Is Outpatient Supraglottoplasty Possible? Examination of Perioperative Comorbidities and Outcomes.

OBJECTIVE: Supraglottoplasty (SGP) is necessary in approximately 10% to 15% of infants with congenital laryngomalacia (LM). Postoperative management strategies vary and this study examines outcomes and readmission rates. STUDY DESIGN: This is a retrospective chart review. SETTING: A single tertiary care institution's patients undergoing SGP from 2012 to May 2022. METHODS: Patients were assigned to 1 of 3 groups based on length of stay (LOS) including 0, 1, or 2+ days. Preoperative data included age, prevailing symptoms, and comorbidities at the time of surgery. Postoperative data included level of care, perioperative complications, readmission rates, and SGP failure. Descriptive statistics were used to evaluate differences between the 3 groups. RESULTS: Following SGP in 975 patients, 23.8% were discharged the same day (Group 0), 63.2% were observed overnight (Group 1), and 13.0% required 2+ days (Group 2). Four of the 616 patients in Group 1 were admitted to an intensive care unit (ICU) setting, and 11 patients in Group 2. The patients in Group 2 had a higher incidence of Pepcid use, previous intubation, cardiac anomalies, prior gastrostomy tube (G tube), subglottic stenosis, cyanosis, failure to thrive, and prematurity. The significant differences in the group admitted to the ICU were those admitted for airway, cerebral palsy, prior G tube, PSG, prior intubation, or cardiac anomaly (P = <.05). Readmission rates from Group 0 versus 1 were 1.3% and 2.9%, respectively, though this was not statistically significant. CONCLUSION: SGP is a safe procedure that can be performed in the outpatient setting in select patients.

Myringoplasty Using Human Birth Tissue Allografts to Repair Large Tympanic Membrane Perforations.

OBJECTIVE: Examine outcomes among a series of pediatric patients who underwent myringoplasty using human birth tissue (BT) for repair of large tympanic membrane (TM) perforations. STUDY DESIGN: Case series. SETTING: Single-institution pediatric hospital. METHODS: Retrospective chart review of patients treated with BT during a 4-year study period. Subjects who underwent myringoplasty for large (size 40% or greater) TM perforations were included for this study. Patients with a stable perforation of at least 1 month's duration preoperatively who then followed up for at least 3 months postoperatively met inclusion criteria. RESULTS: Six subjects were included in this study. One subject underwent bilateral repair; thus, this series includes a total of 7 perforations. TM perforations ranged from 40% to 70% of the TM. At initial follow-up (median of 2 months), 5 of the 7 perforations had healed. One of these 5 had evidence of a 10% recurrent perforation at 5 months, which subsequently healed. Of the 2 patients not healed at initial follow-up, 1 had only a residual pinpoint perforation that subsequently healed; the other had a persistent 30% perforation that was possibly related to their postoperative recovery period, which was complicated by a respiratory viral illness. CONCLUSION: For large TM perforations, myringoplasty with BT grafts may be a viable alternative to longer, more invasive procedures like tympanoplasty. Larger, randomized, prospective studies are needed.

Blue Rubber Bleb Nevus Syndrome: A Rare Case of Gastrointestinal Hemorrhage Necessitating Bowel Resection.

Blue Rubber Bleb Nevus Syndrome is a congenital rarity that manifests as vascular malformations throughout the body, including the gastrointestinal tract. With fewer than 300 cases reported, the etiology and clinical course is poorly understood; however, the literature suggests TEK mutations on chromosome 9 result in unregulated angiogenesis. We present the case of a young female treated for anemia of unknown etiology who presented in hemorrhagic shock due to gastrointestinal hemorrhage necessitating small bowel resection, with cutaneous, intestinal, hepatic, and lingual vascular malformations associated with a single somatic pathologic TEK mutation. Although uncommon, this case suggests that Blue Rubber Bleb Nevus Syndrome should be considered in the differential of a patient with persistent anemia and cutaneous lesions, carrying the potential for multiple gastrointestinal vascular malformations progressing to hemorrhage necessitating operative management. Additionally, a severe phenotype can occur without a double-hit TEK mutation.

Impact of Oral Steroids on Tonsillectomy Postoperative Complications and Pain.

