Pediatric hyperparathyroidism: review and imaging update.

Hyperparathyroidism, due to increased secretion of parathyroid hormones, may be primary, secondary or tertiary. Most pediatric patients with sporadic primary hyperparathyroidism will be symptomatic, presenting with either end-organ damage or nonspecific symptoms. In younger patients with primary hyperparathyroidism, there is a higher prevalence of familial hyperparathyroidism including germline inactivating mutations of the calcium-sensing receptor genes that result in either neonatal severe hyperparathyroidism or familial hypocalciuric hypercalcemia. Parathyroid scintigraphy and ultrasound are complementary, first-line imaging modalities for localizing hyperfunctioning parathyroid glands. Second-line imaging modalities are multiphase computed tomography (CT) and magnetic resonance imaging. In pediatrics, multiphase CT protocols should be adjusted to optimize radiation dose. Although, the role of these imaging modalities is better established in preoperative localization of hyperfunctioning parathyroid glands in primary hyperparathyroidism, the same principles apply in secondary and tertiary hyperparathyroidism. In this manuscript, we will review the embryology, anatomy, pathophysiology and preoperative localization of parathyroid glands as well as several subtypes of primary familial hyperparathyroidism. While most of the recent imaging literature centers on adults, we will focus on the issues that are pertinent and applicable to pediatrics.

Preoperative Imaging and Surgical Findings in Pediatric Frontonasal Dermoids.

OBJECTIVE: To review cases of congenital frontonasal dermoids to gain insight into the accuracy of preoperative computed tomography (CT) and magnetic resonance imaging (MRI) in predicting intracranial extension. METHODS: This retrospective study included all patients who underwent primary excision of frontonasal dermoids at an academic children's hospital over a 23-year period. Preoperative presentation, imaging, and operative findings were reviewed. Receiver operating characteristic (ROC) statistics were generated to determine CT and MRI accuracy in detecting intracranial extension. RESULTS: Search queries yielded 129 patients who underwent surgical removal of frontonasal dermoids over the study period with an average age of presentation of 12 months. Preoperative imaging was performed on 122 patients, with 19 patients receiving both CT and MRI. CT and MRI were concordant in the prediction of intracranial extension in 18 out of 19 patients. Intraoperatively, intracranial extension requiring craniotomy was seen in 11 patients (8.5%). CT was 87.5% sensitive and 97.4% specific for predicting intracranial extension with an ROC of 0.925 (95% CI [0.801, 1]), whereas MRI was 60.0% sensitive and 97.8% specific with an ROC of 0.789 (95% CI [0.627, 0.950]). CONCLUSION: This is the largest case series in the literature describing a single institution's experience with frontonasal dermoids. Intracranial extension is rare and few patients required craniotomy in our series. CT and MRI have comparable accuracy at detecting intracranial extension. Single-modality imaging is recommended preoperatively in the absence of other clinical indications. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1961-1966, 2024.

Delaying Invasive Treatment in Unilateral Head and Neck Lymphatic Malformation Improves Outcomes.

