Although previous studies have shown that dexamethasone is useful in croup, the optimal dosage has not been clarified yet. The aim of this study was to investigate the effectiveness of dexamethasone 0.15 mg/kg single dose compared with the recommended dose of 0.6 mg/kg for treatment of hospitalized children with moderate to severe croup.
Forty-one children aged 6 months to 5 years admitted from March 2001 to October 2003 at the pediatric ward of Hatyai Hospital with moderate to severe croup were recruited for the study. After random allocation, each child received a single intravenous injection of either dexamethasone 0.6 mg/kg (maximum dose 12 mg) or 0.15 mg/kg (maximum dose 3 mg). All children were given a single dose of epinephrine nebulization at the beginning of the study. The croup scores were measured at 0, 1, 2, 3, 4, 6, 8, 10 and 12 h after the dexamethasone injection.
One hour following the injection, the mean croup scores were significantly reduced from baseline values in both groups (p<0.01). There was no difference in the mean croup scores between the two groups at any time. Median times to croup scores equal or less than two in both high- and low-dose groups were 8 and 7.9 h, respectively. No patient required intubation or was removed from the study because of a rising croup score after enrollment. There was no significant adverse reaction from dexamethasone treatment in either group.
Dexamethasone 0.15 and 0.6 mg/kg are equally effective in the treatment of moderate to severe croup.
We reported a 2-year-old boy with developmental delay, mild mental retardation, and severe craniofacial malformation, including facial asymmetry with hypoplasia of the left zygoma, maxilla, and mandible, and left anophthalmia and anotia. A genome-wide screen revealed a 1.38Mb duplication on chromosome 1q31.1, which was absent in his parents and 27 healthy controls. The duplication region contains two Refseq genes, PLA2G4A and C1orf99, which have not been reported to be implicated in craniofacial malformation. Functional studies of these genes and additional clinical analysis are necessary to elucidate the pathogenesis of craniofacial malformation.
The aim of our study was to evaluate the effectiveness of combining newborn hearing screening with screening for genetic mutations associated with deafness.
Ten thousand forty-three newborn babies, born between December 2009 and April 2011 in Gansu province, China, were screened for hearing loss using the otoacoustic emissions test or automatic auditory brainstem response test and genetic mutations associated with deafness using a standard protocol.
In the hearing screening, the referral rate for hearing loss in the first-step screening was 14.4% (1409/9786), decreasing significantly to 3.8% (362/9506) upon retesting. After the second-step screening, a total of 537 newborns were lost to follow-up. The genetic screening found that about 2.29% (230/10,043) individuals carried one or more recessive risk alleles or the mitochondrial mutation. Among them, 18 babies had the pathogenic mitochondrial DNA mutation, 92 babies were SLC26A4 heterozygote carriers, one case with both SLC26A4 and 12S rRNA 1555A>G mutation, 117 babies were GJB2 heterozygote carriers, and two babies were GJB2 homozygote carriers. However, 83.5% (192/230) neonates passed the conventional hearing screening among these carriers.
It might be effective to complement the conventional hearing screening with gene screening for the purpose of early diagnosis and discovery of the late-onset hearing loss.
For children with Down syndrome, the incidence of hearing loss may be as high as 78% , therefore the American Academy of Pediatrics recommends regular screening for the presence of hearing abnormalities. Tympanometry is used as an indication of middle ear pathology. In our experience, Down's patients' tympanograms do not always correlate with otoscopic findings. Down's patients have joint laxity, small ear canals, anterior tympanic membrane orientation and softer tissue composition, all factors thought to affect tympanogram results in infants. Because the use of the 1000 Hz tympanometry is widely recognized as standard procedure in the evaluation of infants aged 0-6 months, we propose it may have greater reliability in testing patients with Down syndrome.
Compare the results of visual inspection of the tympanic membrane by a Pediatric Otolaryngologist to the results of tympanometry at traditional probe tone (226 Hz) and at the infant probe tone (1000 Hz).
Institutional Review Board - approved prospective study of 26 subject-ears in patients with Down syndrome aged 6 months-18 years but recent stable middle ear/Eustachian tube function using physical examination and tympanometric probe tones at 226 Hz and 1000 Hz. Subject-ears were examined with record of "clear of effusion," showed the presence of "fluid," or were to be "excluded." Blinded to ear exam results, tympanometry was then completed with record of which Jerger classification tympanogram was found at each frequency.
Although the sensitivity of each test was 1, the specificity of the 1000 Hz tympanometry (100%) in this study was markedly improved compared to the specificity of the 226 Hz tympanometry (71%) (p=0.016).
This pilot study demonstrated evidence that tympanometry in children with Down syndrome may be more reliable at 1000 Hz than at 226 Hz in detecting the presence of middle ear effusion beyond infancy. Use of the 1000 Hz probe tone yielded fewer false positives for disease (type B tympanograms in the setting of absent middle ear disease). Further studies of a larger patient population are needed to corroborate these results.
Universal newborn hearing screening (UNHS) has significantly reduced the age of children undergoing audiological examinations. Middle ear function is usually evaluated with tympanometry using a probe tone of 226 Hz, although higher frequencies are recommended in infants. The aim of this study was to compare the feasibility of 226 and 1000 Hz tympanometry for different trace classification systems in relation to age, risk factors for hearing loss and ear canal volume.
Data from 577 infants (915 ears) <1 year were analyzed. Tympanograms were classified according to the classification systems of Jerger, Marchant et al. and Kei et al. and correlated with ear microscopy as the gold standard. Test quality parameters of tympanometry with probe tones of 226 and 1000 Hz were compared in four different age groups.
The trace classification following Kei et al. presented the best correlation to ear microscopy and reduced the number of unclassified tympanograms. The use of probe tones of 226 Hz in infants below the age of nine months showed a poor level of sensitivity.
We recommend the use of a 1000 Hz probe tone in infants at least up to the age of nine months. In this age group, 226 Hz tympanometry is inappropriate. In children with craniofacial abnormalities and smaller ear canal volumes, 1000 Hz tympanometry could be taken into consideration, even for older children. High-frequency tympanograms should be evaluated according to the classification system of Kei et al., which differentiates between peaked (normal) and flat (abnormal) curves.
