Noise in Endoscopic Sinus and Skull Base Surgery Operating Rooms.

BACKGROUND: Noise in the operating room (OR) contributes to miscommunication among team members and may negatively impact patient outcomes. OBJECTIVES: This study aimed to quantify noise levels during endoscopic sinus and skull base surgery. The secondary aim was to understand how OR team members perceive noise during endoscopic sinus and skull base surgery. METHODS: Noise levels were measured using the validated phone application SoundMeter X 10.0.4 (r1865) (Faber Acoustical, Utah, USA) at the ear-level of the surgeon, scrub nurse, circulating nurse, and anesthesiologist. At the end of each surgery, OR team members were asked to complete a six-question questionnaire about noise during that surgery. RESULTS: One thousand four hundred and two noise measurements were recorded across 353 trials. The loudest mean noise measurement was 84.51 dB and maximum noise measurement was 96.21 dB at the ear-level of the surgeon. Noise was significantly higher at the ear-level of the surgeon and scrub nurse in comparison to the circulating nurse (p = .000) and anesthesiologist (p = .000). Forty percent of questionnaire respondents believed noise was a problem and 38% stated that noise caused communication issues during surgery. CONCLUSION: Surgeons and scrub nurses have significantly higher noise exposure in comparison to circulating nurses and anesthesiologists during endoscopic sinus and skull base surgery. For these members of the OR team, noise is also identified as problematic and causing issues with communication. Mechanisms to reduce potential noise may be implemented to improve communication and patient outcomes in endoscopic sinus and skull base surgery.

Genotype-phenotype correlation in children with hereditary spherocytosis.

Hereditary spherocytosis (HS) is a common inherited haemolytic anaemia attributed to disturbances in five different red cell membrane proteins. We performed a retrospective study of 166 children with HS and describe the clinical phenotype according to the genotype. In 160/166 (97%) children with HS a disease-causing mutation was identified. Pathogenic variants in ANK1, SPTB, SLC4A1 and SPTA1 were found in 49%, 33%, 13% and 5% of patients. Children with SLC4A1-HS had the mildest phenotype, showing the highest haemoglobin (P < 0·001), lowest reticulocyte counts (P < 0·001) and lowest unconjugated bilirubin levels (P = 0·006), and none required splenectomy in childhood (P < 0·001). Conversely, children with autosomal recessive SPTA1-HS had the most severe clinical phenotype, with almost all patients undergoing splenectomy in early childhood. Patients with ANK1 and SPTB variants showed a similar clinical phenotype. Within each gene, variant type or location did not predict disease severity or likelihood of splenectomy. Among patients with a genetic diagnosis, 47 (29%) underwent splenectomy (23 partial; 24 total) while 57 (36%) underwent cholecystectomy. Total splenectomy led to greater improvements in haemoglobin (P = 0·02). Select use of genetic testing (especially in patients without a family history) may help predict clinical phenotype in childhood and guide family counselling.

Supraglottic p16+ squamous cell carcinoma during pregnancy: a case report and review of the literature.

BACKGROUND: Head and neck cancer during pregnancy is uncommon. Specifically, laryngeal cancer in pregnancy has only been previously reported 10 times. HPV p16+ supraglottic cancer during pregnancy has never been described in the literature prior to this case report. This case is important to report to understand the most effective and safe diagnostic, treatment and follow-up options available for pregnant patients with laryngeal cancer. CASE PRESENTATION: This report describes a case of a 33-year-old patient who was 24 weeks pregnant presenting with dysphonia and odynophagia. After laryngeal biopsy and MRI she was diagnosed with T3N1M0, stage three p16+ squamous cell carcinoma of the supraglottis. After inter-disciplinary consultation as well as extensive patient discussion, an awake tracheostomy, PEG tube placement and then elective C-section at 28 weeks' gestation was completed. This was followed by chemoradiotherapy. The patient has remained free from disease with a healthy child at four years post-treatment. CONCLUSION: Supraglottic cancer during pregnancy is rare with only four previous cases reported in the literature. This case report elucidates the importance of including multiple specialities as well as patient preference in the decision-making process regarding treatment for patients with supraglottic cancer during pregnancy. Furthermore, diagnostic and treatment guidelines for pregnant patients with head and neck cancers should be established to promote the best possible oncological, obstetrical and neonatal care.

Mild Hereditary Spherocytosis without Accompanying Hereditary Haemochromatosis: An Unrecognised Cause of Iron Overload.

Hereditary spherocytosis (HS) is a common inherited haemolytic anaemia and has great variability in its presentation. Non-transfusion iron overload in HS has only been reported with co-inheritance of hereditary haemochromatosis (HHC). We present 4 unrelated patients of East Asian ethnicity with mild HS and significant non-transfusion iron overload in the absence of known disease-causing mutations in HHC genes. We hypothesise that, in patients with mild HS, life-long chronic haemolysis and erythropoietic drive may promote iron absorption. This suggests that mild HS may not be entirely benign, and that patients with mild HS should be monitored for iron overload.

Results after laparoscopic partial splenectomy for children with hereditary spherocytosis: Are outcomes influenced by genetic mutation?

BACKGROUND: Laparoscopic partial splenectomy (LPS) theoretically maintains long-term splenic immune function for children with hereditary spherocytosis (HS). Our goal was to review our results after LPS and to determine if specific genetic mutations influence outcome. METHODS: All children with HS undergoing LPS between 2005 and 2016 were reviewed. RESULTS: Thirty-one children underwent LPS (16 male) at a median age of 9 (range 2-18) years. All experienced an increase in hemoglobin and decrease in reticulocyte count early after LPS and at last follow-up. Twenty-two were sent for genetic analysis. Mutations in α-spectrin, ß-spectrin, and Ankyrin were identified in 6, 5, and 11 patients, respectively. Gene mutation was not correlated with complications, perioperative transfusion, length of hospital stay, or median hemoglobin, platelet, or reticulocyte counts. Three children required completion splenectomy at 10.9, 6.9, and 3.2years post-LPS, each with a different gene mutation. CONCLUSIONS: LPS is effective in reversing anemia and reducing reticulocytosis. So far less than 10% have required completion splenectomy, and those children did benefit from delaying the risks of asplenia. In this preliminary analysis, genetic mutation did not influence outcome after LPS. A larger multicenter study is necessary to further investigate potential correlations with specific genetic mutations. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: IV.