Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence.

OBJECTIVE: To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS). DESIGN: Retrospective cohort study. SETTING: Tertiary-care children's hospital. PATIENTS, PARTICIPANTS: Consecutive patients with PRS born between January 2010 and June 2018. MAIN OUTCOME MEASURES: Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data.126 patients with PRS were included; 54% had an associated syndrome, 64% had an overt cleft palate, and 22% had a submucous cleft palate. 64/126 were noted to have laryngomalacia (51%). Patients with concurrent PRS and laryngomalacia were significantly more likely to have submucous cleft palate (P = .005) and present with aspiration with cough (P = .01) compared to patients with PRS without laryngomalacia. Patients with concurrent laryngomalacia and PRS showed a significant decrease in apnea-hypopnea index (AHI) and obstructive AHI (OAHI) after mandibular distraction, with a median AHI and OAHI improvement of 22.3 (P = .001) and 19.8 (P = .002), respectively. Patients who underwent only SGP did not show significant improvement in these parameters (P = .112 for AHI, P = .064 for OAHI).The prevalence of laryngomalacia in our PRS cohort was 51%. Patients with PRS and laryngomalacia are more likely to present with overt aspiration compared to patients with PRS without laryngomalacia. These data support that laryngomalacia does not appear to be a contraindication to pursuing MDO.

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.

Outcomes of Furlow Double-Opposing Z-Plasty Palatoplasty for the Treatment of Symptomatic Overt and Occult Submucous Cleft Palate: A Comparison Study.

BACKGROUND: The submucous cleft palate can be overt or occult and may require surgical repair. The double-opposing Z-plasty (Furlow repair) is the authors' center's preferred approach. This study evaluated complication rates, differences in outcome between overt and occult types, and patient factors associated with surgical failure. METHODS: This retrospective study reviewed documentation on all patients who underwent Furlow Z-plasty for submucous cleft palate at a single center between 2004 and 2018. Speech pathology was quantified using the Pittsburgh Weighted Speech Score. RESULTS: A total of 351 patients were included (125 overt and 226 occult cases). Furlow Z-plasty was successful (postoperative Pittsburgh Weighted Speech Score <7 without recommendation for secondary speech surgery) in 291 patients (82.1 percent). Apart from those requiring secondary surgery, there were no documented complications. Occult-type patients were 7.5 years old at palatoplasty with a speech score of 14.1; overt-type patients were 6.5 years old with a score of 15.7. Postoperative speech scores were similar for both groups. Secondary speech surgery patients had a higher preoperative score (16.9 versus 14.2). Age at time of palatoplasty and submucous cleft palate type were not predictive of the need for secondary surgery. Syndromic patients had higher preoperative and postoperative speech scores (15.6 and 7.5, respectively) than nonsyndromic patients (14.3 and 4.3) and needed secondary surgery more often (24.4 percent versus 9.2 percent). V-shaped velar vaulting on preoperative assessment was present in 92 percent of occult-type patients. CONCLUSIONS: Furlow palatoplasty is a safe and effective means of repairing submucous cleft palate. Patients with the occult type presented later with a lower Pittsburgh Weighted Speech Score. High preoperative speech score and syndromic status were associated with the need for secondary speech surgery. V-shaped velar vaulting is a reliable sign of occult submucous cleft palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

The Association Between Age at Palatoplasty and Speech and Language Outcomes in Children With Cleft Palate: An Observational Chart Review Study.

