Disordered breathing during sleep in patients with mucopolysaccharidoses

OBJECTIVE: Obstructive sleep apnoea (OSA) has been reported as a feature of children with mucopolysaccharidoses (MPS). However, the incidence and severity of OSA with respect to disease type is poorly defined. The aim of the present study was to measure objectively the degree of OSA in a group of children with a range of MPS syndromes. METHODS: In a cross-sectional study, cardiopulmonary sleep studies were performed during unsedated sleep in 26 children with MPS over a period of 2 years. Scores of OSA severity based upon clinical history and upon objective sleep study data were made in each case and compared. RESULTS: OSA was present in 24/26 patients, and ranged in severity from mild to severe. OSA was most marked in MPS type IH (Hurler syndrome) followed by types IHS (Hurler--Scheie syndrome) and II (Hunter syndrome). Frequent arousals and poor sleep quality, not suspected clinically, were noted in several patients. There was agreement between the clinical and objective scoring systems in only 17/26 patients (65%) with clinical history scores tending to underestimate the most severe cases (5/26 cases) and overestimate the severity in the mild cases (4/26 cases). CONCLUSIONS: Obstructive respiratory problems are frequent in MPS patients and there are differences in severity of OSA between the different MPS types. Assessments of the severity of OSA based upon clinical history alone are inadequate. Our results suggest that objective sleep studies are necessary to evaluate these cases, to monitor clinical outcome and to assess the effects of therapeutic intervention. Prospective studies in larger numbers of patients are needed to validate these observations.     

Paediatric powered endonasal dacryocystorhinostomy

Dacryocystorhinostomy (DCR) is indicated in children with persistent nasolacrimal duct obstruction (NLDO). Endoscopic endonasal DCR has been successfully performed in both adults and children, with success rates similar to that of external DCR. This paper aims to evaluate the outcomes of paediatric patients undergoing endonasal endoscopic DCR at a district general hospital. A retrospective review of all patients attending a joint lacrimal clinic undergoing endonasal endoscopic DCR between October 2001 and December 2008 was performed. Sixteen children (17 eyes) aged between 2 and 14 were analysed. The main outcome measure was resolution of symptoms. Fourteen cases (82%) presented with epiphora with or without stickiness. Three cases had recurrent dacryocystitis. Three patients had Down syndrome. There were no operative complications. During a mean post-operative period of 12 months, all but one case showed an improvement of symptoms. While 60% of the patients had a complete resolution of symptoms, the remaining were minimally symptomatic. Of those with residual symptoms, two had Down syndrome. Endonasal DCR is a safe and effective intervention for children with persistent NLDO. Success is higher in children with no craniofacial abnormality. Joint care under a consultant Oculoplastic and ENT surgeon provides optimum care for these patients.     

Extensive intratemporal cholesteatoma: surgical strategy

OBJECTIVE: To evaluate the decisional elements in the surgical strategy for extensive intratemporal cholesteatomas. STUDY DESIGN: A retrospective review of cases followed up between 1985 and 1996. SETTING: Tertiary referral center. PATIENTS: Nineteen patients with temporal bone cholesteatoma extending beyond the middle ear limits and surgically treated were included. Preoperative imaging distinguished apical (8), infralabyrinthine (3), supralabyrinthine (3), retrolabyrinthine (1), and translabyrinthine (4) cholesteatomas. INTERVENTION: Apical and supralabyrinthine lesions were treated through a middle fossa approach. Infralabyrinthine and translabyrinthine locations were exposed through a subtotal petrosectomy or a transotic route, depending on the preoperative audiovestibular status and labyrinthine destruction on computed tomography. The retrolabyrinthine lesion was approached through a retrolabyrinthine route. MAIN OUTCOME MEASURES: Patients were assessed for postoperative audiologic and facial functions and for recurrence of tumor. RESULTS: The facial nerve was neither rerouted nor interrupted during surgery. Among the 12 patients with preoperative facial palsy (FP), 5 cases of improvement (42%), 6 cases of stable function (50%), and 1 case of mild deterioration (8%) were observed postoperatively. In patients without preoperative FP, facial function remained unchanged postoperatively. The labyrinth could be preserved in three patients (16%), with postoperative stable hearing function in two (11%), and a 40-dB mean auditory deterioration in one (5%). Complete macroscopic resection was obtained in all patients. Two cases (11%) of postoperative recurrence were observed. CONCLUSION: The surgical strategy, principally based on cholesteatoma location and preoperative auditory function, yielded a high rate of local disease control and facial function preservation.     