To determine the differences between tracheal foreign body aspiration and bronchial foreign body aspiration.
This retrospective study includes 1007 patients with the diagnosis of foreign body aspiration according to rigid bronchoscopy. Patients were divided into two groups: tracheal and bronchial foreign body groups. Age, sex, clinical presentation, pre-operative radiographic findings, rigid bronchoscopy findings, types of foreign body, and the complications of each group were observed and analyzed.
Out of 989 study patients, there were 146 patients (14.5%) in the tracheal foreign body group and 843 patients (83.7%) in the bronchial foreign body group. Eighteen patients with FBs located in the larynx and presenting with multiple FBs located in the tracheal and bronchial regions were excluded from this study. The majority of the patients were under the age of three for both groups. The male to female ratio was significantly higher in the bronchial foreign body group (P<0.001). More patients in the bronchial foreign body group were misdiagnosed upon first clinical visit (P=0.001), referred from another hospital (P<0.05), or had delayed diagnosis (P<0.05). The most prominent symptom in both groups was cough, followed by decreased breath sounds, wheezing, and dyspnea. More patients in the bronchial foreign body group experienced decreased breath sounds (P<0.001), while more patients in the tracheal foreign body group experienced dyspnea (P<0.05). Chest fluoroscopy abnormalities were observed at a higher frequency in the bronchial foreign body group (P<0.001). Lateral neck X-ray results showed higher frequencies of abnormalities in the tracheal foreign body patients. Out of 30 patients in the tracheal foreign body group received a CT scan, 27 had abnormal scan results, while all 253 patients in the bronchial foreign body subgroup had abnormal results. The majority of foreign bodies were organic materials and were removed by rigid bronchoscopy at the first clinical session in 96.6% of tracheal foreign body cases and 96.0% of bronchial foreign body cases (P=0.727). Major complications, including one death, were observed only in the bronchial foreign body group.
The nature of tracheal foreign body aspiration is different from bronchial aspiration. Clinical presentation and pre-operative radiographic findings are helpful for diagnosis. The clinician should understand the differences between tracheal and bronchial FB cases and provide the appropriate management when either is presented.
Occurrence of foreign bodies (FBs) in ear, nose and throat (ENT) in children are not uncommon in clinical practice. We described our experience with ENT foreign bodies in Melanesian children.
The study was carried out at the tertiary referral center which is also a University teaching hospital. The 15-year period from 1990 to 2004, 1037 cases of ENT foreign bodies were managed. The clinical, operative and follow up data of these patients were collected from available clinic cards and admission charts. A retrospective analysis was made with these data.
Foreign bodies in the external auditory canal constituted 711 (68.6%) cases. It was followed by nose in 258 (24.9%), pharynx in 26 (2.5%), esophagus in 21 (2.0%) and laryngotracheobronchial (LTB) tree in 21 (2.0%) cases. Innumerable varieties of foreign bodies were removed from the ear. The common ones were stones in 277 (39.0%), seeds in 142 (20.0%), plastic ornament beads in 70 (9.8%) and cotton in 48 (6.8%) cases. Six hundred and thirteen (86.2%) of them were removed under microscope at the clinic without any complications. The difficult ones in uncooperative children were removed in the operation theatre. They numbered 98 (13.8%). The nasal foreign bodies were exclusively limited up to the age of 12 years. Common nasal foreign bodies were foam in 54 (20.9%), seeds in 51 (19.8%), stone in 28 (10.9%) and ornament beads in 24 (9.3%) cases. Two hundred and forty-six (95.3%) of them were removed in the clinic. Only 12 (4.7%) were removed in the operation theatre. Fishbone was the commonest (23, 90.2%) foreign body encountered in the pharynx of the children. Coins were the exclusive esophageal foreign bodies in children and all were removed in the operation theatre under general anesthesia. In the LTB tree the FBs were mostly localized to the right main bronchus which occurred in 11 (52.4%) cases. This was followed by the trachea in four (19.1%) cases. The different types of FBs encountered were coffee beans in eight (38.0%), peanuts in five (23.8%), plant seeds in three (14.2%) cases. We encountered two mortalities in these LTB FB patients.
Otolaryngological foreign bodies in children are common. For early diagnosis a high index of suspicion has to be maintained by pediatric otolaryngologist.
The aim of the study was to assess the speech development of children with at least 2 years' hearing experience with a cochlear implant (CI).
One hundred and six children were tested, all of whom had used the CI for at least 2 years. Receptive and expressive language development were tested using the scales of early communication skills for hearing-impaired children after Geers and Moog and the Reynell developmental language scales III. In addition, free-field audiometry was performed. Pre-postoperative measurements were compared statistically, and a linear regression analysis was carried out.
Clear improvements in the gain in functional hearing 2 years after receiving the CI as well as clear improvements in both speech perception and speech production were ascertained. All deviations were statistically highly significant (P<0.000). The receptive and expressive speech test results correlate positively to a great extent. The results of logistic regressive analysis indicate that the speech production measured depends decisively on age at implantation, age at time of test, speech production before implantation, and additional handicaps.
Based on the conception of the WHO, a statistically significant improvement of both impairment and disability can be confirmed. In the present study, the regression analysis established the age at implantation as the most important prognostic factor. While the percentage of children with good speech development is larger with early implanted children than with late implanted children, some of the children among the early implanted group show unsatisfactory speech development of unknown origin. Improvements in speech development are likely to be achieved when more children are diagnosed and implanted early. This finding urgently requires the introduction of a general newborn screening program. Thorough anamnesis and assessment during parent counseling of the manifold factors described are a prerequisite for the precise estimation of the difficulties to be met and the expected effectiveness of the implant in each individual case.
The aim of this study was to analyze the incidence and etiologic factors of non-use and limited use of cochlear implants. The patients' age, gender, duration of implantation and additional disabilities were investigated.
