Search

OBJECTIVE To describe malformations associated with pediatric congenital cholesteatomas of the middle ear. STUDY DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS One hundred and seventy-three cases of middle ear congenital cholesteatoma (CC) in 171 children operated between 2007 and 2017. INTERVENTIONS Demographic, clinical, and surgical data were collected from operative reports. MAIN OUTCOME MEASURES We first described the type and rate of malformations associated with CC. Secondly, we compared cholesteatoma features in two subgroups: anterior superior (AS) versus posterior superior (PS) starting point. Third, we compared demographic, clinical, and surgical data between patients with and without malformation. RESULTS CC was associated with malformations in 17 cases (17/173; 9.8%). The main malformation was preauricular fistula (8/173; 4.6%). Other malformations were: one first branchial cleft, two labio palatine cleft, one nasal cyst, two preauricular fibrochondroma, and five other malformations. PS congenital cholesteatomas were diagnosed in older children (4.6 versus 8.6 years, p

Summary Objectives The focus of this report is hearing screening of newborns transferred from the regular nursery to a specialized area. The purpose of the study undertaken was: (1) to determine whether screening coverage in this population was achieved; (2) to establish whether the linkage between neonatal screening and the diagnostic follow-up was carried out correctly; (3) to better determine the incidence of permanent childhood hearing impairment (PCHI) in this at-risk population. Methods Six population centres averaging 12,000 births annually participated (Bordeaux, Lille, Paris, Marseille, Toulouse and Lyon). Automated auditory brainstem response (AABR) (Natus ALGO 3i ® ) screening was performed in two stages: i.e. infants with initial “positive” results were screened a second time using the same technique. Of the 117,103 babies born during the study period, 4972 neonates were “transferred” and comprised the population for this report (4.2% of the total births). Results and discussion Screening results for 4972 “transferred” neonates were compared with those of non-transferred neonates ( N = 112,131). Screening coverage of eligible infants was significantly lower (75.4%) in “transferred” neonates (3750 infants screened) compared to 97.5% coverage of non-transferred neonates (109,349 infants screened). The rate of positive results after the first stage AABR was higher in the “transferred” population (11.1%) than in the non-transferred population (6.5%). Of the 415 “transferred” newborns with initial positive screens, 91.3% were rechecked as stipulated in the project protocol. The second pre-discharge AABR ascertained that in half of the cases auditory function had normalized in the day. Of the 183 “transferred” infants whose result remained suspect at the conclusion of both stages of the neonatal screen (4.9% of the tested population), only 70.5% returned to the audiology centre for diagnostic follow-up. The incidence of bilateral PCHI was markedly higher (4/1000) in “transferred” infants than in the non-transferred population (1.08/1000). Conclusions The difficulty of obtaining universal screening coverage in “transferred” infants was, unfortunately, verified in this prospective, multicentre study. Further, the diversity of our “transferred” population was not much greater than that revealed by careful analysis of published hearing screening studies in neonatal intensive care unit (NICU) infants. The influence of risk factors and their more or less complex combinations is apparent.

Annales Francaises d'Oto-Rhino-Laryngologie et de pathologie cervico-faciale - Vol. 125 - N° 2 - p. 72-77

Objectives To assess the diagnostic strategy, treatment and outcome of acquired subglottic cysts. Materials and methods Retrospective, monocentric, tertiary referential center study of 172 preterm neonates assessed by endoscopic examination over a 10 years period. Identification of patients presenting with subglottic cysts. Results 17 children were diagnosed with subglottic cysts. Among them, 98% were prematurates (28 ± 4 weeks of gestation), and 76% had a history of hyaline membrane disease or a bronchopulmonary dysplasia. All patients were intubated during the neonatal period, for a mean duration of 14 days. Mean age at diagnosis was 8 months. An associated laryngotracheal anomaly was diagnosed in 30% of cases. Six procedures, including flexible controls, were needed to achieve full recovery. We used cold steel microinstruments, CO 2 or Thulium LASER. Mean follow up was 3 years. Conclusions Acquired subglottic cysts concern early preterm infants. Children treated for subglottic cysts should undergo a long term follow up, as there is a trend for cysts to recur, as well as a risk of secondary subglottic stenosis.