儿童阻塞性睡眠呼吸暂停低通气综合征手术疗效分析

目的探讨儿童阻塞性睡眠呼吸暂停低通气综合征(obstructive sleep apnea hypopnea syndrome,OSAHS)手术成功和失败的原因,以提高手术预评估水平,提高手术疗效。方法将我院2003年3月～2006年3月手术治疗的112例临床资料完整的OSAHS患儿进行疗效分析。其中,扁桃体加腺样体切除56例;单纯扁桃体切除41例,包括单侧扁桃体切除2例;单纯腺样体切除15例。结果治愈102例(91.1%),包括单侧扁桃体切除后对侧扁桃体代偿增生肥大者2例,再次行对侧扁桃体切除后治愈;显效9例(8.0%),包括肥胖4例,咽淋巴增生1例,鼻部阻塞4例;好转1例(0.9%),为肥胖伴下颌骨后缩者;无效0例。结论儿童OSAHS大多手术疗效较好,少数肥胖、鼻部阻塞、咽淋巴环增生及下颌后缩者疗效欠佳。在腺样体、扁桃体手术前同时应考虑解除其他部位阻塞或无创通气治疗,方可进一步提高手术疗效。对肥胖患儿还应加以减肥治疗。     

Effect of methylphenidate on auditory event related potential in boys with attention deficit hyperactivity disorder

OBJECTIVE: Event related brain potentials (ERPs) is a non-invasive technique giving knowledge about neural activity associated with sensory and cognitive information processing. The aims of the present study were to investigate amplitude and latency of P100, N200, and P300 in parietal and frontal areas in children with attention deficit hyperactivity disorder (ADHD), and in healthy children, and to determine the effect of methylphenidate (MPH) on these ERPs indices in ADHD group. METHODS: ERP indices, latencies of parietal P3 (PP3L), P1 (PP1L), N2 (PN2L), and frontal P1 (FP1L), N2 (FN2L), P3 (FP3L), and amplitudes of parietal P3 (PP3A), P1 (PP1A), N2 (PN2A), and frontal P1 (FP1A), N2 (FN2A), and P3 (FP3A), using an auditory oddball paradigm were recorded before and under MPH treatment in boys with ADHD, and in 23 healthy children. RESULTS: Before MPH treatment, PP3L was significantly longer and PP3A, PN2A, FN2A, and FP3A smaller in children with ADHD compared to healthy children (all P values < .05). No significant difference was found in PP1L, PP1A, PN2L, FP1L, FP1A, FN2L, and FP3L between ADHD and control group (all P values > .05). MPH treatment resulted in a significant decrease in PP3L, PN2L, and FP3L, and increase in PP3A, PP1A, and FP3A (all P values < .05). There was no significant difference in PP1L, PN2A, FP1L, FP1A, FN2L, and FN2A between before MPH and under MPH treatment in ADHD subjects (all P values > .05). Under MPH treatment, PP3L, PP3A, PP1L, PP1A, PN2L, FP1L, FP1A, FN2L, FP3L, and FP3A were not significantly different between children with ADHD and healthy controls (all P values > .05). However, PN2A and FN2A were significantly smaller in ADHD subjects compared to controls (both P values < .05). CONCLUSION: This study provides indirect evidence that ADHD subjects are associated with abnormalities in signal detection (inattention) and discrimination, and information processing. In addition, present study has shown that except FN2A and PN2A, MPH normalizes ERP indices, which suggested that MPH may be effective on impaired information processing in ADHD, but not on the receiving information.     

