Cell-intrinsic differences between human airway epithelial cells from children and adults.

The airway epithelium is a protective barrier that is maintained by the self-renewal and differentiation of basal stem cells. Increasing age is a principle risk factor for chronic lung diseases, but few studies have explored age-related molecular or functional changes in the airway epithelium. We retrieved epithelial biopsies from histologically normal tracheobronchial sites from pediatric and adult donors and compared their cellular composition and gene expression profile (in laser capture-microdissected whole epithelium, fluorescence-activated cell-sorted basal cells, and basal cells in cell culture). Histologically, pediatric and adult tracheobronchial epithelium was similar in composition. We observed age-associated changes in RNA sequencing studies, including higher interferon-associated gene expression in pediatric epithelium. In cell culture, pediatric cells had higher colony formation ability, sustained in vitro growth, and outcompeted adult cells in a direct competitive proliferation assay. Our results demonstrate cell-intrinsic differences between airway epithelial cells from children and adults in both homeostatic and proliferative states.

Head and neck teratomas in children--A series of 23 cases at Great Ormond Street Hospital.

OBJECTIVE: Head and neck teratomas are rare and malignant change is rarer still. This is a report detailing all of the teratomas treated by the ear, nose and throat or craniofacial teams over the last 28 years at our institution. Examining the common presenting and radiological features as well as our success rates compared to the current literature. METHOD: A retrospective review of all cases presenting to our institution with a head and neck teratoma were analysed. Data regarding the following characteristics were collected: antenatal history, clinical features, biological serum makers, radiological and pathological characteristics. Surgical treatment, rates of reoccurrence and the degree of post-operative follow up were also analysed. RESULTS: 23 cases in total were included: 8 cervical, 6 nasopharyngeal, 5 thyroid, 2 thymus, 2 temporal. One had malignant change. The majority of children presented at birth with respiratory distress, 5 cases were picked up antenatally and one case presented at 10 years of age. All were treated surgically; with complete excision in 20 patients. No clinical recurrence occurred but further surgery was performed for radiologically suspected residual disease in one case. DISCUSSION: This is the largest detailed case series in literature in regard to head and neck teratomas. Illustrating that this is frequently a benign disease process disease in the head and neck region and has an excellent long term prognosis following surgery. In the situation of incomplete resection careful meticulous follow up with radiological imaging and a multidisciplinary team approach is a safe and viable alternative.

Primary cervical ganglioneuroblastoma.

OBJECTIVES: Ganglioneuroblastomas represent a histological subgroup of the rare neuroblastic tumours with intermediate malignant potential arising from neural crest progenitor cells of sympathetic nerves. Diagnosis can often be difficult based on imaging alone. We describe 4 cases of children presenting with a solitary neck mass with histology ultimately revealing ganglioneuroblastoma. METHODS: A retrospective case note review was carried out of all patients with cervical ganglioneuroblastoma seen at Great Ormond Street Hospital, UK. RESULTS: Mean age at presentation was 5 years. Based on imaging, the initial diagnoses for three of the cases were: lymphatic malformation, carotid body tumour, paraganglioma, respectively, whilst the remaining case had an immediate incisional biopsy revealing the correct diagnosis. All cases were managed by surgical excision with no evidence of recurrence after a median follow up of 6 years. CONCLUSION: Otolaryngologists should be aware of ganglioneuroblastoma when establishing the differential diagnosis of a child presenting with a neck mass. Biopsy is recommended as the gold standard investigation to avoid an incorrect diagnosis.

The clinical application of electrolaryngography in a tertiary children's hospital.

The assessment and management of children's voice disorders poses clinical challenges at many levels. Accurate diagnosis and treatment requires access to a range of tools and methods appropriate for use with paediatrics. The application of electrolaryngography to the management of children's voice disorders in a tertiary children's hospital is described, with examples drawn from four case studies. Measures of fundamental frequency, contact quotient, and irregularity are used routinely to clarify the nature of the vocal impairment and to evaluate treatment outcome. Real-time displays provide biofeedback to assist the development of new skills within therapy programmes.

Multimodality treatment of pediatric lymphatic malformations of the head and neck using surgery and sclerotherapy.

OBJECTIVES: To describe a multimodality approach to the management of pediatric head and neck lymphatic malformations using surgery, sclerotherapy, or both and to review the outcomes of these approaches. DESIGN: Retrospective case series. SETTING: A single pediatric tertiary care referral center. PATIENTS: Ninety-seven pediatric patients (aged 1 month to 16 years) diagnosed as having lymphatic malformations of the head and neck during a 7-year period. Follow-up ranged from 3 months to 7 years. INTERVENTIONS: All of the patients underwent clinical and radiologic (magnetic resonance imaging) assessment. Treatment modality was selected according to disease location, cyst size, and parental preference. Treatments included surgery (open excision, tongue reduction, electrocautery, and laser treatment), sclerotherapy with OK-432 (Picibanil) or a fibrosing agent (Ethibloc), and a combination of modalities. MAIN OUTCOME MEASURES: Clinically determined responses to treatment, complications, and number of treatments required. RESULTS: All isolated neck disease had complete or near-complete responses, with no nerve palsies sustained. Although most patients achieved complete or near-complete responses, disease with parotid, laryngopharyngeal, or oral components had poorer outcomes and frequently required multiple treatments. Significant long-term neural injury was sustained in 3 of 6 surgical patients for mediastinal disease and in only 4% (n = 4) of other surgical procedures. CONCLUSIONS: Surgery retains an important role in the treatment of pediatric head and neck lymphatic malformations despite the advent of sclerotherapy. Isolated neck disease has an excellent outcome with either modality. Treatment decisions were made via a problem-based approach and were individualized according to anatomical location and disease classification.