OBJECTIVES: To analyze the impact of steroids on postoperative tonsillectomy recovery and implement findings for improvement in postoperative management. METHODS: Institutional review board approved prospective study with retrospective analysis of private practice setting tonsillectomy patients (November 2015 to January 2017). A questionnaire was provided postoperatively to patients undergoing tonsillectomy with or without adenoidectomy. The study population was separated into 2 groups: patients who received steroids (3 days of either dexamethasone or prednisolone), postoperative steroid (POS), versus patients who did not receive steroids (PONS). RESULTS: The questionnaire had a return rate of 27.3% (254/931). Nine of the 254 responses were disqualified for lack of information; therefore, the total number of responses was 245. Of these, 115 were POS and 130 were PONS. The groups were similar in mean age (POS: 13.2 ± 10.4 years, PONS: 14.7 ± 12.1 years, P = .32) and sex (POS: Male 40.0%, PONS: Male 40.0%, P = .97). There was an overall decrease of pain and nausea/vomiting (N/V) in the steroid group (P = .0007). There was reduction in pain (P < .05) from postoperative day (POD) 2, 3, 4, and 6 in the POS group. Otherwise, there was no significant reduction in pain from POD 7 to 14, day-by-day rate of N/V, bleeding, or rate of emergency department (ED) or clinic visit (P > .05). CONCLUSION: Postoperative steroid reduced overall pain and N/V, as well as daily pain on POD 2, 3, 4, and 6. Pain from POD 7 to 14, rate of ED or clinic visit, or daily N/V and bleeding rate were not significantly different between cohorts.

Extracranial arteriovenous malformations demonstrate dysregulated TGF-ß/BMP signaling and increased circulating TGF-ß1.

Extracranial arteriovenous malformations (AVMs) are characterized by anomalous arterial-to-venous connections, aberrant angiogenesis, local inflammation and hypoxia, and disorganized histological architecture; however, the precise molecular perturbations leading to this phenotype remain elusive. We hypothesized that extracranial AVM tissue would demonstrate deregulation of the TGF-ß/BMP signaling pathway, which may serve as a potential target in the development of molecular-based therapies for AVMs. AVM tissue was harvested during resection from 10 patients with AVMs and compared to control tissue. Blood was collected from 14 AVM patients and 10 patients without AVMs as controls. Expression of TGF-ß/BMP pathway components was analyzed using RT-PCR, western blotting, and immunohistochemistry. Circulating levels of TGF-ß1 were analyzed by ELISA. Paired t tests were utilized to perform statistical analysis. The mRNA levels of TGF-ß1, ALK1, Endoglin (ENG), Smad6, Smad7, and Smad8 were significantly elevated in AVM tissue when compared to controls. Protein levels of TGF-ß1 and Smad3 were elevated in AVM tissue while protein levels of BMP-9, ALK1, Smad1, Smad6, and Smad8 were significantly decreased in AVMs. Immunohistochemistry demonstrated increased TGF-ß1 in the perivascular cells of AVMs compared to normal controls, and circulating levels of TGF-ß1 were significantly higher in AVM patients. Patients with AVMs demonstrate aberrant TGF-ß/BMP expression in AVM tissue and blood compared to controls. Targeting aberrantly expressed components of the TGF-ß/BMP pathway in extracranial AVMs may be a viable approach in the development of novel molecular therapies, and monitoring circulating TGF-ß1 levels may be a useful indicator of treatment success.

Laser Treatment of Vascular Anomalies.

Lasers are a safe and effective tool for the treatment of vascular anomalies. There are many laser options available. Matching laser parameters with the characteristics of the vasculature in these lesions can selectively deliver energy to the abnormal tissue. This can lead to reduction in size and symptoms of vascular malformations and hemangiomas.

Infantile Hemangioma Treated with Propranolol Readmission Trends, Complications of Therapy, and Cost: A PHIS Database Study.