OBJECTIVE: To determine whether timing of palatoplasty (early, standard, or late) is associated with speech and language outcomes in children with cleft palate. DESIGN: Retrospective case series. SETTING: Tertiary care children's hospital. PARTICIPANTS: Records from 733 children born between 2005 and 2015 and treated at the Cleft Craniofacial Clinic of a tertiary children's hospital were retrospectively reviewed. Exclusion criteria were cleft repair at an outside hospital, intact secondary palate, absence of postpalatoplasty speech evaluation, syndromes, staged palatoplasty, and introduction to clinic after 12 months of age. Data from 232 children with cleft palate ± cleft lip were analyzed. INTERVENTIONS: Palatoplasty. MAIN OUTCOME MEASURES: Speech/language delays and disorders at 20 months and 5 years of age based on formal hospital or community-based testing or screening evaluation in the Cleft Craniofacial Clinic; additional speech surgery. RESULTS: Median age at palatoplasty was 12.6 months (range: 8.8-21.9 months). Age at palatoplasty was classified as early (<11 months, n = 28), standard (11-13 months, n = 158), or late (>13 months, n = 46). Late palatoplasty was associated with increased odds of speech/language delays and speech therapy at 20 months, and language delays at 5 years, compared with standard or early palatoplasty (P < .05 for all comparisons). However, speech sound production disorders, velopharyngeal incompetence, tube replacement, and hearing loss were not significantly associated with age at palatoplasty. CONCLUSIONS: Late palatoplasty may be associated with short- and long-term delays in speech/language development. Future studies with standardized surgical technique/timing and outcome measures are required to more definitively describe the impact of age at palatoplasty on speech/language development.

Airway anomalies in patients with craniosynostosis.

OBJECTIVES: 1) Characterize the spectrum of airway anomalies in patients with craniosynostosis, and 2) identify clinical characteristics of these patients that may be associated with the development of airway anomalies. METHODS: This study is a retrospective case series assessing the type and frequency of airway anomalies in all patients with craniosynostosis seen at a tertiary-care children's hospital between 2000 and 2016. Cohort analyses were then performed to identify differences in airway anomalies dependent on syndromic associations, multisutural fusion, and location of suture fusion. Clinical characteristics examined included demographics and additional neurologic and craniofacial abnormalities. RESULTS: Four hundred and ninety-six patients with craniosynostosis (83.5% white, 64.5% male; 33.9% sagittal, 28.8% metopic, 11.5% coronal, 1.2% lambdoid, and 24.6% multisutural) were included. Notable airway anomalies included the following: 13.3% adenotonsillar hypertrophy, 8.9% laryngomalacia, 7.3% tracheomalacia, 7.1% subglottic stenosis, 4.0% bronchomalacia, 3.8% laryngeal cleft, and 1.2% vocal fold paresis. Multisutural craniosynostosis patients (n = 122) were more likely to have obstructive sleep apnea (P = 0.005), adenotonsillar hypertrophy (P = 0.014), tracheomalacia (P = 0.011), subglottic stenosis (P < 0.001), and epiglottic/base of tongue collapse (P = 0.003) and require tracheostomy (P = 0.001) and mechanical ventilation (P = 0.017) compared with single suture craniosynostosis. Syndromic craniosynostosis patients (n = 33) were more likely to have obstructive sleep apnea (P < 0.001), laryngomalacia (P = 0.047), and subglottic stenosis (P = 0.009) compared with nonsyndromic patients. CONCLUSION: Airway anomalies are prevalent in patients with craniosynostosis; patients with multisutural or syndromic types have an increased risk of developing certain abnormalities. There should be a lower threshold for referral for airway evaluation in these populations. LEVEL OF EVIDENCE: 4. Laryngoscope, 129:2594-2602, 2019.

The Impact of Timing of Tympanostomy Tube Placement on Sequelae in Children With Cleft Palate.

