Karthik Balakrishnan

Lymphatic malformations: Diagnosis and management

Lymphatic malformations are benign vascular lesions that arise from embryological disturbances in the development of the lymphatic system. They encompass a wide spectrum of related abnormalities, including cystic lymphatic lesions, angiokeratoma, lymphatic malformations that occur in bones (Gorham-Stout Syndrome), lymphatic and chylous leak conditions, and lymphedema. This article will focus only on lymphatic malformation mass lesions, whereas other related disease entities will be covered elsewhere in this journal issue. Lymphatic malformations occur frequently in lymphatic-rich areas such as the head and neck region, but they can also be found on any anatomical site in the body. In general, lymphatic malformations are categorized into macrocystic, microcystic, or combined depending on the size of the cysts contained within the lesion. Lymphatic malformations can cause both deformation of the anatomical site involved and functional deficits. The goal of this article is to discuss the etiology, epidemiology, treatment modalities, and comorbidities associated with lymphatic malformations.

Functional and Symptom Impacts of Pediatric Head and Neck Lymphatic Malformations Developing a Patient-Derived Instrument

Objective Lymphatic malformations cause significant symptoms and functional deficits. Patients seek care for functional and symptomatic effects of their disease, but current disease burden and treatment outcome measures focus primarily on anatomy and histopathology. The authors describe disease impacts reported by patients and parents as a step toward more comprehensive disease burden assessments. Study Design Cross-sectional. Setting Children’s hospital vascular anomaly clinic. Subjects and Methods Participants were recruited through a pediatric vascular anomaly clinic. A panel of senior pediatric otolaryngologists and an outcomes scientist developed interview questions based on clinical and research experience and available literature. The outcomes scientist conducted parent and adolescent interviews. The panel reviewed responses to define relevant items within functional domains. Participants rated impact on daily life for each domain. Results Thirty-one participants represented all 5 de Serres stages (mean [SD] age, 9 [6] years; n = 11 adolescents and 20 parents). Adolescents reported frequent sickness as the domain with greatest impact. Sleep was more affected in adolescents with higher stage lesions. Parents of younger children reported greatest impact on breastfeeding. For adolescents, lesion stage predicted perceived social stigma (controlling for age), whereas increasing age was associated with greater impact from swelling (controlling for stage). For parents, stage predicted breastfeeding impact (controlling for stage). Conclusion This is the first detailed assessment of patient- and parent-reported functional and symptomatic impacts of head and neck lymphatic malformations. Both adolescent patients and parents of younger children reported significant symptom and functional effects of this disease.

Primary Surgery vs Primary Sclerotherapy for Head and Neck Lymphatic Malformations

IMPORTANCE The optimal treatment for head and neck lymphatic malformations (LMs) is unknown. To our knowledge, this is the first head-to-head comparison of primary surgery and sclerotherapy for this condition. OBJECTIVE To compare surgery and sclerotherapy as initial treatment for head and neck LMs. DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study including patients in 2 pediatric vascular anomaly programs receiving treatment for head and neck LMs. INTERVENTIONS Primary surgery or primary sclerotherapy and any subsequent therapy within 1 year. MAIN OUTCOMES AND MEASURES Treatment effectiveness was measured by (1) need for further therapy after first treatment and within 1 year and (2) change in Cologne Disease Score (CDS). Resource utilization was reflected by total intervention number, hospital and intensive care unit (ICU) days, and tracheostomy placement. RESULTS A total of 174 patients were studied. Their mean (SD) age at presentation was 4.2 (4.7) years; 45.1% were female. The initial treatment was surgery in 55.8%, sclerotherapy in 35.1%, and other interventions in 9.1%. The LM stage ranged from 1 to 5, with similar distributions (P = .15) across initial treatment types; 31.2% of LMs were macrocystic, 34.8% were microcystic, and 33.9% were mixed, with similar distributions across treatment types. Patients receiving sclerotherapy had worse pretreatment CDS subscores for respiration, nutrition, and speech (all P ≤ .02). In univariate analysis, initial surgery and initial sclerotherapy had similar effectiveness after the first intervention (P = .21) and at 1 year (P = .30). In multivariate analysis controlling for lesion stage and type, initial surgery and sclerotherapy did not differ in effectiveness after the first intervention (P = .28) or at 1 year (P = .97). Total CDS and subscale changes were similar between treatment types except for the nutrition subscale. Treatment type did not predict total number of interventions (P = .64), total hospital days (P = .34), total ICU days (P = .59), or higher likelihood of subsequent tracheostomy (P = .36). Higher LM stage predicted more hospital and ICU days and higher likelihood of tracheostomy (all P ≤ .02). CONCLUSIONS AND RELEVANCE In this multisite comparison, initial surgery and sclerotherapy for head and neck LMs were similar in effectiveness and resource utilization. Higher stage predicted greater resource utilization.

Applications and outcomes of orbital and transorbital endoscopic surgery.

Objective. To prospectively evaluate the safety, effectiveness, and utility of orbital and transorbital endoscopic surgery. Study Design. Case series with planned data collection. Setting. Level 1 trauma center and tertiary academic hospital. Subjects and Methods. Consecutive sample of 107 patients undergoing orbital or transorbital endoscopic operations. Main Outcome Measures. Ability to achieve intraoperative goals using endoscopic approach; occurrence of predetermined intraoperative or postoperative complications. Results. One hundred seven patients (aged 6-83 years) underwent orbital or transorbital endoscopic surgery for 6 different indications. Seven incisions were used. Endoscopic orbitotomies were made through all 4 orbital walls to access surrounding structures. Intraoperative goals were achieved endoscopically in 106 patients. Mean follow-up was 3 months (mean ± SD, 3.0 ± 3.5). No complication was directly related to surgical approach or use of endoscopy. Seventeen complications were detected in 2 categories: persistent diplopia and persistent vision change. No patient had vision loss. No nonfracture patient suffered a complication. Subgroup analysis demonstrated no difference in surgical success rates when compared with transnasal and transantral medial orbital wall and orbital floor repair and cerebrospinal fluid leak repair. Endoscopic visualization was advantageous in several respects: superior visualization and lighting, particularly posterior to the equator of the globe; image magnification; and video monitoring for education and operating room staff involvement. It also facilitated surgical navigation and computer-aided reconstruction. Conclusion. Orbital and transorbital endoscopy are versatile, effective, and safe approaches useful for addressing diverse urgent and elective problems. In appropriate clinical situations, these procedures may offer better access and visualization than open or transnasal approaches.