OBJECTIVES: Large (De Serres stage [IV-V]) head and neck lymphatic malformations (HNLMs) often have multiple, high-risk, invasive treatments (ITs) to address functional compromise. Logically reducing HNLM ITs should reduce treatment risk. We tested whether delaying HNLM ITs reduces total IT number. MATERIALS: Consecutive HNLM patients (n = 199) between 2010 and 2017, aged 0-18 years. METHODS: ITs (surgery or sclerotherapy) were offered for persistent or dysfunction causing HNLMs. Treatment effectiveness categorized by IT number: optimal (0-1), acceptable (2-5), or suboptimal (>5). Clinical data were summarized, and outcome associations tested (χ2 ). Relative risk (RR) with a Poisson working model tested whether HNLM observation or IT delay (>6 months post-diagnosis) predicts treatment success (i.e., ≤1 IT). RESULTS: Median age at HNLM diagnosis was 1.3 months (interquartile range [IQR] 0-45 m) with 107/199(54%) male. HNLM were stage I-III (174 [88%]), IV-V (25 [13%]). Initial treatment was observation (70 [35%]), invasive (129 [65%]). Treatment outcomes were optimal (137 [69%]), acceptable (36 [18%]), and suboptimal (26 [13%]). Suboptimal outcome associations: EXIT procedure, stage IV-V, oral location, and tracheotomy (p < 0.001). Stage I-III HNLMs were initially observed compared with stage I-III having ITs within 6 months of HNLM diagnosis, had a 82% lower relative treatment failure risk ([i.e., >1 IT], RR = 0.09, 95% CI 0.02-0.36, p < 0.001). Stage I-III HNLMs with non-delayed ITs had reduced treatment failure risk compared with IV-V (RR = 0.47, 95% CI 0.33-0.66, p < 0.001). CONCLUSION: Observation and delayed IT in stage I-III HNLM ("Grade 1") is safe and reduces IT (i.e., ≤1 IT). Stage IV-V HNLMs ("Grade 2") with early IT have a greater risk of multiple ITs. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:956-962, 2023.

Clinical Outcomes of Diffuse Sclerosing Variant Papillary Thyroid Carcinoma in Pediatric Patients.

OBJECTIVES/HYPOTHESIS: The diffuse sclerosing variant of papillary thyroid carcinoma (DSV) may be more aggressive than conventional well-differentiated non-DSV related papillary thyroid carcinomas (N-PTC). STUDY DESIGN: Retrospective chart review. METHODS: Retrospective review of clinical outcomes for patients 21 years of age or younger who underwent initial surgery for PTC at a single institution from January 1, 2005 to April 1, 2020. Genomic analysis was performed using targeted next-generation sequencing. Data were analyzed using Fischer's exact test and Kaplan-Meier curve log-rank test. RESULTS: Our cohort consisted of 72 patients, nine with DSV and 63 with N-PTC. Age at diagnosis was comparable (15.4 vs. 16.2 years, respectively, P = .46). DSV were more likely to be in the high-risk American Thyroid Academy pediatric risk group (100% vs. 41.3%, P = .004), to present with regional cervical lymph node metastases (100% vs. 60.3%, P = .036), and to present with distant metastases (67% vs. 22%, P = .005). No mortality seen in either group over 27.5 (interquartile range 14.8, 46.00) months average follow-up. Throughout the follow-up period, DSV were more likely to experience progression than N-PTC (hazard ratio = 5.7 [95% confidence interval 1.7-20.0; P = .0056]). In a subset of 19 patients with aggressive disease who had molecular testing as part of clinical care we detected RET fusions in nearly all DSV compared to a minority of N-PTC (83% vs. 15.4%, P = .0095). CONCLUSIONS: Pediatric patients with DSV have more advanced disease at diagnosis and are more likely to experience progression of disease compared to patients with N-PTC. The prevalence of RET fusions in our cohort recapitulates the frequency of this alteration described in prior studies. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1132-1138, 2022.

Somatic activating BRAF variants cause isolated lymphatic malformations.

Somatic activating variants in PIK3CA, the gene that encodes the p110α catalytic subunit of phosphatidylinositol 3-kinase (PI3K), have been previously detected in ∼80% of lymphatic malformations (LMs).1 , 2 We report the presence of somatic activating variants in BRAF in individuals with LMs that do not possess pathogenic PIK3CA variants. The BRAF substitution p.Val600Glu (c.1799T>A), one of the most common driver mutations in cancer, was detected in multiple individuals with LMs. Histology revealed abnormal lymphatic channels with immunopositivity for BRAFV600E in endothelial cells that was otherwise indistinguishable from PIK3CA-positive LM. The finding that BRAF variants contribute to low-flow LMs increases the complexity of prior models associating low-flow vascular malformations (LM and venous malformations) with mutations in the PI3K-AKT-MTOR and high-flow vascular malformations (arteriovenous malformations) with mutations in the RAS-mitogen-activated protein kinase (MAPK) pathway.3 In addition, this work highlights the importance of genetic diagnosis prior to initiating medical therapy as more studies examine therapeutics for individuals with vascular malformations.

