N. Leboulanger

Management specificities of congenital laryngeal stenosis: external and endoscopic approaches.

This study aimed to report our experience in the management of congenital laryngeal stenosis and to compare our series and results to published data in the literature.

Langerhans cell histiocytosis: therapeutic strategy and outcome in a 30‐year nationwide cohort of 1478 patients under 18 years of age

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 < 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.

Idiopathic bilateral vocal cord paralysis in infants: Case series and literature review.

Vocal fold paralysis is the second most common congenital laryngeal anomaly in newborns. Bilateral paralysis is a severe condition and often remains of unknown etiology. We report our experience of congenital idiopathic bilateral vocal fold paralysis in newborns and infants, and discuss the therapeutic options.

Pediatric sinogenic epidural and subdural empyema: The role of endoscopic sinus surgery

AIM To analyze the indications and outcomes of open neurosurgical approaches (ONA) and endoscopic transnasal approaches (ETA) in the surgical management of pediatric sinogenic subdural and epidural empyema. MATERIAL AND METHODS Retrospective single-center study design within a tertiary care referral center setting. Children less than 18 years of age consecutively operated on between January 2012 and February 2014 for drainage of a sinogenic subdural empyema (SE) or epidural (EE) empyema were included. MAIN OUTCOME MEASURES success of first surgical procedure, persistent symptoms and sequelae at the end of the follow-up period. RESULTS Nine SE (53%) and 8 EE (47%) were observed. Neurological symptoms, especially seizures, were more frequent in the SE group. Perioperative pus samples were positive in 67% of the SE group and in 75% of the EE group. The most frequently isolated bacteria belonged to the Streptococcus anginosus group. CT or MR imaging showed that most empyema probably originated from the frontal sinus. However, two cases resulted from an ethmoiditis and one case from a Potts puffy tumor, without any direct contact with the paranasal sinus. In cases of SE, the most effective surgical technique was ONA with craniotomy. Associated endoscopic sinus drainage was useful for the purpose of bacteriological diagnosis. In cases of EE, effectiveness was noted in both ONA and ETA techniques. In two cases of EE, the ETA procedure encompassed direct drainage of the empyema through the posterior wall of the frontal sinus (Draf III approach). The number of patients successfully treated after a single surgical procedure was higher in the EE group (p=0.05). Regarding outcomes, no mortalities were observed. Persistent disorders at the end of the follow-up period, especially headaches, cognitive, concentration or schooling problems, tended to be more frequent in the SE group than in the EE group (67% vs 29%), and were more commonly observed in cases requiring several surgical procedures (75% vs 12.5%) (p=0.05). DISCUSSION Endoscopic sinus surgery plays a critical role in the surgical management of pediatric sinogenic SE and EE. In cases of small volume EE, the endoscopic approach associated with antibiotherapy may be sufficient to treat the infectious process.

Head and neck teratoma: From diagnosis to treatment

INTRODUCTION Head and neck teratoma is a rare entity. Its prognosis mostly depends on the risk of neonatal respiratory distress, its extension and potential malignancy. Surgical management must be as complete as possible to avoid recurrences and malignant transformation. The authors present a retrospective analysis of 6 cervicofacial teratomas and a review of the literature. The aim of the study was to analyse prenatal, neonatal and postnatal management of teratoma. MATERIALS AND METHODS Charts of children presenting with a head and neck teratoma, managed by our maxillofacial and plastic surgery unit, were analysed and antenatal, clinical, biological, radiological and pathological characteristics were collected. Surgical treatment, recurrences and surgical outcomes were analysed. RESULTS Six patients were included: 2 with a cervical teratoma, 2 with a facial teratoma and 2 with intraoral teratomas. In 2 cases, the lesions were diagnosed antenatally and both patients required neonatal resuscitation. All the patients underwent early surgery, and 3 with complete excisions. All patients with an initial incomplete excision eventually presented a recurrence and therefore second look surgery. No malignant transformation was noted. CONCLUSION Early prenatal diagnosis is crucial to neonatal care. Early surgery and meticulous follow-up are critical in the long-term favourable outcome.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts

INTRODUCTION The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition. OBJECTIVE To provide recommendations for the diagnosis and management of type I laryngeal clefts. METHODS Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method. SETTING Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts. CONCLUSIONS This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

Outcome measures for pediatric laryngotracheal reconstruction: International consensus statement: Pediatric Airway Outcomes Consensus

Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies.

