Persistent Obstructive Sleep Apnea Post-adenotonsillectomy in Children.

Background Childhood obstructive sleep apnea (OSA) is a common disorder in children mostly due to adenotonsillar hypertrophy. Therefore, adenotonsillectomy is the mainstay of treatment. However, the outcome of adenotonsillectomy is limited in some patients who develop persistent OSA (POSA). We aim to evaluate the prevalence, risk factors, and treatments of POSA in the pediatric population in Qatar. Methodology This is a retrospective review of medical electronic records of patients aged 1-18 years, who underwent adenoidectomy and/or tonsillectomy at Sidra Medicine (Doha, Qatar) between June 2017 and September 2022. Demographic, clinical, and polysomnography (PSG) data were collected. POSA was defined as the persistence of at least one of the following OSA symptoms: snoring, gasping, mouth breathing or witnessed sleep apnea during post-surgery clinic visits, and/or post-surgical diagnosis of OSA by PSG. The prevalence of POSA was defined as the number of patients who had persistent symptoms divided by patients who were followed at outpatient clinics (ENT/pulmonology) post-surgery. Risk factors for POSA were evaluated using multivariate regression analysis. Results A total of 410 patients (259 males and 151 females) underwent adenotonsillectomy during the study period. The average age at surgery was 3.6 ± 2.5 years. The majority of patients (85.9%) had no history of underlying medical conditions. The rest of the patients (14.1%) were diagnosed with chromosomal abnormalities or neuromuscular disorders. All patients (100%) had a history of snoring before surgery, and 32.4% of patients had a history of witnessed sleep apnea. A total of 52 patients had persistent symptoms four months post-surgery. POSA prevalence was estimated at 15.4%. Univariate analysis showed young age at the time of surgery (p = 0.015), history of asthma (23%, 12/52) (p = 0.002), allergic rhinitis (13%, 7/52) (p = 0.001), gastroesophageal reflux disease ((11%, 6/52) (p < 0.001), and genetic syndromes (17%, 9/52) (p < 0.005) as significant risk factors for POSA. Multiple regression analysis showed that syndromic disorders and allergic rhinitis were significantly correlated with persistent OSA (p = 0.021 and p = 0.000, respectively). Conclusions POSA is prevalent in children post-tonsillectomy and adenoidectomy, especially in patients with genetic syndromes and those with symptoms of allergic rhinitis. Future studies are needed to better define the condition and provide evidence-based diagnostic and therapeutic approaches.

Endoscopic-Assisted Cochlear Implantation via a "Microfacial Recess".

Syndromic patients can have severely anomalous anatomy significantly challenging conventional cochlear implant (CI) surgery. This case report describes a 20-year-old with brachio-oto-renal syndrome with a severely anomalous facial nerve completely covering the round window and preventing a traditional posterior tympanotomy CI. This is the first report to illustrate the performance of an endoscopic trans-canal bony cochleostomy with insertion of the CI performed via a "microfacial recess." We describe the performance of an endoscopic trans-canal bony cochleostomy and a modification of the conventional wide posterior tympanotomy usually performed into a 1 mm "microfacial recess," which allowed a full insertion to take place. Laryngoscope, 2024.

Poland-Möbius syndrome: a case report implicating a novel mutation of the PLXND1 gene and literature review.

BACKGROUND: Möbius (Moebius) and Poland's syndromes are two rare congenital syndromes characterized by non-progressive bilateral (and often asymmetric) dysfunction of the 6th and 7th cranial nerves and hypoplasia of the pectoral muscles associated with chest wall and upper limb anomalies respectively. Manifest simultaneously as Poland-Möbius (Poland-Moebius) syndrome, debate continues as to whether this is a distinct nosological entity or represents phenotypic variation as part of a spectrum of disorders of rhomboencephalic development. Etiological hypotheses implicate both genetic and environmental factors. The PLXND1 gene codes for a protein expressed in the fetal central nervous system and vascular endothelium and is thus involved in embryonic neurogenesis and vasculogenesis. It is located at chromosome region 3q21-q22, a locus of interest for Möbius syndrome. CASE PRESENTATION: We present the first report of a patient with Poland-Möbius syndrome and a mutation in the PLXND1 gene. A child with Poland-Möbius syndrome and a maternally inherited missense variant (NM_015103.2:ex14:c.2890G > Ap.V964M) in the PLXND1 gene is described. In order to contextualize these findings, the literature was examined to identify other confirmed cases of Poland-Möbius syndrome for which genetic data were available. Fourteen additional cases of Poland-Möbius syndrome with genetic studies are described in the literature. None implicated the PLXND1 gene which has previously been implicated in isolated Möbius syndrome. CONCLUSIONS: This report provides further evidence in support of a role for PLXND1 mutations in Möbius syndrome and reasserts the nosological link between Möbius and Poland's syndromes. LEVEL OF EVIDENCE: Level V, Descriptive Study.

An Orbital Abscess Secondary to Intraoral Impalement.

Penetrating orbital trauma in the pediatric population is rare. Even more unusual is a secondary orbital infection following penetrating trauma. Here we present a highly unusual case of fulminant facial cellulitis with an orbital abscess in an otherwise healthy 3-year-old boy following a penetrating injury to the orbit from a point of entry on the gingiva-buccal sulcus, sustained during a fall while carrying a wooden lollipop stick. Examination of the retina revealed a focal injury at the inferior pole of the globe. The organisms cultured from pus sampled from the abscess and from the discharging intraoral wound revealed the same oral commensals while the MRI revealed a track in continuity with the orbital collection.

Ectopic Intratracheal Thyroid: A Rare Cause of Airway Obstruction.

Ectopic intratracheal thyroid tissue (EITT) is a rare abnormality with only limited cases reported so far. The presenting symptoms can be very similar to those of bronchial asthma. We discuss the case of a 29-year-old man with subglottic ectopic thyroid, with a history of thyroid surgery for goiter, which has been managed with laser-assisted endoscopic approach. We have also included presenting symptoms, pathophysiology, diagnosis, and management of EITT. We aim to include EITT in the differentials of airway obstruction, particularly in those patients who have goiter or previous thyroid surgeries.