Outcomes in Pediatric Thyroidectomy: Results From a Multinational, Multi-institutional Database.

OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.

Diagnosis and treatment of primary immunodeficiency disease: the role of the otolaryngologist.

OBJECTIVE: The objective of the study was to review the diagnosis and treatment of primary immunodeficiency disease (PID) and the role of otolaryngologists in the management of PID. METHODS: A search was conducted of PubMed and the Web sites of organizations for PID patients for literature pertaining to the diagnosis and treatment of PID, with an emphasis on the role of otolaryngologists. The reference lists of selected articles were reviewed for additional articles. RESULTS: Patients with PID commonly present with respiratory tract infections (eg, recurrent ear, nose, or throat infections) and chest disease. Diagnostic delays or inadequate treatment of PID may lead to significant morbidity and premature mortality. Immunoglobulin (Ig) replacement is the cornerstone of therapy for most patients with PID. Although intravenous Ig is the most popular route of administration in the United States, subcutaneous Ig administration may be appropriate for patients with poor venous access, those who are unable to tolerate intravenous Ig, or those who prefer the independence and flexibility of self-administration. CONCLUSIONS: Recognition and diagnosis of PID by otolaryngologists are critical to optimizing patient outcomes. Several therapeutic regimens for Ig replacement are now available that offer patients increased flexibility and independence.

Impact of Eliminating Local Anesthesia on Immediate Postoperative Analgesia in Pediatric Ambulatory Adenotonsillectomy.

Our goal was to standardize intraoperative analgesic regimens for pediatric ambulatory tonsillectomy by eliminating local anesthetic use and to determine its impact on postoperative pain measures, while controlling for other factors. METHODS: We assembled a quality improvement team at an ambulatory surgery center. They introduced a standardized anesthetic protocol, involving American Society of Anesthesiologists Classification 1 and 2 patients undergoing adenotonsillectomy. Local anesthesia elimination was the project's single intervention. We collected pre-intervention data (79 cases) from July 5 to September 17, 2019 and post-intervention data (59 cases) from September 25 to December 17, 2019. The intervention requested that surgeons eliminate the use of local anesthetics. The following outcomes measures were evaluated using statistical process control charts and Shewhart's theory of variation: (1) maximum pain score in the post-anesthesia care unit, (2) total post-anesthesia care unit minutes, and (3) postoperative opioid rescue rate. RESULTS: No special cause variation signal was detected in any of the measures following the intervention. CONCLUSIONS: Our data suggest that eliminating intraoperative local anesthetic use does not worsen postoperative pain control at our facility. The intervention eliminated the added expenses and possible risks associated with local anesthetic use. This series is unique in its standardization of anesthetic regimen in a high-volume ambulatory surgery center with the exception of local anesthesia practices. The study results may impact the standardized clinical protocol for pediatric ambulatory adenotonsillectomy at our institution and may hold relevance for other centers.

Active Observation as an Alternative to Invasive Treatments for Pediatric Head and Neck Lymphatic Malformations.