Patients and methods:
Of the 413 (200 males, 213 females) pediatric patients (age under 16) implanted in our clinic between January 2000 and December 2011, 12 limited user/non-user cochlear implanted patients were selected who had a follow-up of at least 24 months. Preoperative and postoperative listening progress profile (LiP) and meaningful auditory integration scale (MAIS) tests were performed to analyze the auditory performances of the patients.
In total of 12 recipients (2.90%) (7 male and 5 female patients; age range, 5-13 years), 4 (0.96%) patients were non-users and 8 (1.93%) patients were limited users. The patients had some additional disabilities as autism, cerebral palsy, moderate mental retardation, attention deficit/hyperactivity disorder, ossified cochlea due to meningitis and learning disability-lack of family interest. None had experienced device failure. In the postoperative 24th month, listening progress profile and meaningful auditory integration scale test scores were better in the limited users as expected.
It should always be considered in patients with additional factors like autism, mental-motor retardation, learning disabilities that they will show limited development from cochlear implantation. These patients are potential limited/non-users. These patients require unique rehabilitation and provide high family and educational interest.
This study explores the experience at Sydney Children's Hospital (SCH) managing children with acute mastoiditis and establishes a robust treatment algorithm.
Retrospective review of all patients admitted to SCH with an ICD-10 coding of "Mastoiditis" from 1 January 1996 through 31 December 2006 inclusive. Criteria assessed included demographic characteristics, clinical features, symptom duration and treatment initiated by the general practitioner. The results of investigations at SCH were reviewed including white blood cell count, microbiology and imaging. The presence of complications was determined and the results of medical and surgical treatment were assessed.
Seventy-nine episodes of acute mastoiditis were managed in 76 patients. Treatment prior to SCH was commenced by the family practitioner or district hospital doctor in 53/79 patients. The mean duration of community initiated treatment before presentation to SCH was 3.7 days. In 33 episodes a previous history of acute otitis media was noted (42%). In the remaining 46 episodes (58%) mastoiditis was the initial diagnosis. Complications were found in 30 episodes (38%) and 36 episodes (46%) required surgical treatment.
Mastoiditis often develops rapidly but may be treated very effectively. The potential for significant morbidity remains high but excellent outcomes can be expected for those who are managed without delay. Children with acute mastoiditis should be managed in centres where timely and complete medical and surgical treatment is available.
The ability to understand speech requires processing of rapidly changing acoustic information. Much more is known about processing the rapid spectro-temporal properties of speech than is known about processing of intensity, even though intensity is a fundamental cue for accurate speech perception. The purpose for the current study was to characterize, in 9-11-year-old typically language-developing children, the auditory event-related brain potentials elicited by different tone intensities when presented in complex environments (i.e., varying in frequency and intensity) at rapid rates.
Pure tones of four different intensity levels (66, 74, 78, and 86dB SPL) and five different stimulus frequencies were presented at a stimulus rate of 10Hz. The latency and amplitude of the auditory event-related brain potentials were measured.
At this fast rate, a positive (P1) followed by a negative component was elicited. The lowest intensity sound elicited the lowest P1 amplitude and the highest intensity sound elicited the highest P1 amplitude. The P1 elicited by the two middle tone intensities had amplitudes that fell between the lowest and highest amplitudes but they were not significantly different from each other. The negative component following the P1 was unaffected by intensity variation.
Intensity variation of sounds presented in a complex environment at a rapid rate modulated only the amplitude of the earliest obligatory auditory component (P1), consistent with our previous studies in which only the P1 could follow the rapid stimulation rate. P1 amplitude changes reflected the relative differences among the sounds, not the absolute differences in loudness among the sounds presented together in the sequence. The results suggest that the environment, or context, within which rapid sounds occur, influences the relative amplitude of the P1 in children.
The objective of this paper is to assess the treatment protocols used in our institution for the management of airway obstruction among patients with Robin sequence and to suggest a rationale for management based on the findings.
A retrospective study of 110 children with Robin sequence seen from 1988 to 1997 at the Chang Gung Memorial Hospital was done. Results of the management in airway obstruction and feeding difficulty were studied.
Prone posturing was effective in the treatment of mild airway obstruction in 82 patients who had noisy breathing sounds. Twenty-eight infants required endotracheal intubation due to severe respiratory distress. Seven received a tongue to lip adhesion (TLA) operation. Three of these patients showed a relief of airway obstruction, while four of them needed a tracheotomy to maintain a patent airway because of wound dehiscence. Two other patients underwent tracheotomy without TLA. All the six patients who received tracheotomy were eventually decannulated. A nasopharyngeal tube was inserted in two other patients, and one of them improved only temporarily. With regards to feeding difficulties, 46 patients needed nasogastric tube feeding, while the rest were aided through the use of a cleft palate bottle and nipple. A gastrostomy was not needed in any of the patients in this series.
The morbidity and mortality among Robin sequence patients had been widely decreased through the teamwork of the pediatrician, anesthesiologist, otolaryngologist, dentist, and plastic surgeon. Based on our experience, conservative management was sufficient for those patients with mild airway obstruction, while endotracheal intubation was required for patients with cyanosis, respiratory failure and sleep apnea. Tracheotomy was a better option than TLA for airway management among patients with failed extubation.
The purpose of this study is to investigate the early myelination patterns of brainstem auditory nuclei and pathway on magnetic resonance imaging compared with past histological research. We aimed to identify the time course difference in myelination of the brainstem auditory nuclei and pathway between magnetic resonance imaging and histological research results.
Subjects were 192 infants ranging in age from -4 to 224 corrected postnatal weeks. Images were obtained using a 1.5 T magnetic resonance unit. In four sites (cochlear nucleus, superior olivary nucleus, lateral lemniscus, inferior colliculus) of the brainstem auditory nuclei and pathway on four cross-sections obtained perpendicular to the long axis of the brainstem, signal changes of T1- and T2-weighted magnetic resonance images were analyzed using a region-of-interest methodology according to corrected postnatal age.
The cochlear nucleus and superior olivary nucleus showed myelinated intensity change from -3 to 13 corrected postnatal weeks on T2-weighted images. The lateral lemniscus showed myelinated intensity change from -3 to 8 corrected postnatal weeks on T1-weighted images and from -1 to 13 corrected postnatal weeks on T2-weighted images. The inferior colliculus showed myelinated intensity change from -2 to 39 corrected postnatal weeks on T2-weighted images.
