Cartilage tympanoplasty for retraction pocket of the tympanic membrane in children.

Background: Retraction pocket (RP) of the tympanic membrane (TM) is a common pathology in children that can cause ossicular chain erosion, cholesteatoma formation, and potentially life-threatening complications of cholesteatoma. This study assessed the functional and anatomical results of cartilage grafting in children with severe RP of the TM. Methods: This was a retrospective review of 212 children from a tertiary referral center. Results: We identified significant differences in hearing results, indication criteria, and location of TM fixation between stages II and III of RP (according to Charachon). We observed a significantly higher incidence of RP in boys than in girls. Conclusions: Cartilage tympanoplasty for retraction pocket of the tympanic membrane in children is a safe procedure with good anatomical and hearing results.

Two Bonebridge bone conduction hearing implant generations: audiological benefit and quality of hearing in children.

PURPOSE: The study aimed to evaluate audiological benefits, quality of hearing and safety of two Bonebridge generation: BCI601 and BCI602 (MED-EL, Innsbruck, Austria) in children. METHODS: Twelve children were implanted: five BCI601 and seven BCI602 comprising of ten conductive hearing loss, and two single sided deaf SSD subjects. Audiological outcomes tested were sound field audiometry, functional gain, speech recognition threshold (SRT50), speech recognition in noise (SPRINT) and localisation abilities. Subjective measures were Speech, Spatial and Qualities of Hearing Scale (SSQ12). RESULTS: The mean FG with the BCI601 was 25.0 dB and with the BCI602 28.0 dB. The benefit in SRT50 was 23.2 dB and 33.8 dB, respectively. The mean benefit in SPRINT was 15% and 6.7% and the localisation ability improved from 33.3° to 16° and from 26.2° to 17.6°, respectively. The two SSD subjects reported a FG of 17 dB, a benefit in SRT50 of 22.5 and a benefit in SPRINT of 20%. Subjective outcomes improved significantly and even exceeded the values of their age-and sex matched normal hearing peers. One revision was reported: a retroauricular emphysema above the implant occurred 12 months post-OP, it was resolved operatively with the implant still being functional. CONCLUSION: The pediatric cohort reports significant audiological benefit, even exceeding that of the age- and sex matched control. The combination of the high safety and audiological benefit makes the Bonebridge a comfortable and effective option in hearing rehabilitation in children.

Extensive Parapharyngeal Abscess in a 4-Month-Old Infant.

Parapharyngeal abscess in an infant is a very rare condition. We present the case of a 4-month-old girl with large masses on the neck's left side. Computed tomography showed an extensive parapharyngeal abscess. Left tonsillectomy was performed under general anesthesia from a transoral approach, followed by an incision and evacuation of the abscess from the parapharyngeal space. Microbiological analysis identified a massive occurrence of Streptococcus intermedius.

Videolaryngoscopy vs. Direct Laryngoscopy for Elective Airway Management in Paediatric Anaesthesia: A prospective randomised controlled trial.

BACKGROUND: The indirect visualisation of the glottic area with a videolaryngoscope could improve intubation conditions which may possibly lead to a higher success rate of the first intubation attempt. OBJECTIVE: Comparison of videolaryngoscopy and direct laryngoscopy for elective airway management in paediatric patients. DESIGN: Prospective randomised controlled trial. SETTINGS: Operating room. PARTICIPANTS: 535 paediatric patients undergoing elective anaesthesia with tracheal intubation. 501 patients were included in the final analysis. INTERVENTIONS: Patients were randomly allocated to the videolaryngoscopy group (n  = 265) and to the direct laryngoscopy group (n  = 269) for the primary airway management. MAIN OUTCOME MEASURES: The first attempt intubation success rate was assessed as the primary outcome. The secondary outcomes were defined as: the time to successful intubation (time to the first EtCO2 wave), the overall intubation success rate, the number of intubation attempts, the incidence of complications, and the impact of the length of the operator's clinical practice. RESULTS: The study was terminated after the planned interim analysis for futility. There were no significant demographic differences between the two groups. The first attempt intubation success rate was lower in the videolaryngoscopy group; 86.8% (n  = 211) vs. 92.6% (n  = 239), P = 0.046. The mean time to the first EtCO2 wave was longer in the videolaryngoscopy group at 39.0 s ±â€Š36.7 compared to the direct laryngoscopy group, 23.6 s ±â€Š24.7 (P < 0.001). There was no difference in the overall intubation success rate, in the incidence of complications nor significant difference based on the length of the clinical practice of the operator. CONCLUSIONS: The first attempt intubation success rate was lower in the videolaryngoscopy group in comparison to the direct laryngoscopy group. The time needed for successful intubation with videolaryngoscopy was longer compared with direct laryngoscopy. TRIAL REGISTRATION: Clinicaltrials.gov identifier: NCT03747250.

