computed tomography and magnetic resonance imaging in the 1-year second-stage diagnosis of cholesteatoma.

Published
2022

10. Juvenile-Onset Recurrent Respiratory Papillomatosis Aggressiveness: In Situ Study of the Level of Transcription of HPV E6 and E7

Author

Charles Lépine, Thibault Voron, Dominique Berrebi, Marion Mandavit, Marine Nervo, Sophie Outh-Gauer, Hélène Péré, Louis Tournier, Natacha Teissier, Eric Tartour, Nicolas Leboulanger, Louise Galmiche, and Cécile Badoual

Subjects
Juvenile-onset recurrent respiratory papillomatosis, HPV 6, HPV 11, RNA, chromogenic in situ hybridization, lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens, lcsh:RC254-282, Article
Abstract

Simple Summary Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is a condition related to HPV 6 and 11 infection which is characterized by the repeated growth of benign exophytic papilloma in the respiratory tract of children. Disease progression is unpredictable leading sometimes to airway compromise and death. The aim of this study was to explore p16INK4a and expression of the RNA of HPV genes E6 and E7 with a chromogenic in situ hybridization (CISH) as biomarkers of JoRRP aggressiveness on a bicentric cohort of forty-eight children. CISH was scored semi-quantitatively as high (2+ score) and low (1+ score) levels of transcription of E6 and E7. Patients with a 2+ score had a more aggressive disease compared to those with a 1+ score. These data are a first step towards the use of biomarkers predictive of disease severity in JoRRP, this could improve the disease management, for example, by implementing adjuvant treatment at the early stages. Abstract Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is a condition related to HPV 6 and 11 infection which is characterized by the repeated growth of benign exophytic papilloma in the respiratory tract. Disease progression is unpredictable: some children experience minor symptoms, while others require multiple interventions due to florid growth. The aim of this study was to explore the biomarkers of JoRRP severity on a bicentric cohort of forty-eight children. We performed a CISH on the most recent sample of papilloma with a probe targeting the mRNA of the E6 and E7 genes of HPV 6 and 11 and an immunostaining with p16INK4a antibody. For each patient HPV RNA CISH staining was assessed semi-quantitatively to define two scores: 1+, defined as a low staining extent, and 2+, defined as a high staining extent. This series contained 19 patients with a score of 1+ and 29 with a score of 2+. Patients with a score of 2+ had a median of surgical excision (SE) per year that was twice that of patients with a score of 1+ (respectively 6.1 versus 2.8, p = 0.036). We found similar results with the median number of SE the first year. Regarding p16INK4a, all patients were negative. To conclude, HPV RNA CISH might be a biomarker which is predictive of disease aggressiveness in JoRRP, and might help in patient care management.

Published
2020

11. COVID-19 and ENT Pediatric otolaryngology during the COVID-19 pandemic. Guidelines of the French Association of Pediatric Otorhinolaryngology (AFOP) and French Society of Otorhinolaryngology (SFORL)

Author

Natacha Teissier, Anne-Laure Mansbach, A. Fanous, Neck Surgery Head, Nicolas Leboulanger, Charlotte Celerier, S. Pondaven, Briac Thierry, T. Sagardoy, Mohamed Akkari, Romain Luscan, François Simon, Vincent Couloigner, S. Ayari-Khalfallah, Richard Nicollas, Eric Moreddu, P. Fayoux, and Emmanuel Lescanne

Subjects
Suction (medicine), medicine.medical_specialty, medicine.diagnostic_test, business.industry, General surgery, Incidence (epidemiology), Context (language use), medicine.disease, Endoscopy, medicine.anatomical_structure, Otorhinolaryngology, Medicine, Surgery, Foreign body, business, Personal protective equipment, Nose
Abstract

Objective Joint guidelines of the French Pediatric Otolaryngology Society (AFOP) and of the French Society of otorhinolaryngology–head and neck surgery (SFORL) on the management of paediatric otolaryngology patients in the context of the COVID-19 pandemic. Methods A nation-wide workgroup drew guidelines based on clinical experience, national and local recommendations and scientific literature. Proposals may have to be updated on a day-to-day basis. Results In children, incidence of symptomatic COVID-19 (1–5%) is low and of good prognosis. The indications for nasal flexible endoscopy should be drastically limited. If undertaken, full Personal Protective Equipment (PPE) including FFP2 masks are required, as well as use of a sheath. Saline nose wash done by caregivers other than parents at home should require PPE. Unless foreign body tracheobronchial aspiration is clinically obvious, CT-scan should be performed to confirm indication of endoscopy. Surgical indications should be limited to emergencies and to cases that cannot be delayed beyond 2 months (especially endonasal, endopharyngeal laryngo-tracheobronchial procedures). Postponement should ideally be a group decision and recorded as such in the medical file. Surgical techniques should be adapted to limit the risk of viral dissemination in the air, avoiding the use of drills, microdebriders, monopolar cautery or lasers. Continuous suction should be placed near the operating field. In case of confirmed Covid-19 cases, or suspected cases (or in some centres systematically), PPE with FFP2 mask should be worn by all staff members present in the operating room.

