3D printing as surgical planning and training in pediatric endoscopic skull base surgery - Systematic review and practical example.

BACKGROUND: Pediatric endoscopic skull base surgery is challenging due to the intricate anatomy of the skull base and the presence of tumors with varied pathologies. The use of three-dimensional (3D) printing technologies in skull base surgeries has been found to be highly beneficial. A systematic review of the literature was performed to investigate the published studies that reported the effectiveness of 3D printing in pediatric endoscopic skull base surgery. METHODS: Pub Med, Embase, Science Direct, The Cochrane Library, and Scopus were searched from January 01, 2000, until June 30, 2022. Original articles of any design reporting on the effectiveness of 3D printing in pediatric endoscopic skull base surgery were included. Information related to study population, conditions, models used, and key findings of study were extracted. Quality of included studies was evaluated using the Joanna Briggs Institute's (JBI) Critical Appraisal Checklist for Studies. To exemplify the use of 3D technology in this scenario, we report a complex clival chordoma case. RESULTS: Six research articles were retrieved and included for qualitative analysis. Four of the six studies were conducted in the United States, followed by two in China. According to these studies, 3D reconstruction and printed models were more beneficial than CT/MRI images when discussing surgery with patients. In clinical training, these models were more helpful than 2D images in understanding the pathology when used in conjunction with image-guiding systems. It has been found that patient-specific 3D modeling, simulations, and rehearsal are the most efficient preoperative planning techniques, particularly in the pediatric population, for the treatment of complicated skull base surgeries. All the studies had a moderate risk of bias. CONCLUSION: 3D printing technologies assist in printing complex skull base tumors and the structures around them in three dimensions at the point of care and at the time needed, enabling the choice of the appropriate surgical strategy, thus minimizing surgery-related complications.

Vestibulo-ocular reflex assessed with Video Head Impulse Test in children with Vestibular Migraine: Our experience.

Vestibular Migraine in children can mimic other disorders, especially at presentation. Outcome is hard to predict and management may be challenging due to the fact that many of the patients are too young to describe their symptoms and these are not always accompanied by headache. OBJECTIVE: To assess vestibulo-ocular reflex (VOR) in pediatric patients who meet criteria for defined Vestibular Migraine and to compare results to healthy controls. METHODS: Twenty-one patients aged 11-16 years were included in this prospective multicentric study. VOR was assessed using the video Head Impulse Test by EyeSeeCam®(Interacoustics, Denmark). RESULTS: Patients with Vestibular Migraine (VM) have higher values of gain compared to asymptomatic patients. CONCLUSION: Video Head Impulse Test (vHIT) is a useful and relatively fast-to-perform examination in children compared to other vestibular tests. Patients with VM seem to have higher values of gain at vHIT.

Juvenile recurrent respiratory papillomatosis treated with combined erlotinib and celecoxib: Initial report.

Recurrent respiratory papillomatosis (RRP) is a chronic disease caused by human papillomavirus (HPV). RRP is a clinical challenge because of the high recurrence rate, poor surgery response, extension to tracheobronchial tree and because of the risk of malignancy in some cases. There is no consensus on which adjuvant therapy is better for those patients with highly recurrent course. Because papilloma cells overexpress the epidermal growth factor receptor (EGFR), together with an increased expression of COX-2 and prostaglandin E2, the combination of erlotinib and celecoxib seems plausible, and could be proposed for patients with poor response to previous lines of treatment.

Influence of nasal septum deformity on nasal obstruction, disease severity, and medical treatment response among children and adolescents with persistent allergic rhinitis.

