Evaluation of Balance with Computerized Dynamic Posturography in Children with Otitis Media.

BACKGROUND: Otitis media with effusion (OME) frequently leads to vestibular symptoms in children. However, young children face difficulty expressing their symptoms due to their limited language abilities. METHODS: The balance of study and patient group evaluated with computer dynamic posturography, single-leg stance test with eyes closed and regular Head Impulse Test. The study group was assessed once again after the insertion of a ventilation tube two months later. RESULTS: In the Sensory Organization Test, the scores for conditions 5, 6, and composite equilibrium of the preoperative patient group were notably lower compared with both the control and postoperative patient groups (p < 0.05). Additionally, a significant correlation was found between single-leg stance test with eyes closed results and conditions 5, 6, and composite equilibrium scores. CONCLUSION: The impact of OME on the vestibular system is negative. This effect can be objectively assessed using Computer Dynamic Posturography and following tube insertion, there is a notable improvement in vestibular function. Furthermore, the single-leg stance (SLS) test with eyes closed has shown its reliability in assessing balance disorders, notably in children with OME. LEVEL OF EVIDENCE: Level 2 Laryngoscope, 2024.

Evaluation of the effects of three different tracheotomy techniques on tracheal complications and decannulation.

OBJECTIVE: Compared to adults, pediatric tracheotomy is associated with a higher incidence of complications. Various surgical techniques have been described to minimize complications. The aim of this study is to investigate the effects of three different tracheotomy techniques on tracheal complications and decannulation. METHODS: Pediatric patients who underwent tracheotomy at Hacettepe University Department of Otorhinolaryngology were retrospectively analyzed. 62 patients were included in the study. Tracheotomy related complications and direct laryngoscopy images of all patients were examined in the postoperative period. The patients were divided into 3 groups according to the tracheotomy technique; vertical incision traction suture (VITS) technique (n: 24), vertical incision maturation suture (VIMS) technique (n: 19), inferior based flap (Björk) technique (BT) (n: 19). RESULTS: The rate of severe suprastomal pathology was detected as 29.2% in the VITS group, 15.8% in the VIMS group and 26.3% in the BT group (p = 0.656). The accidental decannulation rate was detected as 20.8% in the VITS group, 26.3% in the VIMS group and 10.5% BT group (p = 0.442). The rate of life-threatening occurrence until recannulated was detected as 60% in the VITS group, 20% in the VIMS group and 0% in the BT group (p = 0.394). The rate of tracheocutaneous fistula was detected as 13.3% in the VITS group, 40% in the VIMS group and 33.3% in the BT group (p= 0.337). CONCLUSIONS: There were no differences in complication rates based on techniques, and that stomal complications were common in small children requiring tracheotomies.

Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance.

INTRODUCTION: Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA. METHODS: Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. RESULTS: A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). CONCLUSION: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.

Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance.

INTRODUCTION: Mucopolysaccharidosis (MPS) VI or Maroteaux-Lamy syndrome (253200) is an autosomal recessive lysosomal storage disorder caused by deficiency in N-acetylgalactosamine-4-sulfatase (arylsulfatase B). The heterogeneity and progressive nature of MPS VI necessitates a multidisciplinary team approach and there is a need for robust guidance to achieve optimal management. This programme was convened to develop evidence-based, expert-agreed recommendations for the general principles of management, routine monitoring requirements and the use of medical and surgical interventions in patients with MPS VI. METHODS: 26 international healthcare professionals from various disciplines, all with expertise in managing MPS VI, and three patient advocates formed the Steering Committee group (SC) and contributed to the development of this guidance. Members from six Patient Advocacy Groups (PAGs) acted as advisors and attended interviews to ensure representation of the patient perspective. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with expertise and experience managing patients with MPS VI and the manuscript has been evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. RESULTS: A total of 93 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions. Consensus was reached on all statements after two rounds of voting. The greatest challenges faced by patients as relayed by consultation with PAGs were deficits in endurance, dexterity, hearing, vision and respiratory function. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). CONCLUSION: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS VI and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.

