International Journal of Pediatric Otorhinolaryngology

Editorial Board

2010-03-01

Epidemiology, natural history, and risk factors: Panel report from the Ninth International Research Conference on Otitis Media

2010-03-01

Abstract: The 2007 Recent Advances in Otitis Media Research Conference Panel Report provides an update on otitis media (OM) research published from 2003 to 2007. This report summarizes important trends in disease incidence and prevalence, describes established and newly identified risk factors for acute and chronic OM and OM with effusion, and conveys information on newly discovered genetic factors. In this report, researchers have described declining rates of OM diagnosis, antibiotic prescriptions, offices visits for OM, and middle ear surgery since the licensure and routine use of pneumococcal conjugate vaccine in infants. The panel report also recommends short and long term goals for current and future OM research.

Effects of adeno-tonsillectomy on polysomnography patterns in Down syndrome children with obstructive sleep apnea: A comparative study with children without Down syndrome

Mona M. Shete, Rose Mary S. Stocks, Merry E. Sebelik, Robert A. Schoumacher — 2010-03-01

Abstract: Objective: To determine if adeno-tonsillectomy (T&A) in children with Down syndrome (DS) improves breathing, measured by apnea hypopnea index (AHI), rapid eye movement AHI (REM-AHI) and the lowest oxygen desaturation (SaO2), and sleep disruption, measured by arousal index (ArI) and time spent in stages 1–4 and rapid eye movement (REM) sleep and compare these results with a group of non-DS children with obstructive sleep apnea (OSA).Study design: Retrospective chart review at pediatric sleep center.Patients: Eleven DS and nine non-DS children underwent pre- and post-T&A polysomnography between 1997 and 2005.Outcome measures: Pre- and post-T&A polysomnography parameters were compared using paired t-test and independent samples test.Results: Mean age in DS group was 101 months and non-DS group was 80 months (64% males in DS and 88% in non-DS group). The average BMI was 29.8 and 27.6 for DS and non-DS group. The total AHI showed significant improvement after T&A but this was not as marked as the non-DS group. REM-AHI and lowest SaO2 did not show significant change in the DS children. The non-DS group showed significant improvement in all respiratory parameters. Both groups showed mild improvement in sleep parameters. With the modest overall improvement, 27% of the DS children required no further treatment. However, 73% required CPAP, BiPAP or oxygen for persistent OSA.Conclusion: This study supports the fact that T&A in DS children improves some parameters of OSA, however not as markedly as in non-DS children.

Supraglottoplasty outcomes in relation to age and comorbid conditions

Stephen R. Hoff, James W. Schroeder, Jeff C. Rastatter, Lauren D. Holinger — 2010-03-01

Abstract: Objective: To determine if age and comorbid conditions effect outcomes in children undergoing supraglottoplasty for severe laryngomalacia.Design: Retrospective study.Setting: Urban tertiary-care children's hospital.Patients: Children undergoing supraglottoplasty for severe laryngomalacia between February 2004 and July 2008. 56 patients were identified.Outcome measures: Persistence of upper airway obstruction, revision surgery (supraglottoplasty), and additional surgery (tracheostomy).Results: 33/56 (58.9%) patients had no comorbid conditions and 23/56 (41.1%) patients had comorbid conditions. In noncomorbid patients, 36.4% of those less than 2 months of age at the time of surgery required revision supraglottoplasty, compared to 5.3% of patients between 2 and 10 months (p<0.05). Compared to the 2–10-month age group, there was a significantly higher percentage of patients with comorbid conditions in the >10-month group (32.1% vs. 79%, p<0.01). Patients with comorbid conditions were diagnosed at a significantly later age than those without (6mo vs. 2mo, respectively), and had significantly higher rates of revision supraglottoplasty (47.8% vs. 18.2%) and tracheostomy (39.1% vs. 0.0%). 70% of children with neurological conditions required revision surgery, with 60% requiring tracheostomy. The revision surgery and tracheostomy rates were significantly higher compared to the noncomorbid group (p<0.01 and p<0.0001). Children with cardiac conditions had a higher rate of tracheostomy than noncomorbid children (30% vs. 0%, p<0.01). 16.7% of children with genetic conditions required supraglottoplasty, and none required tracheostomy.Conclusions: In noncomorbid patients, those undergoing supraglottoplasty less than 2 months of age had a significantly higher rate of revision supraglottoplasty. Patients with neurologic and cardiac comorbidities require tracheostomy at a significantly higher rate than noncomorbid patients.

