Otorhinologic Disorders in 22q11.2 Deletion Syndrome.

OBJECTIVE: Investigate incidence and natural history of otologic and sinonasal disease associated with 22q11.2 deletion syndrome. STUDY DESIGN: Case series. SETTING: Tertiary care children's hospital. METHODS: Charts from consecutive children born 2000 to 2018 with a diagnosis of 22q11.2 deletion, DiGeorge, or velocardiofacial syndrome based on the International Classification of Diseases (ICD)-9 and ICD-10 codes were reviewed. Otologic and rhinologic diagnoses and surgeries and immune and microbiologic laboratory findings were collected from the medical record. RESULTS: After the exclusion of patients with no 22q11.2 deletion (n = 101), otologic care at an outside hospital (n = 59), and loss to follow-up prior to 3 years of age (n = 22), 128 were included. Males comprised 80 (62.5%) patients, 115 (89.8%) were white, and the median age at genetic confirmation of 22q11.2 deletion was 119 days (range 0 days to 14.6 years). Recurrent acute otitis media (RAOM), chronic otitis media with effusion, chronic rhinosinusitis, and recurrent acute sinusitis were diagnosed in 54 (42.2%), 37 (28.9%), 10 (7.8%), and 8 (6.3%), respectively. Tympanostomy tubes were placed in 49 (38.3%). Adenoidectomy and sinus surgery were performed in 38 (29.7%) and 4 (3.1%), respectively. Neither immunoglobulin nor cluster of differentiation deficiency increased the odds of RAOM diagnosis, tympanostomy tube placement, or chronic/recurrent sinusitis. Methicillin-resistant Staphylococcus aureus was the most common organism in sinus cultures (4/13, 30.8%). Streptococcus pneumonia dominated otorrhea cultures (11/21, 52.4%). CONCLUSION: Approximately half of children with 22q11.2 deletion may experience otologic disease that often requires surgical management. Future studies will utilize a larger cohort to examine the role of immunodeficiency in otologic and rhinologic disease in this population.

The limits of pandemic precautions: Tympanostomy tube placement in children with cleft palate during COVID-19.

PURPOSE: Coronavirus Disease-2019 (COVID-19) mitigation measures have led to a sustained reduction in tympanostomy tube (TT) placement in the general population. The present aim was to determine if TT placement has also decreased in children at risk for chronic otitis media with effusion (COME), such as those with cleft palate (CP). MATERIALS AND METHODS: A cohort study with medical record review was performed including consecutive children, ages 0-17 years, undergoing primary palatoplasty at a tertiary children's hospital February 2019-January 2020 (pre-COVID) or May 2020-April 2021 (COVID). Revision palatoplasty (n = 29) was excluded. Patient characteristics and middle ear status pre-operatively and at palatoplasty were compared between groups using logistic regression or Wilcoxon rank-sum. RESULTS: The pre-COVID and COVID cohorts included 73 and 87 patients, respectively. Seventy (44%) were female and median age at palatoplasty was 13.5 months for CP ± cleft lip (CP ± L) and 5.5 years for submucous cleft palate (SMCP). In patients with CP ± L, TT were placed or in place and patent at palatoplasty in 28/38 (74%) pre-COVID and 37/50 (74%) during COVID (P = 0.97). In patients with SMCP, these proportions were 5/35 (14%) and 6/37 (16%), respectively (P = 0.82). Examining only patients <2 years of age also revealed no difference in TT placement pre-COVID versus COVID (P = 0.99). Finally, the prevalence and type of effusion during COVID was similar to pre-COVID. CONCLUSIONS: Reduced infectious exposure has not decreased TT placement or effusion at palatoplasty. Future work could focus on non-infectious immunologic factors underlying the maintenance of COME in these children.

Improved Understanding of Acute Incisional Hernia Incarceration: Implications for Addressing the Excess Mortality of Emergent Repair.

BACKGROUND: Incisional hernia develops in up to 20% of patients undergoing abdominal operations. We sought to identify characteristics associated with poor outcomes after acute incisional hernia incarceration. STUDY DESIGN: We performed a retrospective cohort study of adult patients with incisional hernias undergoing elective repair or with acute incarceration between 2010 and 2017. The primary end point was 30-day mortality. Logistic regression was used to determine adjusted odds associated with 30-day mortality. The American College of Surgeons Surgical Risk Calculator was used to estimate outcomes had these patients undergone elective repair. RESULTS: A total of 483 patients experienced acute incarceration; 30-day mortality was 9.52%. Increasing age (adjusted odds ratio 1.05; 95% CI, 1.02 to 1.08) and bowel resection (adjusted odds ratio 3.18; 95% CI, 1.45 to 6.95) were associated with mortality. Among those with acute incarceration, 231 patients (47.9%) had no documentation of an earlier surgical evaluation and 252 (52.2%) had been evaluated but had not undergone elective repair. Among patients 80 years and older, 30-day mortality after emergent repair was high (22.9%) compared with estimated 30-day mortality for elective repair (0.73%), based on the American College of Surgeons Surgical Risk Calculator. Estimated mortality was comparable with observed elective repair mortality (0.82%) in an age-matched cohort. Similar mortality trends were noted for patients younger than 60 years and aged 60 to 79 years. CONCLUSIONS: Comparison of predicted elective repair and observed emergent repair mortality in patients with acute incarceration suggests that acceptable outcomes could have been achieved with elective repair. Almost one-half of acute incarceration patients had no earlier surgical evaluation, therefore, targeted interventions to address surgical referral can potentially result in fewer incarceration-related deaths.