Magnetic resonance imaging revealed the signal intensity change by myelination 11-18 weeks later than those reported in the histological literature. This time lag suggests that apart from histological research, the necessity for the milestones of auditory pathway maturation using MRI is suggested to evaluate the development of brainstem auditory pathway using MRI. This result suggests that myelination does not take place suddenly but happens gradually, so definite myelination, namely the complete change of myelin sheath ingredients, loss of water, and gain of lipids, is needed to be detected by magnetic resonance imaging.
Between 1985 and 1990, 113 children were diagnosed as having congenital vocal cord paralysis. Most of them were still being followed up in June 1994. Fifty two had bilateral paralysis, 61 had unilateral paralysis: 41 were on the left side and 20 on the right side. Forty two were idiopathic, 29 were associated with neurologic disorders, six were associated with heart malformations. Fifteen children were born after difficult delivery. Among the newborns with unilateral paralysis that occurred after an abnormal delivery, 73% recovered spontaneously; likewise 70% of the neurologic group and 74% of the idiopathic group recovered spontaneously. The prognosis of bilateral paralysis was worse with only 52% of spontaneous recovery in the neurologic disorders group and the idiopathic group. Seven percent of the children underwent a surgical procedure. They were all decanulated or extubated. In view of our experience the prognosis is poor for bilateral idiopathic laryngeal paralysis or those with neurological context.
Functional endoscopic sinus surgery (FESS) is not frequently performed in children. The aim of our retrospective analysis was to determine symptoms, surgical extent, complications and outcome of operated children.
For a period from 1994 to 2004 the clinical records of the Department of Otorhinolaryngology, LMU Munich, Germany, were screened for pediatric FESS procedures. Subsequently, a retrospective chart review was performed in all cases. For assessment of outcome, symptom- and quality of life-related questionnaires were sent out to all patients.
115 children had a FESS procedure due to CRS, 77 were boys and 38 girls. The response rate of the questionnaires was 64% (73 of 115); the mean follow up of these was 5.4 (+/-1.8) years. 76% of the patients reported an improvement of their chief symptoms and 71% of their general quality of life. The overall quality of life had improved significantly (p<0.01) on VAS. In CRS patients nasal obstruction was completely relieved in 62.3%, facial pain in 65.5% and postnasal drip in 72.5%. Improvement of primary nasal symptoms (PNS) of CRS in patients with CF, asthma or allergies as well as in youngsters who had started or continued to smoke 35 out of 73 (48%) was significantly less.
Self-assessment after FESS showed in 76% improvement of chief symptoms in children with CRS. Besides known negative prognostic factors for long-term outcome as CF, asthma, nasal polyps, allergies and previous surgery, smoking was correlated with less favourable improvement.
Review a large series of patients with Robin sequence to document the incidence of (1) associated syndromic diagnoses; (2) co-morbid conditions; (3) frequency and type of operative management for airway compromise and feeding difficulties; and (4) possible differences in treatment between syndromic and nonsyndromic infants.
Retrospective case-review of 115 patients with Robin sequence managed between 1962 and 2002 at two tertiary-care teaching hospitals for evaluation of demographic information, clinical findings, and treatment interventions.
Fifty-four percent (N=63) of patients were nonsyndromic. Syndromic patients included: Stickler syndrome (18%), velocardiofacial syndrome (7%), Treacher-Collins (5%), facial and hemifacial microsomia (3%), and other defined (3.5%) and undefined (9%) disorders. There was no statistical difference between the syndromic and nonsyndromic patients with regard to need for operative airway management (Fisher's exact test, p=0.264). Forty-two percent of patients required a feeding gastrostomy tube to correct feeding difficulties. Patients with a syndromic diagnosis were more likely to be developmentally delayed. Fifty-one (44%) patients underwent operative airway management: 61% underwent tongue-lip adhesion and 39% underwent tracheotomy. Fifteen percent of patients initially had tongue-lip adhesion subsequently required tracheotomy. While the preferred treatment for respiratory compromise differed between the two institutions, the percentage of patients requiring operative intervention was similar.
The pathogenesis of Robin sequence is multifactorial and syndromic in nearly half of the patients. Operative treatment of respiratory failure was required in 44% of infants; the rate was similar in both hospitals. The operative approach differed significantly between the institutions, however, based on the philosophy and training of the managing surgical specialty. Co-morbid factors such as baseline cardiopulmonary and neurologic status did not play a significant role in surgical decision making.
The purpose of this study was to evaluate our 25-year experience of pediatric salivary tumors.
Patients less than 19 years old with a diagnosis of salivary tumor were identified at Oral-maxillofacial Head and Neck Tumor Center, China Medical University from 1987 to 2011.
122 patients were included in our study, 105 tumors were benign and 17 were malignant. The most common involved site was parotid gland, pleomorphic adenoma represented 91.4% in benign group, and mucoepidermoid carcinoma represented 47.1% in malignant group. All tumors in submandibular gland were benign. Warthin's tumor was diagnosed in one patient. In our follow-up, no regional recurrences occurred in patients without neck dissection. The 5- and 10-year overall survival rates of patients with malignant salivary tumors were 81.8% and 66.7% respectively.
Epithelial salivary tumor was rare, parotid gland was most common involved site, pleomorphic adenoma and mucoepidermoid carcinoma were the most common benign and malignant tumor respectively. The prognosis of patients with malignant salivary tumors was not very favorable.
During 10 years, 127 children were admitted to the pediatric ward because of aspiration of foreign bodies (0.56% of all admissions). Eighty-one percent of the children were under 3 years of age. One hundred and one children (80%) had a positive history of foreign body aspiration. Vegetable substances, particularly peanuts and grains, were the commonest type of foreign body removed. Clinical signs and radiological studies were, in most children, pathognomonic, but sometimes not conclusive. Chest X-rays were normal in 18%; fluoroscopy was diagnostic in 92%. Rigid bronchoscopy and subsequent removal of the foreign body was the treatment instituted. Pneumonia (the commonest complication) developed before and after bronchoscopy in 28% of the children. Pneumomediastinum and subcutaneous emphysema were present on admission in 2 children. Cardiac arrhythmias, bronchospasm, and cardiac arrest were recorded during bronchoscopy. Bronchiectasis developed in one, and persistent intractable pneumonia, requiring lobectomy, developed in another patient. One death occurred.