OBJECTIVE: To describe the impact of timing of tympanostomy tube insertion on the number of tubes received and complications in children with routine tube placement. DESIGN: Retrospective case series. SETTING: Tertiary care children's hospital. PARTICIPANTS: Records from a consecutive sample of 401 children with cleft palate were reviewed. Sixty-five patients with isolated cleft palate and 82 patients with cleft lip and palate had follow-up until 5 years of age and were included. INTERVENTIONS: Tympanostomy tubes. MAIN OUTCOME MEASURE(S): Number of tubes received and tube-related complications. The hypothesis was formulated prior to data collection. RESULTS: Males comprised 55.8% of included patients, and tubes were placed in 98.6% of patients at a median age of 6.5 months. Effusion was documented at first tube placement for 96.5% of patients. Most (67.4%) patients required replacement of tubes, and 10.6% required long-term tubes. Complications included otorrhea (71.0%), myringosclerosis (35.2%), granulation (22.8%), perforation (17.9%), retained tubes (5.5%), and cholesteatoma (1.4%). Cleft lip and palate (P < .001) and otorrhea (P = .023) were associated with tube placement before palatoplasty. Patients with tube placement before palatoplasty (P = .033), genetic disorders (P = .007), failed newborn hearing screen (P = .012), otorrhea (P < .001), and granulation (P < .001) received more tubes. CONCLUSIONS: Nearly universal effusion in patients with cleft palate supports the need for routine tube placement. The potential for otorrhea and requiring more tubes should be weighed against the risks associated with prolonged effusion when considering tube placement before palatoplasty.

Laryngomalacia in Patients With Craniosynostosis.

OBJECTIVE: To characterize differences in the clinical presentation and treatment outcomes of laryngomalacia in patients with and without craniosynostosis. METHODS: A retrospective cohort study was performed comparing all patients with concomitant laryngomalacia and craniosynostosis seen at a tertiary care children's hospital between 2000 and 2016 with a control group of patients with isolated laryngomalacia. Thirty-two patients with craniosynostosis (59% male) and 68 control patients (56% male) were included. There were no significant differences in age of diagnosis or incidence of prematurity. Symptom presentation, disease severity, swallowing function, comorbidities, treatment modalities, and outcomes were examined using logistic regression. RESULTS: Patients with craniosynostosis had increased odds of presenting with stertor (odds ratio [OR] = 3.41, P = .022), increased work of breathing (OR = 18.8, P = .007), obstructive sleep apnea (OR = 8.48, P = .003), dysphagia (OR = 3.40, P = .008), and aspiration (OR = 40.2, P < .001) and decreased odds of presenting with stridor (OR = 0.0804, P < .001) compared with controls. Patients with craniosynostosis had increased odds of severe laryngomalacia (OR = 5.00, P = .031) and other airway anomalies such as tracheomalacia (OR = 5.73, P = .004), bronchomalacia (OR = 15.5, P = .013), and subglottic stenosis (OR = 2.75, P = .028). Treatment of patients with craniosynostosis was more likely to include tracheostomy (OR = 24.8, P < .001) and gastrostomy tube (OR = 88.4, P < .001). There were no significant differences in rates of supraglottoplasty. CONCLUSION: Clinical presentations, comorbidities, and treatments of laryngomalacia are significantly different in the context of craniosynostosis.

Diagnosing Tongue Base Obstruction in Pierre Robin Sequence Infants: Sleep vs Awake Endoscopy.

OBJECTIVE: To investigate whether awake endoscopy can diagnose base-of-tongue obstruction as reliably as sleep endoscopy in infants with Pierre Robin sequence (PRS). DESIGN: The study was retrospective with the clinicians blinded to patient identity. Endoscopy findings were assessed and measured by the performing pediatric otolaryngologist. SETTING: Tertiary care children's hospital. PATIENTS: All infants with PRS managed between January 2005 and July 2015 were included. There were 141 patients, of which 35 underwent both awake endoscopy (AE) and drug-induced sleep endoscopy (DISE). INTERVENTIONS: Bedside AE and DISE in the operating room. MAIN OUTCOME MEASURES: Presence of moderate or severe base-of-tongue collapse was assessed. Sensitivity, specificity, and positive likelihood ratio of AE findings as well as intertest differences between AE and DISE were calculated. RESULTS: AE had 50.0% sensitivity (95% confidence interval [CI] 27.2%-72.8%) and 86.7% specificity (95% CI 59.5%-98.3%) for detecting base-of-tongue obstruction compared to DISE; false negative rate was 50.0% (n = 10). Positive likelihood ratio was 3.75 (CI 0.96-14.65). Compared to AE, DISE demonstrated significantly more cases of base-of-tongue obstruction ( P = .039). CONCLUSIONS: Bedside AE has low sensitivity for detecting base-of-tongue collapse in infants with PRS. Because of the substantial false negative rate, AE may not be a reliable diagnostic modality for ruling out base-of-tongue obstruction in this susceptible population. DISE may be indicated in high-risk patients to avoid underdiagnosing upper airway obstruction.