Objective: To examine admission trends, complications, and costs for inpatient infantile hemangioma (IH) associated with propranolol therapy utilizing the Pediatric Health Information System (PHIS) database. Study Design. A retrospective cohort study was completed using the PHIS database. The PHIS database was queried from 2008 to 2020 for children without cardiac disease and between the ages of three weeks and one year who were admitted with a diagnosis of IH and administered propranolol. Admissions were trended annually and by geographic region. Primary outcomes were length of stay (LOS), readmission, mortality, propranolol-related complications, and costs. Bivariate and multivariable analyses were employed to identify predictors of the primary outcomes. Results: A total of 2290 unique patient encounters were identified. Admissions steadily decreased after 2011, with variations by geographic region. There was no mortality and only 60 (2.6%) propranolol-related complications. African-American race (odds ratio (OR) 1.20 [95% CI: 1.02-1.41]), respiratory comorbidities (OR 2.04 [95% CI: 1.42-2.93]), neurologic conditions (OR 1.34 [95% CI: 1.09-1.59]), admission to an intensive care unit (OR 1.31 [95% CI: 1.09-1.59]), bronchospasm (OR 1.37 [95% CI: 1.22-1.55]), and hyperkalemia (OR 1.86 [95% CI: 1.08-3.20]) were associated with increased LOS. Neurologic conditions (OR 2.87 [95% CI: 1.76-4.67]) and respiratory comorbidities (OR 2.48 [CI: 1.43-4.30]) were associated with readmission. Average cost per admission was $5,158 ($3,259 to $8,560 range). Conclusion: There is an overall national decline in rate of admissions for IH propranolol therapy. Inpatient admission may be beneficial for patients with neurologic or respiratory conditions.

Multimodality treatment of vascular anomalies of the head and neck.

BACKGROUND: Vascular anomalies affect up to 5% of children with the majority affecting the head and neck. They present at different ages as a wide variety of lesions. A careful evaluation with history, physical examination, and imaging assists in the proper diagnosis. Depending upon the condition treatment options for vascular anomalies include topical therapy, selective photothermolysis, sclerotherapy, embolization, surgical excision, and targeted systemic therapy. CONCLUSION: Staged multimodal therapeutic regimens have proven to best control disease and allow for the preservation of function and aesthetics. The timing, sequence, and combination of therapies are best determined by a multidisciplinary vascular anomalies team. Patients and families need to be counseled on anticipated positive outcomes following a protracted course of treatment for the majority of vascular anomalies.

Pharmacokinetics of bleomycin sclerotherapy in patients with vascular malformations.

Bleomycin, a chemotherapy agent that inhibits synthesis of DNA, has been increasingly utilized in sclerotherapy for patients with vascular malformations. A serious long-term risk of intravenous bleomycin is dose-dependent interstitial pneumonitis. Little is known about absorption and circulating levels of bleomycin when used in sclerotherapy for patients with vascular malformations. This is an Institutional Review Board (IRB)-approved prospective study on patients receiving bleomycin sclerotherapy in the management of vascular malformations. Depending on the type of vascular malformation, bleomycin was administered either in the lumen or interstitial space of the involved lesion. A bleomycin assay measured serum bleomycin plasma concentrations versus time at seven intervals following treatment. Pharmacokinetic parameters were obtained for each participant and included peak plasma concentration (Cmax ), time to reach peak plasma concentration (Tmax ), volume of distribution (Vd ), elimination half-life (t1/2 ), the volume of plasma cleared of the drug per unit time (CL), and total systemic exposure area under the curve (AUC). Fifteen patients were enrolled (5: lymphatic, 4: venous, 6: arteriovenous malformations). Bleomycin was administered interstitially (IS) in 11 patients and intraluminal (IL) in four; median age of 13 years (range: 2-67). Pharmacokinetic analysis revealed terminal elimination half-life (t1/2λz ) of 88.51 (±23.09) and 111.61 (±37.75) minutes for the IS and IL groups, respectively. Vd was 4.86 L (±6.74) and 1.55 L (±0.54) for the IS and IL groups, respectively. AUC was 53.9 (±23.45) and 129.17 (±93.57) mg min/L for the IS and IL groups, respectively. There were no statistically significant differences in t1/2λz , Vd , or AUC parameters between groups. Bleomycin is absorbed systemically when used as a sclerosant for vascular malformations when injected either IS or IL.

Update on Pediatric Tracheostomy: Indications, Technique, Education, and Decannulation.