Published
2020
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12. Life-Threatening Obstructive Sleep Apnea Caused by Adenoid Hypertrophy in an Infant with Noonan Syndrome

Author

Sonia Khirani, Nicolas Leboulanger, Adriana Ramirez, and Brigitte Fauroux

Subjects
Pediatrics, RJ1-570
Abstract

Adenoidectomy is a commonly performed surgery in children, even though its effectiveness is still under investigation. However, in children with risk factors such as age under 3 years old, associated comorbidities, or severe obstructive sleep apneas, a high postoperative respiratory morbidity is possible. We report the case of a 15-month-old boy with Noonan syndrome and a complex clinical history, who presented with a life-threatening obstructive sleep apnea due to hypertrophy of the adenoids which resolved completely after adenoidectomy.

Published
2012
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13. International Pediatric ORL Group (IPOG) Robin Sequence consensus recommendations

Author

Sam J. Daniel, Michael J. Rutter, Alan Cheng, Michelle Wyatt, Cory M. Resnick, Dimple Goel, Richard Nicollas, Carolyn R. Rogers-Vizena, John Russell, Reza Rahbar, Pierre Fayoux, Gi Soo Lee, An Boudewyns, George H. Zalzal, Alessandro de Alarcon, Nicolas Leboulanger, Eric Moreddu, Karthik Balakrishnan, Catherine K. Hart, Gregory C. Allen, Harlan R. Muntz, and Richard J.H. Smith

Subjects
Male, medicine.medical_specialty, Consensus, medicine.medical_treatment, Otolaryngology, 03 medical and health sciences, 0302 clinical medicine, Swallowing, 030225 pediatrics, medicine, Humans, In patient, Airway Management, 030223 otorhinolaryngology, Intensive care medicine, Robin Sequence, Pierre Robin Syndrome, business.industry, Infant, Newborn, Infant, General Medicine, Airway obstruction, respiratory system, medicine.disease, Airway Obstruction, Otorhinolaryngology, Expert opinion, Practice Guidelines as Topic, Pediatrics, Perinatology and Child Health, Female, Airway management, Pediatric otolaryngology, Human medicine, Airway, business
Abstract

Objective To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management. Conclusion Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.

Published
2020

14. International Pediatric Otolaryngology Group (IPOG): juvenile-onset recurrent respiratory papillomatosis consensus recommendations

Author

Riaz Y. Seedat, Shazia Peer, Marlene Soma, Douglas R. Sidell, Marilena Trozzi, Reza Rahbar, John Russell, Alessandro deAlarcon, Nicolas Leboulanger, Catherine K. Hart, Roger C. Nuss, George H. Zalzal, Sergio Bottero, An Boudewyns, Karen B. Zur, Richard Nicollas, Dana M. Thompson, Craig Derkay, Richard J.H. Smith, John Carter, Karthik Balakrishnan, Michael J. Rutter, Christopher J. Hartnick, Paolo Campisi, Alan Cheng, Seth M. Pransky, Eric Moreddu, Jean Michel Triglia, Harlan R. Muntz, Alejandro Cocciaglia, Julie E. Strychowsky, Pierre Fayoux, Claire M. Lawlor, and Michelle Wyatt

Subjects
medicine.medical_specialty, Consensus, MEDLINE, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Health care, Pediatric surgery, medicine, Humans, Child, 030223 otorhinolaryngology, Intensive care medicine, Referral and Consultation, Respiratory Tract Infections, Pulmonologists, Postoperative Care, Disease surveillance, Respiratory tract infections, business.industry, Papillomavirus Infections, General Medicine, Otorhinolaryngology, Chemotherapy, Adjuvant, Population Surveillance, Pediatrics, Perinatology and Child Health, Human medicine, Recurrent Respiratory Papillomatosis, business, Pediatric anesthesia
Abstract

Objectives To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. Results Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP. Conclusions Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.