Videofluoroscopic Swallow Study in Diagnostics of H-Type Tracheoesophageal Fistula in Children.

Esophageal atresia remains one of the most challenging congenital anomalies of the newborn. It can occur with or without tracheoesophageal fistula (TEF), and to date, there are still no universally recommended diagnostic procedures. The so-called H-type TEF is that without esophageal atresia, and its prevalence is lower than 5% of all TEFs. We present a case report of a newborn with regurgitation, vomiting, feeding problems, dyspnea, and repeated aspiration bronchopneumonia. A wide range of diagnostics procedures had been performed with negative results until we used videofluoroscopy, which revealed the H-type TEF and allowed appropriate treatment of the patient.

Novel mutations in TRPM6 gene associated with primary hypomagnesemia with secondary hypocalcemia. Case report.

BACKGROUND: Primary hypomagnesemia with secondary hypocalcemia (HSH) is a rare genetic disorder. Dysfunctional transient receptor potential melastatin 6 causes impaired intestinal absorption of magnesium, leading to low serum levels accompanied by hypocalcemia. Typical signs at initial manifestation are generalized seizures, tetany, and/or muscle spasms. CASE REPORT: We present a 5 w/o female manifesting tonic-clonic seizures. Laboratory tests detected severe hypomagnesemia and hypocalcemia. The molecular genetic analysis revealed two novel mutations within the TRPM6 gene c.3308dupC (p.Pro1104Thrfs*28) (p.P1104Tfs*28) and c.3958C>T (p.Gln1302*) (p.Q1302*) and the patient was successfully treated with Mg supplementation. CONCLUSION: Ion disbalance should be taken into account in the differential diagnosis of infantile seizures. Accurate diagnosis of HSH together with appropriate treatment are crucial to prevent irreversible neurological outcomes.

Pathogenesis of Retraction Pocket of the Tympanic Membrane-A Narrative Review.

Several theories describe the development of the retraction pocket of the tympanic membrane (RP). Many authors suggest that the negative middle ear pressure is the main reason responsible for developing this condition. A narrative review has been undertaken, and conclusions are drawn reflecting a current knowledge with our new observations in the histological and immunohistochemical study. Recent studies show the important role of inflammation in the development and progression of RP. A review of the available literature shows that the inflammation plays a key role in pathogenesis of the RP and its progression to the cholesteatoma. We support this statement with our new results from histological and immunohistochemical analysis of the RPs.

Risk Factors for Postoperative Bleeding after Adenoidectomy.

IMPORTANCE: Postoperative bleeding is a common and potentially life-threatening complication. Precise identification of risk factors in addition to the basic ones, such as coagulation parameters, is certainly very desirable. OBJECTIVE: The aim of this study was to identify other possible risk factors for bleeding after adenoidectomy in children. DESIGN: This observational prospective study enrolled children undergoing adenoidectomy from October 2019 to February 2020, then evaluated the influence of possible risk factors for bleeding. SETTING: Tertiary pediatric otorhinolaryngology center. PARTICIPANTS: A total of 288 children aged 0-18 years undergoing adenoidectomy for recurrent upper respiratory tract infections, recurrent acute otitis media, secretory otitis media, and obstructive sleep apnea syndrome. MAIN OUTCOMES AND MEASURES: Increased blood pressure and time of surgery were identified as risk factors for bleeding after adenoidectomy. RESULTS: Elevated systolic (p = 0.046), diastolic (p = 0.012), and mean arterial blood pressure (p = 0.007) (Mann-Whitney U test) as adjusted for age-specific distributions and with corrections for height and weight, as well as time length of surgery (p < 0.001) (Fisher's exact test) were revealed as statistically significant risk factors for postoperative bleeding. Atmospheric pressure, surgeon's level of experiences, chronic inflammatory content in adenoid vegetation (AV), size of AV, recidivism of AV, recurrent infections of the upper respiratory tract, type of anesthesia, long-term using of drugs, and positive coagulation questionnaire or pathology in standard coagulation tests were not found to be risk factors for bleeding after adenoidectomy. CONCLUSIONS AND RELEVANCE: In this prospective study within a well-defined population of children, we evaluated increased blood pressure and time of surgery as risk factors for bleeding after adenoidectomy. These data bring new information that complements current knowledge in this field.

Retroauricular Emphysema as a Late Complication After Bonebridge Implantation: Case Report.