Should Children With Cleft Palate Receive Early Long-Term Tympanostomy Tubes: One Institution's Experience.

OBJECTIVES: To determine whether children with cleft palate might benefit from early long-term tympanostomy tubes with the hypothesis that receiving multiple tubes is associated with shorter duration of first tubes. DESIGN: Retrospective cohort study. SETTING: Tertiary care children's hospital. PARTICIPANTS: Records from 401 consecutive children with cleft palate ± cleft lip, born April 2005 to April 2010, were reviewed. After exclusion of children with cleft repair at an outside hospital, no follow-up after 5 years of age, intact secondary palate, no tubes, or tube replacement at palatoplasty, 105 children remained. MAIN OUTCOME MEASURE: Number of tubes. RESULTS: Armstrong grommet tubes were placed at a median age of 6.7 months (range 2.3-19.6 months). Tubes were replaced in 55.3% of patients, with 34.0% receiving ≥3 sets. Duration of first tubes was significantly longer for children with 1 set of tubes compared with those with multiple sets (median 26 vs 19 months, P = .004). Otorrhea, but not perforation, was associated with longer duration of first tubes (median 27 vs 20.5 months, P = .028). Cleft type did not impact the proportion of patients with multiple tubes. Median age at last tube placement for children with multiple tubes was 5.0 years (range 1.9-8.7 years). CONCLUSION: Short duration of first tubes is associated with receiving multiple tubes. Because most patients require repeat tubes and many require tubes until school age, there is a significant need for controlled, prospective trials of early long-term tube placement in this population.

The Impact of Tympanostomy Tubes on Speech and Language Development in Children with Cleft Palate.

Objective Describe the impact of hearing loss, tympanostomy tube placement before palatoplasty, and number of tubes received on speech outcomes in children with cleft palate. Study Design Case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods Records from 737 children born between April 2005 and April 2015 who underwent palatoplasty at a tertiary children's hospital were reviewed. Exclusion criteria were cleft repair at an outside hospital, intact secondary palate, absence of postpalatoplasty speech evaluation, sensorineural or mixed hearing loss, no tubes, first tubes after palatoplasty, or first clinic after 12 months of age. Data from 152 patients with isolated cleft palate and 166 patients with cleft lip and palate were analyzed using Wilcoxon rank-sum, χ2, and Fisher exact test and logistic regression. Results Most patients (242, 76.1%) received tubes before palatoplasty. Hearing loss after tubes, but not before, was associated with speech/language delays at 24 months ( P = .005) and language delays ( P = .048) and speech sound production disorders (SSPDs, P = .040) at 5 years. Receiving tubes before palatoplasty was associated with failed newborn hearing screen ( P = .001) and younger age at first posttubes type B tympanogram with normal canal volume ( P = .015). Hearing loss after tubes ( P = .021), language delays ( P = .025), SSPDs ( P = .003), and velopharyngeal insufficiency ( P = .032) at 5 years and speech surgery ( P = .022) were associated with more tubes. Conclusion Continued middle ear disease, reflected by hearing loss and multiple tubes, may impair speech and language development. Inserting tubes before palatoplasty did not mitigate these impairments better than later tube placement.

Ear Infection in Isolated Cleft Lip: Etiological Implications.