1274 T-tubes were inserted for persistent otitis media with effusion in 661 patients over a 4-year period. The peak ages for insertion were between 4 and 7 years, and by 11 years the condition is uncommon. If a tube has not extruded spontaneously after 30 months it becomes increasingly less likely to do so. Complications were persistent perforations (32.6%), tympanosclerosis (23.6%), repeated otorrhoea or tympanic membrane granulations (21%), and impacted wax (10.9%). Forty-eight percent of patients experienced one or more complication in the study period, and the complication rate increased dramatically in patients whose tubes had remained in situ for longer than 36 months. After this time surgical removal is recommended.
Since the widespread availability and use of antibiotics the prevalence of Lemierre syndrome (L.S.) has decreased. It is a well-described entity, consisting of postanginal septicaemia with thrombophlebitis of the internal jugular vein with metastatic infection, most commonly in the lungs. The most common causative agent is a gram-negative, non-spore-forming obligate anaerobic bacterium, Fusobacterium necrophorum (F.n.). We describe the unusual clinical features of a 12-year-old boy with Lemierre syndrome with isolated hypoglossal nerve palsy - the latter symptom is an extremely rare manifestation of this disease.
To express a 13-year experience in transnasal endoscopic repair of congenital choanal obliteration and to analyze the different predictors that may affect the surgical outcome.
Retrospective clinical study.
Otolaryngology Department, Tanta University Hospital, Egypt.
Over a 13-year period (from January 1996 to December 2008), 54 children aged between 7 day and 14 year old at the time of surgery underwent transnasal endoscopic repair of an unilateral (32 cases) and bilateral (22 cases) congenital choanal obliteration using conventional instruments and microdebrider/drill to remove the atretic plate, bony boundaries, and vomer. A laterally based mucosal flap was routinely used to resurface most of the raw areas of the lateral and superior borders of the created choana followed by shortened period stenting (5-7 days). Patients were then clinically and endoscopically monitored for nasal obstruction and healing for a mean follow-up of 46+/-6 months. Demographic characteristics of these patients, associated anomalies, surgical technique, postoperative stenting, complications, postoperative care, and surgeons' learning curve were discussed in the light of the findings of the surgical outcomes.
There were 33 females (61.1%) and 21 males (38.9%). A total of 12 patients (22.2%) presented with associated anomalies. There were no major intra-operative or postoperative complications. The success rate for bilateral choanal obliteration was 86.4% and for unilateral choanal obliteration 96.9% with an overall success rate 92.3%. Revision surgery of the relapsed cases using the same technique without mucosal flap was successful 100%.
Transnasal endoscopic repair of congenital choanal obliteration is an easy and evolving technique with adequate safety and high success rate. Early diagnosis and good preparation of the patients for surgery, adequate resection of the bony boundaries of the obliterated choana and vomer, shortened period nasal stenting, strict early and late follow-up, and the growing experience of the surgeons, all were prognostic factors for success. Associated anomalies and use of mucosal flap did not seem to affect the prognosis. Inadequate bone resection, bilaterality, pure bony obliteration, the younger the age of the patient, and early developing experience of the surgeons, all were possible predictive factors for relapse.
Allergic rhinitis is one of the most frequent chronic diseases among children. The objective of the study was to assess the prevalence of rhinoconjunctivitis and determine clinical, social and environmental associated factors, among school children aged 6-7 years and adolescents aged 13-14 years in Bogotá, Colombia.
We used ISAAC (International Study of Asthma and Allergies in Childhood) phase III study methodology and questionnaire for design and data collection, and we did a secondary analysis of these data. The sample consisted of 3830 registers from adolescents between 13 and 14 years old and 3256 registers from children between 6 and 7 years.
The prevalence of rhinoconjunctivitis symptoms in the last year was 17.2% (95% CI, 15.9-18.5) in children vs. 24.9% (95% CI, 23.6-26.3) in adolescents. In both groups there was a female predominance in children (17.8% vs. 16.5%), and in the teenagers group (28.0% vs. 21.4%), though the differences were not statistically significant. The factors associated with rhinoconjunctivitis in the group of 6-7 years where: asthma [OR 3.9; (95% CI, 2.8-5.4)], atopic dermatitis [OR 2.3; (95% CI, 1.7-3.1)], use of acetaminophen in the last year [OR 2.6; (95% CI, 1.4-4.9)], use of antibiotics in the first year of live [OR 1.7; (95% CI, 1.3-2.3)], higher maternal education [OR 1.5; (95% CI, 1.0-2.3)] and cesarean delivery [OR 1.6; (95% CI, 1.2-2.1)]. Among the 13-14 year age-group, factors associated with rhinoconjunctivitis included: asthma [OR 2.6; (95% CI, 2.0-3.4)], atopic dermatitis [OR 1.8; (95% CI, 1.4-2.3)], use of acetaminophen in the last year [OR 1.8; (95% CI, 1.4-2.4)], consumption of fast-food three times or more per week [OR 1.5; (95% CI, 1.2-2.0), ever smoked [OR 1.4; (95% CI, 1.2-1.7)] and meat consumption was protective factor [OR 0.7; (95% CI, 0.5-0.9)].
In both studied groups, the estimated prevalence of rhinoconjunctivitis symptoms was relatively high. Future in-depth research is needed to assess the complex interactions between allergic rhinoconjunctivitis symptoms and social and environmental factors.
Subglottic stenosis in young children represents a formidable management problem. The role of radiological evaluation, endoscopy, endotracheal intubation, tracheotomy, decannulation and surgery in the overall approach to this group of patients is discussed. Experience with laryngotracheal reconstruction in 13 patients is related; decannulation was achieved in 11 children. In general, 3 types of surgical procedures for laryngotracheal reconstruction have been described in the literature. Surgery is indicated in a minority of children with persistent significant subglottic stenosis. Specific contraindications to surgery are delineated. Appropriate management of endotracheal tubes and avoidance of aggressive endoscopy will minimize the development of subglottic stenosis.