OBJECTIVES: An increasing number of treatment modalities for lymphatic malformations are being described, complicating therapeutic decisions. Understanding lymphatic malformation natural history is essential. We describe management of head and neck lymphatic malformations where decisions primarily addressed lesion-induced functional compromise (ie, breathing, swallowing) to identify factors associated with invasive treatment and active observation. We hypothesize that non-function threatening malformations can be observed. STUDY DESIGN: Retrospective case series. METHODS: Retrospective case series of consecutive head and neck lymphatic malformation patients (2000-2017) with over 2 years of follow-up. Patient characteristics were summarized and associations with invasive treatment (surgery or sclerotherapy) tested using Fisher's exact. In observed patients, factors associated with spontaneous regression were assessed with Fisher's exact test. RESULTS: Of 191 patients, 101 (53%) were male, 97 (51%) Caucasian, and 98 (51.3%) younger than 3 months. Malformations were de Serres I-III 167 (87%), or IV-V 24 (12%), and commonly located in the neck (101, 53%), or oral cavity (36, 19%). Initial treatments included observation (65, 34%) or invasive treatments such as primary surgery (80, 42%), staged surgery (25, 13%), or primary sclerotherapy (9, 5%). Of 65 initially observed malformations, 8 (12%) subsequently had invasive treatment, 36 (58%) had spontaneous regression, and 21 (32%) elected for no invasive therapy. Spontaneous regression was associated with location in the lateral neck (P = .003) and macrocystic malformations (P = .017). CONCLUSION: Head and neck lymphatic malformation treatment selection can be individualized after stratifying by stage, presence of functional compromise, and consideration of natural history. Recognizing the spectrum of severity is essential in evaluating efficacy of emerging treatments, as selected malformations may respond to observation. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1392-1397, 2021.

Targeted Oncogene Therapy Before Surgery in Pediatric Patients With Advanced Invasive Thyroid Cancer at Initial Presentation: Is It Time for a Paradigm Shift?

Importance: Initial data suggest the effectiveness of oncogene-specific targeted therapies in inducing tumor regression of diverse cancers in children and adults, with minimal adverse effects. Observations: In this review, preliminary data suggest that systemic therapy may be effective in inducing tumor regression in pediatric patients with unresectable invasive thyroid cancer. Although most pediatric patients with thyroid cancer initially present with operable disease, some children have extensive disease that poses substantial surgical challenges and exposes them to higher than usual risk of operative complications. Extensive disease includes thyroid cancer that invades the trachea or esophagus or encases vascular or neural structures. Previous efforts to manage extensive thyroid cancer focused on surgery with near-curative intent. With the recent development of oncogene-specific targeted therapies that are effective in inducing tumor regression, with minimal drug-associated adverse effects, there is an opportunity to consider incorporating these agents as neoadjuvant therapy. In patients with morbidly invasive regional metastasis or with hypoxia associated with extensive pulmonary metastasis, neoadjuvant therapy can be incorporated to induce tumor regression before surgery and radioactive iodine therapy. For patients with widely invasive medullary thyroid cancer, in whom the risk of surgical complications is high and the likelihood of surgical remission is low, these agents may replace surgery depending on the response to therapy and long-term tolerance. Conclusions and Relevance: With oncogene-specific targeted therapy that is associated with substantial tumor regression and low risk of adverse reactions, there appears to be an opportunity to include children with advanced invasive thyroid cancer in clinical trials exploring neoadjuvant targeted oncogene therapy before or instead of surgery.

Pediatric Otolaryngology Divisional and Institutional Preparatory Response at Seattle Children's Hospital after COVID-19 Regional Exposure.

Coronavirus disease 2019 (COVID-19) is a novel coronavirus resulting in high mortality in the adult population but low mortality in the pediatric population. The role children and adolescents play in COVID-19 transmission is unclear, and it is possible that healthy pediatric patients serve as a reservoir for the virus. This article serves as a summary of a single pediatric institution's response to COVID-19 with the goal of protecting both patients and health care providers while providing ongoing care to critically ill patients who require urgent interventions. A significant limitation of this commentary is that it reflects a single institution's joint effort at a moment in time but does not take into consideration future circumstances that could change practice patterns. We still hope dissemination of our overall response at this moment, approximately 8 weeks after our region's first adult case, may benefit other pediatric institutions preparing for COVID-19.

PHACES syndrome: otolaryngic considerations in recognition and management.

