Use of an Orthodontic and Otolaryngological Approach in an Infant with Holoprosencephaly.

Holoprosencephaly is a complex human brain malformation resulting from incomplete cleavage of the prosencephalon into both hemispheres. Congenital nasal pyriform aperture stenosis (CNPAS) is sometimes found in patients with mild forms of holoprosencephaly. Surgical treatment is required. Low-invasive surgical approaches involve balloon dilation of the pyriform opening. We present the case of an 8-day-old girl diagnosed with holoprosencephaly, CNPAS, and the presence of a solitary median maxillary central incisor. Once examined by neonatologist, geneticist, pneumologist, otolaryngologist, and pediatric dentist, a combined otolaryngological-orthodontic approach was used. The obstruction of the right nasal cavity was treated by widening the nasal cavities and stabilizing them with a balloon dilation technique. After surgery, the respiratory space was increased by applying a neonatal palatal expander plate (NPEP) considering the palatal deformity: ogival shaped, anterior vertex growth direction, reduction of transverse diameters. The NPEP promoted distraction of the median palatine suture and assisted the nasal dilation. Therefore, after the insertion of NPEP, the physiological sucking-swallowing mechanism was activated. In infants with CNPAS, NPEP can be useful to ensure the safe stability of nasal dilation. A multidisciplinary approach is fundamental. In our experience, the close collaboration between an otolaryngologist and orthodontist is essential for the management of the patient with CNPAS.

Oral splint therapy in patients with Menière's disease and temporomandibular disorder: a long-term, controlled study.

PURPOSE: To assess the effect of oral splint therapy on audio-vestibular symptoms in patients with Menière's disease (MD) and temporomandibular disorder (TMD). METHODS: Retrospective case-control study. Treatment group: 37 patients with MD and TMD who received gnatological treatment. CONTROL GROUP: 26 patients with MD and TMD who had never received gnatological treatment. The number of vertigo spells in 6 months (primary endpoint), pure-tone audiometry average (PTA), MD stage, functional level, Dizziness handicap Index (DHI), Tinnitus handicap Index (THI) and Aural Fullness Scale (AFS) were compared at baseline and after 24 months according to groups. Analysis of Covariance was used to determine the treatment effect. RESULTS: Groups were comparable for demographic, clinical data, baseline PTAs and the number of vertigo spells. Analysis of covariance showed a significant effect of gnathological treatment on number of vertigo spells ([Formula: see text] = 0.258, p < 0.001), PTA ([Formula: see text] = 0.201, p < 0.001), MD stage ([Formula: see text] = 0.224, p < 0.001), functional level ([Formula: see text] = 0.424, p < 0.001), DHI ([Formula: see text] = 0.421, p < 0.001), THI ([Formula: see text] = 0.183, p < 0.001), but not for AFS ([Formula: see text] = 0.005, p = 0.582). The treatment group showed vertigo control of class A in 86.5% and class B in 13.5% of patients. In the control group, vertigo control was of class A in 19.2% of patients and class B in 11.5%, class C in 30.8%, class D in 11.5%, class E in 19.2% and class F in 7.7%. Classes of vertigo control differed significantly (X2 test, p < 0.001). CONCLUSIONS: Oral splint therapy could represent a viable treatment in patients with TMD and uncontrolled MD disease. The effects are maintained at least after 2 years.

Temporomandibular Joint and Otitis Media: A Narrative Review of Implications in Etiopathogenesis and Treatment.

Otitis media (OM) and its recurring (rAOM), effusive (OME), and chronic forms, represent a frequent clinical challenge. The middle ear, the mandible, and the temporomandibular joint (TMJ) share several embryological and anatomical connections. Despite that, the role of mandibular malposition and TMJ dysfunction is frequently overlooked in the management of otitis media. In this narrative review, we present current evidence supporting the etiopathogenetic role of a dysfunctional stomatognathic system in the onset of OM and the effectiveness of orthognathic treatment in preventing rAOM and OME. In particular, a focus on the influence of TMJ on Eustachian tube function is provided.

Association between upper airway obstruction and malocclusion in mouth-breathing children.