PURPOSE OF REVIEW: Tracheostomy in a child demands critical pre-operative evaluation, deliberate family education, competent surgical technique, and multidisciplinary post-operative care. The goals of pediatric tracheostomy are to establish a safe airway, optimize ventilation, and expedite discharge. Herein we provide an update regarding timing, surgical technique, complications, and decannulation, focusing on a longitudinal approach to pediatric tracheostomy care. RECENT FINDINGS: Pediatric tracheostomy is performed in approximately 0.2% of inpatient stays among tertiary pediatric hospitals. Mortality in children with tracheostomies ranges from 10-20% due to significant comorbidities in this population. Tracheostomy-specific mortality and complications are now rare. Recent global initiatives have aimed to optimize decision-making, lower surgical costs, reduce the length of intensive care, and eliminate perioperative wound complications. The safest road to tracheostomy decannulation in children remains to be both patient and provider dependent. SUMMARY: Recent literature provides guidance on safe, uncomplicated, and long-term tracheostomy care in children. Further research is needed to help standardize decannulation protocols.

Propranolol for Treatment of Infantile Hemangioma: Efficacy and Effect on Pediatric Growth and Development.

PURPOSE: Propranolol has been successful in treating problematic infantile hemangiomas (IH) but concerns regarding its effect on normal growth and development have been raised. This study examines physical growth, developmental milestones, and human growth hormone (hGH) levels in infants receiving propranolol for problematic IH. METHOD: Monthly heights and weights of children undergoing propranolol therapy for IH were prospectively collected and tabulated. Data analysis and comparison to World Health Organization (WHO) weight-for-age and weight-to-length z-scores was performed. Questionnaires regarding milestones, efficacy, and guardian satisfaction were performed, and a combination of both chart results and phone conducted surveys was tabulated. Serum from a small representative cohort of age-matched children with IH treated and not treated with propranolol was collected. RESULTS: A total of 185 children receiving propranolol therapy between 2008 and 2013 for IH were assigned to this study. The children were divided into two cohorts based on the presence of comorbidities or risk factors that may affect growth and development (n = 142 no comorbidities, n = 43 with comorbidities). Neither cohort demonstrated deviation from normal weight in comparison to WHO normative data. There was a significant deviation for BMI-for-age and weight-for-age z-scores in our population, especially in patients on propranolol for more than 7 months. Based on data from participants, via either completed questionnaires or chart results, most children met their developmental milestones at or before target ages, regardless of the presence of comorbidities. Eighty percent of guardians noticed clinical improvement of the IH, with 91% either happy about or neutral to using the medication. hGH levels were higher in patients receiving propranolol therapy, but not significantly different. CONCLUSION: Propranolol therapy is effective and well tolerated in the treatment of infantile hemangiomas. This study suggests that propranolol does not impair growth and has no impact on normal pediatric development.

Process improvement strategy to implement an outpatient surgery center efficiency model in an academic inpatient setting.

OBJECTIVES: By utilizing process improvement methodology, we aim to: 1) create an ambulatory surgical efficiency model (SEM) confined to an inpatient setting, and 2) reduce patient wait time and improve patient flow within the operating room. METHODS: A prospective cohort of all otolaryngology cases performed from May 2016 to October 2017 at a tertiary, academic, pediatric hospital. Intraoperative timestamps were collected to determine turnover times. Time to procedure was collected from clinic visit to the day of operation. RESULTS: A total of 5955 patients were enrolled. 3393 cases were performed prior to the implementation of SEM and 2562 after. Of the 2562 cases, 819 were deemed appropriate for the SEM. Prior to the SEM, the average number of working days between the clinic visit and operating room (WD) was 31.1 days (95% CI 30.7-31.4). After the SEM, the WD for non-SEM cases was 30.0 days (95% CI 29.7-30.2), and the WD for SEM cases was 14.4 days (95% CI 14.2-14.6). The average turnover time was significantly less for SEM cases at 11.4 min (95% CI 10.7-12.2) vs. non-SEM cases at 24.4 min (95% CI 23.9-25.0) (p < 0.0001). CONCLUSION: Process improvement methodology is effective in improving perioperative patient flow. This quality improvement project decreased the average time from diagnosis to surgical procedure, as well as decreased the average turnover time between cases. Patient flow can improve with a high-volume SEM within an inpatient hospital operating room setting. This strategy can be instrumental in improving patient care by providing increased access to the operating room.