OBJECTIVES: To describe the otolaryngic manifestations of PHACES and evaluate current diagnostic and management principles for these patients. METHODS: A retrospective review was performed within a tertiary children's hospital. Children with segmental facial hemangiomas of infancy and one extracutaneous manifestation comprising PHACES (posterior fossa malformation, arteriovenous malformations, cardiac/aortic defects, eye anomalies, and sternal defect) were identified. Otolaryngic problems were evaluated with physical examination, audiogram, swallow evaluation, polysomnography, and laryngoscopy. Extracutaneous manifestations were diagnosed using radiology, echocardiogram, and EEG. Treatment for cutaneous and airway hemangiomas included oral and intralesional steroids, CO(2) or pulse-dye laser, tracheotomy, and surgical excision. Management of extracutaneous problems was system-dependent. RESULTS: Of 246 children with segmental facial hemangiomas of infancy evaluated since January 2000, 5 girls (2.0%) met diagnostic criteria for PHACES. Mean age at last follow-up was 2.6 years (range 0.4-5.8). Each child had one extracutaneous manifestation of aortic anomaly (2/5), sternal clefting (2/5), and brain malformation (1/5). Otolaryngic abnormalities included middle ear atelectasis (1/5), tympanic membrane hemangiomas with conductive hearing loss (3/5), skin and cartilage ulceration (2/5), dysphagia (4/5), and airway hemangiomas with stridor (3/5). Three children received oral steroids and required pulse-dye laser for cutaneous hemangiomas of infancy. One child underwent tracheotomy. Additional interventions included tympanostomy tubes and resection of nasal hemangioma. CONCLUSIONS: Diagnosis of PHACES requires awareness of the association of facial hemangiomas of infancy with systemic and airway problems. Otolaryngology-related manifestations of PHACES are not commonly described, and management should be tailored to the individual patient.

Clinical and radiographic findings in children with spontaneous lymphatic malformation regression.

OBJECTIVE: Evaluate clinical and radiographic characteristics of spontaneously regressing lymphatic malformations ("lesions"). SUBJECTS AND METHODS: Retrospective review of 104 consecutive patients with cervicofacial lesions, with 1-year follow-up. DATA COLLECTED: patient's age; lesion stage, location, radiographic characteristics; treatment. Data analysis using descriptive and Fischer exact tests. RESULTS: Spontaneously regressing lesions were identified in 13 of 104 (12.5%) patients. Five of 13 had in utero lesions, which persisted at birth; presenting age in the remaining eight patients was 2 to 138 months. Lesions regressed within 2 to 7 months. Lesion stage: I (7 of 13), II (2 of 13), III (4 of 13). Lesion location: left neck (9 of 13), right neck (4 of 13), posterior neck (10 of 13). All 13 resolving lesions were macrocystic with fewer than five septations in 11 of 13. Comparison of a resolving lesion cohort with a nonresolving lesion cohort demonstrated that disappearing lesions are more likely to have fewer than five septae and to be macrocystic (P < 0.05). Treatment was none in seven of 13, antibiotics in four of 13, and redundant skin excision in two of 13. CONCLUSION: Spontaneous lesion regression can occur, and these lesions have distinct features. Lesions with these characteristics can be observed.

Facial nerve anatomy, dissection and preservation in lymphatic malformation management.

OBJECTIVE: To describe facial nerve anatomy and surgical techniques used for safe lymphatic malformation resection of malformation involving the facial nerve. METHODS DESIGN: retrospective case series. SETTING: tertiary pediatric hospital. SUBJECTS: record review of lymphatic malformation patients after facial nerve dissection, from 1996 to 2005. Data collected included: facial nerve function, relationship of lymphatic malformation to facial nerve, facial nerve anatomy, dissection extent and clinical outcome. RESULTS: Sixteen patients who met inclusion criteria underwent a total of 21 facial nerve dissections. Mean age at dissection was 48 months (range 1-72 months). Mean follow-up was 38 months (range 8-144 months). Pre-operative lymphatic malformation stage by patient: II=7/16, III=4/16, IV=2/16 and V=3/16. Higher stage lymphatic malformations required more extensive dissections (p=0.026). Pre-operative facial nerve function was House-Brackmann grade (HBG)-1 in 20, and HBG-6 in 1. Eight months postoperatively, facial nerve function was HBG-1 in 18, HBG-2 in 1, and HBG-6 in 2. The facial nerve was surrounded by lymphatic malformation in 10/21, deep to the lymphatic malformation in 5/21, superficial to the lymphatic malformation in 4/21, and not identified in 2/21. Imaging studies predicted facial nerve position in 15/21 procedures. Antegrade nerve dissection was performed in 10/21, retrograde in 7/21 and not done in 2/21. Abnormally elongated facial nerve was identified in 11/21 cases and required more extensive dissection (p=0.040). Facial nerve monitoring was used in 15/21 dissections. Clinical outcomes were felt to be good in 19/21 dissections. CONCLUSIONS: In lymphatic malformation surgery, the facial nerve is often abnormally elongated and encompassed by malformation. Pre-operative imaging, facial nerve identification and dissection allow excellent postoperative facial nerve function.