Bonebridge (BB) is the first active implantation system for bone conduction that is placed fully under the skin. Experience suggests that BB is characterized by low incidence of postoperative complications. This case report presents a rare case of a 16-year-old girl with incidence of emphysema occurring over the implant 1 year after operation. We performed a computed tomography scan that showed pockets of gas above the floating mass transducer so we provided the revision surgery and sealed the artificial opening with fat from the earlobe and fibrin glue. Since that time, no air has collected in the retroauricular area and the implant has been fully functional.

Salivary Gland Choristoma of the Middle Ear in a Child: A Case Report.

Salivary gland choristoma is an extremely rare middle ear pathology. We present the case of a 10-year-old girl with unilateral conductive hearing loss. Tympanotomy showed a nonspecific middle ear mass, absence of stapes, anomaly of incus, and displaced facial nerve. It was not possible to remove the mass completely. Histology confirmed salivary gland choristoma. The hearing in this case can be improved with a bone-anchored hearing aid.

Difficult airway prediction in paediatric anaesthesia (Diffair): Prospective observational study.

BACKGROUND: The incidence of difficult airway in paediatric patients is lower than that the adult population, and the majority should be predictable. AIMS: The primary aim of this trial was to evaluate the incidence of difficult airway in pediatric patients. The secondary aim was to predict difficult airway in these patients. METHODS: Paediatric patients undergoing elective surgery under general anaesthesia in a tertiary university hospital were examined, and a panel of difficult airway prediction tests was performed. We recorded the incidence, risk factors for difficult airway and events associated with difficult airway together with the sensitivity and specificity of tests for difficult airway and events associated with difficult airway prediction. RESULTS: We prospectively included 389 paediatric patients. The incidence of difficult airway was 3.6%; the incidence of events associated with difficult airway was 10%. The sensitivity for prediction of events associated with difficult airway during the pre-anaesthesia evaluation was 5.3% with the specificity 93.3%. In the operating room, the sensitivity of prediction was 15% with 97.8% specificity. CONCLUSION: We found minimal efficacy for preanaesthesia difficult airway prediction.

Experience with cholesteatoma behind an intact tympanic membrane in children.

INTRODUCTION: To systematically investigate all surgeries for cholesteatoma behind an intact tympanic membrane at our department. To identify predictive factors that can help the surgeon to plan surgery, surgical techniques, and follow-up treatment. MATERIAL AND METHODS: This retrospective study evaluates 21 child patients, who were operated in the period 2007-2017 on for cholesteatoma behind an intact tympanic membrane. RESULTS: A total of 202 primary operations were performed for cholesteatoma. In 21 cases (10,4%) there was a cholesteatoma behind an intact tympanic membrane and in 11 (5,45%) cases of it there was the congenital cholesteatoma. The most frequently affected area was the anterior-superior quadrant. The preoperative hearing loss increased significantly with disease severity (I-IV by Potsic). CONCLUSIONS: The classification system according to Potsic is sufficient and fully corresponds to the surgeon's needs. It has been clearly shown that a higher CC stage is associated with worse postoperative hearing results.

Immunohistochemical analysis of retraction pocket pars tensa of tympanic membrane in children.

AIMS: Immunohistochemical analysis of retraction pocket pars tensa of tympanic membrane in children. Identification of signs typical for cholesteatoma and support of retraction theory of cholesteatoma. STUDY DESIGN: a prospective study analysing 31 surgically removed retraction pockets. DEPARTMENT: University Hospital, Children's Medical Centre Methods: Retraction pockets processed by a standard process for immunohistochemical analysis. The observed findings were specified using antibodies CD45 LCA (leukocyte common antigen), CD31 (platelet endothelial cell adhesion molecule), D2-40 (marker of lymphatic endothelium), MMP9 (marker of degradation of connective tissue extracellular matrix) and Ki67 (cellular marker of proliferation). RESULTS: All observed parameters except for MMP9 had a significantly higher incidence in retraction pocket stage III compared to stage II according to Charachon. CONCLUSION: We described immunohistochemical signs of retraction pocket pars tensa of tympanic membrane in children resulting in cholesteatoma. All the observed signs occur in the structure of matrix and perimatrix of cholesteatoma. A significantly higher incidence of all observed parameters except from MMP9 was proved in retraction pocket stage III, unlike in stage II. This observation proves the fact that retraction pocket is a progressive disease and is a procholesteatoma stage.

Laryngopharyngeal Reflux Is a Potential Risk Factor for Juvenile-Onset Recurrent Respiratory Papillomatosis.