Published
2020

15. Desmoid-type fibromatosis of the head and neck in children: A changing situation

Author

Hélène Boutroux, Nadège Corradini, Louise Galmiche, Aurore Coulomb, Vincent Couloigner, Erea-Noel Garabedian, Françoise Denoyelle, Marie-Julie Blouin, Veronique Minard-Colin, Antoine Paul, Thierry Van Den Abbeele, Daniel Orbach, Nicolas Leboulanger, Département d'hématologie [Gustave Roussy], Institut Gustave Roussy (IGR), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), and Hospices Civils de Lyon (HCL)

Subjects
Male, medicine.medical_specialty, Adolescent, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Antineoplastic Agents, Disease, 03 medical and health sciences, 0302 clinical medicine, Postoperative Complications, Biopsy, medicine, Bell Palsy, Humans, Parotid Gland, Head and neck, Child, Watchful Waiting, 030304 developmental biology, Retrospective Studies, 0303 health sciences, Chemotherapy, Palsy, medicine.diagnostic_test, business.industry, Fibromatosis, Infant, General Medicine, medicine.disease, Combined Modality Therapy, 3. Good health, Parotid gland, Surgery, Fibromatosis, Aggressive, Mandibular Neoplasms, medicine.anatomical_structure, Otorhinolaryngology, Tumor progression, Head and Neck Neoplasms, 030220 oncology & carcinogenesis, Child, Preschool, Pediatrics, Perinatology and Child Health, Disease Progression, Female, business
Abstract

Introduction Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies. Methods This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. All medical files were reviewed and their outcomes analyzed according to the initial therapies provided. Results Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. Conclusion HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

Published
2019
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16. Langerhans cell histiocytosis: therapeutic strategy and outcome in a 30-year nationwide cohort of 1478 patients under 18 years of age

Author

Abdellatif Tazi, Jean-François Emile, Yves Reguerre, Jean Donadieu, Khê Hoang-Xuan, Charlotte Rigaud, Yves Bertrand, Jean Miron, Caroline Thomas, Patrick Lutz, Mohamed Barkaoui, Claire Galambrun, Despina Moshous, Aurore Coulomb, Nathalie Aladjidi, Ludovic Mansuy, Virginie Gandemer, Corinne Armari-Alla, Anne Lambilliotte, Anne Deville, Geneviève Plat, Sébastien Héritier, Eric Jeziorski, Sylvie Fraitag, Nicolas Leboulanger, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hématologie et immunologie pédiatrique, Hospices Civils de Lyon (HCL)-CHU Lyon-Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL)-Hôpital Femme-Mère-Enfant (HFME), Service d'hématologie pédiatrique-oncologie, Hôpital Jeanne de Flandre [Lille]-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service d'Hémato-oncologie Pédiatrique, CHU Bordeaux [Bordeaux]-Hôpital Pellegrin, CIC - Bordeaux, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU de Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service d'Hématologie pédiatrique, Hôpital de la Timone, Marseille, Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service d'Hématologie et d'Oncologie Pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Hôpital de la Tronche, Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'Anatomie et cytologie pathologiques [CHU Pitié-Salpêtrière] (ACP), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut de Génétique et Développement de Rennes (IGDR), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), CHU Pontchaillou [Rennes], Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-CHU Pitié-Salpêtrière [AP-HP], Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], OncoNeuroTek [Paris], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-CHU Pitié-Salpêtrière [AP-HP], Service de pneumologie [Saint-Louis], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Pathology, Groupe hospitalier Ambroise Paré, Registre français des neutropénies chroniques sévères, Institut National de la Santé et de la Recherche Médicale, Association Histiocytose France, Association la Petite Maison dans la Prairie, PHRC 2001 CHU Nantes, French Ministry of Health, RMHE, GARDRAT family, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Service d'anatomie pathologique [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP]-Sorbonne Université (SU), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Centre National de la Recherche Scientifique (CNRS), Service de neurologie Mazarin, CHU Pitié-Salpêtrière [APHP], Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Hospices Civils de Lyon ( HCL ) -CHU Lyon-Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] ( IHOPe ), Hospices Civils de Lyon ( HCL ) -Hôpital Femme-Mère-Enfant (HFME), Hôpital Jeanne de Flandre [Lille]-Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Assistance Publique - Hôpitaux de Marseille ( APHM ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ), Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), CHU Félix Guyon, Service d'anatomie pathologique, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Institut Mondor de Recherche Biomédicale ( IMRB ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 ( UPEC UP12 ), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute ( ICM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ) -CHU Pitié-Salpêtrière [APHP], Hôpital de la Salpétrière, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ) -CHU Pitié-Salpêtrière [APHP]-Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ) -CHU Pitié-Salpêtrière [APHP], Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité ( CRESS (U1153 / UMR_A 1125) ), Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Institut National de la Recherche Agronomique ( INRA ), Service d'Anatomie et cytologie pathologiques = Service de Pathologie [CHU Pitié-Salpêtrière] (ACP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-CHU Pitié-Salpêtrière [AP-HP], Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], and Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)