BACKGROUND AND HYPOTHESIS: Chronic ear infections are a common occurrence in children with orofacial clefts involving the secondary palate. Less is known about the middle ear status of individuals with isolated clefts of the lip, although several studies have reported elevated rates of ear infection in this group. The purpose of this retrospective study was to test the hypothesis that chronic ear infections occur more frequently in isolated cleft lip cases (n = 94) compared with controls (n = 183). METHODS: A questionnaire was used to obtain information on history of chronic ear infection. The association between ear infection status (present/absent) and cleft lip status (cleft lip case/control) was tested using both chi-square and logistic regression. RESULTS AND CONCLUSIONS: The reported occurrence of chronic ear infection was significantly greater in cleft lip cases (31%) compared with unaffected controls (11%). After adjusting for age and sex, having a cleft lip increased the odds of being positive for ear infection by a factor greater than 3 (odds ratio = 3.698; 95% confidence interval = 1.91 to 7.14). Within cleft lip cases, there was no difference in the occurrence of ear infection by defect laterality or by the type of clefting present in the family history. Although velopharyngeal insufficiency was present in 18.4% of our cleft lip sample, there was no statistical association between ear infection and abnormal speech patterns. These results may have potential implications both for the clinical management of isolated cleft lip cases and for understanding the etiology of orofacial clefting.

Speech Outcomes After Clinically Indicated Posterior Pharyngeal Flap Takedown.

BACKGROUND: Velopharyngeal insufficiency affects as many as one in three patients after cleft palate repair. Correction using a posterior pharyngeal flap (PPF) has been shown to improve clinical speech symptomatology; however, PPFs can be complicated by hyponasality and obstructive sleep apnea. The goal of this study was to assess if speech outcomes revert after clinically indicated PPF takedown. METHODS: The cleft-craniofacial database of the Children's Hospital of Pittsburgh at the University of Pittsburgh Medical Center was retrospectively queried to identify patients with a diagnosis of velopharyngeal insufficiency treated with PPF who ultimately required takedown. Using the Pittsburgh Weighted Speech Score (PWSS), preoperative scores were compared to those after PPF takedown. Outcomes after 2 different methods of PPF takedown (PPF takedown alone or PPF takedown with conversion to Furlow palatoplasty) were stratified and cross-compared. RESULTS: A total of 64 patients underwent takedown of their PPF. Of these, 18 patients underwent PPF takedown alone, and 46 patients underwent PPF takedown with conversion to Furlow Palatoplasty. Patients averaged 12.43 (range, 3.0-22.0)(SD: 3.93) years of age at the time of PPF takedown, and 58% were men. Demographics between groups were not statistically different. The mean duration of follow-up after surgery was 38.09 (range, 1-104) (SD, 27.81) months. For patients undergoing PPF takedown alone, the mean preoperative and postoperative PWSS was 3.83 (range, 0.0-23.0) (SD, 6.13) and 4.11 (range, 0.0-23.0) (SD, 5.31), respectively (P = 0.89). The mean change in PWSS was 0.28 (range, -9.0 to 7.0) (SD, 4.3). For patients undergoing takedown of PPF with conversion to Furlow palatoplasty, the mean preoperative and postoperative PWSS was 6.37 (range, 0-26) (SD, 6.70) and 3.11 (range, 0.0-27.0) (SD, 4.14), respectively (P < 0.01). The mean change in PWSS was -3.26 (range, -23.0 to 4.0) (SD, 4.3). For all patients, the mean preoperative PWSS was 5.66 (range, 0.0-26) (SD, 6.60) and 3.39 (range, 0.0-27) (SD, 4.48), respectively (P < 0.05). The mean change in PWSS was -2.26 (range, -23.0 to 7) (SD, 5.7). There was no statistically significant regression in PWSS for either surgical intervention. Two patients in the PPF takedown alone cohort demonstrated deterioration in PWSS that warranted delayed conversion to Furlow palatoplasty. Approximately 90% of patients, who undergo clinically indicated PPF takedown alone, without conversion to Furlow Palatoplasty, will show no clinically significant reduction in speech. CONCLUSIONS: Although there is concern that PPF takedown may degrade speech, this study finds that surgical takedown of PPF, when clinically indicated, does not result in a clinically significant regression of speech.

Characteristics of sleep apnea in infants with Pierre-Robin sequence: Is there improvement with advancing age?