Acute mastoiditis is a potential complication of acute otitis media (AOM), with Streptococcus pneumoniae historically the most common pathogen isolated. Following the release of the 7-valent pneumococcal conjugate vaccine in 2000, a marked decline in invasive pneumococcal disease and a smaller reduction in pneumococcal AOM were observed, but data regarding its impact on acute mastoiditis are limited. With the recent introduction of the 13-valent pneumococcal conjugate vaccine (PCV13), it is anticipated that pneumococcal AOM and invasive disease will further diminish. We report a case of acute mastoiditis from a multidrug-resistant serotype 19A S. pneumoniae in an immunocompetent child who had received three PCV13 vaccinations.
To determine the microbiology of otitis media (OM) since the introduction of the 13-valent pneumococcal conjugate vaccine (PCV13) in February 2010.
Middle ear effusion from a pediatric Otolaryngology population undergoing pressure equalization tube (PET) placement was obtained and sent for aerobic culture and antibiotic susceptibility testing between August 2012 and April 2013. Vaccination records were obtained and statistical analysis was completed.
During the 8-month period, 236 ears were evaluated, and of those 39 ears were found to have positive cultures. The single nonvaccine Streptococcus pneumoniae (serotype 16) isolate was obtained from a PCV7-only vaccinated patient and was penicillin susceptible. The three most common isolates were Staphylococcus coagulase negative (57%), Haemophilus influenzae (17%), and Moraxella catarrhalis (7%).
This study is the first to assess the bacteriology of OM in a pediatric population undergoing PET placement in the immediate post-PCV13 era. Our study is limited by sample size; however, the lack of S. pneumoniae cultures indicates that PCV13 has had a significant impact on pneumococcal infections during these initial years following licensure.
To describe temporal bone histopathology in an infant with partial trisomies of chromosomes 13 and 14.
Temporal bones were taken at autopsy from a 7-day-old neonate who has both partial trisomy 13 and partial trisomy 14. The right temporal bone was embedded in celloidin and sections were cut for microscopic examination. The left temporal bone was studied by microdissection. The middle ear was examined and the inner ear sensory organs dissected for study by light microscopy.
The external auditory canal was stenotic in both ears. Remnants of mesenchymal tissue were present in the middle ear cavity. The middle ear ossicles were normal except that both stapes were malformed with a single crus and a small footplate. Both facial nerve canals were dehiscent in the region of the oval window. The cochlea was malformed bilaterally; a scala communis was present and the basilar membrane was abnormally short. No loss of sensory cells was observed in either cochlea. Blood vessels were found traversing scala vestibuli and there were cystic lesions in the stria vascularis and spiral ligament. In the middle cochlear turn, the bony wall of scala vestibuli and the osseous spiral lamina were covered by a substantial layer of connective tissue which appeared to be an extension of the spiral ligament. This is an unusual finding which, to the authors' knowledge, has not been previously reported. In the vestibular apparatus a wide communication was present between the saccule and utricle.
As this study demonstrates, abnormalities of the external, middle, and inner ear may occur in cases of partial trisomy 13 and partial trisomy 14. Both temporal bone findings and clinical features in partial trisomy 13 and partial trisomy 14 to some degree overlap with those of trisomy 13, partial trisomy 13 and partial trisomy 14.
Congenital choanal atresia (CCA) is the developmental failure of the nasal cavity to communicate with the nasopharynx. Surgical repair is recommended in the first weeks of life in bilateral cases, because in newborns this is a life-threatening situation. This is a case report of a 13-year-old patient complaining of long-term bilateral nasal obstruction and rhinorrea, in whom bilateral choanal atresia was diagnosed by endoscopic exploration and CT scan, and who was treated by an endonasal endoscopic surgical technique. Bilateral choanal atresia is a life-threatening disease in newborns; however, it can be diagnosed in adults with bilateral nasal obstruction and rhinorrea.
Impalement injuries of the soft palate and oropharynx are common injuries especially in children. Devastating neurologic sequelae secondary to thrombus propagation in the internal carotid artery are rare but well-documented complications of these innocuous appearing injuries. Neurologic sequelae have been reported anywhere from 3 to 60 hours after the injury. Protocols that include hospitalization up to 72 hours have been advocated. In order to evaluate the effectiveness and practicality of such protocols, all hospital admissions for palate injuries in the past 17 years were reviewed. Of the 131 cases reviewed, no complications were discovered during hospitalization, nor were there any complications in these patients seen in follow up. Based on this review, the majority of soft plate and oropharyngeal impalement injuries can be managed on an outpatient basis. Parental counseling, similar to instructions given in instances of mild head trauma, as well as close follow up in the office are felt to be a more reasonable and as well as a safe treatment plan. A suggested instruction form is presented.
Laryngobronchoscopy (LBS), using both rigid and flexible bronchoscopes, has become a frequently performed operation in children. A data base was established to enable retrospective evaluation of a large number of LBSs carried out in a pediatric center. Experience with 1332 cases of LBS involving 808 patients over an 8-year period is presented. The main indications for LBS were inspiratory stridor, atelectasis, and suspected foreign body aspiration. Most frequent diagnoses at LBS were bronchopneumonia, intubation trauma, tracheomalacia, laryngomalacia, and foreign body aspiration. Only 25 complications occurred (1.9%) including two cases of xylometazoline intoxication.
To identify and characterize food items with high risk of airway obstruction in children younger than 15 years.
This retrospective study collected injury data from 1989 to 1998 for 26 pediatric hospitals in the United States and Canada. Aspiration, choking, ingestion, and insertion injuries due to food items were analyzed. The data included 1429 infants and children. Results were compared with fatality data published by the American Association of Pediatrics in 1984.
The 10 food objects with the highest frequency for both injuries and fatalities were identified. Peanuts caused the highest frequency of injury, and hot dogs were most often associated with fatal outcomes. The severity of respiratory distress prior to hospital evaluation varied for different foods. Age younger than 3 years was the highest-risk factor. Key characteristics such as bite size, shape, and texture were analyzed and found to demonstrate relationships with severity of clinical outcomes.
