Neuraxial and regional anesthesia in surgical patients with osteogenesis imperfecta: a narrative review of literature.

BACKGROUND AND OBJECTIVES: Regional and neuraxial anesthesia techniques have become instrumental in the perioperative period yet have not been well described in patients with osteogenesis imperfecta (OI), a congenital connective tissue disorder characterized by skeletal dysplasia and fragility. Patients with skeletal dysplasia present unique perioperative challenges that warrant consideration of these techniques despite their relative contraindication in this population due to reports of increased bleeding with surgery, skeletal fragility concerns with positioning, and risk of spinal cord injury with continuous neuraxial catheters. The aim of this narrative review was to evaluate literature describing the use of regional and neuraxial techniques in patients with OI and any associated clinical outcomes. METHODS: All available literature from inception to July 2020 was retrieved, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, from MEDLINE, Embase, Google Scholar and The Cochrane Library. Three authors reviewed all references for eligibility, abstracted data, and appraised quality. RESULTS: Of 412 articles initially identified, 42 met our inclusion criteria, yielding 161 cases with regional and/or neuraxial techniques described. In 117 (72.6%) of the 161 cases, neuraxial technique was performed, including 76 (64.9%) epidural, 7 (5.9%) caudal, 5 (4.2%) combined spinal epidural, and 29 (24.7%) spinal procedures. In 44 (27.4%) of the 161 cases, the use of regional anesthesia was described. Our review was confounded by incomplete data reporting and small sample sizes, as most were case reports. There were no randomized controlled trials, and the two single-center retrospective data reviews lacked sufficient data to perform meta-analysis. While complications or negative outcomes related to these techniques were not reported in any of the cases, less than half specifically discuss outcomes beyond placement and immediate postoperative course. CONCLUSIONS: There is insufficient evidence to validate or refute the potential risks associated with the use of regional and neuraxial techniques in patients with OI. This review did not uncover any reports of negative sequelae related to the use of these modalities to support relative contraindication in this population; however, further research is needed to adequately assess clinically relevant outcomes such as complications and opioid-sparing effect.

Predictive Factors for Vestibular Loss in Children With Hearing Loss.

PURPOSE: The aim of this study was to determine if there are factors that can predict whether a child with hearing loss will also have vestibular loss. METHOD: A retrospective chart review was completed on 186 children with hearing loss seen at Boys Town National Research Hospital for vestibular testing from 1999 to 2015 through neurosensory genetics clinic or cochlear implant candidacy. Each child's medical chart was reviewed to obtain the following data: vestibular loss severity (classified as normal, bilateral, or mild to moderate), degree of hearing loss (bilateral pure-tone average [PTA]), imaging abnormalities (classified as "normal" or "abnormal"), parental concerns for gross motor delay (classified as "yes, there is concern" or "no, there is not a concern"), parent report of age when their child sat (months) and walked independently (months), comorbidities (classified as "yes" if there were 1 or more comorbidities or "no" if there were no comorbidities), and score on the Developmental Profile-3. RESULTS: Children were grouped according to vestibular loss severity; 115 children had normal vestibular function, 31 had bilateral vestibular loss, and 40 had mild-to-moderate vestibular loss. As severity of vestibular loss increased, children (a) sat and walked later, (b) scored more poorly on the Developmental Profile-3 physical subscale, (c) had more severe hearing loss, (d) had parents who more frequently reported concern for gross motor delay, and (e) were more likely to have other comorbidities. Of these factors, age-to-sit, age-to-walk, PTA, and parental concerns for gross motor developmental delay had the greatest ability to differentiate children with vestibular loss from children with normal vestibular function. For age-to-sit, using a cutoff value of 7.25 months yielded a sensitivity of 62% and a specificity of 81%. For age-to-walk, a cutoff value of 14.5 months yielded a sensitivity of 78% and a specificity of 77%. For PTA for the neurosensory genetics group, a cutoff value of 40 dB yielded a sensitivity of 80% and a specificity of 55%; however, a cutoff value of 66 dB yielded a sensitivity of 33% and an improved specificity of 91%. CONCLUSIONS: A referral for vestibular evaluation should be considered for children whose hearing loss is greater than 66 dB and particularly those who sit later than 7.25 months or walk later than 14.5 months or whose parents report concerns for gross motor development. Collectively, these factors appear to be more sensitive for identifying children with bilateral vestibular loss compared with children with mild-to-moderate vestibular loss. Because of the benefit of physical therapy, children identified with vestibular loss should then be referred to physical therapy for further evaluation and treatment.

Gender-based comorbidity in benign paroxysmal positional vertigo.

It has been noted that benign paroxysmal positional vertigo (BPPV) may be associated with certain disorders and medical procedures. However, most studies to date were done in Europe, and epidemiological data on the United States (US) population are scarce. Gender-based information is even rarer. Furthermore, it is difficult to assess the relative prevalence of each type of association based solely on literature data, because different comorbidities were reported by various groups from different countries using different patient populations and possibly different inclusion/exclusion criteria. In this study, we surveyed and analyzed a large adult BPPV population (n = 1,360 surveyed, 227 completed, most of which were recurrent BPPV cases) from Omaha, NE, US, and its vicinity, all diagnosed at Boys Town National Research Hospital (BTNRH) over the past decade using established and consistent diagnostic criteria. In addition, we performed a retrospective analysis of patients' diagnostic records (n = 1,377, with 1,360 adults and 17 children). The following comorbidities were found to be significantly more prevalent in the BPPV population when compared to the age- and gender-matched general population: ear/hearing problems, head injury, thyroid problems, allergies, high cholesterol, headaches, and numbness/paralysis. There were gender differences in the comorbidities. In addition, familial predisposition was fairly common among the participants. Thus, the data confirm some previously reported comorbidities, identify new ones (hearing loss, thyroid problems, high cholesterol, and numbness/paralysis), and suggest possible predisposing and triggering factors and events for BPPV.

Menopause and benign paroxysmal positional vertigo.

OBJECTIVE: This study was designed to examine the age and sex distribution and the effects of menopause in a large cohort of participants diagnosed with benign paroxysmal positional vertigo (BPPV). METHODS: We analyzed 1,377 BPPV patients and surveyed 935 women from this group-all diagnosed at the Boys Town National Research Hospital in the last decade. RESULTS: A detailed age and sex distribution analysis of BPPV onset showed that aging had a profound impact on BPPV occurrence in both sexes, and that perimenopausal women were especially susceptible to BPPV (3.2:1 female-to-male ratio). The latter is a novel finding and was confirmed by a direct survey of female BPPV patients (168 participated). In addition, there was a pronounced female preponderance (6.8:1 female-to-male ratio) in BPPV in the teenage group despite its low prevalence in this age group. CONCLUSIONS: Data suggest that hormonal fluctuations (especially during menopause) may increase the tendency to develop BPPV.