Objective First branchial cleft anomalies are rare congenital head and neck lesions. Literature pertaining to classification, work up and surgical treatment of these lesions is limited and, in some instances, contradictory. The goal of this work is to provide refinement of the classification system of these lesions and to provide guidance for clinicians to aid in the comprehensive management of children with first branchial cleft anomalies. Materials and Methods Delphi method survey of expert opinion under the direction of the International Pediatric Otolaryngology Group (IPOG) was conducted to generate recommendations for the definition and management of first branchial cleft anomalies. The recommendations are the result of expert consensus and critical review of the literature. Results Consensus recommendations include evaluation and diagnostic considerations for children with first branchial cleft anomalies as well as recommendations for surgical management. The current Work classification system was reviewed, and modifications were made to it to provide a more cogent categorization of these lesions. Conclusion The mission of the International Pediatric Otolaryngology Group (IPOG) is to develop expertise-based recommendations based on review of the literature for the management of pediatric otolaryngologic disorders. These consensus recommendations are aimed at improving care of children presenting with first branchial cleft anomalies. Here we present a revised classification system based on parotid gland involvement, with a focus on avoiding stratification based on germ layer, in addition to guidelines for management.
OBJECTIVES:To describe operative techniques using rigid bronchoscopy and ferromagnetic bronchoscopic equipment to retrieve magnetic foreign bodies in distal tertiary bronchi beyond the reach of traditional optical instrumentation. METHODS:A 13-year-old presented to the Emergency Department following aspiration of three backing magnets from a magnetic nose ring. Chest radiographs demonstrated a 4 mm × 3 mm foreign body in the right lower lobe 0.5 cm from diaphragm on expiratory film. She was taken to the operating room for removal of an airway foreign body. RESULTS:The foreign body was visualized with direct laryngoscopy followed by rigid bronchoscopy in the distal right lower tertiary bronchus. Attempts to pass optical instruments were limited both by distance of the object and size of instrumentation compared to the diameter of the tertiary bronchus. A salivary wire basket and ureteral stone retrieval basket were then passed with endoscopic visualization into the tertiary bronchus but were unable to engage the foreign body. An attempt was made to pass a 2 French Fogarty embolectomy catheter distally, but the catheter was too large to bypass the foreign body. Finally, ferromagnetic pulmonary rat tooth biopsy forceps were advanced into the tertiary bronchus and successfully attracted the magnetic foreign body for safe removal through our rigid bronchoscope. CONCLUSION:We present a novel method of utilizing ferromagnetic flexible bronchoscopic instruments to safely remove magnetic foreign bodies in the tertiary bronchi beyond the reach of traditional bronchoscopic instruments. Laryngoscope, 135:920-922, 2025.
OBJECTIVE:To investigate whether perioperative calcium and 1,25 OH vitamin D supplementation (PCDS) influences the rates of postoperative hypocalcemia and length of stay (LOS) following pediatric thyroidectomy.STUDY DESIGN:Retrospective Cohort Review.SETTING:Tertiary children's hospital.METHODS:94 patients who underwent completion or total thyroidectomy with or without concomitant neck dissection from 2010 to 2020 at a single institution were included. Patients with pre-existing hypocalcemia or preoperative vitamin D insufficiency were excluded. Rates of postoperative hypocalcemia and LOS were compared for patients receiving PCDS to those receiving no supplementation.RESULTS:Thirty percent of patients with PCDS had documented postoperative hypocalcemia compared to 64% of patients without PCDS (p = 0.01). Patients with PCDS had a median LOS of 30 h compared to 36 h (p = 0.002). Multivariable analyses confirmed that patients with PCDS had lower odds of postoperative hypocalcemia (OR: 0.32, CI: 0.11, 0.89) and shorter LOS by 17 h (SE: 8, p = 0.04) after adjustment for confounders.CONCLUSION:PCDS is associated with significantly lower risk of hypocalcemia and shorter LOS. Standardizing preoperative care for pediatric patients undergoing thyroidectomy may decrease variability and improve outcomes following surgery.