Preoperative Facial Nerve Mapping to Plan and Guide Pediatric Facial Vascular Anomaly Resection.

Importance: Facial vascular anomalies are surgical challenges due to their vascularity and facial nerve distortion. To assist facial vascular anomaly surgical treatment, presurgical percutaneous facial nerve stimulation and recording of compound motor action potentials can be used to map the facial nerve branches. During surgery, the nerve map and continuous intraoperative motor end plate potential monitoring can be used to reduce nerve injury. Objective: To investigate if preoperative facial nerve mapping (FNM) is associated with intraoperative facial nerve injury risk and safe surgical approach options compared with standard nerve integrity monitoring (NIM). Design, Setting, and Participants: This investigation was a historically controlled study at a tertiary vascular anomaly center in Seattle, Washington. Participants were 92 pediatric patients with facial vascular anomalies undergoing definitive anomaly surgery (from January 1, 1999, through January 1, 2015), with 2 years' follow-up. In retrospective review, a consecutive FNM patient cohort after 2005 (FNM group) was compared with a consecutive historical cohort (1999-2005) (NIM group). Main Outcomes and Measures: Postoperative facial nerve function and selected surgical approach. For NIM and FNM comparisons, statistical analysis calculated odds ratios of nerve injury and operative approach, and time-to-event methods analyzed operative time. Results: The NIM group had 31 patients (median age, 3.3 years [interquartile range, 2.2-11.4 years]; 20 [65%] male), and the FNM group had 61 patients (median age, 4.4 years [interquartile range, 1.5-11.0 years]; 26 [43%] male). In both groups, lymphatic malformation resection was most common (19 of 31 [61%] in the NIM group and 32 of 61 [52%] in the FNM group), and the median anomaly volumes were similar (52.4 mL; interquartile range, 12.8-183.3 mL in the NIM group and 65.4 mL; interquartile range, 18.8-180.2 mL in the FNM group). Weakness in the facial nerve branches at 2 years after surgery was more common in the NIM group (6 of 31 [19%]) compared with the FNM group (1 of 61 [2%]) (percentage difference, 17%; 95% CI, 3%-32%). Anterograde facial nerve dissection was used more in the NIM group (27 of 31 [87%]) compared with the FNM group (28 of 61 [46%]) (percentage difference, 41%; 95% CI, 24%-58%). Treatment with retrograde dissection without identification of the main trunk of the facial nerve was performed in 21 of 61 (34%) in the FNM group compared with 0 of 31 (0%) in the NIM group. Operative time was significantly shorter in the FNM group, and patients in the FNM group were more likely to complete surgery sooner (adjusted hazard ratio, 5.36; 95% CI, 2.00-14.36). Conclusions and Relevance: Facial nerve mapping before facial vascular anomaly surgery was associated with less intraoperative facial nerve injury and shorter operative time. Mapping enabled direct identification of individual intralesional and perilesional nerve branches, reducing the need for traditional anterograde facial nerve dissection, and allowed for safe removal of some lesions after partial nerve dissection through transoral or direct excision.

Clinical outcomes in lymphocytopenic lymphatic malformation patients.