儿童上气道咳嗽综合征误诊140例

目的 探讨上气道咳嗽综合征（UACS）的临床特点、诊断及治疗。方法 回顾性分析UACS患者140例病历资料,诊断过程中曾误诊为慢性支气管炎102例、咳嗽变异性哮喘25例、抽动症13例,确诊后对所有患者按照鼻部疾病进行治疗。结果 经治疗14d后咳嗽消失或好转的有129例,总缓解率92.1%（129/140）。结论 UACS容易误诊,鼻咽内窥镜检查（鼻内镜或电子鼻咽镜）是诊断UACS的有力工具。明确诊断后对因治疗,UACS预后良好。     

UK and Ireland experience of bone anchored hearing aids (BAHA) in individuals with Down syndrome

OBJECTIVE: To evaluate the indications, surgical techniques and post-operative problems seen in children with Down syndrome fitted with bone anchored hearing aids (BAHA). METHODS: A postal survey of all registered United Kingdom and Ireland BAHA centres. RESULTS: There was a 98% response rate to the survey. Eighty-one centres in the United Kingdom and Ireland undertake BAHA surgery. Eighteen centres were identified to have undertaken BAHA surgery on individuals with Down syndrome. A total of 43 individuals were implanted. Twenty-four cases were under the age of 16. Soft tissue complications were encountered in 21 patients (49%). Osseointegration failures were encountered in four cases (9%). There was a high level of satisfaction with the BAHA system amongst patients, parents and carers. CONCLUSIONS: BAHA is a valuable method of hearing amplification in children with Down syndrome. It should be considered not as a primary method of amplification, but in the overall management of individuals with Down syndrome after conventional hearing aids and/or ventilation tubes have been considered or already failed. The survey shows a high patient and carer satisfaction with the system, despite short term early soft tissue complications.     

Varicella-zoster virus load and cochleovestibular symptoms in Ramsay Hunt syndrome

OBJECTIVES: The mechanism by which varicella-zoster virus (VZV) reactivation causes cochleovestibular symptoms (CVSs) in patients with Ramsay Hunt syndrome (RHS) remains to be elucidated. The present study analyzed the relationship between VZV load and the onset of CVSs in RHS. METHODS: The subjects consisted of 56 patients with RHS; 29 exhibited CVSs and facial paralysis (FP; group 1), and 27 exhibited FP without CVSs (group 2). The VZV DNA copy number in the saliva was measured with a quantitative polymerase chain reaction. Anti-VZV antibodies were assayed by an enzyme-linked immunosorbent assay with paired sera. RESULTS: There was no significant difference in maximum viral copy number between the two groups. In group 1, CVSs occurred at various times between the early phase and the regression phase of VZV reactivation. In some patients, CVSs occurred in the early phase of VZV reactivation, before the onset of zoster lesions and FP. CONCLUSIONS: There are various different patterns in the development of eighth cranial nerve dysfunction, which is caused by progression of neuritis or labyrinthitis following VZV reactivation. Our data suggest that CVSs in RHS may also be caused by reactivation of VZV in the spiral and/or vestibular ganglia.     

