S. Roman

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.

Primary cricotracheal resection in children: indications, technique and outcome

OBJECTIVEnTreatment of subglottic stenosis has benefited greatly from development of grafting techniques such as larygontracheoplasty. Meanwhile, cricotracheal resection of the stenotic area and a major part of the cricoid cartilage have been shown to give excellent results in adults.nnnPATIENTS AND METHODSnFrom June 1993 to June 2000, we performed cricotracheal resection, as the primary surgical procedure, for acquired (n=13), congenital (n=2) or mixed (n=l) subglottic stenosis in 16 pediatric patients comprising seven boys and nine girls. At the time of surgery, the mean weight was 16 kg and the mean age was 5 years. Degree of stenosis was classified as grade II in one case, grade III in 12, and grade IV in three. Eight patients underwent two-stage procedures with postoperative tracheostomy. A rolled reinforced silastic stent was placed for a mean period of 20 days and the tracheostomy tube was removed within a mean period of 44 days after stent removal. Eight patients underwent single-stage procedures. The Portex endotracheal tube was used for a mean period of 4 days.nnnRESULTSnAll patients underwent regular clinical and endoscopic postoperative examination. Mean follow-up was 38 months. No interference with laryngotracheal growth has been noted in any case, including the five with follow-ups longer than 5 years.nnnCONCLUSIONSnThe indications for laryngotracheoplasty and cricotracheal resection in children with subglottic stenosis are still unclear. Decisions must be made on a case-by-case basis. In this article the authors discuss decisional factors in terms of clinical findings, surgical techniques, potential complications, and outcome.

Primary pediatric neuroblastic tumors of the neck

Neuroblastic tumors are the third most common cause of solid tumors in early childhood. Cervical tumors account for only 5% of cases. In this report, we describe a series of four pediatric neuroblastic tumors of the neck. The histological diagnosis was ganglioneuroblastoma in three cases and neuroblastoma in one case. Presenting signs were solitary cervical mass in two cases and respiratory distress in association with Claude-Bernard Horners syndrome in two cases. Mean age at presentation was 15 months. Cervical computed tomography scan and/or magnetic resonance imaging depicted calcifications within the tumor in 50% of cases and allowed accurate assessment of extension. Increased urine catecholamine levels were observed only in the patient with neuroblastoma. Scintigraphy with [131]iodine-methyliodobenzylguanidine demonstrated selective uptake by the tumor in two cases. Amplification of N-myc oncogene, a documented unfavorable prognostic sign, was not found in any case. Surgical treatment was performed in all patients. Neoadjuvant chemotherapy was performed in one case. All patients underwent regular surveillance. No evidence of recurrence has been observed with a mean follow-up period of 7 years.

Head and neck manifestation and prognosis of Langerhans’ cell histiocytosis in children

OBJECTIVEnTo appreciate the several head and neck manifestations of Langherans cell histiocytosis (LCH) in children and their multidisciplinary management and outcome.nnnSTUDY DESIGNnRetrospective study.nnnPATIENTS AND METHODSnClinical reports of 42 patients with LCH treated in the Departments of Paediatric Haematology, Paediatric Oncology and Paediatric Otorhinolaryngology of a tertiary care center were analyzed. Only cases where the disease was localized to the head and neck were considered. The age at diagnosis, gender, clinical presentation, extension of disease as well as response to treatment and outcome were recorded from the charts of each of these patients.nnnRESULTSnOf the 42 patient charts reviewed, 31 (73.8%) presented with head and neck localization. 10 of these had an exclusive head and neck presentation. Multisystem LCH was mostly found in infants under 3-year-old (mean age: 2-year-old), and bony manifestations in older. All treatments delivered to patients were well-tolerated and the evolution good.nnnDISCUSSION AND CONCLUSIONnHead and neck involvement is known to be very frequent in LCH. There is no consensus about treatment but authors highlight that all teams in charge of patients presenting with LCH agree to remain as conservative as possible. For solitary large lesions looking like a tumor which resection could result in functional or cosmetic morbidity, it would be important to get first a biopsy. For multisystemic LHC, therapeutic trials with chemotherapy agents still in process should increase the rate of success.

Management of subglottic stenosis in infancy and childhood.