OBJECTIVE:To show the safety and efficacy of the endoscopic endonasal approach (EEA) for skull base surgery in pediatric patients through descriptive analysis of cases over an 11-year period.METHODS:The study comprised 94 patients undergoing EEA for skull base surgery, between January 2007 and June 2018, at 2 tertiary pediatric hospitals. Descriptive statistics are presented regarding the presentation, intraoperative details, and complications.RESULTS:Over the study period, 130 surgeries were performed in 94 patients: 94 primary surgeries and 36 reoperations. The mean patient age was 13.8 years and 48.9% of patients were female. Presenting signs/symptoms included endocrinopathies (56.4%), vision abnormalities (37.2%), and cranial nerve deficits (20.2%). EEA alone was used in 95.7% of primary surgeries and 91.7% of reoperations. Diseases treated included craniopharyngioma (18.1%), pituitary adenoma (17.0%), Rathke cleft cyst (13.8%), chordoma (9.6%), osteosarcoma (5.3%), juvenile nasopharyngeal angiofibroma (4.3%), skull base fracture (4.3%), and encephalocele (3.2%). A lumbar drain was used in 20.2% of primary surgeries and 25% of reoperations. A nasoseptal flap was used in 36.2% of primary surgeries and 25% of reoperations. Postoperative complications included cerebrospinal fluid leak (12.8%), sinusitis (7.4%), bacterial meningitis (3.2%), and carotid artery injury in 1 reoperation.CONCLUSIONS:EEA for anterior cranial base disease is safe and efficacious in pediatric patients and can be used to treat many of the rare and heterogenous diseases that arise in this anatomic location. Management strategies and rates of sinonasal and intracranial complications including cerebrospinal fluid leak rate are similar to those reported in adult cohorts.
PURPOSE OF REVIEW:The purpose of this review is to describe the development of pediatric skull base surgical techniques and illustrate the advantages of pediatric endonasal skull base surgery (ESBS) when applied in appropriate settings. Additionally, this manuscript endeavors to define the pediatric skull base team components, highlight circumstances amenable to the development of a pediatric skull base surgery team, and describe the relative advantages of independent pediatric teams versus incorporation with adult skull base practices. RECENT FINDINGS:Multiple series published within the last decade have described the application of ESBS to the pediatric population, demonstrating adoption of these interventions in many academic centers. Most series include relatively small numbers of patients, highlighting the relative infrequency of anterior skull base pathology in the pediatric patient. Given the relatively low volume and high technical demands of this skillset, general guidelines for the timing, suggested training, and volume necessary to support a pediatric skull base team are offered. SUMMARY:The interest in pediatric ESBS continues to expand though case volumes may limit maintenance of skills in lower volume centers. The development of a dedicated pediatric skull base team in areas where sufficient volume exists facilitates concentration of expertise and interdisciplinary relationships necessary to provide the highest level of care. Collaborating with adult skull base teams can enhance the pediatric team experience, increasing exposure to complex surgical planning and radiologic nuances. However, a pediatric-focused skull base team can tailor treatment to meet the specific psychosocial and developmental needs of children.
BACKGROUND AND OBJECTIVES: Lesions located in the retrosellar region, interpeduncular cistern, and petroclival region are among the most difficult to access in neurosurgery. Transcranial approaches are useful; however, the large distance between the surgeon and the lesion as well as the presence of major neurovascular structures surrounding the lesion may limit surgical exposure. A midline transsphenoidal route avoids transgression of the neurovascular plane and provides direct access to the interpeduncular cistern. To safely access the interpeduncular fossa, it requires mobilization of the pituitary gland. The pituitary hemitransposition technique permits mobilization of the gland, while preserving its venous drainage and arterial supply to the gland on one of its sides, preserving gland function. The authors aim to describe the intradural pituitary hemitransposition technique and to demonstrate its safe application for resection of skull base tumors in the retrosellar space. METHODS: The authors describe the surgical technique and illustrate its application in 5 cases of different types of skull base tumors, including a video demonstrating all the steps to perform this approach. In addition, the authors discuss the advantages and limitations of this technique compared with other approaches to the retrosellar space. RESULTS: The intradural pituitary hemitransposition technique was used to safely resect a chondrosarcoma, chordoma, craniopharyngioma, teratoma, and meningioma involving the parasellar and retrosellar spaces, while minimizing endocrine morbidity. We had one patient with mild, albeit permanent hyperprolactinemia and hypothyroidism after surgery. No other patients had permanent dysfunction related to surgery. CONCLUSION: The endonasal endoscopic intradural pituitary hemitransposition approach is an effective technique for resection of lesions located within the retrosellar and petroclival regions, allowing adequate exposure while potentially optimizing the preservation of the pituitary function.