OBJECTIVES: To investigate changes in obstructive sleep apnea (OSA) and central sleep apnea (CSA) in infants with Pierre-Robin sequence (PRS) with advancing age and after mandibular distraction osteogenesis (MDO). METHODS: Charts from 141 infants with PRS that presented to our tertiary-care children's hospital between 2005 and 2015 were retrospectively reviewed. Forty-five patients received a polysomnogram (PSG) prior to surgical intervention. Linear regression was utilized to compare age at pre-operative PSG with apnea-hypopnea index (AHI), obstructive apnea-hypopnea index (OAHI), and central apnea index (CAI). We then analyzed a subset of 9 patients who underwent MDO with pre- and post-operative PSGs. Wilcoxon signed-rank test was utilized to examine differences in pre- and post-operative OSA and CSA scores. RESULTS: Forty-five patients received pre-operative PSGs. Of these, 80.0% demonstrated severe sleep apnea (AHI≥10), 68.9% demonstrated severe obstructive sleep apnea (OAHI≥10), and 55.6% demonstrated central sleep apnea (CAI≥1). There was no significant pattern of decrease in AHI, OAHI, and CAI with increased age up to 1 year. Among the 9 patients who underwent MDO with pre- and post-operative PSGs, significant reductions in AHI, OAHI, CAI, and percentage of total sleep time with arterial oxygen saturation (SaO2) <90% and significant increases in SaO2 nadir were identified after MDO. CONCLUSIONS: Contrary to previously examined literature in non-PRS patients, we did not find a decreased severity of central or obstructive sleep apnea with advancing age. Infants with PRS who underwent MDO demonstrated significant decreases in both obstructive and central apnea indices.

Improving speech outcomes after failed palate repair: evaluating the safety and efficacy of conversion Furlow palatoplasty.

BACKGROUND: Velopharyngeal insufficiency occurs in a nontrivial number of cases following cleft palate repair. We hypothesize that a conversion Furlow palatoplasty allows for long-term correction of VPI resulting from a failed primary palate repair, obviating the need for pharyngoplasty and its attendant comorbidities. METHODS: A retrospective review of patients undergoing a conversion Furlow palatoplasty between 2003 and 2010 was performed. Patients were grouped according to the type of preceding palatal repair. Velopharyngeal insufficiency was assessed using Pittsburgh Weighted Speech Scale (PWSS). Scores were recorded and compared preoperatively and postoperatively at 3 sequential visits. RESULTS: Sixty-two patients met inclusion criteria and were grouped by preceding repair (straight-line repair (n = 37), straight-line repair with subsequent oronasal fistula (n = 14), or pharyngeal flap (n = 11). Median PWSS scores at individual visits were as follows: preoperative = 11, first postoperative = 3 (mean, 114.0 ± 6.7 days), second postoperative = 1 (mean, 529.0 ± 29.1 days), and most recent postoperative = 3 (mean, 1368.6 ± 76.9 days). There was a significant difference between preoperative and postoperative PWSS scores in the entire cohort (P < 0.001) with overall improvement, and post hoc analysis showed improvement between each postoperative visit (P < 0.05) with the exception of the second to the most recent visit. There were no differences between postoperative PWSS scores in the operative subgroupings (P > 0.05). Eight patients failed to improve and showed no differences in PWSS scores over time (P > 0.05). Patients with a PWSS score of 7 or greater (n = 8) at the first postoperative visit (0-6 months) displayed improvement at the most recent visit (P< 0.05). CONCLUSIONS: Conversion Furlow palatoplasty is an effective means for salvaging speech. Future studies should elucidate which factors predict the success of this technique following failed palate repair.

Intraoperative high-dose epinephrine infiltration in cleft palate repair.