Objective To assess the efficiency of autologous fat transfer (AFT/Coleman procedure) in the management of velopharyngeal insufficiency (VPI). Settings Tertiary academic center, retrospective case series over a 4 year period. Pre- and post-speech assessment by a speech pathologist using the Borel-Maisonny scale. Patients and method Twenty-five (25) procedures were performed on 22 patients during the considered period. Mean age at surgery was 12.4 ± 4.1 years-old. Main associated conditions were 22q11 deletion ( n = 6 including 2 with cleft palate), isolated cleft palate ( n = 3), and Robin sequence ( n = 2). Indications were VPI grade 2a ( n = 5), 2b ( n = 11) and 3 ( n = 6), despite prolonged speech therapy (pre-op mean duration: 4.2 years) and previous surgery (velopharyngoplasty, n = 13). Four patients had a contraindication of velopharyngoplasty (aberrant internal carotid arteries). Results and conclusion Fat harvesting sites were umbilicus ( n = 23) and buttock ( n = 2). Mean injected fat volume was 7.8 ml, in the posterior wall of the pharynx ( n = 25) the soft palate ( n = 15), the peritonsillar arches ( n = 3), and the pre-existing flap ( n = 3). Mean follow-up was 17 months. Two patients relapsed once and one patient twice, requiring additional injections. Final post-operative examination 1 year after the last procedure showed an improvement of speech in 90% of cases (grade 1, n = 2; 1/2a, n = 5; 2a n = 10; 2b, n = 5). AFT is a safe technique indicated in the primary and secondary management of VPI, with stable results on speech. However, if a complete return to normal is difficult to achieve, its simplicity allows multiple procedures in the same patient.

Summary Objective To review the experience with ganglioneuromas in the head and neck of children including presentation, diagnostic testing, treatments, and outcomes. Design Case series. Retrospective chart review. Setting Tertiary care hospital. Patients or other participants All patients with a history of ganglioneuroma of the neck in each authors practice were reviewed. All pathologically confirmed occurrences were eligible for inclusion, and five patients met these criteria. Results Five patients underwent surgical excision of head and neck ganglioneuromas between 1988 and 2004. There were no occurrences of secretory tumors, therefore all of the patients presented with enlarging masses. In all cases, the tumor arose from the cervical sympathetic chain, and thus, patients had subsequent ipsilateral Horner's Syndrome following resection. No synchronous tumors were noted, nor has a recurrent tumor been observed to this point. Complete excision was possible in all cases via a transcervical, or transoral approach, without mandibulotomy. Conclusions Ganglioneuroma of the neck is a rare tumor that most commonly presents as an enlarging neck mass. Complete surgical excision is the treatment of choice, and in this series of children was possible with transcervical approach, and once via transoral approach. This tumor may be suspected in children who are otherwise asymptomatic, and present with long history of enlarging neck masses.

Two previous case reports described two sibs affected with both sensorineural hearing loss and oligodontia. Here, we report a similar syndrome in a male patient with an, as yet, undescribed vestibular aqueduct enlargement on tomodensitometry. The analysis of the parent's audiograms is consistent with the suggested autosomal recessive mode of inheritance of this disorder.

Corrigendum to ‘Autologous fat transfer in velopharyngeal insufficiency: Indications and results of a 25 procedures series’ [International Journal of Pediatric Otorhinolaryngology 75 (2011) 1404–1407] Nicolas Leboulanger *, Marion Blanchard , Francoise Denoyelle , Fergal Glynn , Jean-Baptiste Charrier , Gilles Roger , Jean-Paul Monteil , Erea-Noel Garabedian a,d,e Otolaryngology, Head and Neck Surgery Department, Armand-Trousseau Children Hospital, Paris, France Otolaryngology, Head and Neck Surgery Department, Bicetre Hospital, Bicetre, France Otolaryngology, Head and Neck Surgery Department, Lariboisiere Hospital, Paris, France Assistance Publique, Hopitaux de Paris, France UPMC, Universite Pierre et Marie Curie Paris VI, France