Reoperative Cardiac Surgery Is a Risk Factor for Long-Term Mortality.

BACKGROUND: Reoperative cardiac surgery has been associated with increased morbidity and mortality. Large propensity-matched series comparing all first-time and redo cardiac operations are lacking. The primary objective of the current study was to provide detailed outcomes and risk factors for mortality and readmissions after reoperative cardiac surgery. METHODS: All patients who underwent cardiac surgery from 2011 to 2017 were included. Propensity matching yielded equitable cohorts. Multivariable Cox regression analysis was performed to identify independent predictors of 30-day, 1-year, and 5-year mortality and readmissions. RESULTS: A total of 14,151 patients underwent cardiac surgery, of which 1700 (12%) had reoperative cardiac surgery. There were significantly (P < .001) more comorbidities in the reoperative cardiac surgery group. Propensity matching yielded 1696 patients in each cohort. After propensity matching, operative mortality (8.37% vs 6.07%; P = .01), blood product transfusion (54.7% vs 46.2%; P < .001), and prolonged ventilator requirements (>24 hours) (20% vs 17%; P = .02) were increased for the reoperative cohort. On multivariable analysis for propensity-matched cohorts, reoperation was an independent predictor of mortality at 30 days (hazard ratio [HR], 1.36; 95% confidence interval [CI], 1.05-1.75; P = .02), 1 year (HR, 1.30; 95% CI, 1.09-1.55; P = .004), and 5 years (HR, 1.30; 95% CI, 1.14-1.5; P = .002). CONCLUSIONS: After risk-adjusting for baseline characteristics, the need for reoperation was an independent predictor of both short-term and long-term mortality after reoperative cardiac surgery. These data are relevant when considering alternative therapies such as percutaneous coronary or transcatheter valve interventions.

Impact of Perioperative Stroke on Midterm Outcomes After Transcatheter Aortic Valve Replacement.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) has evolved as an alternative therapy to open aortic valve replacement in most patients with aortic stenosis. Stroke associated with TAVR can be a devastating complication in the short term; however, little is known regarding midterm outcomes. METHODS: All patients undergoing TAVR at the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania from 2011 to 2018 were included. Modified Rankin Scale values as a measurement of stroke-related disability were extracted for patients who had neurologic deficits. RESULTS: Neurologic events (NEs) developed in 51 (4.3%) of the 1193 patients during the study period (32 [2.7%] had disabling strokes; 19 [1.6%] had nondisabling strokes, including 5 [0.4%] transient ischemic attacks). Patients who had TAVR-related NEs were older (85.8 ± 4.2 years vs 81.5 ± 7.9 years; P < .001) and predominantly female (68.6% vs 31.4%; P = .007), but they were comparable in terms of The Society of Thoracic Surgeons predicted mortality score and vascular access. Patients with NEs had increased short term and midterm mortality (15.7% vs 2.6%, 29.4% vs 13.9%, and 47.1% vs 35.7% at 30 days, 1 year, and 3 years, respectively). Severity of disability, determined by the modified Rankin Scale, was a risk factor for 30-day mortality (HR, 5.8; P = .003), 1-year mortality (HR, 2.1; P < .001) and 3-year mortality (HR, 1.8; P < .001). Predictors of TAVR NEs were older age (odds ratio [OR] per year of age, 1.11; P = .001), low body surface area (OR per m2, 0.22; P = .050), procedural duration (OR per minute, 1.01; P = .024), and administration of blood products (OR, 3.23; P = .002). CONCLUSIONS: Stroke increases short-term and midterm mortality after TAVR. Risk prediction for neurologic events in TAVR could aid the framework for patient selection and further improve outcomes.

Perceptions of HIV Preexposure Prophylaxis Among Young Pregnant Women from Rural KwaZulu-Natal, South Africa.

Young sub-Saharan women are at particularly high risk of HIV acquisition during pregnancy and the postpartum period and would potentially benefit from preexposure prophylaxis (PrEP). From June to August 2016, we interviewed 187 HIV negative pregnant women 18-24 years old in Tugela Ferry, Kwazulu-Natal province, a rural and among the poorest subdistricts in South Africa. Demographic data, HIV and PrEP knowledge, HIV risk, and readiness for oral tenofovir-based PrEP were collected using an information-motivation-behavior model-formatted instrument. Mean age was 20.3 years, 179 (95.7%) were unemployed, and 137 (73.3%) reported sex with one partner in the last month. Most were concerned that their sexual partner (95.2%) potentially had HIV or had other sexual partners in the last month (36.4%). Despite this, only 7 (3.7%) women reported that condoms had been used consistently during sex; most (97.3%) felt powerless to negotiate condom use with their partner. There was widespread interest in taking PrEP (97.3%), and most women (>97%) reported possessing the skills to take pills regularly, would commit to monthly visits, and were motivated to remain HIV negative to take care of their families. Young pregnant rural South African women are cognizant of their HIV risk and interested in prevention. Impending motherhood may portend increased interest in HIV prevention. We identified three potential obstacles to successful PrEP rollout among young pregnant women: hesitation about PrEP effectiveness (46%), perceived HIV stigma (53.5%), and risk compensation through decreased condom use (9.6%). Comparative studies of motivations, skills, and rates of initiation and adherence among pregnant and nonpregnant women are needed to inform optimal implementation efforts.