OBJECTIVE: Local infiltration of epinephrine before surgical procedures is a well-accepted technique to promote vasoconstriction. Typically, the dose of epinephrine is limited by the co-administration of local anesthetic as well as the risk for arrhythmogenesis and hemodynamic changes. In addition, some controversy exists regarding the acceptable dose of epinephrine given to children. This retrospective review examines the use and safety of "high-dose" epinephrine in palatoplasty at our cleft-craniofacial center. DESIGN: A retrospective review of epinephrine use in primary palatoplasty at a tertiary children's hospital from 2003 to 2007 was performed. Operative and anesthetic records were reviewed for hypertension (systolic blood pressure, >120 or diastolic blood pressure, >70) and tachycardia (>190 beats per min) as defined by the American Heart Association guidelines, as well as dysrhythmias, intraoperative complications, and postoperative complications. RESULTS: A total of 102 patients who underwent consecutive primary palatoplasties performed by a single surgeon were identified. After the induction of anesthesia and before incision, the patients received an initial epinephrine infiltration (without local anesthetic) up to a maximum 10 µg/kg. The average total dose of epinephrine administered during palatoplasty was 12.8 µg/kg (range, 3.2-75.0 µg/kg). Doses up to a maximum of 10 µg/kg were administered as needed at 30-minute intervals. No instances of clinically unstable tachycardia or hypertension occurred. A total of 21.6% of the patients (22/102) experienced an instance of hypertension, and only 13.7% of these (14/102) were related to epinephrine administration. One (1%) postoperative fistula was identified. CONCLUSIONS: Locally infiltrated high-dose epinephrine during palatoplasty can be safely used as a means of vasoconstriction. Doses reaching a maximum of 10 µg/kg, administered as needed at 30-minute intervals, do not seem to be a significant risk for hemodynamic instability, intraoperative complications, or postoperative complications.

Exploring high frequency temporal fluctuations in the terminal aneurysm of the basilar bifurcation.

Cerebral aneurysms are a common cause of death and disability. Of all the cardiovascular diseases, aneurysms are perhaps the most strongly linked with the local fluid mechanic environment. Aside from early in vivo clinical work that hinted at the possibility of high-frequency intra-aneurysmal velocity oscillations, flow in cerebral aneurysms is most often assumed to be laminar. This work investigates, through the use of numerical simulations, the potential for disturbed flow to exist in the terminal aneurysm of the basilar bifurcation. The nature of the disturbed flow is explored using a series of four idealized basilar tip models, and the results supported by four patient specific terminal basilar tip aneurysms. All four idealized models demonstrated instability in the inflow jet through high frequency fluctuations in the velocity and the pressure at approximately 120 Hz. The instability arises through a breakdown of the inflow jet, which begins to oscillate upon entering the aneurysm. The wall shear stress undergoes similar high-frequency oscillations in both magnitude and direction. The neck and dome regions of the aneurysm present 180 deg changes in the direction of the wall shear stress, due to the formation of small recirculation zones near the shear layer of the jet (at the frequency of the inflow jet oscillation) and the oscillation of the impingement zone on the dome of the aneurysm, respectively. Similar results were observed in the patient-specific models, which showed high frequency fluctuations at approximately 112 Hz in two of the four models and oscillations in the magnitude and direction of the wall shear stress. These results demonstrate that there is potential for disturbed laminar unsteady flow in the terminal aneurysm of the basilar bifurcation. The instabilities appear similar to the first instability mode of a free round jet.

Hemodynamics of the mouse abdominal aortic aneurysm.

The abdominal aortic aneurysm (AAA) is a significant cause of death and disability in the Western world and is the subject of many clinical and pathological studies. One of the most commonly used surrogates of the human AAA is the angiotensin II (Ang II) induced model used in mice. Despite the widespread use of this model, there is a lack of knowledge concerning its hemodynamics; this study was motivated by the desire to understand the fluid dynamic environment of the mouse AAA. Numerical simulations were performed using three subject-specific mouse models in flow conditions typical of the mouse. The numerical results from one model showed a shed vortex that correlated with measurements observed in vivo by Doppler ultrasound. The other models had smaller aneurysmal volumes and did not show vortex shedding, although a recirculation zone was formed in the aneurysm, in which a vortex could be observed, that elongated and remained attached to the wall throughout the systolic portion of the cardiac cycle. To link the hemodynamics with aneurysm progression, the remodeling that occurred between week one and week two of the Ang II infusion was quantified and compared with the hemodynamic wall parameters. The strongest correlation was found between the remodeled distance and the oscillatory shear index, which had a correlation coefficient greater than 0.7 for all three models. These results demonstrate that the hemodynamics of the mouse AAA are driven by a strong shear layer, which causes the formation of a recirculation zone in the aneurysm cavity during the systolic portion of the cardiac waveform. The recirculation zone results in areas of quiescent flow, which are correlated with the locations of the aneurysm remodeling.