Prevalence of DFNB1 mutations in Argentinean children with non-syndromic deafness. Report of a novel mutation in GJB2

2010-03-01

Abstract: Objective: Mutations in DFNB1 locus, containing GJB2 (connexin 26) and GJB6 (connexin 30) genes, are the most common cause of autosomal recessive non-syndromic hearing loss. More than 100 mutations in GJB2 have been reported worldwide. Two deletions in GJB6, del(GJB6-D13S1830) and del(GJB6-D13S1854), have been found to be frequent in the Spanish population. The aim of this study was to determine the prevalence of GJB2 mutations and both GJB6 deletions in Argentinean children with non-syndromic deafness.Methods: This study included 94 unrelated children with moderate to profound non-syndromic sensorineural hearing impairment. Molecular analysis was performed using a tiered approach. All DNA samples were screened for c.35delG mutation by PCR/RFLP. Samples from patients who were not homozygous for c.35delG were analysed for the presence of GJB6 deletions by PCR multiplex. The samples that remained unresolved after screening were further analysed by direct sequencing of GJB2 coding region. Finally, the splice site mutation IVS1+1G→A was analysed by PCR/RFLP.Results: Sequence variations in the GJB2 and GJB6 genes were found in 49 of the 94 unrelated patients. The most prevalent GJB2 mutation, c.35delG, was found in 40 of the 68 pathogenic alleles with the second most common allele being p.R143W (4/68). Fourteen sequence variations other than c.35delG were identified. Seven already described mutations were present in more than one allele; among them, IVS1+1G→A, the rare splice site mutation flanking exon 1. In addition to known disease-related alterations, a novel GJB2 mutation, c.262G>C (p.A88P), was also identified. Six alleles were identified carrying GJB6 deletions; the most prevalent was del(GJB6-D13S1830). The frequency of the latter was found to be as high as that found in Spain from where Argentina has received one of its major immigration waves.Conclusions: The overall frequency of GJB2/GJB6 mutations in the present sample is in agreement with other Caucasian populations. As expected, c.35delG was the most prevalent mutation. The deletion del(GJB6-D13S1830) was the second most common mutation. These findings reinforce the importance of the study of GJB2/GJB6 genes in diagnosis to provide early treatment and genetic counselling.

HPV types causing juvenile recurrent laryngeal papillomatosis in South Africa

R.Y. Seedat, M. Thukane, A.C. Jansen, I. Rossouw, D. Goedhals, F.J. Burt — 2010-03-01

Abstract: Objectives: To identify human papillomavirus (HPV) types associated with juvenile onset recurrent laryngeal papillomatosis (RLP) in southern Africa, to determine if there is a correlation between HPV type and disease aggressiveness and to determine the diagnostic and prognostic value of rapid molecular techniques for detection and typing of HPV using laryngeal biopsies.Methods: Laryngeal biopsies from patients undergoing surgery for RLP were screened for HPV using conventional and real-time PCR techniques. Amplicons were sequenced to determine the HPV type involved. Clinical features were correlated with HPV type.Results: HPV was identified in papillomata from 18 out of 19 patients. Only HPV-6 and HPV-11 were identified, with no co-infections. There was 100% concordance between conventional and real-time PCR techniques. Patients with HPV-11 disease required more procedures and tended to have higher Derkay scores than those with HPV-6 disease. The HPV types identified in our patients were genetically similar to HPV types from geographically distinct regions.Conclusions: RLP in our patient population appears to be exclusively due to HPV-6 or HPV-11. HPV-11 disease appears to be more aggressive than HPV-6 disease. Identification of the HPV types provides motivation for inclusion of vaccines against these types in vaccination programs to protect women against infection and subsequently reduce the incidence of RLP.