Abstract During the 12-year period between June 1987 and June 1999, 141 children underwent curative treatment for subglottic laryngeal stenosis at La Timone Children’s Hospital in Marseille, France. Ninety-six children (68%) were under the age of 5 years; 106 (75%) presented with acquired stenosis and 93 had narrowing involving over 70% of the subglottic lumen. Endoscopic laser surgery was performed in 25 cases and open surgery in 116. Open surgical techniques included laryngotracheoplasty with autologous cartilage interposition in 83 cases, laryngotracheal split in 22, and cricotracheal resection in 11. After decannulation, 132 children (94%) were able to breathe normally through the upper airway. Perspectives for development of new techniques and improvement of conventional methods are discussed.

Intralaryngeal manifestation of thyroglossal duct cyst

Laryngeal expression of a thyglossal duct cyst is extremely rare, only 10 cases were reported up to this time. All the cases were located either in the hyoid bone or in the supraglottal region. Moreover, all of them were reported in adults. This is the first case of thyroglossal duct cyst in a young child presenting as a pure noninflamed subglottal cyst. A 2-year-old boy was referred to our ENT consultation with laryngeal dyspnea. These respiratory troubles appeared progressively for 1 month after a common cold. The patient had an inspiratory dyspnea, could eat well, and had normal throat, ears, and nose. A little mass was noted on palpation of the larynx between the thyroid cartilage and the cricoid ring. The boy’s parents said that this “little pea” was always noted but its size never increased nor decreased. The flexible laryngoscopy performed during the initial consultation showed vocal cords and supraglottis free of anomaly. The subglottal region could not be seen well, but it seems that a mass was obstructing its lumen. An endoscopy with the patient under general anesthesia was then performed during the following days. An anterior and depressible mucosa-lined subglottal mass was found obstructing more than 80% of the lumen (Fig 1). The mucosa that covered the mass had a regular aspect and was free of hyperhemia. Because there was an extralaryngeal component noted during the physical examination, it was decided to perform a CT-scan. A typical cystic well-delimited lesion was visualized through the cricothyroid membrane (Fig 2). A surgical removal with an external approach was decided on and performed. The cricoid ring was heavily eroded by the cyst that could be separated from the subglottal mucosa that was not breached. It was noted during the dissection that the cyst had a tract leading superiorly to the body of the hyoid bone. The mass was then removed with the central part of the hyoid bone and a complete Sistrunk procedure was performed. The histologic features were consistent with a benign thyroglossal duct cyst. A new endoscopy was performed 6 weeks later and showed a normal airway.

Rhythmic Priming Enhances Speech Production Abilities: Evidence From Prelingually Deaf Children

OBJECTIVEnFollowing recent findings that rhythmic priming can enhance speech perception, the aim of this experiment was to investigate whether this extends to speech production.nnnMETHODnThe authors measured the influence of rhythmic priming on phonological production abilities in 14 hearing impaired children with hearing devices. Children had to repeat sentences that were or were not preceded by a rhythmical prime. In addition, this rhythmic prime either matched or mismatched the meter (i.e., stress contrasts) of the sentence.nnnRESULTSnMatching conditions resulted in a greater phonological accuracy of spoken sentences compared to baseline and mismatching conditions. Cochlear implant users were also more sensitive to rhythmic priming than hearing aid users.nnnCONCLUSIONSnThese results suggest that musical rhythmic priming can enhance phonological production in HI children via an enhanced perception of the target sentence. Overall, these findings suggest that musical rhythm engages domain-general expectations which can enhance both in perception and production of speech.

Native language shapes automatic neural processing of speech

The development of the phoneme inventory is driven by the acoustic-phonetic properties of ones native language. Neural representation of speech is known to be shaped by language experience, as indexed by cortical responses, and recent studies suggest that subcortical processing also exhibits this attunement to native language. However, most work to date has focused on the differences between tonal and non-tonal languages that use pitch variations to convey phonemic categories. The aim of this cross-language study is to determine whether subcortical encoding of speech sounds is sensitive to language experience by comparing native speakers of two non-tonal languages (French and English). We hypothesized that neural representations would be more robust and fine-grained for speech sounds that belong to the native phonemic inventory of the listener, and especially for the dimensions that are phonetically relevant to the listener such as high frequency components. We recorded neural responses of American English and French native speakers, listening to natural syllables of both languages. Results showed that, independently of the stimulus, American participants exhibited greater neural representation of the fundamental frequency compared to French participants, consistent with the importance of the fundamental frequency to convey stress patterns in English. Furthermore, participants showed more robust encoding and more precise spectral representations of the first formant when listening to the syllable of their native language as compared to non-native language. These results align with the hypothesis that language experience shapes sensory processing of speech and that this plasticity occurs as a function of what is meaningful to a listener.