OBJECTIVES: To evaluate the association between upper airway obstruction and occlusal anomalies in mouth-breathing children. METHODS: 356 mouth-breathing children were evaluated by ENT physicians and specialists in orthodontics. ENT examination included nasal endoscopy to assess the adenoidal hypertrophy, tonsillar grading and presence of nasal septum deviation. Clinical orthodontic examination was performed to record occlusal variables. Univariate and multivariable logistic regression were performed to study the association between registered variables. RESULTS: 221 patients (mean age ± sd = 6.2 ± 2.5 years) met inclusion criteria. 81.4% of children presented malocclusion. A significant association between tonsillar grade 2 and the presence of malocclusion, Class II relation and increased overjet was shown. Tonsillar grade 4 showed a significant association with the presence of malocclusion and increased overjet. Adenoidal hypertrophy and nasal septum deviation did not show any association with occlusal findings. CONCLUSIONS: A high frequency of orthodontic problems was seen in mouth-breathing children. Our results suggested that severe tonsillar hypertrophy may determine presence of malocclusion and increased overjet. On the other hand, the association between mild tonsillar hypertrophy and many occlusal anomalies in mouth-breathers suggest an important role of malocclusion in the onset of oral breathing in children.

Needle aspiration of lateral neck abscess in children: A simple and effective treatment.

BACKGROUND: Cervical abscesses frequently occurred in pediatric patients. Surgical drainage is currently recommended in the cases not responding to medical therapy. Needle aspiration may represent a simple, effective and minimally invasive treatment for acute neck suppuration in selected cases. OBJECTIVE: To evaluate the effectiveness of needle aspiration in the treatment of cervical abscesses in pediatric age as an alternative to surgical drainage. METHODS: From November 2013 to November 2019, at the ENT Department of the "Bambino Gesù" Pediatric Hospital, Rome, we treated 47 children with cervical abscess by performing aspiration with a 19 G needle in wakefulness after applying local anesthetic. Parenteral antibiotic treatment was associated with all patients both before and after treatment. RESULTS: Complete regression of the abscess occurred in 46 children with no evidence of recurrence in the follow-up period. Surgical drainage was required in only one patient due to the lack of resolution of the abscess episode. No complications such as hematomas or hypertrophic scars were found in any patient. CONCLUSIONS: Our results confirm the effectiveness of needle aspiration in the treatment of cervical abscesses in children as an alternative to surgical drainage.

Juvenile recurrent parotitis: Diagnostic and therapeutic effectiveness of sialography. Retrospective study on 110 children.

INTRODUCTION: Juvenile recurrent parotitis (JRP) is characterized by recurrent episodes of painful parotid swelling, generally associated with non-obstructive sialectasia of the parotid gland. The aim of this study was to evaluate the diagnostic and therapeutic effectiveness of sialography in children affected by JRP. METHODS: Clinical records of 110 outpatients with a diagnosis of JRP followed up from 2008 to 2017 at the Unit of Paediatric Otorhinolaryngology, Surgery Department of the Bambino Gesù Children's Hospital of Rome, were retrospectively reviewed. Data on demographics, number of acute episodes/year, course of disease, site of symptoms and duration of follow up were collected. The inclusion criteria were: at least two or more episodes of intermittent swelling of the parotid glands on one side or both sides during the last 6 months, age <16 years. Exclusion criteria were: obstructive lesions, dental malocclusion, Sjogren syndrome, congenital IgA immunodeficiency, and relevant systemic diseases. Outcome of the procedure was measured by evaluating number and degree of episodes of parotid swelling before and after sialography. RESULTS: Sialography has been shown in all cases to be a valid method in the diagnosis of JRP. Following the execution of the sialography, in 98 patients (89% of cases) there was a statistically significant improvement of disease with a mean reduction of 67.4% of episodes of parotid swelling (p < 0.05). In 75 patients there was marked improvement of the symptomatology (p < 0.05). In 23 patients partial resolution occurred (p < 0.05); in 12 patients there was no resolution or a reduction less than 30% of episodes (p > 0.05). There was a statistically significant relationship between the number of attacks/year and the degree of glandular function, pre and post-sialography in 2-way ANOVA test (p < 0.05). CONCLUSION: Sialography is effective method not only as a diagnostic procedure but also as a therapeutic procedure in treatment of JRP. It is a method that can be carried out in ambulatory setting, without anaesthesia, with a minimum cost and with a very low rate of complications. In a disease with tendency to spontaneous resolution like JRP, sialography represent a therapeutic option alternative to more invasive treatment.