UK and Ireland experience of cochlear implants in children with Down Syndrome

P.S. Hans, R. England, S. Prowse, E. Young, P.Z. Sheehan — 2010-03-01

Abstract: Objectives: Down Syndrome (DS) is associated with a high incidence of hearing loss. The majority of hearing loss is conductive, but between 4 and 20% is sensorineural, which in the main is mild or moderate and is managed with conventional behind-the-ear hearing aids. Cochlear implantation is an elective invasive procedure, performed to provide some form of hearing rehabilitation in individuals with severe to profound sensorineural hearing loss, and initially candidacy criteria were strict—excluding patients with additional disabilities. With good results and expanding experience, more candidates with additional disabilities are now being implanted. A survey of UK and Ireland Cochlear Implant Programmes sought to identify the number of individuals with DS who have been implanted with a cochlear implant (CI) and to provide relevant information on outcomes of implantation in these individuals.Methods: E-mail survey of all programmes within the British Cochlear Implant Group (BCIG). Postal questionnaire to programmes identified to have implanted a child with Down Syndrome, with data collection on pre-operative assessment, surgical and post-operative outcomes. Case series review.Results: Three of 23 BCIG programmes have implanted a child with Down Syndrome. Four children have received implants. No intraoperative or post-operative surgical complications were encountered. All children had middle ear disease, but no problems with implantation were associated with their middle ear condition. All children remain implant users, 12 months to 4 years post-implantation.Conclusion: Cochlear implantation is an option for a child with Down Syndrome and associated severe to profound sensorineural hearing loss. Clinicians caring for these children and their families should consider referral for assessment by a Cochlear Implant Programme.

Qualitative analysis of parents’ experience with receiving the news of the detection of their child's hearing loss

Peter Gilbey — 2010-03-01

Abstract: Objective: Despite the fact that clinicians are responsible for delivering bad news, they have been shown to lack both confidence and skill in performing this basic task. The time immediately after the detection of childhood hearing loss is perceived as stressful. We conducted a qualitative study to assess parents’ experiences with receiving the bad news of the detection of their child's hearing loss.Study design: Semi-structured interviews were conducted with families of children with hearing loss identified during early childhood.Setting: A rehabilitation center treating pre-school children in the north of Israel.Patients: 14 families/parents of children diagnosed prior to the implementation of a universal screening program.Main outcome measure: Parents’ perceptions of the manner in which the information regarding the detection of their child's hearing loss was given, and what their feelings were at the time.Results: 50% of parents expressed dissatisfaction with the process of the breaking of the bad news. ABR is perceived by parents and health professionals alike as the definitive moment of diagnosis. The emotions experienced by parents at the moment of the breaking of the bad news were predominantly shock and upset. The meaning of the news was perceived differently under different circumstances. Information given bluntly, without empathy, was a frequent complaint. Parents repeatedly stated the importance of the formulation of a plan for the future.Conclusions: Qualitative enquiry provided valuable information. Effective strategies for the breaking of bad news should become an integral part of universal neonatal screening programs.