Children younger than 3 years remain at greatest risk of food injury and death. We found that hard, round foods with high elasticity or lubricity properties, or both, pose a significant level of risk. Consideration of the key characteristics of the most hazardous foods may greatly decrease airway obstruction injuries. Food safety education can help pediatricians and parents select, process, and supervise appropriate foods for children younger than 3 years to make them safer for this highest-risk population.
To assess the rate of tracheocutaneous fistula requiring surgical repair and the complications and outcomes following it in the ENT department of a tertiary referral university paediatric hospital.
A retrospective review of all children requiring airway support with a tracheostomy between 1995 and 2009 and subsequently requiring closure of tracheocutaneous fistula.
One hundred and ninety-three children underwent 196 tracheostomies. Seventy-three children were successfully decannulated until now. Twenty-three children (11.9%) required subsequent surgical closure of their tracheocutaneous fistula. In all these children the age at tracheostomy was less than 1 year old, and the median age at decannulation was 4 years old, (range 2-9 yo). Surgical repair was undertaken 6-12 months after decannulation. There were 4 minor complications in the postoperative period (wound infection, haemorrhage and early recurrence) and no major complications. None of the patients have experienced any degree of significant airway stenosis and there was no need for a repeat tracheotomy in any of the tracheocutaneous fistula closure patients. The cosmetic results were deemed to be good.
Our rate of tracheocutaneous fistula compares well with the reported rates in the literature (13-43%). There appears to be a significant relation to age at tracheostomy and duration of tracheostomy.
Public Law (P.L.) 94-142, although greatly increasing the physician's role in delivery of services to handicapped children, falls short in meeting the needs of hearing impaired children from birth to 36 months in that it does not mandate referral services until age three years. The literature has shown that both conductive and sensorineural hearing losses cause delay in the development of normal speech and language. Further, those children identified and treated earlier demonstrate higher skill levels on speech and language tasks than those children identified late. The High Risk Register for hearing loss has been found a reliable means to identify such children. Given that the most critical period for learning language is from birth to three years and that at least two objective and reliable procedures for evaluating the hearing of infants and pre-school children, Impedance Audiometry and Brainstem Evoked Response Audiometry, are becoming more readily available in the United States, it is no longer acceptable to support the "wait and see" approach in referring suspected hearing impaired children for auditory, speech, and language diagnostics and habilitative intervention.
The GJB2 and MTRNR1 1555A>G mutations are the prevalent causes of hearing loss worldwide. However, the mutation profiles of the two genes are dependent on the ethnic or geographic origins. Therefore, this study was to characterize the forms and frequencies of the two genes in 813 students with hearing loss in Hubei province, Central China.
Blood samples from 813 students were obtained with informed consent. Genomic DNA was extracted from peripheral blood leukocytes. The target fragments were amplified by polymerase chain reaction (PCR). Sequencing (or enzyme digestion) was applied to identify sequence variations.
Ten different mutations were identified in GJB2 in 146 of the 813 (17.96%) patients and 11.81% (96/813) patients had homoplasmic mtDNA 1555A>G mutation.
This study demonstrated the high prevalence of GJB2 and mtDNA 1555A>G mutations in Central Chinese population. Therefore, it will be effective to perform GJB2 and mtDNA 1555A>G mutation analysis for genetic screening for hearing loss in this population.
Mutations in mitochondrial DNA (mtDNA), especially the A1555G transition in the 12S rRNA gene, are one of the causes of both aminoglycoside-induced and non-syndromic sensorineural hearing loss.
The aim of this study was to determine the prevalence of the A1555G mitochondrial mutation in Moroccan patients.
We performed molecular characterization by PCR-RFLP and direct sequencing of one hundred and sixty four patients (84 unrelated familial and 80 sporadic cases) with a congenital sensorineural non-syndromic hearing loss and one hundred normal hearing controls for the occurrence of the A1555G mutation.
Mutational analysis of the mtDNA showed the presence of the homoplasmic A1555G mutation in three families, leading to a frequency of 3.6% similar to that reported for European-populations. No A1555G mutation was detected in sporadic and controls cases. However, we detected in twenty normal hearing controls a novel polymorphism A1557C, which was not found in patient samples. We further evidenced the presence of the A1438G mitochondrial polymorphism in four patients with sensorineural hearing loss and in five controls.
Our results show that the occurrence of the A1555G mutation in hearing impaired patient's accounts for 3.6% in a Moroccan patients and those novel mtDNA polymorphisms might contribute to a novel sub-haplogroup specific of the Magrheb.
Serefeddin Sabuncuoglu is known to be the author of the first illustrated surgery book, Cerrahiyyetu'l Haniyye (Imperial Surgery), which was written in Turkish in 1465 a.d. at the age of 80. The purpose of this paper is to describe his contributions to pediatric otorhinolaryngology.
Cerrahiyyetu'l Haniyye consists of three chapters totaling 412 pages, in which there are 191 sections dealing with a wide range of surgical specialties, including pediatric otorhinolaryngologic surgery. Sabuncuoglu explains some of child diseases and their treatment in a whole section, and gives information on the same subject in relevant part of sections. The illustrations he provides are not at a state of the art level with those of his contemporaries, but they bear a great importance considering the attitudes of Islam to the art of painting. For each section, a single, poetic sentence describes in detail the diagnosis, classification and surgical technique. Some authors have claimed that he only translated Ebu Kasim-al Zahravi (Albucasis)'s Al-Tasrif (Textbook of Surgery) and added the illustrations of the surgical techniques. Even if this is accepted, the illustrations are enough to entitle this work as a milestone. Serefeddin Sabuncuoglu describes medical and surgical management pediatric otorhinolaryngologic diseases such as harelip, hypertrophy of adenoids, atresia of external auditory canal, a foreign body in external auditory canal, short frenulum, abscess of head, mass and abscess of neck in children and unpleasant odor in nose in his textbook.