Objective With few studies investigating the effectiveness of telemedicine (TM) in pediatric otolaryngology (ear, nose, and throat; ENT), its role in clinical practice is unclear. The objective of this study was to investigate provider perspectives regarding utility of TM in pediatric ENT practice. Methods A survey gauging the relative merits of TM visits for common pediatric ENT chief complaints and postoperative visits was distributed to all pediatric ENT providers at a tertiary care, free-standing children’s hospital. Respondents were asked to assess the effectiveness of TM visits compared with in-person visits for completing the following tasks: history collection, physical examination, medical decision-making, and patient counseling. Results Providers rated TM visits as less useful than in-person visits for completing the most predefined tasks but did identify advantages in history taking via TM for the majority of complaints. Compared with providers with ≥10 years of experience, those with <10 years of experience found TM to be more effective than the in-person appointment for making clinical decisions for patients presenting with recurrent/chronic pharyngitis, neck masses, and stridor/noisy breathing. Opinions regarding the utility of TM for postoperative visits were mixed, with adenoidectomy, tonsillectomy and superficial procedures being most frequently deemed appropriate for TM. Conclusions The introduction of TM to pediatric ENT faces limitations in detailed examination of areas not accessible without specialized instrumentation. Due to its strength in history taking, results suggest an asynchronous, ‘store and forward’ encounter followed by an in-person physical examination to confirm the diagnosis and treatment plan could be beneficial.
Abstract Background Cushing disease (CD) is uncommon in the pediatric population and is usually caused by benign pituitary corticotroph microadenomas. In some adults with CD, Crooke cell adenoma (CCA), a more aggressive histologic variant of corticotroph adenomas, has been described. CCA has rarely been reported in children as the etiology of CD and its course is not well described. Objectives To report a pediatric case of CCA, the youngest to our knowledge Case An 11-year-old African American female presented to the endocrinology clinic with concerns for diabetes, excessive weight gain and linear growth arrest. Workup for possible etiology showed: midnight salivary cortisol 0.924 (normal < 0.112 µg/dL), ACTH 119(6-48 pg/mL), morning cortisol 8.5 (normal < 1.8 µg/dL) post 1 mg dexamethasone mg/dL, consistent with the diagnosis of CD. Magnetic resonance imaging (MRI) showed a 4 mm pituitary microadenoma in the left anterior side of the adenohypophysis for which she underwent endoscopic endonasal transsphenoidal resection. Histopathologic examination revealed pituitary adenomatous tissue consisting of a uniform population of basophilic cells with granular cytoplasm, arranged in trabeculae and small nests with bundles encircling the nuclei, findings pathognomonic of CCA. Given persistence of hypercortisolemia, another transsphenoidal gross total resection of residual adenomatous tissue was performed 4 months later. This led to resolution of CD as evidenced by weight loss and linear growth. Twelve months following the second surgery, the patient experienced rapid weight gain and a decelerated linear growth accompanied by pubertal arrest. Another pituitary MRI showed a recurrent left sided adenoma (8×8×7 mm). Biochemical investigation this time again showed elevated midnight salivary cortisol and 24- hour urine free cortisol (UFC). In addition, AM cortisol was unsuppressed following 1 mg overnight dexamethasone administration. In response, repeat transsphenoidal gross total resection was performed, however residual microscopic disease in the cavernous sinus wall was highly suspected intraoperatively. A spine MRI did not show disseminated disease. Despite this, hypercortisolemia persisted both clinically and biochemically. The patient subsequently underwent proton beam radiation therapy in combination with adjuvant adrenolytic therapy with mitotane. The patient responded to these interventions with normalization of 24-hour UFC, resumption of linear growth, and pubertal progression. The treatment course was complicated by the development of isolated TSH deficiency requiring thyroid hormone replacement. Conclusion CCA is an aggressive entity described in the adult literature as a cause of CD. The current report adds to the limited pediatric cases of this disease. CCA can be equally aggressive in children and refractory to surgical treatment. Here, we show that it may respond to proton beam radiation and adrenolytic therapy. Pediatric endocrinologists should be aware of the rare occurrence of CCA in children and tailor treatment accordingly using a multi-disciplinary approach. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
OBJECTIVE: The pediatric skull base may present anatomic challenges to the skull base surgeon, including limited sphenoid pneumatization and a narrow nasal corridor. The rare nature of pediatric skull base pathology makes it difficult to gain experience with these anatomic challenges. The objective of this study was to create a 3-dimensionally (3D) printed model of the pediatric skull base and assess its potential as a training tool. METHODS: Twenty-eight participants at various stages of training and practice were included in our study. They completed a pre- and postdissection questionnaire assessing challenges with endoscopic endonasal skeletonization of the carotid arteries and sella face using the 3D printed model. RESULTS: The majority of participants had completed a skull base surgery fellowship (60.7%), were <5 years into practice (60.7%), and had <10 cases of pediatric skull base experience (82.1%). Anticipated challenges included limitation of maneuverability of instruments (71.4%), narrow nasal corridor and nonpneumatized bone (57.1%). On a scale of 0-10, 10 being very difficult, the average participant expected level of difficulty with visualization was 6.89 and expected level of difficulty with instrumentation was 7.3. On postdissection assessment, there was a non-statistically significant change to 6.93 and 7.5, respectively. Participants endorsed on a scale of 0-10, 10 being very realistic, an overall model realism of 7.0 and haptic realism of 7.1. CONCLUSIONS: A 3D printed model of the pediatric skull base may provide a realistic model to help participants gain experience with anatomic limitations characteristic of the pediatric anterior skull base.