Otitis media with effusion: Recurrence after tympanostomy tube extrusion

2010-03-01

Abstract: Objective: The incidence rate of recurrence of otitis media with effusion (OME) in children after tympanostomy tube extrusion and the relationship between recurrence and tube retention time was investigated. Also relationship between recurrence rate and initial age of tube insertion was analysed.Study Design: A retrospective case series of patients who had tympanostomy tube insertion.Methods: A total of 91 children (169 ears) were reviewed. Ears of children divided into three groups according to retention time of tympanostomy tubes. Group I: tympanostomy tube retention time less than 6 months. Group II: tympanostomy tube retention time 6 months to 12 months. Group III: tympanostomy tube retention time 12 months or more.Results: OME recurrence rate after tube extrusion was 20.7% in the study. The longer the tympanostomy tube retention time was the lower was the recurrence rate of OME. The comparison of the three groups indicated that recurrence rates were higher in group I (36.54%), than in group II (17.74%) and in group III (9.1%). There were statistically significant differences in recurrence rates between group I and group III, and between group I and II (p<0.05, p<0.05). However, the difference in recurrence rates between group II and group III was statistically not significant (p>0.05). In the preschool age group and school age group, the recurrence rates were 5.5% and 15.4%, respectively. There was no significant difference between these two groups (p>0.05). OME recurrence was observed in 9.9% of males and in 11% of females. There was no significant difference in recurrence rates between males and females (p>0.05).Conclusion: After extrusion of tympanostomy tube, children should be followed-up regularly for recurrence of OME. The shorter the retention time of tympanostomy tubes was the higher was the recurrence rate. For the treatment of OME the ideal type of tubes should have the lowest complication and recurrence rates. Further studies are needed to ascertain the relationship between the incidence of OME and optimal tympanostomy tubes duration of tube stay in tympanic membrane. Therefore, new studies with larger series are necessary to investigate the correlation between the recurrence rates and different tympanostomy tubes after extrusion of tubes.

Epidemiological analysis of Microtia: A retrospective study in 345 patients in China

Jinfang Wu, Ruhong Zhang, Qun Zhang, Zhicheng Xu, Wenxin Chen, Datao Li — 2010-03-01

Abstract: Objective: To study the epidemiologic characteristics of microtia in China and to investigate the possible risk factors with respect to the classification of microtia.Methods: A total of 345 patients with microtia were studied. All patients were taken an intentional physical examination and classified into five types. A detailed questionnaire concerning the maternal conditions during pregnancy was filled out by patient's mother. The frequencies of the relative factors were counted and the variables were statistically analyzed using Chi Square and Fisher's tests in the five types of microtia.Results: Most cases of microtia (88.12%) were sporadic and 72.75% of all cases occurred in males. It was more seen unilateral, especially affected on the right side (55.94%). A total of 195 patients (56.52%) were isolated microtia. In the rest of non-isolated microtia patients, 37.97% of the cases had hemifacial microsomia, which was the most common associated deformity. Thirty-four patients (9.86%) belonged to typical familial microtia. Three maternal factors showed significant differences in the five types of microtia, which were perinatal virus infection, high prior miscarriages and prevention treatments for threatened abortion.Conclusions: The majority of microtia cases in China are sporadic and usually more common in males. Mothers who have prior miscarriages over 3 times or perinatal virus infection seem to be more likely to have severe microtia infants.

Ulcerative tracheitis among intubated neonates—A clinicopathologic study

2010-03-01

Abstract: Objective: To determine frequency of ulcerative tracheitis (UT) among intubated neonates and identify groups of neonates at greater risk of UT.Methods: Medical histories and autopsy findings from 232 neonates between 1995 and 2006 were reviewed retrospectively. All neonates were treated at the Department of Neonatology, Clinical Hospital Center Split. Autopsies and histological examinations were performed at the Clinical Department of Pathology, Forensic Medicine and Cytology. Neonates were classified into groups based on their sex, gestational age, survival time and cause of death, duration and reasons for intubation. Simplified Wigglesworth classification was used to determine cause of death. Chi-squared test and multiple logistical regression analysis were used for statistical analysis.Results: UT was found in 44 cases (18.96%). The main risk factor for UT development was duration of intubation, especially if it lasted over 96h (P=0.005). Higher frequency of UT was noted in neonates with gestational age over 37 weeks (P=0.002) and birth weight over 2500g (P=0.115).Conclusion: Our study has shown that the main risk factor for UT development was intubation duration exceeding 96h. Term-borns, and neonates with normal birth weight both have higher risk of UT. High risk groups should be observed carefully and alternative respiratory support treatment, such as continues positive airway pressure (CPAP) may be considered.