BACKGROUND: To determine if lymphocytopenia in patients with lymphatic malformation (LM) is associated with rates of infection and poor clinical outcomes. MATERIALS AND METHODS: This is a retrospective case series at a tertiary pediatric hospital, of 21 consecutive patients (11 male and 10 female) undergoing LM treatment. Clinical data (i.e., age, clinical LM stage, presence of tissue hypertrophy, frequency/type of medical therapy, and number of hospitalizations) obtained from LM patients with lymphocytopenia (n = 6) was compared to LM patients without lymphocytopenia (n = 15). RESULTS: The average age at the time of detailed leukocyte analysis was 67 months (Range 1-231). Six patients with lymphocytopenia (below 1500/cm(3)) were compared with 15 without lymphocytopenia (above 1500/cm(3)). All six patients with lymphocytopenia had large bilateral LM and normal neutrophil and platelet counts. The total number of hospital admissions was two times greater in lymphocytopenic patients (mean 8.3) compared to nonlymphocytopenic patients (mean 4.09) Chi square analysis revealed a statistical difference in lymphocytopenic patients. They were more likely to have had central line placement, central line infection, bacteremia, prophylactic antibiotics, admission at birth, infections distant from the lymphatic malformation and a treatment complication compared to nonlymphocytopenic patients. Univariate logistic regression revealed that, independent of LM stage, the use of prophylactic antibiotics, the need for a central line, the occurrence of a line infection, and the hospital admission rate were significantly increased in lymphocytopenic patients. CONCLUSION: Patients with LM-associated lymphocytopenia have increased hospitalization requirements, rate of infection, and receive more intensive antibiotic therapy compared to nonlymphocytopenic LM patients.

Lymphocytopenia in children with lymphatic malformation.

OBJECTIVE: To determine whether an immunologic abnormality exists in patients with lymphatic malformation (LM). DESIGN: Retrospective case series. SETTING: Tertiary care pediatric hospital. PATIENTS: Twenty-one consecutive patients (11 male and 10 female) undergoing LM treatment. INTERVENTIONS: Clinical data (ie, age, clinical LM stage, radiographic appearance, and histologic findings) were correlated with complete blood cell count and detailed lymphocyte differential. Complete blood cell counts and lymphocyte subsets were measured in 21 and 18 patients, respectively. RESULTS: The average age at the time of testing was 67 months (range, 1-231 months). The patients were categorized according to LM stage, including 4 (19%) with stage 1, 4 (19%) with stage 2, 4 (19%) with stage 3, 7 (33%) with stage 4, and 2 (10%) with stage 5 disease. Radiographic LM appearance was macrocystic in 6 patients (29%), mixed macrocystic and microcystic in 8 (38%), and microcystic in 7 (33%). Complete blood cell count data demonstrated lymphocytopenia in 6 patients (29%). The results of the lymphocyte subset tests showed concomitant T-, B-, and natural killer (NK)-cell deficiency in 6 (33%) of 18 patients. All 6 patients with T-cell lymphocytopenia had normal neutrophil and platelet counts. Spearman rank and chi(2) analyses showed that LM stage 4 or 5 and microcystic LM were significantly associated with lymphocytopenia (P = .002 and P = .008, respectively). Histologic analysis did not demonstrate increased lymphocytes in any LM specimens. CONCLUSION: We found T, B, and NK lymphocytopenia in patients with large bilateral or microcystic LM. Although the relationship between lymphocytopenia and infection was not addressed in this study, the recognition of lymphocytopenia in patients with LM may have important clinical and prognostic implications.

Recurrent thyroglossal duct cysts: a 23-year experience and a new method for management.

