J.-M. Triglia

Congenital cysts and fistulas of the neck

This retrospective study describes a series of 191 children treated for congenital cysts and fistulas of the neck between 1984 and 1999 in the pediatric ORL Department of La Timone Childrens Hospital. Preauricular fistulas and cystic hygromas were not included. The anomalies in this series were classified as either malformations of the midline or malformations of laterocervical region. Malformations of the midline included the thyroglossal duct cysts (n=102) and dermoid cysts (n=21). The most common malformations of the laterocervical region were cysts and fistulas of the second cleft (n=37) followed by those of the first cleft (n=20),those of the fourth pouch (n=7), and thymic cysts (n=4). Diagnosis of malformations of the midline is usually straightforward. However, diagnosis of malformation of the laterocervical region can be problematic. Misdiagnosis often leads to inadequate treatment with recurrence and functional as well as cosmetic sequelae.

Middle ear implant in conductive and mixed congenital hearing loss in children

PURPOSE Active middle ear implant can be used in children and adolescents with congenital hearing loss. The authors report their experience with the semi implantable Medel Vibrant Soundbridge(®) (VSB) in the audiologic rehabilitation of such patients. METHODS In this retrospective study, audiological and surgical data of 10 children (10.5±4 years) implanted with 12 VSB in 2 tertiary cares ENT Departments were analysed. RESULTS Two children with bilateral external auditory canal (EAC) atresia and mixed hearing loss (mean air conduction (AC) thresholds=65dB HL) were bilaterally implanted. Eight children presented with microtia associated with EAC atresia bilaterally (n=3) and unilaterally (n=5). All of them had a conductive hearing loss in the implanted ear (mean (AC) thresholds were 58.75dB HL preoperatively). The Floating Mass Transducer was crimped on the long process of the incus (n=8) or on the suprastructure of the stapes (n=4). There were no intra- or postoperative surgical complications. All the children wore their implants after 5 weeks. Postoperative mean bone conduction (BC) thresholds were unchanged. The mean aided thresholds with VSB (four frequencies warble tones at 0.5, 1, 2 and 4 kHz) were 28dB HL (± 10). Word discrimination threshold in quiet conditions in free field with the VSB unilaterally activated was 50% at 38dB SPL (± 9). CONCLUSION The results indicate that satisfaction of the children and their parents is very encouraging but surgeons should be cautious with this new approach in relation to the pinna reconstruction and to possible risks to inner ear and facial nerve.

Middle ear implant for mixed hearing loss with malformation in a 9-year-old child

OBJECTIVE The aim was to report the results of the first case in France of pediatric auditory rehabilitation with a middle ear implant and to discuss the putative indications with this new therapeutic option in children. PATIENT AND METHODS A prospective study over 18 months on clinical and audiometric results after a middle ear implantation with a Vibrant Med-El(R) implant in a 9-year-old child with mixed hearing loss. RESULTS Postoperative unaided pure tone audiometry (PTA) was unchanged by the surgical procedure. After 18 months of implant use, the mean PTA loss in free-field warble tone audiometry was 33.75 dB and the intelligibility threshold was 30 dB. After 18 months of follow-up, the intelligibility threshold was improved by 25 dB in comparison with the preoperative results with two hearing aids. The implant worked perfectly well and the child did not show any complication during this period. CONCLUSION The reliability of the implant and the quality of the auditory results obtained in this case and in a limited number of cases in the world make the Vibrant Med-El(R) a new therapeutic option in hearing loss in children with bilateral auricular atresia.