Temporal bone abnormalities associated with hearing loss in Waardenburg syndrome

OBJECTIVES/HYPOTHESIS: The objectives were to correlate audiometric thresholds with radiological findings and to determine the prevalence of inner ear radiological abnormalities in patients with hearing loss and Waardenburg syndrome. STUDY DESIGN: The study was a retrospective review of patients with Waardenburg syndrome identified in a pediatric hearing-impaired population and human genetics clinic. METHODS: Nine children with Waardenburg syndrome were identified. Eighty-nine children without sensorineural hearing loss served as control subjects. Clinical data, audiometric thresholds, and radiographic temporal bone measurements in these children were analyzed. RESULTS: Seven children were identified with hearing loss and Waardenburg syndrome. Four children had Waardenburg syndrome type 1, and three children had Waardenburg syndrome type 2. The overall prevalence of hearing loss in the total study population with Waardenburg syndrome was 78%. The mean pure-tone average was 99 dB. All of the children had sensorineural hearing loss. The hearing outcome was stable in 86% of the children. Twelve temporal bones were available for radiological analysis by computed tomography. Enlargement of the vestibular aqueduct was found in 50% of the CT scans. There was a significant difference in measurements of vestibular aqueduct width at the midpoint between the patients with Waardenburg syndrome and the control group (P <.05). There were also significant differences in the measurements of the vestibule (P =.0484), internal auditory canal (P =.0092), and modiolus (P =.0045) between the children with Waardenburg syndrome and the control group. CONCLUSION: A profound sensorineural hearing loss was characteristic of the study population with Waardenburg syndrome. Overall, 100% of patients with hearing loss and Waardenburg syndrome had temporal bone anomalies on at least one measurement of their inner ear, and 50% had an enlargement of the vestibular aqueduct at the midpoint. As shown by computed tomography, enlargement of the vestibular aqueduct and the upper vestibule, narrowing of the internal auditory canal porus, and hypoplasia of the modiolus are features of Waardenburg syndrome.     

Hörvermögen bei Kindern und Jugendlichen mit Down-Syndrom

Background

There are controversial opinions about the incidence of hearing impairment of children and adolescents with Down’s syndrome as an additional cause for obstruction in social and communicative live. Next to congenital diseases of the inner ear, functional limitations can often lead to conductive hearing disorders in patients with Down’s syndrome.

Patients and methods

The hearing ability, otoscopic and audiometric characteristics of 115 patients aged 3.2±2.9 years were analysed retrospectively over 42 months.

Results

A total of 86 children showed morphologic otoscopic peculiarities: transitory evoked otoacoustic emissions (TEOAE) were often non-reproducible, even in patients with normal hearing abilities(14.1% of cases), and were not evaluable in 32.8% of cases. Half of the patients showed hearing loss, 82% of whom suffered from a conductive hearing loss, 6.9% from a combined, and 8.6% from isolated sensorineural hearing loss. Hearing loss was first diagnosed when the children were 4.6±3.4 years old.

Conclusion

The frequent occurrence of hearing loss justifies and requires an early and continuous pedaudiological examination. As a common screening examination, TEOAE is limited due to its cost, however, it fulfils its purpose as a means of distinguishing healthy children from those with possible hearing disorders. In most cases, objective and subjective audiometric methods must be combined in order to precisely define the hearing ability.     

Syndromic etiology in children at schools for the deaf in Turkey

OBJECTIVE: The aim of this study was to determine the syndromic etiology of bilateral severe sensorineural hearing disorders in children and current etiological causes to reduce the cases in the unknown group. METHODS: This study was conducted on 550 students of five schools for the deaf in Istanbul and Zonguldak, Turkey. Otologic, audiologic, dysmorphologic, ophthalmologic and dental examinations were performed in all children. Familial and medical histories were obtained. RESULTS: The etiology of hearing loss was genetic in 346 (62.90%), acquired in 107 (19.45%) and unknown in 97 (%17.63) cases. A total of 619 malformations were defined in 550 children and 99 of them belonged to a syndrome. We identified 33 different syndromes for these 99 syndromic children. Syndromic etiology was found in 18.0% of the total and 28.61% of the subjects with genetic etiology. Most common syndrome was Waardenburg syndrome which occurred in 33 children. CONCLUSION: The incidence of hereditary hearing impairment is very high in developing countries compared to developed countries. Prevention is essential to reduce the incidence, multidisciplinary approach and genetic counselling are necessary in this regard.     