Velopharyngeal insufficiency managed by autologous fat grafting in patients with aberrant courses of internal carotid arteries

INTRODUCTION Velopharyngeal insufficiency (VPI) is usually managed, besides speech therapy, by performing a velopharyngoplasty. An alternative approach is autologous fat grafting (AFG) of the posterior pharyngeal wall. About 5% of the population has internal carotid arteries (ICA) with an aberrant course. This anatomic variation can be responsible for surgical difficulties while when performing a velopharyngoplasty, and therefore lead surgeons to only consider a speech reeducation of VPI. However, AFG is does not bear such surgical morbidity. OBJECTIVE The aim of this study is to retrospectively determine AFG efficiency on VPI in patients with aberrant ICAs courses who cannot benefit from a velopharyngoplasty, by comparing pre- and postoperative Borel Maisonny score (BMS) and intelligibility (Intell). METHODS We conducted a retrospective study in 2 centers, including children with VPI and aberrant ICAs courses who underwent an AFG of the posterior pharyngeal wall from 2004 to 2015, in addition to speech therapy. RESULTS Nine patients (4-11 years old) underwent the surgical procedure, 8 of them presenting a 22q11 microdeletion. All improved their VPI by AFG of the pharyngeal wall according to BMS and Intelligibility after a 10 months follow-up. The effect was stable after 3 years of follow-up. No severe complication (apnea, vascular injury) occured. CONCLUSION AFG of the pharyngeal wall, associated with speech therapy, seems to be a safe procedure for patients with VIP and aberrant ICAs courses. Multiple procedures are possible if needed.

Otogenic Temporomandibular Arthritis in Children

Importance Septic arthritis of the temporomandibular joint (SATMJ) is a very rare but potentially severe complication of pediatric middle ear infections because it presents risks of TMJ ankylosis. Objective To describe the clinical, radiological, biological, and microbiological characteristics and evolution of SATMJ complicating middle ear infections (otogenic SATMJ) in children. Design, Setting, and Participants This multicenter retrospective study included all children younger than 18 years referred between January 1, 2005, and December 31, 2015, for otogenic SATMJ or for TMJ ankylosis that occurred a few months to a few years after an acute mastoiditis. Nine children were included in the study. Review of the children’s medical charts was conducted from February 1, 2016, to April 1, 2016. Main Outcomes and Measures Patients’ demographic characteristics and symptoms; radiological, biological, and bacteriological findings, including reanalysis of initial imaging; and treatment and outcome of SATMJ. Results Of the 9 children, 6 were boys and 3 were girls; the mean age was 2.1 years (range, 6 months to 4.7 years). In 7 cases (78%), the primary middle ear infection was acute mastoiditis. Clinically, 5 children (55%) had preauricular swelling and only 1 (11%) had trismus. Associated thrombophlebitis of the lateral sinus or intracranial collections was present in 7 cases (78%). An initial computed tomographic scan was performed for all but 1 patient, and second-line analysis detected clear signs of TMJ inflammation in all 8 children who had a computed tomographic scan. However, SATMJ was diagnosed in only 3 cases at the time of the initial middle ear infection, leading to the recommendation of TMJ physical therapy for several months. The most frequently involved bacteria was Fusobacterium necrophorum, which was found in 4 cases. Long-term ankylosis was identified in 6 cases (67%), and 5 of these children required surgical treatment. Conclusions and Relevance Clinicians and radiologists must thoroughly look for signs of SATMJ in children with acute mastoiditis to detect this complication, which can lead to disabling and hard-to-treat TMJ ankylosis.

Metastatic Squamous-Cell Carcinoma of the Lung Arising in a 12-Year-Old Boy with Juvenile Recurrent Respiratory Papillomatosis of Neonatal Onset

Abstract Juvenile recurrent respiratory papillomatosis is the most common benign neoplastic disease of the larynx in children, characterized by numerous squamous papillomas caused by Human Papilloma Virus type 6 and 11. HPV is thought to be acquired at the time of vaginal delivery from maternal genital condylomas. Juvenile recurrent respiratory papillomatosis can be protracted by surgical interventions performed to avoid airway obstruction and extend below the vocal cords as far as the main stem bronchi. Lung involvement in Juvenile recurrent respiratory papillomatosis seems to be more prevalent than non-systematic reviews have reported until now and progression to cancer occurs in a significant proportion of these cases at a younger age than previously reported. This would suggest that closer attention should be paid to these children. We report a case of malignant transformation in a 12 year-old boy followed-up since the birth for an invasive juvenile recurrent respiratory papillomatosis with pulmonary involvement. The presence of HPV 6/11 was demonstrated by PCR analysis performed on material obtained from a metastatic vertebral lesion. ÖZ Juvenil tekrarlayan respiratuvar papillomatozis, Human Papilloma virüs tip 6 ve 11’in neden olduğu çok sayıda skuamöz papillomlarla karakterli, çocuklarda larenksin en sık görülen benign neoplastik hastalığıdır. HPV enfeksiyonunun vaginal doğum sırasında maternal genital kondilomdan kazanıldığı düşünülmektedir. Juvenil tekrarlayan respiratuvar papillomatozis’te havayolu tıkanıklığını ve vokal kordun altıdan ana bronkusa uzanımını önlemek için cerrahi girişimler uygulanmaktadır. Juvenil tekrarlayan respiratuvar papillomatozis’de akciğer tutulumu şimdiye kadar raporlanandan daha sıktır ve olguların çoğunda önceki raporlara göre daha erken yaşta kanser gelişimi görülmektedir. Bu nedenle, çocuklar yakın takibe alınmalıdır. Hastamız doğduğundan itibaren invaziv juvenil tekrarlayan respiratuvar papillomatozis tanısıyla takipli, akciğer tutulumu olup malign transformasyon gelişen 12 yaşında erkek çocuktur. HPV 6/11 varlığı vertebra metastazından alınan örnekte çalışılan PCR ile gösterilmiştir.