Although not recognized and rewarded in his time, Serefeddin Sabuncuoglu was a great surgeon in Turkish medical history. The sections on children in his book are of great importance in pediatric otorhinolaryngology which became a different speciality in the last century. Writing his book at his 80's, he deserves to be considered as a distinguished scientist, medical doctor and illustrator of his era, considering the insulting and discriminating ideas toward women and children, especially girls, at that period.
Few studies exist which document the early speech development of German-speaking children or German-speaking children who are deaf and using cochlear implants. The current study aims to: (1) document the pre-canonical and canonical speech development of German-speaking children who are deaf and receive cochlear implants by the age of 16 months and (2) compare these children's results with those of children with normal hearing.
This longitudinal study included 5 German-speaking children with normal hearing and 5 with sensorineural deafness. All children from the deaf group received hearing amplification before cochlear implantation, received their first implant by 16 months of age, and became bilateral implant users by 31 months of age. The pre-canonical and canonical vocalisations of each child were recorded on video- and audiotapes in a semi-standardised playing situation every 4 weeks over a span of 1 year. In the cochlear implant group, the recording started 4-5 days postoperatively (first implant); in the normal hearing group it began between the ages of 4 and 5 months. The video and audio recordings were analysed using EUDICO Linguistic Annotator version 2.4 (Nijmegen, The Netherlands) and International Phonetic Alphabet transcription.
Both groups showed individual patterns of babbling acquisition, though the groups' patterns of acquisition were similar when analysed for consonant manner and place. Some children started with plosives and others, with nasals, but all acquired fricatives and laterals next. Onset of canonical babbling for children in the cochlear implant group began 0-4 months after first fitting of the first device, while children from the normal hearing group demonstrated an onset of canonical babbling between 4 and 9 months of age.
Our results show that deaf children who receive cochlear implants at an early age are capable of reaching the canonical babbling milestone in a shorter time than children with normal hearing typically do and that their consonant phoneme acquisition follows a similar sequence to normal hearing peers'. These results are consistent with the literature indicating that early identification and intervention are important for allowing children with cochlear implants the opportunity to catch up to hearing peers.
Few studies have specifically assessed the risk factors for persistence or recurrence of OME in a cohort of school-age children. The generally accepted etiological factors for OME occurrence may not apply in the same way when the presence of OME over a year from original diagnosis is assessed.
A cohort of 250 school-age children with unilateral or bilateral OME, identified through screening of 5121 asymptomatic children was re-examined 16 months later. All were assessed for a variety of demographic, family and medical factors. Measures included tympanometry, acoustic reflexes and a complete otolaryngologic examination.
At 16 months after initial confirmation of OME, 56 out of 250 children (22.4%) suffered from OME, 21 bilateral and 31 unilateral. Presence of OME at 16 months was not associated with gender, blood group, gestational age and weight, history of breast feeding, paternal education level and smoking history, history of allergy, previous use of antibiotics, or with surgery (myringotomy, insertion of ventilation tubes or adenotonsillectomy). In multiple backward-eliminating logistic regression, the only factors associated with OME presence after 16 months were episodes of AOM during the study period (odds ratio 2.75 (95% CI: 1.13-8.17), p=0.04) and younger age (odds ratio 0.53 (95% CI: 0.32-0.79), p=0.002 for each 2 years of increase in age).
Seventy-eight percent of school-age children identified with OME through screening will be free of disease 16 months later. The threshold for referral, or surveillance could however justifiably be lower in children who (a) have once been identified with OME and (b) are (relatively) younger, or have experienced an episode of acute otitis media.
To compare the characteristics of acute mastoiditis in children in different age groups in order to identify risk groups and risk factors for acute mastoiditis.
Records for all children aged 0-16 years treated for acute mastoiditis during 1993-2007 at 33 Ear, Nose and Throat departments in Sweden were reviewed retrospectively according to defined criteria for acute mastoiditis.
A total of 678 cases fulfilled the inclusion criteria. Acute mastoiditis was most common in children younger than two years of age and this group was characterized by less prior history of other diseases and ear diseases, fewer visits to health care centers and less antibiotic treatment before admission, shorter duration of symptoms before admission, hospitalization for fewer days and lower frequency of complications and mastoidectomies. These children also showed a higher incidence of clinical findings, increased inflammatory markers such as fever and heightened counts of C-reactive protein and white blood cells compared with older children. They also tested positive for significantly more samples of Streptococcus pneumoniae while the older children more often exhibited growth of Streptococcus pyogenes or Pseudomonas aeruginosa or no microbial growth.
The characteristics of pediatric acute mastoiditis differed significantly between age groups. Acute mastoiditis was most common in children younger than two years of age. They showed more rapid progress of symptoms and more distinct signs of acute mastoiditis. This is probably the reason why parents rapidly seek medical care for small children and hospital treatment thus starts earlier in the youngest children, which may in turn explain the excellent outcome. This study showed that younger children have neither more severe acute mastoiditis nor more complications than older ones. The differences between age groups suggest that there are distinctions in the pathophysiology behind the onset and course of acute mastoiditis in younger and older children.
The preferred treatment of pediatric intraoral ranulas remains controversial. We present our experience with ranulas at the Gyeongsang National University Hospital and review the literature.
The study involved 17 children under 15 years of age who visited our hospital from 2001 to 2008 and were diagnosed with a ranula exceeding 2 cm in diameter. The age and gender of the patients, the surgical procedures, presurgical observation period, postsurgical follow-up period, and complications of each case were determined. The unruptured specimens were all subjected to detailed pathological analysis.
The patients were on average 9.3 years and there were 10 girls and 7 boys. Spontaneous resolution was not detected in any of the cases during the presurgical observation period. Indeed, in two cases, the ranula had increased in size. The ranula and sublingual gland (SG) were resected in all cases. The average operation time was 1h. Recurrence and complications were not detected in any of the cases. Pathological analyses revealed that there was no communication of the ranula with the SG in any of the cases.
Our experiences suggest that the presurgical observation period need not be longer than 3 months and that the resection of ranulas along with the ipsilateral SG is a safe and effective primary treatment for symptomatic pediatric intraoral ranulas that exceed 2cm in diameter.