Characteristics of hearing impairment in Yemeni children with chronic suppurative otitis media: A case–control study

2010-03-01

Abstract: Background: Chronic suppurative otitis media (CSOM) is a serious disorder particularly in low resource settings. It can lead to disabling hearing impairment and sometimes life-threatening infective complications.Objective: The aim of the present study was to describe the characteristics of hearing impairment associated with CSOM in Yemeni children.Methods: A case–control study of 75 children with CSOM and 74 healthy controls. Hearing was assessed by behavioural testing and audiometry.Results: Cases had lower academic performance than controls (OR 15.31, 95% CI 1.99–322.14, p<0.001). Disabling hearing impairment >30dB was present in 51.5% (right ear) and 66.7% (left ear) of children with CSOM.Conclusion: Disabling hearing impairment was identified as a major health problem in these Yemeni children with CSOM. There is a need for investment to reduce the burden of CSOM and its complications in these communities. Greater attention to the chronic disabling effects of CSOM in children is required in poor communities and low resource settings.

Early resection and reconstruction of head and neck masses in infants with upper airway obstruction

2010-03-01

Abstract: Objective: Congenital airway obstruction can be caused by tumors or lesions arising from the neck, tongue and oral cavity. Neonates may require prolonged intubation or tracheostomy before curative resection and reconstruction. The aim of the study was to carry out surgical resection and reconstruction with locoregional flap and free bone graft in the neonatal and early infantile period for definitive management of head and neck masses and treatment of potential airway obstruction.Methods: Newborns with obstructive head and neck masses in Queen Mary Hospital, University of Hong Kong Medical Centre between 2006 and 2009 were operated on in the neonatal period.Results: There were one obstructive neck teratoma, two intraoral teratomas and one neuroglial heterotopia. All tumors were resected within the first 3 months of life without major complication. A local cervical cutaneous flap was first used to reconstruct the lateral pharyngeal wall defect in a neonate with a huge neck teratoma, followed by another infant with a neuroglial heterotopia. A piece of cranial bone was used for reconstruction of the skull base defect. None of them required tracheostomy or prolonged intubation. Oral feeding was resumed in the early postoperative period.Conclusion: Surgical resection and reconstruction with locoregional flap and bone graft can be performed safely in neonatal and early infantile period as management of head and neck masses and treatment of upper airway obstruction.

Otoplasty in children younger than 5 years of age

Murat Songu, Hamit Adibelli — 2010-03-01

Abstract: Objective: The prominent ear is the most common congenital deformity of the auricle. It is often recommended that prominent ears be surgically repaired before children start school and most surgeons seem to perform the surgery after 5 years of age. The aim of our study is to summarize the rationale of performing otoplasty procedure in children under the age of 5, to discuss the advantages and disadvantages, and to review the patient (parent) satisfaction.Methods: A retrospective study was performed on 10 children under the age of 5 who underwent otoplasty procedure and was followed for over a year.Results: Ten patients (3 boys and 7 girls) between the ages of 48 months and 59 months, with a median age of 51.5 months were evaluated. Otoplasty was bilateral in 8 patients and unilateral in 2 patients. Global Aesthetic Improvement Scales of the patients were rated as “improved” or “better” at 52 weeks. The patient (parent) satisfaction was measured by a telephone survey. Parents revealed that 9 out of 10 were “very” or “completely” satisfied with the appearance and symmetry of their children's ears. We did not observe any visible disturbance or growth restriction in our patients, even in the unilateral operated group.Conclusion: Timing of surgery is an issue of concern with regard to otoplasty in children. There may be significant psychosocial benefit to early intervention, particularly in light of changing norms for interaction with peers at ages considerably earlier than what had previously been thought of as the “school age.” Our preference is to plan the otoplasty as young as four years of age, after the child has expressed some concern about the deformity.

Preliminary outcomes of cholesteatoma screening in children using non-echo-planar diffusion-weighted magnetic resonance imaging