Congenital nasal pyriform aperture stenosis: Elaboration of a management algorithm from 25 years of experience

INTRODUCTION Congenital nasal pyriform aperture stenosis (CNPAS) is a rare disease presenting with neonatal respiratory distress, often associated with other anomalies. MATERIALS AND METHODS This study reports the clinical and radiological characteristics of the patients managed in The Department of Pediatric Otolaryngology Head and Neck Surgery of La Timone Childrens Hospital in Marseille between 1988 and 2014. Pyriform aperture (PA) widths were measured on CT-scans, obtained by using hand calipers at the largest portion of the PA in a plan parallel to the Francfort plan. RESULTS 10 patients were included. Average PA width was 6.6mm, 5/10 patients presented with single central maxillary median incisor, 6/10 patients had associated abnormalities. 8 patients underwent a surgical intervention and 2 patients were medically managed. All the patients had satisfactory nasal airway permeability on late follow-up. A management algorithm was elaborated. CNPAS should be evoked when breathing difficulties are associated with impossibility of passing fiberscope or nasogastric tube at the nasal inlet. Craniofacial CT-scanning is necessary to make the diagnosis and look for associated abnormalities. Medical treatment associating nasal wash and decongestants should be performed. Surgical intervention is necessary when failure of the medical management. DISCUSSION AND CONCLUSIONS Our results were close to those found in the literature in terms of clinical characteristics, associated abnormalities and PA width. However, no objective criterion to decide whether a surgical intervention is necessary or not, has been established so far. The algorithm we propose offers guidelines from diagnosis to treatment, but the management should be adapted based on clinical tolerance.

Prenatal diagnosis of congenital dacryocystocele

Congenital bilateral dacryocystocele was diagnosed prenatally by ultrasonography in 3 female fetuses at 32.5 weeks gestation. After birth, first baby developed respiratory distress and was treated with endoscopic marsupialization of the cysts; the second baby had no respiratory symptoms and had spontaneous resolution of the cysts without surgery. The last one was expected to have a left dacryocystocele in US but the clinical examination after birth showed a bilateral lesion, with predominance on the right side and underwent an endoscopic marsupialization for nasal obstruction. Prenatal diagnosis with ultrasonography facilitated the education of the mothers and staff and helped minimize the risk of potential complications.

Value of systematic aetiological investigation in children with sensorineural hearing loss.

UNLABELLED Sensorineural hearing loss is the most common form of sensory impairment in children. As a precise aetiological diagnosis has major prognostic and management implications, it is useful to evaluate the contents of the aetiological investigation of sensorineural hearing loss in France. This article presents a retrospective review of professional practices by comparing the aetiological investigation of hearing loss in children with a cochlear implant and children without a cochlear implant. PATIENTS AND METHODS One hundred and seven children under the age of 18 years with unilateral or bilateral sensorineural hearing loss attending the paediatric ENT department for the first time between January 2007 and January 2009 were included in the study. Data from the clinical interview and all complementary investigations were analysed. RESULTS The various aetiologies of hearing loss were classified as genetic, acquired, or unknown in each of the two populations. Hearing loss was of unknown origin in 52% of the 87 non-implanted children and 15% of the 20 children with a cochlear implant. CONCLUSION This study demonstrates the heterogeneous practices in terms of aetiological investigation of sensorineural hearing loss as a function of the target population. A more systematic aetiological investigation was performed in children fitted with a cochlear implant, requiring multidisciplinary management. This study indicates the need to define a standard aetiological investigation for all children with sensorineural hearing loss.

Combined endonasal and neurosurgical resection of a congenital teratoma with pharyngeal, intracranial and orbital extension: Case report, surgical technique and review of the literature

OBJECTIVES This study reports a patient with a large teratoma involving the oropharynx, the nasopharynx and the left orbit, with intracranial extension. This case represents one of the first reported instances of such an association. A literature review reporting head and neck teratomas with intracranial involvement is also presented. CASE REPORT The authors describe a case of a neonate presenting with a huge teratoma causing respiratory distress due to upper airway obstruction. The child was operated on at 3 months of age with a combined neurosurgical and endonasal endoscopic-assisted approach. After more than 1-year follow-up, the child has no recurrence and no complications of surgery. DISCUSSION This type of teratoma is very rare and surgical morbidity is common. The diagnosis may be apparent before birth, which will facilitate the planning of respiratory management. The combined neurosurgical and otolaryngologic approach for a neonatal teratoma has not previously been described. Using an endoscopic-assisted approach for intranasal tumors removal in neonates and infants is a very rare surgical challenge. CONCLUSION Large nasopharyngeal teratomas of infancy with sinonasal and intracranial extension may be managed using a combined endoscopic-assisted endonasal and neurosurgical procedure.