Clinical Significance of Screening Electrocardiograms for the Administration of Propranolol for Problematic Infantile Hemangiomas.

OBJECTIVE: Low-dose nonselective ß blockade is an effective treatment for problematic infantile hemangioma (PIH). Screening electrocardiograms (ECG) are performed prior to the initiation of propranolol to minimize the risk of exacerbating undiagnosed heart block. How ECG results affect subsequent propranolol usage and patient management remains unclear. We examined the value of ECG prior to propranolol therapy in a quaternary pediatric hospital. METHODS: A retrospective chart review was performed on all infants who received propranolol (2 mg/kg/day divided three times daily) to treat PIH at Arkansas Children's Hospital from Sept. 2008 to Sept. 2015. All available demographic, historical, and clinical data were obtained. ECGs and echocardiographic data were reviewed and summarized. A pediatric cardiologist read all ECGs. RESULTS: A total of 333 patients (75% female) received propranolol therapy. ECG information was available for 317 (95%). Abnormal findings were present on 44/317 (13.9%) of study ECGs. The most common abnormal finding was "voltage criteria for ventricular hypertrophy" (n = 35, 76.1%). Two patients had abnormal rhythms; one had first-degree atrioventricular (AV) block, and one had occasional premature atrial contractions. Of the 31 patients who underwent echocardiograms, 20 (35%) were abnormal. 2.9% of infants with PIH treated with propranolol required a follow-up with a cardiologist. No patient was precluded from taking propranolol due to the findings on screening ECG. CONCLUSIONS: Screening ECGs prior to propranolol therapy are abnormal in nearly 14% of patients with PIH but are unlikely to preclude therapy. In the absence of prior cardiac history, this cohort offers further evidence suggesting that screening ECGs may be of limited value in determining the safety of propranolol in otherwise healthy infants with PIH.

Gentle ND:YAG Laser Therapy in the Treatment of Cutaneous Venous Malformations.

Background: Venous malformations (VMs) are congenital vascular malformations that grow progressively and never resolve on their own. Cutaneous VMs are difficult to treat due to risk of injury and deformation. The purpose of this study was to examine the safety and efficacy of a modified neodymium-doped yttrium aluminum garnet laser (Gentle YAG) in the management of cutaneous VMs. Methods: Retrospective chart review of patients undergoing Gentle YAG therapy for cutaneous VMs and a blind prospective evaluation of photographs, performed by 10 reviewers, before and after treatment for growth, stability, improvement, or resolution of VMs. Results: Forty-five patients (18 males and 27 females) who underwent Gentle YAG therapy for a cutaneous VM were identified. Based on photographic review, Gentle YAG therapy elicited improvement in the appearance of VMs in 72% of the patients, χ2 (1, N = 45) = 25.94, p < 0.0001, with reviewers noting complete resolution in 8.2%, significant improvement in 34.5%, some improvement in 29.3%, and no growth or improvement in 20.9% of patients. Growth of the VM was noted in 7.3% of patients. Three (6.7%) patients reported complications from the treatment, which included infection, bleeding, blister, and color change. Four patients (8.9%) reported pretreatment pain, which resolved in three (75.0%) after treatment. Conclusions: Gentle YAG therapy can provide safe and effective treatment for cutaneous VMs and should be considered in the multimodal management of VMs.

Detection of Respiratory Pathogens Does Not Predict Risks After Outpatient Adenotonsillectomy.