OBJECTIVE: To evaluate the impact of different types of nasal septum deformity (NSD) on nasal obstruction, rhinitis severity and response to medical treatment among pediatric persistent allergic rhinitis (PER) patients. METHODS: In a prospective, real-life study, 150 children and adolescents (mean age 13 ± 2.8 years, females 32.6%) diagnosed with PER according to ARIA guidelines were assessed by nasal endoscopy for NSD according to Mladina's classification, their response to medical treatment (intranasal steroids and antihistamines or antileucotriens), the presence of comorbidities, rhinitis severity (modified-ARIA criterion) and nasal obstruction visual analog scale score (VAS). RESULTS: Most patients (87%) had 1 of the 7 types of septal deformities. There was a high prevalence of bilateral (types 4 and 6; 46%) and anterior unilateral (types 1 and 2; 25%) NSD in patients not responding to medical treatment. Type 4 (OR = 6.4; p = 0.005) or type 6 (OR = 4.4; p = 0.03) NSD increased the risk of lack of improvement with medical treatment. Coexistence of anterior unilateral or bilateral NSD with severe turbinate enlargement increased >20-fold the risk of lack of improvement. Patients with bilateral NSD presented greater rhinitis severity. Non-responder adolescents displayed higher prevalence of bilateral NSD than children (53% vs. 23%; p = 0.02). Nasal obstruction VAS was higher for patients with anterior than posterior NSD, and greater for patients with bilateral NSD than any other type of septal morphology. CONCLUSION: Nasal endoscopy shows that bilateral and unilateral anterior nasal septum deformities are strongly associated with a poor response to medical treatment, greater rhinitis severity and higher nasal obstruction VAS. Consequently, nasal endoscopy is necessary in the PER patients to understand the disease severity as well as to plan a specific surgical treatment in order to improve nasal obstruction, disease severity, and patient's quality of life.

Nasal obstructive disorders impair health-related quality of life in adolescents with persistent allergic rhinitis: A real-life study.

BACKGROUND: We previously reported a higher prevalence of nasal obstructive disorders (NOD) in pediatric patients with persistent allergic rhinitis (PER) not responding to medical treatment. The aim of this study was to determine the impact of NOD on quality of life (QoL) in this population. METHODS: Real-life prospective study including 142 patients (41 children, 6-11 years old and 101 adolescents, 12-17 years old) with moderate and severe PER. After 2 months of medical treatment (intranasal steroids and antihistamines), patients were asked whether their symptoms had improved (yes/no) and classified accordingly in R, responders and NR, non-responders. Nasal symptoms (visual analog scale, VAS), NOD (nasal endoscopy), and QoL (PRQLQ, AdolQRLQ) were also assessed. RESULTS: Sixty-nine adolescents and 24 children were included in the NR group. NR presented worse QoL overall scores in adolescents (3.16±1.1 vs 1.63±0.99; P=.00001) and children (2.19±0.82 vs 1.51±0.77, P=.02). Medical treatment failure was associated with worse outcomes in QoL (adolescents OR: 1.6, P<.0001; children OR: 1.04, P=.036). Female adolescents presented worse QoL scores than males (3.19 vs 2.36, P=.001). The presence of obstructive septal deviation (OR: 1.02, P=.005), obstructive turbinate hyperplasia (OR: 1.03, P=.0006), and coexistence of both (OR=2.06, P=.001) was associated with worse QoL in adolescents. A strong and highly significant correlation was found between nasal symptoms VAS and QoL. CONCLUSION: The presence of NOD, particularly in adolescents, is associated with poor QoL outcomes. Assessment of NOD in pediatric PER should be considered an essential approach to determine the response to treatment and its impact on patient's QoL.

Nasal obstructive disorders induce medical treatment failure in paediatric persistent allergic rhinitis (The NODPAR Study).