Cochlear implantation in children with "CHARGE syndrome": surgical options and outcomes

CHARGE syndrome is a rare, polymalformative disease, representing one of the major causes of associated blindness and deafness. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE children. Aim of this study is to present our results in children with “CHARGE syndrome” submitted to cochlear implantation (CI). The frequency of anatomic anomalies, possible variations in the surgical technique of CI, and the audiological/rehabilitative benefits attained in our patients are reported. we submitted 5 children affected by CHARGE syndrome with profound, bilateral, sensorineural hearing loss to CI. Otoacoustic emissions, auditory brainstem response, acoustic impedance testing, cranial computed tomography and magnetic resonance were carried out preoperatively in all children. CI was performed using the mastoidotomy–posterior tympanotomy approach in two cases, and the suprameatal approach in three children. Infant toddler-meaningful auditory integration scale was used to evaluate kid’s audiological performance before and after CI. Intra-operatory findings and postsurgical complications were evaluated. Among our patients, intraoperative anatomical malformations were cochlear hypoplasia (100 %), ossicles malformations (100 %), semicircular canals aplasia (100 %), oval window atresia (60 %), round window atresia (40 %), widening of the aqueduct of the vestibule (20 %), and aberrant course of the facial nerve (20 %). No intra- or postoperative complication was recorded in relation to implant positioning. After a follow-up ranging from 1 to 4.5 years, only 2/5 patients used oral language as the sole mean of communication, 1 started utilizing oral language as the main mean of communication, while 2 patients did not develop any linguistic ability. In conclusion, CI in patients with CHARGE association is feasible and, despite results variability, it should be carried out in CHARGE children with severe hearing loss as soon as possible. Although the selection of a specific surgical technique does not seem to influence the audiological outcome, the suprameatal access is valuable when important surgical landmarks (i.e. lateral semicircular canal and incus) are absent.     

Auditory brainstem responses in young children with Down's syndrome

Auditory brainstem evoked responses (ABRs) were studied in 37 Japanese infants and children with Down's syndrome to determine the level of the lesion causing their unresponsiveness to sound stimuli. ABR thresholds, peak latencies of waves I and the peak interval latency of wave I-V were measured. For comparison with ABR thresholds, behavioral audiometry was performed. Thirty-two percent of the patients (12 cases) with Down's syndrome showed no responses by ABR as well as behavioral audiometry. The other ABR abnormalities with respect to age-matched controls were classified into 6 types: shorter wave I latencies (2 cases, 5%), shorter wave V latencies (7 cases, 19%), shorter wave I-V intervals (8 cases, 22%), wave I prolongation (13 cases, 35%), wave V prolongation (5 cases, 14%) and prolongation of wave I-V peak interval (no case, 0%). Our results demonstrate a high incidence of hearing loss at middle and inner ear levels, and suggest some anomaly within the auditory brainstem in Japanese cases of Down's syndrome.     

Analysis of fifty cases of facial palsy due to otitis media

Fifty cases of facial palsy (FP) due to otitis media treated in our hospital during the 10-year period from 1972 to 1981 were analyzed. The averaged percentage of this incidence was 3.1% of all the FP occurring. FP due to otitis media acuta (OMA) was frequently seen in infants. FP due to otitis media chronica (OMC) tended to occur in combination with cholesteatoma or acute exacerbations of infection. Paralyses were generally mild and bacteria from the otorrhea were similar to those of OMC without FP. When present, sites of dehiscence of the facial canal were most frequently seen in its horizontal portion. Electromyographically, the posterior (mastoid) side of the horizontal portion of the facial nerve bundle seemed to be related to the branch to the orbicularis oculi muscle, while the anterior (stapes) side was directed to the orbicularis oris muscle.     

Surgical aspects of cochlear implantation in syndromic children

Abstract

Objective

The objective of this study was to report surgical results and outcomes of cochlear implantation in a large series of children with syndromes from one centre.

Patients and methods

All syndromic children who underwent cochlear implantation at Great Ormond Street Hospital, from January 2000 to December 2010 were included in this study. The surgical technique was analysed and audiological outcomes were collected.

Results

Over the 10-year period of this study, a total of 88 cochleas in 67 children with syndromes were implanted. The common syndromes implanted in this study were Ushers syndrome (23 patients, 33 cochleas), Wardenburgs syndrome (8 patients, 9 cochleas), Pendreds syndrome (4 patients, 4 cochleas), Jervell?Lange?Neilsen syndrome (3 patients, 4 cochleas), Enlarged vestibular aqueduct syndrome (4 patients, 7 cochleas), Cogans syndrome (3 patients, 4 cochleas), CHARGE (5 patients, 6 cochleas), and Branchio Oto Renal syndrome (3 patients, 4 cochleas). Pre-operative radiological inner ear anatomy was found to be abnormal in 28.4% (25/88) cochleas in this study group. Full insertion of the electrode was achieved in 93.1% (82/88) of cochleas, partial insertion in three cochleas, and insertion was abandoned in three cochleas. Early complications were seen in 6.8% (6/88) of implantations. All the 64/67 children who were implanted are still using the implant.