2010-03-01

Abstract: Objective: Diffusion-weighted (DW) MRI imaging is evolving into an alternative to second look surgery in detection of cholesteatoma recurrence. Insights into the DW MRI appearances of postoperative or inflammatory mucosal changes have recently described using non-echo-planar, turbo spin-echo (TSE) DW MRI which reliably distinguishes between postoperative changes and cholesteatoma. We investigated the use of TSE DW MRI in our pediatric population in order to validate a rapid and cost-effective MRI sequence that can be used to screen for cholesteatoma.Methods: Prospective comparative study with adult and pediatric patients at a tertiary referral centre. Patients in the study underwent TSE DW MRI prior to second look or revision surgery for cholesteatoma. A Siemens 1.5T scanner was employed, using the HASTE sequence (EPI DW MRI) as well as standard echo-planar DWI, T1 and T2 sequences. The MRI findings were then correlated with the intraoperative findings at surgery 9–15 months after primary surgery, or of revision surgery in the cases that were referred from other centres. Detection and localisation of cholesteatoma on TSE DW MRI were compared with the findings at second surgery, long considered the gold standard for detection of residual or recurrent disease. Scanning time between the TSE sequence and the standard planar DW MR were also compared.Results: In a cohort of 92 patients, 21 pediatric patients were identified. 15 patients have had their 15 second look or revision procedures and DW MRI prior to their surgery. TSE DW MRI detected cholesteatoma and reliably identified the location of the cholesteatoma in 2 patients whom all had disease confirmed at surgery. The 13 cases with negative preoperative DW MRI for cholesteatoma were all confirmed to be disease free at surgery. Scanning time of the TSE sequence takes 100s as opposed to 20min using standard echo-planar DW MRI techniques without the requirement of a contrast agent and without the need for a general anaesthetic for any of the children.Conclusion: TSE (HASTE) DW MRI is emerging as a cost effective, noninvasive alternative to second look surgery for detection and screening for cholesteatoma in pediatric patients.

Respiratory tract involvement in a child with epidermolysis bullosa simplex with plectin deficiency: A case report

2010-03-01

Abstract: We report a rare case of a child with epidermolysis bullosa simplex (EBS) with plectin deficiency but without muscular dystrophy, with severe lesions of the oral cavity, oropharyngeal, hypopharyngeal, laryngeal, tracheal and bronchial mucosa. Case report and a review of the world literature are used. The literature review revealed only five similar patients with EBS without muscular dystrophy complicated by respiratory involvement. This paper highlights the potentially serious complications of the EB in the form of breathing, swallowing and speech difficulties and describes the specific problems encountered in the treatment of this patient. Epidermolysis bullosa (EB) is a group of severe hereditary diseases, primarily of the skin, but which can also involve the respiratory and gastrointestinal tract mucosa. Respiratory tract involvement is usually only found in certain types of EB. The oral cavity and oropharynx are involved more frequently than the hypopharynx, larynx and trachea. Involvement of laryngeal and tracheal mucosa is generally associated with an increased morbidity and mortality, numerous complications and therapeutic difficulties, and is more common in junctional EB and dystrophic EB than in EBS. We present a rare case of a child with EBS and plectin deficiency with pronounced lesions of respiratory tract mucosa from the oral cavity to the bronchi and even extending into the trachea. Deciding on tracheotomy requires thorough consideration and should not be taken lightly.

A case report of frontonasal dysplasia

J. Kean, S.S.M. Al-Busaidi, A.A. Quaba — 2010-03-01

Summary: The following report describes a case of mild frontonasal dysplasia, a rare congenital anomaly affecting the nose and frontal bone. Our patient was referred at 2 months of age with a complex nasal skin and tip deformity. This report outlines management of this patient and reviews the literature with regard to presentation and classification of this condition.

Long-term impact of tongue reduction on speech intelligibility, articulation and oromyofunctional behaviour in a child with Beckwith–Wiedemann syndrome