BACKGROUND: Allergic rhinitis (AR) is the most common chronic disease among children. To characterize the disease, a modified classification of severity (m-ARIA) has recently been validated in AR children. When medical treatment fails, surgery for nasal obstructive disorders (NOD) may be a therapeutic option. Our objective was to assess the prevalence of NOD and their influence in medical treatment response among children with persistent AR (PER). METHODS: In a prospective, real-life study, 130 paediatric PER patients (13.1 ± 2.8 years, females 31.5%, severe rhinitis 49%) referred from Allergy to ENT department were assessed for their response (R, responders; NR, non-responders) to medical treatment (intranasal steroids and antihistamines or antileukotrienes) by direct questioning and nasal symptom visual analogue scale, the presence of NOD (septal deformity, turbinate enlargement and adenoidal hyperplasia), comorbidities, nasal symptoms, rhinitis severity (modified ARIA criterion) and asthma control (International Consensus On Pediatric Asthma criterion). RESULTS: After 2 months of treatment, the NR group presented a higher prevalence of obstructive septal deformity and severe inferior turbinate enlargement when compared with the R group. Higher septal deformity and turbinate enlargement scores were strongly associated with treatment refractoriness. The prevalence of severe PER was also higher for the NR group. Higher asthma control scores were associated with the probability of treatment-induced improvement. CONCLUSIONS: In paediatric PER patients, medical therapy refractoriness was associated with NOD, mainly septal deformity and turbinate enlargement. In those patients, ENT examination will facilitate an early NOD diagnosis in order to indicate potential corrective surgery.

Pediatric intranasal lobular capillary hemangioma: Report of two new cases and review of the literature.

BACKGROUND: Lobular capillary hemangioma (LCH) is an acquired benign vascular tumor of unknown origin. It usually affects skin and mucous membranes of the oropharynx. It rarely involves the nasal cavity which most commonly manifests as epistaxis. To our knowledge, only fifteen pediatric intranasal LCH cases have been reported in the literature. None of these occurred in the inferior turbinate. We report two new pediatric cases of LCH, one of them on the inferior turbinate and the other one on the anterior nasal septum. Our principal aim was to highlight the importance of considering this lesion as a differential diagnosis for pediatric unilateral nasal obstruction and epistaxis. METHODS: Retrospective case series and review of current literature regarding the possible causes, diagnosis, and treatment of nasal LCH. DESCRIPTION OF CASES: Two adolescents presented with symptoms of unilateral nasal obstruction and epistaxis. Plain and contrast enhanced computed tomography revealed a well-defined intensely enhancing lesion in both cases. Patients underwent transnasal endoscopic excision and bipolar electrocautery at the base of the tumor for hemostasis. Histopathological examination confirmed the diagnosis of LCH. DISCUSSION: Current epidemiological and pathophysiological data suggests that the development of LCH may be associated to previous nasal trauma or endocrine disorders. LCH should be considered in the differential diagnosis of all pediatric endonasal masses associated with unilateral epistaxis and nasal obstruction. Endoscopic total excision with bipolar electrocautery for hemostasis is an appropriate treatment.

Comorbilidades de los trastornos respiratorios del sueño en los niños / Comorbidities of sleep-disordered breathing in children

Los trastornos respiratorios del sueño en los niños, y especialmente el síndrome de apnea-hipopnea infantil, se asocian a un ramillete de comorbilidades de entre las que destacan las cognitivoconductuales, las metabólicas y de crecimiento, así como las cardiovasculares. Los 2 factores más importantes que contribuyen a su fisiopatología son la hipoxia intermitente y la fragmentación del sueño, que parecen ser las responsables de la respuesta inflamatoria sistémica que acabará produciendo el daño multistémico mencionado(AU)

Sleep-related respiratory disorders in children, especially childhood sleep apnea-hypopnea syndrome, are associated with a wide range of comorbidities affecting the central nervous system, cardiovascular and metabolic systems, and growth. The two most important factors contributing to the physiopathology of this disorder are intermittent hypoxia and sleep fragmentation, which seem to cause the systemic inflammatory response resulting in these end-organ consequences(AU)

[Comorbidities of sleep-disordered breathing in children]. / Comorbilidades de los trastornos respiratorios del sueño en los niños.

Sleep-related respiratory disorders in children, especially childhood sleep apnea-hypopnea syndrome, are associated with a wide range of comorbidities affecting the central nervous system, cardiovascular and metabolic systems, and growth. The two most important factors contributing to the physiopathology of this disorder are intermittent hypoxia and sleep fragmentation, which seem to cause the systemic inflammatory response resulting in these end-organ consequences.