The Murine Angiotensin II-Induced Abdominal Aortic Aneurysm Model: Rupture Risk and Inflammatory Progression Patterns.

An abdominal aortic aneurysm (AAA) is an enlargement of the greatest artery in the body defined as an increase in diameter of 1.5-fold. AAAs are common in the elderly population and thousands die each year from their complications. The most commonly used mouse model to study the pathogenesis of AAA is the angiotensin II (Ang II) infusion method delivered via osmotic mini-pump for 28 days. Here, we studied the site-specificity and onset of aortic rupture, characterized three-dimensional (3D) images and flow patterns in developing AAAs by ultrasound imaging, and examined macrophage infiltration in the Ang II model using 65 apolipoprotein E-deficient mice. Aortic rupture occurred in 16 mice (25%) and was nearly as prevalent at the aortic arch (44%) as it was in the suprarenal region (56%) and was most common within the first 7 days after Ang II infusion (12 of 16; 75%). Longitudinal ultrasound screening was found to correlate nicely with histological analysis and AAA volume renderings showed a significant relationship with AAA severity index. Aortic dissection preceded altered flow patterns and macrophage infiltration was a prominent characteristic of developing AAAs. Targeting the inflammatory component of AAA disease with novel therapeutics will hopefully lead to new strategies to attenuate aneurysm growth and aortic rupture.

Is flow in the common carotid artery fully developed?

The assumption of fully developed or axisymmetric velocity profiles in the common carotid artery (CCA) underlies the straightforward estimation of CCA blood flow rates or wall shear stresses (WSS) from limited velocity data, such as spectral peak velocities acquired using Doppler ultrasound. Using an automated velocity profile classifier developed for this study, we characterized the shape of the CCA velocity profile from cine phase contrast magnetic resonance images acquired as part of an Atherosclerosis Risk in Communities (ARIC) ancillary study, here focusing on 45 participants imaged twice as part of a repeatability protocol. When averaged over the cardiac cycle, roughly 60% of the velocity profiles were classified as skewed, with over half of these exhibiting the crescent shape characteristic of strong Dean-type flow in a curved tube. During early diastole, roughly 80% of the velocity profiles were skewed. In most cases the degree and orientation of skewing were reproduced in the repeat scan, indicating the persistence of these flow features. Fully developed flow thus appears to be the exception rather than the rule in the nominally straight CCA. Implications of this for flow rate and WSS estimation, and perhaps the development and progression of carotid atherosclerosis, warrant further investigation.

A qualitative description of receiving a diagnosis of clefting in the prenatal or postnatal period.

This study investigated the experience of receiving a diagnosis of clefting in the prenatal or postnatal period. Open-ended interviews were conducted with 20 parents of children with cleft lip with or without cleft palate. Interviews were transcribed and analyzed using a qualitative descriptive approach with an emphasis on thematic analysis. Common themes emerged from participants' responses regarding the delivery of the diagnosis, preparation for the birth of their child, advantages and disadvantages of prenatal diagnosis, use of the Internet, views on abortion and genetic testing, among other issues. All participants in the prenatal group indicated they were satisfied they learned of the cleft before the birth of their child. Some participants in the postnatal group would rather have received the diagnosis prenatally, while others were content with learning of the diagnosis in the delivery room. Greater awareness of the parental experience of the timing of receiving a cleft diagnosis may assist health care professionals in providing care for these families.