Conclusion

Cochlear implantation in syndromic children is challenging in both its audiological and surgical aspects. Good surgical results and good audiological and speech outcomes were achieved in this study, and subjective improvement in quality of life was achieved in these patients.     

Major and minor temporal bone abnormalities in children with and without congenital sensorineural hearing loss

OBJECTIVE: To determine the extent of correlation between sensorineural hearing loss (SNHL) and abnormal temporal bone anatomy in children. DESIGN: Axial and coronal high-resolution computed tomographic scans of the temporal bones of 247 children (494 ears) aged 2 months to 15 years with and without SNHL were blindly reviewed. The presence or absence of mild or severe cochlear dysplasias, vestibular dysplasias, and an enlarged vestibular aqueduct (VA) were recorded. The width of the VA was measured. The height, width, and length of the internal auditory canal (IAC) were measured, and abnormalities were described as narrow, widened, or bulbous. Clinical information was then reviewed to determine the presence or absence of a congenital syndrome and/or SNHL, and historical factors that might be responsible for SNHL. MAIN OUTCOME MEASURE: The relationship between radiographic findings and SNHL. RESULTS: One hundred thirteen patients (185 ears) had SNHL. Significant abnormal temporal bone anatomy in children with vs without SNHL included major cochlear and vestibular dysplasias (17% vs 0%; P<.001), enlarged VA (>2 mm) (5% vs 0%; P<.001), and narrow IAC (< or =2 mm) (4% vs 1%; P=.03). The average IAC width (4.85 vs 5.02 mm), height (4.39 vs 4.62 mm), and length (11.22 vs 11.44 mm) were not statistically different between children with vs without SNHL. In children with vs without SNHL, neither a widened (0.5% vs 3.6%) nor a bulbous (9% vs 8%) IAC was seen more often in children with SNHL. In ears with SNHL, the presence of a congenital syndrome significantly increased the risk of cochlear and vestibular abnormalities of the temporal bone (45% vs 14%; P<.001), including IAC abnormalities (30% vs 2%;P<.001), which overall were more commonly seen in children with (20%) vs without (3%) a congenital syndrome regardless of the presence of SNHL. No children with an enlarged VA had a congenital syndrome. CONCLUSIONS: Well-established temporal bone abnormalities such as cochlear and vestibular abnormalities and a grossly enlarged vestibular aqueduct are significantly found in children with SNHL. A narrow IAC is found more often in children with vs without SNHL. No significant correlation is found between SNHL and radiographic findings of a widened or bulbous IAC. In children with a congenital syndrome, more IAC abnormalities were seen, regardless of the presence of SNHL. In children with SNHL, the presence of a congenital syndrome increases the likelihood of a cochlear or vestibular abnormality.     

Diagnosis and therapy for airway obstruction in children with Down syndrome

OBJECTIVES: To document the causes of upper airway obstruction in a population of children with Down syndrome and to highlight the role of associated comorbidities. DESIGN AND SETTING: Review of 23 cases involving children with Down syndrome who were referred for upper airway obstruction over a 2(1/2)-year period to the Pediatric Otolaryngology Service of the University of New Mexico, Albuquerque. METHODS: Data on the following variables were obtained: reason for referral, demographics, diagnosis, surgical procedures, complications, and comorbidities. RESULTS: The children ranged in age from 1 day to 10.2 years (mean age, 1.8 years; median age, 6 months). Thirteen children were male and 10 were female. None of the children had subglottic stenosis. Laryngomalacia was the primary diagnosis in 10 children (43%), 8 of whom were younger than 1 month. Obstructive sleep apnea was the primary diagnosis in 11 children (48%), 8 of whom were older than 2 years. All children with obstructive sleep apnea and 4 children with laryngomalacia had a secondary ear, nose, and throat disorder. Gastroesophageal reflux was a comorbidity in 14 children (61%). CONCLUSIONS: The causes, severity, and presentation of upper airway obstruction in children with Down syndrome are related to the age of the child and to associated comorbidities. The treatment of comorbidities and secondary ear, nose, and throat disorders is an integral component of the surgical management of upper airway obstruction in such cases.     