OBJECTIVES/HYPOTHESIS: To determine whether the presence of detectable upper respiratory infections (URIs) at the time of adenoidectomy/adenotonsillectomy is associated with increased morbidity, complications, and unexpected admissions. STUDY DESIGN: Prospective double-blinded cohort. METHODS: In this prospective cohort study, nasopharyngeal swabs were obtained intraoperatively from 164 pediatric patients undergoing outpatient adenoidectomy/tonsillectomy with or without pressure equalization tubes (PETs) and were analyzed with PCR for the presence of 22 known URIs, including SARS-CoV-2. Surgeons and families were blinded to the results. At the conclusion of the study, rates of detectable infection were determined and intraoperative and postoperative events (unexpected admissions, length of PACU stay, rates of laryngospasm/bronchospasm, oxygen desaturation, bradycardia, and postoperative presentation to an emergency department) were compared between infected and uninfected patients. RESULTS: Of the 164 patients (50% male, 50% female, ages 8 mo-18 y), 136 patients (82.9%) tested positive for one or more URI at the time of surgery. Forty one patients (25.0%) tested positive for three or more URIs concurrently, and 11 (6.7%) tested positive for five or more URIs concurrently. There were no significant differences in admission rates, length of PACU stay, rates of laryngospasm/bronchospasm, oxygen desaturation, bradycardia, or postoperative presentation to an emergency department between positive and negative patients. No patients tested positive for SARS-CoV-2. CONCLUSIONS: A recent positive URI test does not confer any additional intraoperative or postoperative risk in the setting of outpatient adenoidectomy/tonsillectomy in healthy patients. There is no utility in preoperative URI testing, and delaying surgery due to a recent positive URI test is not warranted in this population. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E2074-E2079, 2021.

Analyzing coagulation dynamics during treatment of vascular malformations with thromboelastography.

BACKGROUND/OBJECTIVES: Slow-flow vascular malformations are abnormal vessels that can lead to activation and consumption of coagulation factors and thrombosis, known as localized intravascular coagulopathy (LIC). Most clinical and research evidence of vascular malformation hemostasis relies on conventional coagulation studies, which may not provide a complete picture. Thromboelastograpy (TEG) is a tool that can provide real-time assessment of a patient's coagulation dynamics, and may allow for a more individualized treatment approach. We hypothesized that patients with slow-flow vascular malformations will have changes in TEG parameters peri-procedure that will help predict blood product or medication administration. DESIGN/METHODS: Institutional Review Board approved prospective study of patients with slow-flow vascular malformations undergoing a sedated, minor procedure. TEG and conventional coagulation studies were obtained preprocedure, 15 min, and when possible, at 30 min after the start of the procedure. RESULTS: Twenty-five patients were enrolled. Median age was 15 years (range 3-47 years). Procedures included laser and/or sclerotherapy. There were no changes in TEG parameters from baseline to 15 min or 30 min. The following decreased from baseline to 15 min: fibrinogen 313 to 287 mg/dL (P = .001), D-dimer 1.3 to 1.1 mg/L (P = .02), hemoglobin 12.8 to 11.8 g/dL (P = .001), and platelet count 272 000 to 256 000 (P = .006). No patient had a bleeding/thrombotic complication during or within 1 week postprocedure. CONCLUSION: We saw no change in TEG parameters or bleeding or clotting complications despite significant numerical changes in conventional coagulation profiles, suggesting that conventional studies may not be as useful in determining risks of bleeding or thrombotic complications peri-procedure for minor procedures.

Posterior glottic stenosis: management and outcomes.

PURPOSE OF REVIEW: Pediatric posterior glottic stenosis (PGS) is a challenging clinical entity with multiple treatment options. This review describes the evaluation of patients with PGS and discusses existing surgical techniques. RECENT FINDINGS: PGS secondary to a distinct scar band between the vocal folds can often be effectively managed with endoscopic division and surveillance. More advanced glottic scarring that also involves the interarytenoid mucosa, cricoarytenoid joints, or subglottis merits a more thorough investigation and repair. A postcricoid mucosal advancement flap can be employed in select adolescent or adult PGS, but long-term cricoarytenoid joint mobility is difficult to restore once it has been fixed. Younger pediatric patients have smaller airways and frequent concurrent subglottic stenosis which is better addressed with cartilage grafting. SUMMARY: Surgical success in pediatric PGS depends on careful preoperative airway assessment and the accurate characterization of airway stenosis. A surgical technique should be chosen based on the severity and extent of stenosis.

Update on extracranial arteriovenous malformations: A staged multidisciplinary approach.

