Granulicatella adiacens Causing a Parapharyngeal Abscess in a 10-Month-Old Infant: A Rare-Case Report and Literature Review of Deep Neck Infections (DNIs) in Children.

Granulicatella adiacens is a rare variant of the Streptococcus bacteria. When isolated, G. adiacens has been described in cases of endocarditis and bacteremia, but less commonly seen in isolated pyogenic infections. We report a case of a parapharyngeal abscess caused by G. adiacens in an otherwise healthy 10-month-old infant, which was successfully treated with antibiotics and surgical drainage. To the best of our knowledge, this is the first described case of a pediatric deep soft tissue neck infection caused by G. adiacens with one other report in an adult. Additionally, of all localized infections from this bacteria, this is only the second reported case in the pediatric population. We also include an evidence-based literature review of the clinical presentation, microbiology, imaging modalities, and management approach to deep neck infections (DNIs).

The genomic landscape of rare disorders in the Middle East.

BACKGROUND: Rare diseases collectively impose a significant burden on healthcare systems, especially in underserved regions, like the Middle East, which lack access to genomic diagnostic services and the associated personalized management plans. METHODS: We established a clinical genomics and genetic counseling facility, within a multidisciplinary tertiary pediatric center, in the United Arab Emirates to locally diagnose and manage patients with rare diseases. Clinical genomic investigations included exome-based sequencing, chromosomal microarrays, and/or targeted testing. We assessed the diagnostic yield and implications for clinical management among this population. Variables were compared using the Fisher exact test. Tests were 2-tailed, and P < .05 was considered statistically significant. RESULTS: We present data on 1000 patients with rare diseases (46.2% females; average age, 4.6 years) representing 47 countries primarily from the Arabian Peninsula, the Levant, Africa, and Asia. The cumulative diagnostic yield was 32.5% (95% CI, 29.7-35.5%) and was higher for genomic sequencing-based testing than chromosomal microarrays (37.9% versus 17.2%, P = 0.0001) across all indications, consistent with the higher burden of single gene disorders. Of the 221 Mendelian disorders identified in this cohort, the majority (N = 184) were encountered only once, and those with recessive inheritance accounted for ~ 62% of sequencing diagnoses. Of patients with positive genetic findings (N = 325), 67.7% were less than 5 years of age, and 60% were offered modified management and/or intervention plans. Interestingly, 24% of patients with positive genetic findings received delayed diagnoses (average age, 12.4 years; range 7-37 years), most likely due to a lack of access to genomic investigations in this region. One such genetic finding ended a 15-year-long diagnostic odyssey, leading to a life-threatening diagnosis in one patient, who was then successfully treated using an experimental allogenic bone marrow transplant. Finally, we present cases with candidate genes within regions of homozygosity, likely underlying novel recessive disorders. CONCLUSIONS: Early access to genomic diagnostics for patients with suspected rare disorders in the Middle East is likely to improve clinical outcomes while driving gene discovery in this genetically underrepresented population.

Irradiated homologous costal cartilage grafts for single-stage open airway reconstruction in severe subglottic stenosis for children under the age of one.

We present two cases of premature infants with Cotton-Myer grade 3 subglottic stenosis where endoscopic balloon dilation failed. In an attempt to avoid tracheostomy, both patients underwent open single stage anterior graft larnygotracheoplasty (LTP) with irradiated homologous rib and temporalis fascia grafts. After postoperative reconstruction, the airway was resized to grade 1 in one child, and to grade 2 in the other. One child remained free of stridor post-operatively and avoided the need of a tracheostomy tube. The other child ultimately required a tracheostomy, though she is now in the process of being decannulated. Homologous costal cartilage grafts in open anterior airway LTP may be a reasonable alternative to tracheostomy in young patients with advanced SGS.

Blunt laryngeal trauma presenting as bilateral massive pneumothoraces and subcutaneous emphysema: a multidisciplinary approach to management.

A 3-year-old boy had an unwitnessed fall from a highchair. The child had no loss of consciousness, vomiting, stridor or respiratory distress but within a few minutes had significant swelling in the neck, scalp and around the eyes. He was brought immediately to the emergency room where he deteriorated rapidly and was intubated with a cuffed oral endotracheal tube. A clinical diagnosis of blunt laryngeal trauma was made. Imaging showed no laryngeal disruption, but did reveal massive bilateral pneumothoraces, that were managed with chest tube. A multidisciplinary meeting with family led to a watchful waiting approach. The patient was successfully extubated at 1 week and healed with a clear voice.

Temporary Frontal Paralysis Secondary to Blunt Trauma Frontal Sinus Fracture.

Frontal sinus fractures (FSF) are relatively uncommon and can be challenging for trauma surgeons to manage. Patients with FSF typically present with facial swelling, pain, and nasofrontal ecchymosis. Here we present a rare case of a patient with FSF and anterior table fracture where the main presenting symptom was bilateral frontal paralysis. We outline our management strategy and review the current literature in regard to management of FSF.

Choosing a computer-assisted surgical system for sinus surgery.

OBJECTIVES: Choosing the correct computer-assisted system for sinus surgery can be a formidable task for surgeons and their institutions. A formal trial of an electromagnetic (InstaTrak, Visualization Technologies Incorporated, Woburn, MA) and an optical (LandmarX by Medtronic-Xomed, Jacksonville, FL) system was conducted over a 10-month period at the St. Paul's Sinus Centre in Vancouver, BC. An objective and subjective evaluation method was used to select the system for use at our institution. METHODS: Thirty-nine patients were operated on by the senior author (A.R.J.) using the InstaTrak (23 patients) or the LandmarX (16 patients). The two groups were balanced in terms of age, gender, number of previous surgeries, and extent of surgery. Estimated blood loss, surgical time, and surgical complications were compared between the groups, with all other variables constant. Nursing and radiology personnel as well as the three surgeons who used both systems at the centre were surveyed on patient safety, ease of use, storage, and accessories. RESULTS: There was no statistical difference in objective surgical data. There was a more pronounced learning curve using the LandmarX as defined by a greater decrease in the duration of surgical time by the end of the trial period. The operating room personnel found the InstaTrak easier to use, whereas the computed tomography technologists preferred the LandmarX. The surgeons found the two systems acceptable in terms of navigational accuracy; however, the InstaTrak was felt to be more user friendly. CONCLUSIONS: The InstaTrak system was chosen for our institution mainly because of its ease of use by the operating room staff and the sinus surgeons.