OBJECTIVES: We present an experience in the management of primary and recurrent thyroglossal duct cysts (TGDCs) and describe a novel method for recurrent TGDC removal. METHODS: We performed a retrospective review of TGDC surgery at Children's Hospital in Seattle from 1980 to 2003. The surgical techniques for primary and recurrent TGDCs and the factors associated with TGDC recurrence were evaluated and analyzed. RESULTS: During the study period, 231 patients underwent 296 TGDC surgeries. Thirty-four of the 231 patients (15%) underwent a total of 88 procedures for recurrent TGDCs. Successful procedures used for secondary TGDC management included central neck dissection with directed base of tongue (BOT) excision in 6 of 9 patients (67%), secondary Sistrunk operation with limited BOT resection in 12 of 27 patients (44%), revision Sistrunk operation with BOT dissection in 7 of 11 patients (64%), and suture-guided transhyoid pharyngotomy in 8 of 8 patients (100%). Ten of the 231 patients (4%) had initial TGDC incision and drainage and then underwent a total of 21 procedures, excluding the incision and drainage. The factors associated with TGDC recurrence were inaccurate initial diagnosis (17 of 34 or 50%), infection (5 of 34 or 15%), unusual TGDC presentation (5 of 34 or 15%), and lack of BOT musculature removal (7 of 34 or 20%). The level of surgeon training affected the surgical outcome. CONCLUSIONS: Successful TGDC treatment requires consideration of factors associated with recurrence. Recurrent TGDCs can be treated by several methods, including suture-guided transhyoid pharyngotomy.

Head and neck endocrine surgery in children: 1997 and 2000.

OBJECTIVES: To characterize children undergoing parathyroid, thyroid, and thyroglossal duct cyst surgery in 1997 and 2000 using a nationally representative discharge database to determine whether rates and outcomes of these surgical treatments vary by age, sex, and health care system attributes. DATA SOURCE: The 1997 and 2000 Kids' Inpatient Database, available through the Agency for Healthcare Research and Quality. STUDY SELECTION: All patients 18 years and younger undergoing head and neck endocrine (HNE) procedures were included. DATA EXTRACTION: The sampling scheme of this database allowed for calculation of national and regional estimates using Stata 7.0. DATA SYNTHESIS: An estimated 2077 and 1871 inpatient pediatric HNE procedures were performed nationally in 1997 and 2000, respectively. Most were performed at general (nonpediatric) teaching hospitals. There were an estimated 1102 thyroglossal duct cyst excisions, making this the most common HNE procedure and diagnosis. Thyroid lobectomy was the second most common HNE surgical treatment. Thyroid malignant neoplasm (usually treated by total thyroidectomy) was the second most common diagnosis. Neck dissections were performed in 32% of patients with thyroid malignant neoplasm. These HNE procedures accounted for more than 28 million dollars in hospital charges in 1997 and nearly 38 million dollars in 2000. CONCLUSIONS: Surgical treatment trends for pediatric HNE procedures remained stable between 1997 and 2000. Thyroglossal duct cyst excision and thyroid lobectomy are the most common procedures. There were regional differences in the rates of most HNE surgical treatments. In addition, hospital charges increased between 1997 and 2000.

Laryngeal anatomic differences in pediatric patients with severe laryngomalacia.

OBJECTIVES: To compare the aryepiglottic (AE) length in pediatric patients who have severe laryngomalacia (SL) and are undergoing aryepiglottoplasty with the AE length of a convenience sample of control patients without laryngomalacia. DESIGN: Prospective case-control study. SETTING: A tertiary-care pediatric hospital. RESULTS: The mean AE fold length-glottic length ratio for patients with SL (0.380) was significantly lower than the mean ratio for controls (0.535) (P = .004 in 2-sample t test with unequal variance). For patients with SL, the aryepiglottoplasy procedure resulted in an average AE length increase-glottic length ratio of 0.330. Seven of the patients with SL were also diagnosed as having an underlying neurologic condition, and 18 had a diagnosis of gastroesophageal reflux disease. Two patients with SL required a tracheotomy for treatment of persistent airway obstruction. CONCLUSIONS: In this series, patients with SL had lower AE fold length-glottic length ratios and more frequent occurrence of neuromuscular tone abnormalities (especially gastroesophageal reflux) than controls. These 2 findings may be related in that low intrauterine tone might contribute to anatomic underdevelopment.