Head and neck malignancies are a part of the differential diagnosis of head and neck masses seen in the pediatric age group. It is critical to make prompt diagnosis and employ proper treatment. We will review the most common malignant pathologies as well as their specific clinical presentations and management. Centers that are able to provide multidisciplinary care for pediatric patients with head and neck pathology can help achieve the best outcomes.
This study investigates outcomes of surgical management of pediatric patients with nasal dermoids with prior infection. A retrospective review at Nationwide Children's Hospital, a large free-standing pediatric hospital in the Midwestern USA, was performed. Patients were identified by the Current Procedural Terminology codes 30124 (simple excision of dermoid cyst) and 30125 (complex excision of nasal dermoid cyst) from 2011 to 2016. Demographic, imaging data, surgical findings, microbiological data and recurrence rates were collected for these patients. Descriptive statistical investigation was performed. In total, 14 patients were identified, 4 of the 14 patients (28.5%) had recurrent infection and required additional surgery. Three of seven patients required incision and drainage prior to definitive excision. One of seven patients in the infected group had recurrence. Prior infection does not increase the recurrence rate and almost half of the patients required I&D prior to definitive management.
Abstract Objective Surgery of the pediatric skull base has multiple unique challenges and has seen recent rapid advances. The objective of this review is to assess key issues in pediatric skull base surgery (SBS), including anatomic limitations, surgical approaches, reconstruction techniques, postoperative care, complications, and outcomes. Data Sources PubMed literature review. Review Methods A review of the literature was conducted to assess the challenges, recent advances, and reported outcomes in pediatric SBS. Results The pediatric skull base presents multiple anatomic challenges, including variable patterns of pneumatization, narrow piriform aperture width, and narrow intercarotid distance at the level of the cavernous sinus but not the superior clivus. These issues may be particularly challenging in patients less than 2 years of age. Endoscopic endonasal approaches in the sagittal and coronal plane have been applied to the pediatric skull base while open approaches may still be necessary in the setting of extensive intracranial or orbital disease, as well as disease lateral to critical neurovascular structures. While the nasoseptal flap was initially called into question for pediatric cases, it has been shown through multiple reports to be a feasible and robust reconstructive option. Complications and outcomes often depend upon the pathology. In children, response to noxious stimuli, ability to avoid Valsalva, and adherence to nasal precautions is variable. The use of lumbar drains is more common in pediatric than adult patients. Conclusion While the pediatric skull base presents unique challenges, outcomes data support that endoscopic endonasal approaches are a pertinent surgical technique in appropriately selected patients. Level of Evidence 3a
Objectives Sinusitis is a common pediatric illness that can be complicated by periorbital or intracranial extension. Patients can be managed with antimicrobials alone or in conjunction with surgical intervention. This article examines management patterns and outcomes in pediatric patients presenting with complicated sinusitis. Study Design Case series with chart review. Setting Tertiary care pediatric hospital. Subjects and Methods An evaluation of 168 pediatric patients with complicated sinusitis with periorbital complications presenting at a single institution from 2008 to 2018 was performed. Demographics, disease characteristics, in-hospital management, and outcomes were recorded and analyzed. Results The most common complication was orbital cellulitis, seen in 49% of children. Surgical intervention occurred in 49% of patients, with 36% receiving medical therapy followed by surgery (MTS). Chandler I patients underwent surgical intervention 30% of the time, Chandler II patients 29%, and Chandler III patients 83%. Nineteen percent of initially nonoperative patients started on ampicillin-sulbactam required MTS vs 57% of those started on other antibiotic regimens (P = .01). Twelve percent of initially nonoperative Chandler I to II patients started on ampicillin-sulbactam needed MTS vs 40% started on other antibiotic regimens. Hospital charges for operative patients were $45,056 vs $14,311 for nonoperative patients (P < .01). Hospital charges for patients with surgery followed by medical therapy (SMT) were $45,563 vs $44,393 for MTS (P = .92). Conclusion Nonoperative early stage patients started on ampicillin-sulbactam had a lower risk of MTS. MTS did not cost significantly more than SMT, and there were no significant outcome differences seen.