Pilot study to explore the prophylactic efficacy of oral probiotic Streptococcus salivarius K12 in preventing recurrent pharyngo-tonsillar episodes in pediatric patients.

Background: In the pediatric population, acute pharyngo-tonsillitis represents one of the most frequent causes of access to outpatient treatment and use of antibiotics. In frequent tonsillitis, the pharmacological approach is no longer effective, and, therefore, surgery becomes the treatment of choice. Methods: This study compares the prophylactic efficacy of Streptococcus salivarius K12 (Bactoblis®) in children with recurrent pharyngo-tonsillitis treated vs untreated, with a 12 -month follow-up. The primary objectives are: The incidence of recurrence of pharyngo-tonsillar episodes and the concomitant use of other drugs. Secondary objectives are: tolerability of the treatment, the effectiveness in terms of clinical improvement, days of absence from school, reduction of the use of standard therapies, and cancellation from the surgical planning list. Results: Patients belonging to group A (treated with K12 for 90 days) were 24 males and 26 females, mean age 6.6 years (SD=1.57), those belonging to group B (untreated) were 23 males and 27 females, average age 6.8 years (SD=1.72). In the follow-up, group A reported 26 inflammatory pharyngo-tonsillary episodes in the first trimester, unlike group B, who reported 72 in the second trimester. This has shown a lower incidence (3.38%) of the disease compared to group B (6.66%), for a total of 169 inflammatory pharyngo-tonsillary episodes in group A against 333 in group B. A reduction in days of school absence of 429 days in group A and 927 days in the control group (P<0.01) was also noted. Finally, 14 children of group A (28%) underwent adenotonsillectomy, against the whole group B. No adverse events were reported. Conclusion: The efficacy of K12 on the prevention of pharyngo-tonsillar infections, the decrease in the use of antibiotics and the improvement of the overall quality-of-life was confirmed, with a decreased number of absences from school and fewer patients undergoing surgery.

Audiological findings in a de novo mutation of ANKRD11 gene in KBG syndrome: Report of a case and review of the literature.

KBG syndrome is a rare genetic disorder, due to a mutation of ANKRD11, characterized by specific craniofacial dysmorphism, short stature and macrodontia of upper central incisors, intellectual disability and skeletal anomalies. We report a de novo mutation of ANKRD11 gene in a 7-years old girl, affected by KBG syndrome with bilateral conductive hearing loss. The aim of this article was to review the audiological findings of this syndrome.

Relapsing polychondritis: new therapeutic strategies with biological agents.

Relapsing polychondritis (RP) is a rare disease of unknown etiology characterized by recurrent episodes of inflammation resulting in the destruction of cartilaginous tissues. We describe a young girl with RP unresponsive to conventional therapy.

Otolaryngologic manifestations of pediatric immunodeficiency.

Many adults and children with an underlying immunodeficiency can frequently present to ear, nose and throat (ENT) surgeons. This work deals with the presentation, investigation and management of immuno-compromised children in ENT practise. Both primary immunodeficiencies (PID) and secondary or acquired immunodeficiencies such as human immunodeficiency virus (HIV) infection are here discussed. The aim of this work is to give a complete and exhaustive description of ENT manifestations in immunodeficiency, and to outline basic principles to guide clinical practice.

Head and neck vascular anomalies in children.

Vascular lesions are the most common congenital and neonatal abnormalities. The aim of this work is to point out differences between various vascular anomalies, in order to define accurate diagnosis, and to present different therapeutic options now used for the treatment of the vascular lesions in children. According to biological classification described by the work of Mulliken and Glowacki there are two major types of vascular abnormality: haemangioma and vascular malformation. Haemangioma is a distinct biologic tumour entity characterised by rapid endothelial proliferation shortly after birth. The lesion is absent at birth and growth in early infancy, followed by a spontaneous resolution in childhood. Vascular malformations are structural anomalies that have a normal growth rate and endothelial turnover. According to the morphology of the vessels and the flow rate we distinguish: slow-flow and fast-flow vascular malformation. The authors document their personal experience in diagnosis, clinical evaluation, treatment and follow-up of the vascular lesions.