INTRODUCTION: Human papillomavirus (HPV) causes juvenile-onset recurrent respiratory papillomatosis (JORRP). Although HPV is common in children, the prevalence of JORRP is low. It is likely that other factors contribute to the pathogenesis of JORRP, during either activation or reactivation of a latent HPV infection. There is evidence that laryngopharyngeal reflux (LPR) might be such a risk factor for adult-onset recurrent respiratory papillomatosis. This study investigated if LPR might also be a risk factor for JORRP. MATERIALS AND METHODS: Children with JORRP of the larynx that required microlaryngoscopy at a tertiary referral hospital were included in this prospective case-series study from November 2015 to November 2017. Using immunohistochemistry, HPV infection and pepsin associated with LPR were diagnosed from laryngeal biopsies. RESULTS: Eleven children (aged 4-14 years) were analyzed. No patient had a history of immunodeficiency or tobacco smoke exposure. All patients underwent at least three previous surgeries due to JORRP and had been vaccinated against HPV in the past. Five children were treated using antivirotics and immunomodulators. The only known maternal risk factor was that three mothers were primiparous. All 11 samples were infected with HPV (type 6 or 11). Pathologic LPR was diagnosed in 5/11 children (45.5%). CONCLUSION: LPR may be a risk factor for JORRP, contributing to its development by activating or reactivating a latent HPV infection. Results are in accordance with those from our previous study in adults.

Chronic secretory otitis media and tympanoplasty in children.

The article presents the most frequent type of chronic middle ear inflammation in children and the middle ear surgery. Middle ear reconstructions in childhood represent a complex issue. Their aim is to remove the pathological process in the middle ear and subsequently to restore the transfer function of the middle ear. Autologous and biocompatible materials can be used for reconstruction. Hearing gain is essential, especially in childhood, for the patient's further life. Good hearing in childhood is key for proper development of speech and learning. The quality of life, mental and language skills and children's development are thus positively influenced by the operation. Even in childhood, these operations can achieve good results, both anatomical and functional.

Decreased WNT3 expression in chronic lymphocytic leukaemia is a hallmark of disease progression and identifies patients with worse prognosis in the subgroup with mutated IGHV.

The canonical Wnt pathway, dependent on ß-catenin-controlled transcription, is the most explored Wnt pathway, known to drive the malignant transformation of multiple cell types. Several reports have suggested that this pathway also participates in chronic lymphocytic leukaemia (CLL) pathogenesis. To get a better insight into the role of the Wnt/ß-catenin pathway in CLL we analysed in detail the expression of the most overexpressed Wnt ligand, encoded by the WNT3 gene, in a well-defined cohort of 137 CLL patients. Our analysis demonstrated that (i) untreated patients with more aggressive disease (with a notable exception of patients with 11q deletion) express less WNT3, (ii) WNT3 declines with disease progression in a significant proportion of patients and (iii) low WNT3 was identified as a strong independent marker indicating shorter treatment-free survival in CLL patients with IGHV mutation. Interestingly, CLL-related lymphoid cell lines, but not stromal cells, failed to respond to the ligand-induced activation of the Wnt/ß-catenin pathway. This opens the possibility that CLL cells use Wnt-3 to communicate with the cells in the microenvironment. We thus propose that the Wnt/ß-catenin pathway plays a more complex role in CLL pathogenesis than previously anticipated.

Pott's puffy tumor: a rare complication of acute otitis media in child: a case report.

To describe a rare case of Potts' puffy tumor (PPT) in the zygomatic area, which developed as a complication of acute otitis media in a 6-year-old child. To date, only one case of PPT has been described in the literature as a complication of latent mastoiditis in an adult, and one case of PPT as a complication of acute mastoiditis in a 10-year-old child. Urgent surgical intervention, including evacuation of the purulent lesion, removal of inflamed soft tissue and osteolysis of the involved bone, and antromastoidectomy, intravenous treatment with broad-spectrum antibiotics, including G+, G-, anaerobes and fungi, and local therapy.

Exostosis of the optic canal in a child--a rare diagnosis in a paediatric ear, nose and throat setting: a case report.

INTRODUCTION: Optic nerve compression is an uncommon disorder leading to deterioration or complete loss of vision. CASE PRESENTATION: We describe the case of a 14-year-old Caucasian girl with a gradual deterioration of vision in her right eye. Using modern imaging techniques and endonasal endoscopic surgery, we identified the cause and removed the pathology. CONCLUSIONS: The cause of optic nerve compression was a rare exostosis in the optic canal. An endonasal endoscopic approach is the optimal choice for management of optic nerve pathologies. It is a gentle and minimally invasive surgical technique. Interdisciplinary cooperation, especially with the eye doctor and radiologist, is required in these cases.