Performance of endoscopic repair with endonasal flaps for congenital choanal atresia. A systematic review.

Endoscopic repair of congenital choanal atresia is the gold standard surgical treatment today. Though several controversies on treatment have been reported, surgical techniques for better outcomes are still in discussion. The objective of this study is to evaluate the performance of endoscopic choanal atresia repair with endonasal flaps and no stents. Publications in English in the last 5 years were searched in the PUBMED database and were systematically reviewed. A total of 9 articles were included according to the inclusion criteria, obtaining a total of 266 patients managed for congenital choanal atresia with endoscopic surgery, endonasal flaps, and no stents. Surgical results, type of atresia, atresia laterality, associated pathologies and follow up were evaluated. Successful surgery was obtained in 237 (89%) patients while 29 (11%) patients required a new surgical intervention during the follow-up period. Fourteen percent of the patients were diagnosed with CHARGE syndrome and 5% of the patients had some associated heart disease. Bony-Membranous stenosis was observed in 74% of the patients, while a total bony obstruction was recognized in 26% of the patients. Unilateral atresia was observed in 37% of the cases and 63% of the cases had bilateral atresia. The mean follow-up period was 39.5 months (range 3-168 months). An important functional success rate can be accomplished by correcting congenital choanal atresia using functional endoscopic surgery, covering raw areas with endonasal vascularized flaps, avoiding postoperative endonasal stenting.

Retinal findings in pediatric patients with Usher syndrome Type 1 due to mutations in MYO7A gene.

PURPOSE: To describe retinal alterations detected by swept-source optical coherence tomography (SS-OCT) in paediatric patients with Usher syndrome type 1 (USH1) and to compare these findings to previously published reports. METHODS: Thirty-two eyes from 16 patients (11 males and 5 females) with a genetic diagnosis of USH1 because of MYO7A mutations underwent SS-OCT. Patients ranged in age from 4 to 17 years (mean, 11,13 ± 4,29). The subfoveal and macular area were analysed with SS-OCT at 1050 nm using 12 radial scans of 12.0 mm. Structural abnormalities were evaluated and correlated with best-corrected visual acuity (BCVA). RESULTS: The most common qualitative retinal abnormality was external layer damage in macular area. Specific alterations included external limiting membrane loss/disruption (27 eyes; 84.4%), disruption of the Myoid zone (27 eyes; 84.4%); Ellipsoid zone disruption (28 eyes; 87.5%), and loss of the outer segments (29 eyes; 90.6%). The damage of the retinal pigment epithelium was divided according to the loss of the different layers: phagosome zone (30 eyes; 93.8%), melanosome zone (29 eyes; 90.6%) and mitochondria zone (0 eyes; 0%). The presence of cystoid macular oedema (CMO) was significantly correlated with alterations in photoreceptors. Disruption or absence of the myoid and ellipsoid zones of the photoreceptors were the only variables independently associated with decreased BCVA. CONCLUSIONS: The findings of this study suggest that the physiopathologic basis of early-stage Usher syndrome (USH) may be changes in the outer retinal layer, particularly the photoreceptors, which in turn may cause alterations-such as CMO-in the inner retinal layers. Accordingly, monitoring the condition of photoreceptors during follow-up may be advisable for the early detection of pathologic changes.

Transcanal endoscopic type 1 tympanoplasty in children: Cartilage butterfly and fascia temporalis graft.

INTRODUCTION: Endoscopic type 1 tympanoplasty is every day gaining numerous adepts for tympanic membrane repair. Due to the value of reducing postauricular approaches, decreasing postoperative morbidity and hospitalization time. The objective of this study is to present surgical results of endoscopic type 1 tympanoplasty in the pediatric population using fascia temporalis or cartilage butterfly graft. MATERIALS AND METHODS: Prospective study regarding the pediatric population, mean age of 10.7 years old. Patients diagnosed with chronic otitis media without cholesteatoma and intact ossicular chain. Tympanic membrane reconstruction using inlay cartilage butterfly graft or underlay fascia temporalis graft according to surgical needs. Audiograms were evaluated preoperatively and 6 months after surgery. No postauricular approaches were performed. RESULTS: A total of 54 ears were operated, 25 utilizing underlay fascia temporalis graft and 29 using inlay cartilage butterfly graft. Six months following surgery, dry and closed tympanic membranes were obtained in 54 cases (92.6%). Preoperative and postoperative air conduction (AC) thresholds, bone conduction (BC) thresholds and air-bone gaps (ABG) were assessed. Preoperative AC of 24.6 dB, BC of 8.9 dB and an ABG of 15.5 dB. Postoperative AC of 16.3 dB, BC of 8.9 and an ABG of 6.9 dB. A postoperative ABG reduction of 8.5 dB was reached. CONCLUSION: Transcanal endoscopic type 1 tympanoplasty can be achieved in every pediatric patient with chronic otitis media without cholesteatoma, and, is a safe and efficient procedure.

[Cochlear implant in a patient with dilatation of the endolymphatic sac and vestibular aqueduct]. / Implante coclear en paciente con dilatación del saco endolinfático y del acueducto del vestíbulo.

We present an 8 years patient old with a giant dilatation of the vestibular aqueduct associated with profound and progressive bilateral sensorineural hearing loss. The patient received a cochlear implant and during the surgery a pulsatile clear fluid via the cochleostomy was observed, without gusher. We present all the problems, and offer some advices for this kind of surgery.

[Sensorineural hearing loss due to neonatal hyperbilirubinemia]. / Hipoacusia neurosensorial por hiperbilirrubinemia neonatal.

In this article, the sensorineural hearing loss is presented as a possible sequelae of neonatal hyperbilirubinemia. In our program of early hipoacusia detection, 241 babies were examined from January 1996 until November 1999; 7 cases had a history of hyperbilirubinemia in the neonatal period and 2 of them were diagnosed of sensorineural hearing loss. We discuss how the bilirubin or any other associated factor might have been the cause and this could explain the selective affectation of some children.

[Obstructive sleep apnea in children. Our experience]. / Síndrome de la apnea obstructiva del sueño en el niño. Nuestra experiencia.

We expose our experience in obstructive sleep apnea syndrome (OSAS) in the pediatric population and review the literature. Forty-six nocturnal respiratory polygraphies were performed using a portable device (Eden Trace II Plus) that measures heart rate, chest wall impedance, nasal/oral airflow and oxygen saturation. Seven children have been studied before and six weeks after Adenotonsillectomy. All this children experienced an immediate and important improvement of their obstructive symptoms. After Adenotonsillectomy the number of obstructive sleep apneas disminished up to 87.25% and the number of hypoapneas disminished up to 73.3. The arterial oxygen saturation during the night normalized in the seven patients. The initial treatment of OSAS in children should be the Adenotonsillectomy, although the diagnostic criteria of OSAS in children have to be defined more precisely in the future.

[Intralesional treatment of cutaneous leishmaniasis: a report of two cases]. / Tratamiento intralesional de la leishmaniasis cutánea. A propósito de dos casos.

Two clinical cases of cutaneous leishmaniasis of the outer ear in children are reported. The patients were treated successfully with a single intralesional injection of N-methylglucamine antimonate (Glucantime). Aetiopathogenesis, epidemiology, clinical manifestations, diagnosis, and treatment of cutaneous leishmaniasis are discussed.