K.M. Van Lierde, G. Mortier, E. Huysman, H. Vermeersch — 2010-03-01

Abstract: The purpose of the present case study was to determine the long-term impact of partial glossectomy (using the keyhole technique) on overall speech intelligibility and articulation in a Dutch-speaking child with Beckwith–Wiedemann syndrome (BWS). Furthermore the present study is meant as a contribution to the further delineation of the phonation, resonance, articulation and language characteristics and oral behaviour in a child with BWS. Detailed information on the speech and language characteristics of children with BWS may lead to better guidance of pediatric management programs. The child's speech was assessed 9 years after partial glossectomy with regard to ENT characteristics, overall intelligibility (perceptual consensus evaluation), articulation (phonetic and phonological errors), voice (videostroboscopy, vocal quality), resonance (perceptual, nasometric assessment), language (expressive and receptive) and oral behaviour. A class III malocclusion, an anterior open bite, diastema, overangulation of lower incisors and an enlarged but normal symmetric shaped tongue were present. The overall speech intelligibility improved from severely impaired (presurgical) to slightly impaired (5 months post-glossectomy) to normal (9 years postoperative). Comparative phonetic inventory showed a remarkable improvement of articulation. Nine years post-glossectomy three types of distortions seemed to predominate: a rhotacism and sigmatism and the substitution of the alveolar /z/. Oral behaviour, vocal characteristics and resonance were normal, but problems with expressive syntactic abilities were present. The long-term impact of partial glossectomy, using the keyhole technique (preserving the vascularity and the nervous input of the remaining intrinsic tongue muscles), on speech intelligibility, articulation, and oral behaviour in this Dutch-speaking child with congenital macroglossia can be regarded as successful. It is not clear how these expressive syntactical problems demonstrated in this child can be explained. Certainly they are not part of a more general developmental delay, hearing problems or cognitive malfunctioning. To what extent the presence of expressive syntactical problems is a possible aspect of the phenotypic spectrum of children with BWS is subject for further research. Multiple variables, both known and unknown can affect the long-term outcome after partial glossectomy in a child with BWS. The timing and type of the surgical technique, hearing and cognitive functioning are known variables in this study. But variables such as children's motivation, the contribution of the motor-oriented speech therapy, the parental articulation input and stimulation and other family, school and community factors are unknown and are all factors which can influence speech outcome after partial glossectomy. Detailed analyses in a greater number of subjects with BWS may help further illustrate the long-term impact of partial glossectomy.

Use of zoledronic acid in the treatment of Gorham's disease

2010-03-01

Abstract: Gorham's disease (Gorham–Stout syndrome) is a rare condition of unknown etiology involving a localized endothelial proliferation of lymph vessels resulting in destruction with bone resorption. The syndrome is rarely seen in the facial skeleton and has a large variety of prognoses and treatments. A case of this syndrome in a 9-year-old boy is presented. The clinical aspects are described, together with the treatment involving zoledronic acid. Other treatments described in the literature are reviewed. The authors believe that this report is one of the first cases in which a child afflicted in the first decade of life survives.

Initial experience using propranolol as the sole treatment for infantile airway hemangiomas

Stephen Maturo, Christopher Hartnick — 2010-03-01

Abstract: The objective of this study is to describe the initial use of propranolol as the sole treatment for focal infantile airway hemangiomas, and to report on available literature describing the use of propranolol for airway lesions. This retrospective case series was carried out at a tertiary pediatric medical center. We obtained the following results: two children demonstrated significant response to oral propranolol therapy and avoided not only invasive surgical procedures, but also long-term administration of oral corticosteroids. This is the first report of treating infantile airway hemangiomas with only propranolol without additional surgical intervention or corticosteroid use. Review of literature reveals initial case series with similar, successful results using propranolol as an adjuvant treatment along with other medications and surgical interventions. We conclude that the initial use of propranolol as the sole treatment for infantile airway hemangioma is promising. Literature review reveals that propranolol as the sole treatment for most head and neck hemangiomas shows significant promise based on early case reports. Further studies are needed to determine the long-term effectiveness, dosing strategies, and side effect profile of propranolol treatment for hemangiomas.

KTP laser: An important tool in refractory recurrent tracheo-esophageal fistula in children

2010-03-01

Abstract: Secondary tracheo-oesophageal fistula in delayed primary repair of oesophageal atresia is rare. This paper reports the successful use of the KTP laser in the treatment of this condition in a refractory case. It also recommends the use of direct laryngotracheobronchoscopy (DLTB) in the diagnosis. We recommend the use of this laser in cases of recurrent tracheo-esophageal fistula especially when other means have failed.

Calendar of Events

2010-03-01

Guide for Authors

2010-03-01