Sleep apnea and Down's syndrome

OBJECTIVE: Obstructive sleep apnea has been reported to occur in 20-50% of children with Down's syndrome in case series of patients referred for evaluation of suspected sleep apnea. In this population-based controlled study, we aimed to investigate whether sleep apnea is related to Down's syndrome. MATERIAL AND METHODS: Every child aged 2-10 years with Down's syndrome residing in the Ume? healthcare district (n = 28) was invited to participate in the study, with their siblings acting as controls. Successful overnight sleep apnea recordings and echocardiography were performed in 17/21 children with Down's syndrome and in 21 controls. RESULTS: Obstructive sleep apnea could not be diagnosed, either in children with Down's syndrome or in the control children. The apnea-hypopnea index in the children with Down's syndrome was 1.2 +/- 1.5 and did not differ from that in controls. Snoring and hypertrophy of the tonsils were more common in children with Down's syndrome than in controls. Children with Down's syndrome slept for a shorter time (p < 0.001) and changed body position more often (p < 0.05) than the control children. CONCLUSIONS: Snoring, restless sleep and hypertrophy of the tonsils were common among children with Down's syndrome. Obstructive sleep apnea was, however, not related to Down's syndrome in the present population-based controlled study.     

Thyroid surgery in children and adolescents

Authors present 114 children and adolescents of the total 3865 patients that underwent surgery on the Department of Otorhinolaryngology and Head and Neck Surgery, 1st Faculty of Medicine, Charles University, Prague, Department of Otorhinolaryngology of IPVZ, Prague because of a thyroid disease in years 1991-2000. The male/female ratio in the group was 1:6.5, 16 (13.9%) males and 99 (86.1%) females. For non-malignant disease, the male/female ratio was 1:6.5 and for malignant disease, it was 1:5.4. Thyroid surgery was indicated for non-malignant disease in 82 (71.9%) cases--11 males and 71 females. Most frequently it was indicated because of Graves-Basedow and Hashimoto toxicosis--in 39 children (34.2%), in 5 children (4.4%) for toxic adenoma. Because of the polynodular goiter surgery was indicated in 13 cases (11.4%), in 22 cases (19.3%) for mononodular goiter. Three girls (2.6%) were recommended for surgery because of Hashimoto thyreoiditis and for mechanic syndrome in diffuse goiter one girl (0.9%). Malignant tumor was found in 32 children (28.1%)--5 males and 27 females. Most frequent histological type was papillary cancer--in 25 cases (21.9%). Follicular cancer was diagnosed in four cases (3.5%) and medullar cancer in three cases (2.6%). TNM classification in children and adults is summarized in Table 1. Surgery was carried out in two children with distant metastases (lung localization). In both children the pre-disease stage lasted more than 12 months. The first manifestation of the disease was a finding of enlarged and palpable nodes on the neck. The authors summarize indications for surgery of thyroid diseases in children and adolescents. A treatment of the thyroid gland is a teamwork. Indication for surgery is carried out by a pediatric endocrinologist in cooperation with a surgeon specialized in thyroid surgery. The possible identification of the recurrent laryngeal nerve by a surgery microscope is an unquestionable advance. Children and adults should be centralized into health-care centers capable not only of surgery, but also of endocrinology care. In children with a malignant disease there is a need for cooperation with a pediatric oncologist and a following oncological treatment on a nuclear medicine department. The authors summarize the information about today's thyroid surgery care possibilities for children and adolescents. They point out the differences in indications and the decision about extent of surgery in children and adolescents.