Arteriovenous malformations (AVMs) are vascular lesions that are thought to arise from congenital errors during development of vessels resulting in abnormal connections between arteries and veins. Though most AVMs develop in the brain or spinal cord, they can occur anywhere in the body. These extracranial or peripheral AVMs have a predilection for the head, neck, and limbs. Since these malformations infiltrate normal soft tissue, management requires selective treatment with preservation of normal surrounding architecture. Therefore, they are best addressed through a staged multimodal and multidisciplinary approach, using a combination of different laser, interstitial, intravascular and surgical techniques to specifically target anomalous vessels. The goal of treatment is overall disease improvement and symptom control with interventions that do not result in outcomes worse than the disease itself. Recently, the discovery of somatic and germline mutations in peripheral AVMs have contributed to a better understanding of the pathophysiology, resulting in promising new pharmacologic treatments. Recent evidence suggests that adjuvant medical therapy can enhance and sustain interventional and/or surgical outcomes. Herein we describe how a new understanding of the etiology and physiology of extracranial AVM provides guidance to current treatment approaches.

Surgical resection of macrocystic lymphatic malformations of the head and neck: Short and long-term outcomes.

BACKGROUND: Controversy exists on management of lymphatic malformations, with schools of thought advocating for observation, surgery or sclerotherapy. This study sought to examine outcomes after surgical resection of pediatric cervicofacial macrocystic lymphatic malformations (MLM). METHODS: Case series with planned data collection on pediatric patients with cervicofacial MLM who underwent surgical resection at a tertiary referral center for vascular anomalies from January 1995 to June 2016. For consistency in patient population analysis, patients who had pre-surgical sclerotherapy or had mixed or microcystic disease were excluded. The main outcome was complete response rate (CR) and long-term recurrence-free survival (RFS). RESULTS: Sixty-three patients who underwent excision of MLM were included, 52.4% were female, 77.8% Caucasian. The majority had de Serres stage I-III (96.8%) affecting the neck (71.4%). Patients were discharged the same day (28.6%), or had a 1 day median length-of-stay (interquartile range (IQR) = 2). Surgical complications included seroma/hematoma (9.5%), transient nerve weakness (facial nerve, sympathetic chain, or phrenic nerve, 6.3%), and infection (1.6%). On long-term follow-up (median: 12 months, IQR 1-43 months), a single surgery achieved CR in 90.5% of patients. RFS was achieved in 86% of patients in our observation period of up to 15 years. Most patients requiring a second intervention failed within 6-months of initial procedure (4/5 patients, 90%); associated factors included bilaterality, advanced staging, and partial response at first-follow-up (p = 0.0051, 0.0051, and <0.0001, respectively). CONCLUSIONS: Surgery is safe and effective as first line treatment for selected MLM. For stage I-III MLM CR and long-term RFS can be achieved with a single surgery. A direct and randomized comparison of treatment modalities is needed.

Gastrostomy and Tracheostomy After Complete Repair of Tetralogy of Fallot in Children With 22q11.2 Deletion Syndrome.

OBJECTIVES: Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not been described. We sought to determine the degree to which 22q11.2 deletion is associated with postoperative gastrostomy and/or tracheostomy after repair of tetralogy of Fallot. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System. PATIENTS: Children undergoing complete repair of tetralogy of Fallot (ventricular septal defect closure and relief of right ventricular outflow tract obstruction) from 2003 to 2016. Patients were excluded if they had pulmonary atresia, other congenital heart defects, and/or genetic diagnoses other than 22q11.2 deletion. MEASUREMENTS AND MAIN RESULTS: Two groups were formed on the basis of 22q11.2 deletion status. Outcomes were postoperative tracheostomy and postoperative gastrostomy. Bivariate analysis and Kaplan-Meier analysis at 150 days postoperatively were performed. There were 4,800 patients, of which 317 (7%) had a code for 22q11.2 deletion. There were no significant differences between groups for age at surgery or sex. Patients with 22q11.2 deletion had significantly higher rates of gastrostomy (18% vs 5%; p < 0.001) and higher rates of tracheostomy (7% vs 1%; p < 0.001); there was no difference for mortality. Kaplan-Meier analyses also showed higher rates of gastrostomy (p = 0.024) and tracheostomy (p = 0.037). CONCLUSIONS: The present study establishes rates of postoperative gastrostomy and tracheostomy in children with 22q11.2 deletion after complete repair of tetralogy of Fallot. These data are useful to clinicians for providing families with preoperative counseling.