Subjects
0301 basic medicine, Male, medicine.medical_specialty, Adolescent, [SDV]Life Sciences [q-bio], Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Refractory, Langerhans cell histiocytosis, Internal medicine, medicine, Humans, Cladribine, Child, [ SDV ] Life Sciences [q-bio], business.industry, historical comparisons, Age Factors, Infant, Newborn, Infant, Standard of Care, Hematology, medicine.disease, Combined Modality Therapy, Survival Analysis, Confidence interval, 3. Good health, Vinblastine, Surgery, Histiocytosis, Langerhans-Cell, 030104 developmental biology, Treatment Outcome, 030220 oncology & carcinogenesis, Child, Preschool, Population Surveillance, Cohort, Cytarabine, Female, France, business, Cohort study, medicine.drug, Follow-Up Studies
Abstract

International audience; The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15-year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine. A total of 483 (33%) patients were enrolled before 1998, and 995 (67%) after 1998. Five-year survival was 96·6% (95% confidence interval: 95·4–97·5%) overall, improving from 92% pre-1998 to 99% post-1998 (P \textless 0·001 adjusted to disease extent). This change was supported by an increase in 5-year survival from 60% to 92% in the RO+ group. Survival was particularly associated with cladribine and cytarabine among refractory RO+ patients. Disease reactivation was slightly less frequent after 1998, due to better enrolment of single-system patients, extended therapy duration, and more efficient second-line therapy. The crude rates of endocrine and neurological sequelae (the most frequent sequelae) appeared to improve over time, but this difference was not observed when the analysis was stratified by disease extent

Published
2016
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17. Continuous positive airway pressure titration in infants with severe upper airway obstruction or bronchopulmonary dysplasia

Author

Nicolas Leboulanger, Sabrina Aloui, Arnaud Picard, Adriana Ramirez, Brigitte Fauroux, Sonia Khirani, S2A santé Île-de-France, S2A Santé, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Oxygénothérapie, ADEP assistance, INSERM U955, équipe 13, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), The research of Brigitte Fauroux is supported by the Association Française contre les Myopathies (AFM), Vaincre la Mucoviscidose (VLM), Assistance Publique-Hôpitaux de Paris, Inserm, Université Pierre et Marie Curie-Paris 6, ADEP Assistance, S2A Santé, and IP Santé Domicile., and BMC, Ed.

Subjects
Male, [SDV.MHEP.PHY] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], medicine.medical_treatment, Continuous positive airway pressure, Critical Care and Intensive Care Medicine, Severity of Illness Index, 03 medical and health sciences, 0302 clinical medicine, Severity of illness, Respiratory muscle, [SDV.MHEP.PHY]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], Humans, Medicine, Single-Blind Method, 030212 general & internal medicine, Bronchopulmonary Dysplasia, business.industry, Continuous positive airway pressure titration, Research, Oesophageal pressure, Infant, Airway obstruction, medicine.disease, nervous system diseases, respiratory tract diseases, 030228 respiratory system, Bronchopulmonary dysplasia, Anesthesia, Breathing, Female, Expiratory abdominal activity, business, therapeutics, circulatory and respiratory physiology
Abstract

International audience; INTRODUCTION: Noninvasive continuous positive airway pressure (CPAP) is recognized as an effective treatment for severe airway obstruction in young children. The aim of the present study was to compare a clinical setting with a physiological setting of noninvasive CPAP in infants with nocturnal alveolar hypoventilation due to severe upper airway obstruction (UAO) or bronchopulmonary dysplasia (BPD). METHODS: The breathing pattern and respiratory muscle output of all consecutive infants due to start CPAP in our noninvasive ventilation unit were retrospectively analysed. CPAP set on clinical noninvasive parameters (clinical CPAP) was compared to CPAP set on the normalization or the maximal reduction of the oesophageal pressure (Poes) and transdiaphragmatic pressure (Pdi) swings (physiological CPAP). Expiratory gastric pressure (Pgas) swing was measured. RESULTS: The data of 12 infants (mean age 10 ± 8 mo) with UAO (n = 7) or BPD (n = 5) were gathered. The mean clinical CPAP (8 ± 2 cmH₂O) was associated with a significant decrease in Poes and Pdi swings. Indeed, Poes swing decreased from 31 ± 15 cmH₂O during spontaneous breathing to 21 ± 10 cmH₂O during CPAP (P < 0.05). The mean physiological CPAP level was 2 ± 2 cmH2₂O higher than the mean clinical CPAP level and was associated with a significantly greater improvement in all indices of respiratory effort (Poes swing 11 ± 5 cm H₂O; P < 0.05 compared to clinical CPAP). Expiratory abdominal activity was present during the clinical CPAP and decreased during physiological CPAP. CONCLUSIONS: A physiological setting of noninvasive CPAP, based on the recording of Poes and Pgas, is superior to a clinical setting, based on clinical noninvasive parameters. Expiratory abdominal activity was present during spontaneous breathing and decreased in the physiological CPAP setting.