Myringoplasty in Children for Tympanic Membrane Perforation: Indications, Techniques, Results, Pre- and Post-Operative Care, and Prognostic Factors

Purpose of the ReviewThe aim of this report is to identify the current relevant literature data on myringoplasty for chronic tympanic membrane perforation in children; to expose the different surgical techniques, traditional and recent techniques and to report their indications and results; and to address controversies of age in terms of timing of surgery and as a prognostic factor among many other factors.Recent FindingsMyringoplasty is an easy and reliable surgery when performed in children in order to repair tympanic membrane perforation. Reported success rate is classically between 32 and 95% while some series have 100% rates. Timing of surgery in terms of age is still controversial and varies among teams related to their own experience.SummaryMyringoplasty in children has high rates of anatomical and functional success when well indicated. Age has not been found to affect surgery results.

Laryngotracheal Stenosis in Children and Infants With Neurological Disorders: Management and Outcome.

Introduction: The goal of this retrospective study is to compare the management and outcome of surgical treatment of laryngotracheal stenosis in children and infants with and without an associated neurological disorder. Patients and Method: In a series of children operated on for subglottic stenosis (SGS), patients with an associated neurological disorder were identified. The following criteria were compared in children with and without neurological disease: grade of stenosis, age, technique (Crico-Tracheal Resection (CTR), Laryngo-Tracheo-Plasty (LTP) in single and 2 stage, laser), analyzing duration, preoperative tracheostomy, decannulation rate, preoperative gastrostomy, and number of days in intensive care unit and in hospital. Results: Two hundred twenty-three children were operated on for subglottic stenosis, of whom 68 (30.5%) had an associated neurological disorder. Some criteria were found to be statistically different between the 2 populations: mean age of 43 months in neurological population versus 13 months (P < .001). The distribution of the grades of SGS appeared similar in the 2 groups (P = .088), and the mean duration of stay in hospital and in ICU were not statistically different (respectively, P = .186 and P = .056) between the 2 groups; a 2-stage procedure was performed more frequently than 1-stage in the cases with associated neurological disorder—66.6% versus 36.5% (P = .013); the median duration of stenting was 20 days in those with neurological disease versus 12 (P = .021). Preoperative tracheotomy was noted in 75% of neurological patients versus 47.7% of the others (P < .001). The outcome was considered to be good (decannulation and no further treatment) following a single procedure in 82.4% of patients with neurological disorder, as against 86.5% of neurologically unimpaired subjects. The difference in outcome of surgery was not statistically different (P = .392) between the 2 groups. Discussion and Conclusion: It appears that subglottic stenosis in children with associated neurological disorder is not more severe than in neurologically normal patients. In three-quarters of the neurologically impaired cases, a preoperative tracheostomy was needed, but the rates of failure of postoperative decannulation are not statistically significant between the 2 groups. In our experience, 2-stage techniques are more often performed than single stage in this population in order to allow airway safety, for example after feeding. If properly managed, the final results are similar in the 2 populations.

Cirugía de las malformaciones congénitas cervicales infantiles

Las malformaciones congenitas del cuello pueden diferenciarse en aquellas que afectan a la linea media (quistes del conducto tirogloso, quistes dermoides y fisuras mentoesternales) y en las de asiento laterocervical. Estas ultimas pueden ser de origen branquial (hendiduras primera y segunda, bolsas tercera y cuarta, quistes timicos) o no (linfangiomas quisticos). Un grupo aparte lo forman las fistulas preauriculares. La extirpacion debe ser completa para evitar la recidiva, a excepcion de los linfangiomas quisticos y del tratamiento endoscopico de las cuartas bolsas.