Parotidectomy outcomes, diagnosis and complications on pediatric patients; Twelve years of experiences in a tertiary care center.

The aim of this study is to evaluate parotid masses management in pediatric population. From June 2002 to December 2014, 13 patients under the age of 18 with parotid tumors were treated via surgery at Hacettepe University Department of Otorhinolaryngology. Information on patients' demographics, clinical and histopathologic tumor characteristics and treatment modalities with results was obtained from medical records, retrospectively. The age was ranging between 8-17 years. Ten (76.9%) patients had benign tumors and 8 (61.5%) of them were pleomorphic adenoma. One case had chronic sialadenitis with sialolithiasis and one patient had fluoride follicular hyperplasia. Three patients had malignant tumors; two of them were adenoid cystic carcinoma and one case of malignant melanoma metastasis. Twelve cases had undergone partial parotidectomy. Only one total parotidectomy was performed which was the patient with malignant melanoma. None of the patients had major complications or facial nerve palsy. During the 3 years follow up period, recurrence or Frey syndrome had not been detected but the case with malignant melanoma had distant metastasis during the follow up time. Although pediatric parotid masses are unusual, they can represent a variety of pathological diagnoses, including malignancy. Parotidectomy remains the mainstay of treatment and surgical experience is needed for low complication rates especially in children.

Parapharyngeal giant ganglioneuroma with multifocal bone involvement in a pediatric female patient.

Ganglioneuroma is a rare benign tumor that originates from neural crest. Tumor tends to be slow growing, asymptomatic but can cause symptoms because of pressure to neighboring structures. In the head and neck region they are relatively rarely seen. We hereby present a rare case of multiple ganglioneuromas that were located in parapharyngeal space, iliac bone and other bones in a 13-year-old girl. Patient underwent surgery for the excision of a large mass, extending from parapharyngeal space to neck, with transparotid and transcervical combined approach. After operation, MIBG (iodine-123-meta-iodobenzylguanidine) scintigraphy was performed and involvement of parietooccipital bone, lumbal vertebra, right iliac wing medial cortex and left humerus were detected. No adjuvant therapy was given to the patient. There is no evidence of recurrence in the head and neck region in the following 12 months. In conclusion, complete surgical excision of the tumor, if possible, is the treatment of choice with high success rate. Close clinical and radiological follow-up for these tumors after surgery should be made.

The effect of early routine grommet insertion on management of otitis media with effusion in children with cleft palate.

PURPOSE: The aim of the study is to compare long term otoscopic and audiological findings of cleft palate patients with or without early grommet insertion. METHODS: Cleft palate patients followed-up in Hacettepe University between 2008 and 2013 were included in the study. Age, gender, cleft types and palate surgery data, grommet tube insertion history and otological - audiological evaluations of the patients were recorded. Patients were evaluated in three groups according to grommet insertion history: A-early routine grommet insertion, B-grommet insertion during follow-up, C-no grommet insertion. Otological and audiological findings were compared. RESULTS: There were 154 patients in the study, with a median age of 7.7 years. There were 67 patients in group A (43.5%), 22 patients in group B (14.3%) and 65 patients in group C (42.2%). OME was identified significantly higher in group A and normal otoscopic examination findings were higher in group C. Complications showed a higher rate than other otoscopic findings in group B patients. There was no significant difference for any frequencies in between the groups in terms of mean air-bone gap (ABG) values. There were 20 grade I, 25 grade II, 77 grade III and 32 grade IV patients in the study according to the Veau classification. CONCLUSION: Prophylactic grommet insertion may not be applied as some cleft palate patients with no OME. Wait and see protocol can be recommended for these patients, and they should be followed-up up closely to avoid complications. If the effusion does not recover or tympanic membrane changes occur in follow-up, grommet insertion should be considered.