Midline cervical cleft: a rare congenital anomaly.

OBJECTIVES: A midline cervical cleft (MCC) is a rare congenital anomaly due to failure of fusion of the first and second branchial arches during embryogenesis. It may present as a midline defect of the anterior neck skin with a skin projection or sinus, or as a subcutaneous fibrous cord. This report evaluates the clinical features and surgical management of an MCC. METHODS: We analyzed a series of 4 patients with an MCC successfully treated at Great Ormond Street Hospital for Children in London. RESULTS: Three male patients and 1 female patient between 4 and 11 months of age were found to have an MCC. Each patient presented with an erythematous, fibrous band of tissue extending between the chin and the suprasternal notch. Treatment comprised surgical excision of the lesion and Z-plasty repair. We present the embryology, common clinical presentation, investigations, differential diagnosis, and histology, along with a literature review, of this uncommon malformation of the anterior neck. CONCLUSIONS: An MCC is a differential diagnosis to consider when assessing a child with a midline cervical lesion. Early surgical excision with Z-plasty repair of the soft tissue defect is the treatment of choice to prevent long-term complications.

Angiosarcoma of skull base in a 1-year-old child--a case report.

Angiosarcoma is a rare tumour of endothelial cell origin whilst malignant skull base tumours are highly unusual in paediatric patients. This case reports an angiosarcoma involving the clivus and basi-sphenoid region of the skull base, in a 1-year-old boy. This tumour is extremely rare in childhood, particularly in this site. The histological features were consistent with a high-grade haemangioendothelioma, categorised as an angiosarcoma. The characteristics of this rare malignancy and the challenges in its management are discussed. This is the youngest reported patient, to our knowledge, with an angiosarcoma of the skull base.

Slide tracheoplasty.

PURPOSE OF REVIEW: The current practice of slide tracheoplasty for children with long-segment tracheal stenosis is reviewed. RECENT FINDINGS: In our own series, the mortality for children with long-segment tracheal stenosis managed by slide tracheoplasty in the 5-year period between 1995 and 2000 was 43% (3/7), consistent with other series in the literature. In 2001, we developed a multidisciplinary approach with aggressive postoperative surveillance, intermittent balloon dilatation and selective stenting for salvage. Mortality fell to 11% over the next 5 years (2/18). Since 2005, 25 cases have been treated and hospital mortality has been eliminated (0%, 0/25). There has been one late death due to renal disease. Quality of life for survivors is good. SUMMARY: The outlook has changed substantially in recent years for children with long-segment tracheal stenosis. Previous operations have been superseded by the advent of slide tracheoplasty in combination with a multidisciplinary approach with balloon dilatation and, occasionally, stenting in cases of recurrent stenosis. Survival rates have risen dramatically.

Excision of nasal dermoids with intracranial extension - anterior small window craniotomy approach.

OBJECTIVE: Dermoid cysts are the most common midline congenital nasal masses and may extend intracranially. They commonly become infected, may distort nasal growth, and are cosmetically unacceptable. The treatment of nasal dermoids is complete surgical excision. Removal of any intracranial extension traditionally required a bicoronal incision and frontal craniotomy, with significant associated morbidity. This retrospective study describes a new minimally invasive approach for excision of the intracranial component of the dermoid. METHODS: We present three cases where a brow incision was used. The intracranial part was removed by cutting a small window in the frontal bone directly over the dermoid, minimizing complications of formal craniotomy. RESULTS: Good access allowing complete excision of the dermoid and very low morbidity was achieved in all patients. The dura was breached in one patient at operation but this was easily repaired with a periosteal patch. All patients recovered quickly and hospital stay was short. The resultant scar was cosmetically acceptable. CONCLUSION: The use of a brow incision and small window craniotomy is a successful low morbidity technique for excision of nasal dermoids with intracranial extension.

Glottic scar bands following intubation.

We present two uncommon cases of abnormalities of the pediatric airway, which may present in the first instance to the anesthetist. Glottic scar bands are a result of intubation trauma and are a treatable cause of voice abnormalities and sometimes respiratory distress.

Open excision of subglottic hemangiomas to avoid tracheostomy.

OBJECTIVE: To assess the efficacy of open excision as an alternative to tracheostomy in the management of subglottic hemangioma. DESIGN: A retrospective review of patients undergoing open surgical excision of subglottic hemangiomas over a 10-year period. SETTING: A tertiary pediatric center. PATIENTS: The study included 22 children ranging in age from 2 to 42 months (median age, 5 months) who underwent open excision of subglottic hemangioma over a 10-year period. RESULTS: Twenty-one patients were treated with single-stage procedures, with postoperative endotracheal intubation for an average of 5 days. One patient who had a preexisting tracheostomy was treated with a 2-stage procedure and underwent decannulation 2 months after excision. Seven other patients were tracheostomy dependent at the time of excision and underwent decannulation at the time of the procedure. Cartilage grafts were inserted in 10 patients. There were no problems with subglottic stenosis. Twenty-one patients reported good voice and no airway symptoms after a mean follow-up period of 42 months. Five patients had significant postoperative problems. Three patients required further endoscopic procedures for removal of granulation tissue, and 1 patient, who remains minimally symptomatic, developed an anterior glottic web. One patient required a 6-month course of steroids after surgery to treat residual glottic hemangioma. CONCLUSIONS: Open surgical excision of subglottic hemangiomas can be performed as a single procedure, avoiding a tracheostomy, when modern surgical techniques developed for laryngotracheal reconstruction are incorporated. This approach can avoid repeated endoscopic procedures, prolonged treatment with corticosteroids, and years spent with a tracheostomy waiting for spontaneous involution of the hemangioma.