INTRODUCTION Pathology of the pediatric skull base is rare and ranges from congenital defects to malignancy and traumatic defects. Pediatric patients, particularly those ≤6 years of age, present a unique set of anatomic challenges for the skull base surgeon. The goal of this study was to retrospectively review our experience with reconstruction of pediatric skull base defects with particular emphasis on those ≤6 years of age. METHODS A retrospective chart review was conducted of patients ≤20 years of age who underwent endoscopic endonasal and combined endoscopic and open approaches to address cranial base pathology from 2007 to 2018. Patients were divided into two groups; those ≤6 years of age (group A) and those >6 years of age and ≤20 years of age (group B) and reconstructive techniques and outcomes were analyzed. RESULTS Intraoperative communication with the subarachnoid space (CSF leak) was created in 50% (3/6) of patients ≤ 6 years of age compared to 32.7% (16/49) in patients > 6 years and ≤20 years of age (P < 0.40) in management of their skull base disease. A vascularized flap was utilized for reconstruction in 66.7% (4/6) of patients ≤ 6 years of age and included a nasoseptal flap in 33.3% (2/6). A vascularized flap was utilized for reconstruction in 38.8% (19/49) of patients > 6 years and ≤20 years of age and most commonly included a nasoseptal flap (22.4%, 11/49). A lumbar drain was utilized in 50% (3/6) of patients ≤ 6 years of age compared to 18.4% (9/49) in patients > 6 and ≤20 years of age (P < 0.08). Post-operative CSF leaks were encountered in 16.7% (1/6) of patients ≤ 6 years of age compared to 10.2% (5/49) in patients > 6 and ≤20 years of age (P < 0.63). Behavioral issues were noted to potentially contribute to failed skull base reconstruction in patients <6 years of age. CONCLUSIONS Pediatric patients, particularly those ≤6 years of age, present a unique set of anatomic and behavioral challenges for the skull base surgeon as highlighted in this study.
There has been a rapid global spread of a novel coronavirus, the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), which originated in Wuhan China in late 2019. A serious threat of nosocomial spread exists and as such, there is a critical necessity for well-planned and rehearsed processes during the care of the COVID-19 positive and suspected patient to minimize transmission and risk to healthcare providers and other patients. Because of the aerosolization inherent in airway management, the pediatric otolaryngologist and anesthesiologist should be intimately familiar with strategies to mitigate the high-risk periods of viral contamination that are posed to the environment and healthcare personnel during tracheal intubation and extubation procedures. Since both the pediatric otolaryngologist and anesthesiologist are directly involved in emergency airway interventions, both specialties impact the safety of caring for COVID-19 patients and are a part of overall hospital pandemic preparedness. We describe our institutional approach to COVID-19 perioperative pandemic planning at a large quaternary pediatric hospital including operating room management and remote airway management. We outline our processes for the safe and effective care of these patients with emphasis on simulation and pathways necessary to protect healthcare workers and other personnel from exposure while still providing safe, effective, and rapid care.
Craniopharyngioma has historically been recognized to be a formidable pathology primarily due to its proximity to critical neurovascular structures and the challenging surgical corridors that surgeons have tried to reach this lesion. In this work, we review the medical and surgical management of these tumors with a focus on clinical presentation, diagnostic identification, surgical approach, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to craniopharyngiomas. The management of craniopharyngiomas requires a multidisciplinary team of surgeons, endocrinologists, and neuroanesthesiologists as well as neurocritical care specialists to deliver the most comprehensive and safest surgical resection with minimal postoperative morbidity.