Published
2013
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18. Life-Threatening Obstructive Sleep Apnea Caused by Adenoid Hypertrophy in an Infant with Noonan Syndrome

Author

Brigitte Fauroux, Adriana Ramirez, Sonia Khirani, and Nicolas Leboulanger

Subjects
Pediatrics, medicine.medical_specialty, business.industry, medicine.medical_treatment, lcsh:RJ1-570, lcsh:Pediatrics, Case Report, General Medicine, medicine.disease, Muscle hypertrophy, Obstructive sleep apnea, Clinical history, Adenoidectomy, Respiratory morbidity, medicine, Noonan syndrome, business, Adenoid hypertrophy
Abstract

Adenoidectomy is a commonly performed surgery in children, even though its effectiveness is still under investigation. However, in children with risk factors such as age under 3 years old, associated comorbidities, or severe obstructive sleep apneas, a high postoperative respiratory morbidity is possible. We report the case of a 15-month-old boy with Noonan syndrome and a complex clinical history, who presented with a life-threatening obstructive sleep apnea due to hypertrophy of the adenoids which resolved completely after adenoidectomy.

Published
2012

19. Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery

Author

Gilles Roger, Marie Dupré, Erea-Noel Garabedian, Nicolas Leboulanger, Benoit Godey, Jean Donadieu, Philippe Bordure, Virginie Gandemer, Caroline Thomas, Service d'ORL pédiatrique et Chirurgie Cervico-faciale [CHU Trousseau], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Service d'ORL et de chirurgie maxillo-faciale [Rennes] = ENT Head and Neck Surgery [Rennes], CHU Pontchaillou [Rennes], Service d'hématologie clinique, Hôpital Hôtel-Dieu [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hématogoie pédiatrique, Institut de Génétique et Développement de Rennes (IGDR), Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Service d'oto-rhino-laryngologie (ORL), Hôtel-Dieu, Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], Centre de référence des Histiocytoses, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS), and De Villemeur, Hervé

Subjects
Male, MESH: Temporal Bone, 0302 clinical medicine, Langerhans cell histiocytosis, Temporal bone, Cerebrospinal Fluid Otorrhea, MESH: Otologic Surgical Procedures, 030223 otorhinolaryngology, MESH: Treatment Outcome, MESH: Cerebrospinal Fluid Otorrhea, Cholesteatoma, Middle Ear, medicine.diagnostic_test, Cholesteatoma, MESH: Infant, Sensory Systems, 3. Good health, Treatment Outcome, medicine.anatomical_structure, [SDV.MHEP.OS] Life Sciences [q-bio]/Human health and pathology/Sensory Organs, Child, Preschool, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, Steroids, Otologic Surgical Procedures, MESH: Tomography, X-Ray Computed, Ear Canal, Exploratory surgery, MESH: Ear Canal, medicine.medical_specialty, [SDV.IMM] Life Sciences [q-bio]/Immunology, MESH: Antineoplastic Agents, Phytogenic, MESH: Vinblastine, Vinblastine, Mastoid, MESH: Cholesteatoma, Middle Ear, 03 medical and health sciences, [SDV.MHEP.PED] Life Sciences [q-bio]/Human health and pathology/Pediatrics, MESH: Histiocytosis, Langerhans-Cell, 030225 pediatrics, Biopsy, medicine, otorhinolaryngologic diseases, Humans, Ear canal, [SDV.MHEP.OS]Life Sciences [q-bio]/Human health and pathology/Sensory Organs, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, MESH: Humans, business.industry, MESH: Child, Preschool, Infant, Temporal Bone, medicine.disease, Antineoplastic Agents, Phytogenic, MESH: Steroids, MESH: Male, Surgery, Histiocytosis, Langerhans-Cell, Otorhinolaryngology, MESH: Mastoid, Neurology (clinical), Tomography, X-Ray Computed, Complication, business, MESH: Female
Abstract

International audience; 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.

Published
2009
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