Standardized Outcome and Reporting Measures in Pediatric Head and Neck Lymphatic Malformations

Objective To develop general and site-specific treatment effect and outcome measures to standardize the reporting of head and neck lymphatic malformation (HNLM) treatments. Study Design Consensus statement/expert opinion. Setting Multiple tertiary academic institutions. Subjects and Methods The modified Delphi method is an iterative process of collecting expert opinions, refining opinions through discussion and feedback, statistically aggregating opinions, and using these aggregates to generate consensus opinion in the absence of other data. The modified Delphi method was used by a multi-institutional group of otolaryngology and interventional radiology experts in the field of vascular anomalies to formulate a list of recommended reporting outcomes for the study and treatment of head and neck lymphatic malformations. Results Through 3 rounds of iteration, 10 expert panelists refined 98 proposed outcome measures and 9 outcome categories to a final consensus set of 50 recommended outcome measures in 3 global categories (general, demographics, and treatment complications) and 5 site-specific categories (orbit, oral cavity, pharynx, larynx, and neck). Conclusions We propose the first consensus set of standardized reporting measures for clinical and treatment outcomes in studies of HNLMs. Consistent outcome measures across future studies will facilitate comparison of treatment options and allow systematic review. We hope that these guidelines facilitate the design and reporting of subsequent HNLM studies.

Applying Cost Accounting to Operating Room Staffing in Otolaryngology Time-Driven Activity-Based Costing and Outpatient Adenotonsillectomy

Objectives (1) To describe the application of a detailed cost-accounting method (time-driven activity-cased costing) to operating room personnel costs, avoiding the proxy use of hospital and provider charges. (2) To model potential cost efficiencies using different staffing models with the case study of outpatient adenotonsillectomy. Study Design Prospective cost analysis case study. Setting Tertiary pediatric hospital. Subject and Methods All otolaryngology providers and otolaryngology operating room staff at our institution. Results Time-driven activity-based costing demonstrated precise per-case and per-minute calculation of personnel costs. We identified several areas of unused personnel capacity in a basic staffing model. Per-case personnel costs decreased by 23.2% by allowing a surgeon to run 2 operating rooms, despite doubling all other staff. Further cost reductions up to a total of 26.4% were predicted with additional staffing rearrangements. Conclusion Time-driven activity-based costing allows detailed understanding of not only personnel costs but also how personnel time is used. This in turn allows testing of alternative staffing models to decrease unused personnel capacity and increase efficiency.

Management of head and neck lymphatic malformations.

Lymphatic malformations are congenital vascular malformations most commonly found in the head and neck and typically presenting early in life. Although this disease entity has been recognized for more than a century, there is currently no standardization of disease staging or classification, of treatment paradigms, or of outcome measures. A wide variety of treatment modalities have been proposed. This article reviews diagnostic, evaluation, and treatment strategies for head and neck lymphatic malformations. It also provides the reader specific information to improve treatment outcomes in patients with these lesions. Finally, it advocates for standardization of LM assessment and treatment.

The Lymphatic Malformation Function (LMF) Instrument

Objective The Lymphatic Malformation Function (LMF) instrument is a preliminary parent-report assessment designed to measure outcomes in children with cervicofacial lymphatic malformation (LM). This study aimed to assess the measurement properties of the LMF, refine it, test criterion validity, and evaluate the test-retest reliability. Study Design Cross-sectional. Setting Two pediatric tertiary referral centers. Subjects Parents of 60 children from 6 months to 15 years old with cervicofacial LM. Methods Parents were recruited via mail and online. The LMF was administered on paper or online initially and again within 21 days. Response distributions and interitem correlations were examined for item reduction. Exploratory factor analysis was conducted on retained items. Cronbach’s α, Spearman correlation, and intraclass correlation (ICC) coefficients were calculated to test internal consistency, criterion validity (compared to stage), and test-retest reliability, respectively. Results One item was removed due to a floor effect. The response scale was collapsed from a 5-point scale to a 3-point scale due to skewness. Six items were discarded due to redundancy (interitem correlations >0.7); 2 items were discarded due to factor loadings <0.4. Exploratory factor analysis revealed a 2-factor structure explaining 84% of variance, and the domains Signs and Impacts had good internal consistency (all Cronbach’s α >0.80 and <0.90), significant association with stage (P < .05), and good overall test-retest reliability (ICC, 0.82). Conclusion The LMF has been refined into a 12-item, 2-domain instrument measuring LM-specific signs and impacts with internal consistency, criterion validity, and test-retest reliability.

Endoscopic anterior–posterior cricoid split for pediatric bilateral vocal fold paralysis

Children with bilateral true vocal fold immobility (BTVFI) may present with significant airway distress necessitating tracheostomy. The objective of this study was to review our preliminary experience with the anterior–posterior cricoid split (APCS), an endoscopic intervention used as an alternative to tracheostomy in children with BTVFI.

Structure and functions of pediatric aerodigestive programs: A consensus statement

We present a multi-disciplinary consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.