C.G. Jephson

Stem-cell-based, tissue engineered tracheal replacement in a child: A 2-year follow-up study

BACKGROUND Stem-cell-based, tissue engineered transplants might offer new therapeutic options for patients, including children, with failing organs. The reported replacement of an adult airway using stem cells on a biological scaffold with good results at 6 months supports this view. We describe the case of a child who received a stem-cell-based tracheal replacement and report findings after 2 years of follow-up. METHODS A 12-year-old boy was born with long-segment congenital tracheal stenosis and pulmonary sling. His airway had been maintained by metal stents, but, after failure, a cadaveric donor tracheal scaffold was decellularised. After a short course of granulocyte colony stimulating factor, bone marrow mesenchymal stem cells were retrieved preoperatively and seeded onto the scaffold, with patches of autologous epithelium. Topical human recombinant erythropoietin was applied to encourage angiogenesis, and transforming growth factor β to support chondrogenesis. Intravenous human recombinant erythropoietin was continued postoperatively. Outcomes were survival, morbidity, endoscopic appearance, cytology and proteomics of brushings, and peripheral blood counts. FINDINGS The graft revascularised within 1 week after surgery. A strong neutrophil response was noted locally for the first 8 weeks after surgery, which generated luminal DNA neutrophil extracellular traps. Cytological evidence of restoration of the epithelium was not evident until 1 year. The graft did not have biomechanical strength focally until 18 months, but the patient has not needed any medical intervention since then. 18 months after surgery, he had a normal chest CT scan and ventilation-perfusion scan and had grown 11 cm in height since the operation. At 2 years follow-up, he had a functional airway and had returned to school. INTERPRETATION Follow-up of the first paediatric, stem-cell-based, tissue-engineered transplant shows potential for this technology but also highlights the need for further research. FUNDING Great Ormond Street Hospital NHS Trust, The Royal Free Hampstead NHS Trust, University College Hospital NHS Foundation Trust, and Region of Tuscany.

Branchial anomalies in children

BACKGROUND Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great Ormond Street Hospital over the past 10 years. METHODS All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to December 2010 were included in this study. RESULTS In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated on by a superficial parotidectomy approach with facial nerve identification. Complete excision was achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication (temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were operated on through an elliptical incision around the external opening. Complete excision was achieved in all second cleft cases except one who required a repeat excision. In this subgroup, we had two complications one patient developed a seroma and one had incomplete excision. The three patients with fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening with good outcome. CONCLUSION Branchial anomalies are relatively common in children. There are three distinct types, first cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of the anomaly. Complete excision is essential for good outcomes.

Peri-operative complications after adenotonsillectomy in a UK pediatric tertiary referral centre.

OBJECTIVES Adenoidectomy and/or tonsillectomy are commonly performed in tertiary pediatric hospitals for the management of obstructive sleep apnea, often in children with significant comorbidities. This study examines the peri-operative course of a large series of complex patients undergoing such surgery at a major pediatric centre, reporting particularly cases of respiratory compromise requiring intensive care admission, both electively and unplanned. METHODS This study was conducted by the pediatric ENT department at Great Ormond Street Hospital. All children undergoing adenoidectomy and/or tonsillectomy from July 2003 to December 2010 were included in this study. This involved a retrospective review of the case notes and hospital databases, with particular emphasis on those children requiring admission to the pediatric intensive care unit. RESULTS A total of 1735 consecutive admissions for adenoidectomy and/or tonsillectomy (1627 individual patients aged 4-197 months, median 46 months) were included between 2003 and 2010 (998 adenotonsillectomies, 182 tonsillectomies and 555 adenoidectomies). In this group, 999/1627 patients (61.4%) had a diagnosis of sleep disordered breathing or sleep apnea, including 258 who had polysomnography. 407/1627 (25.0%) had no specific comorbidities which were felt likely to influence their surgical outcome. Established high risk factors included age less than 24 months (292), Down syndrome (99), neuromuscular problems (314), craniofacial abnormalities (94), storage diseases (23), morbid obesity (20), cardiovascular disease (133), respiratory disease (261), hemoglobinopathy (76) and coagulophathy (34). 300/1735 admissions were day cases and 1082/1735 were observed for one night. 353/1735 required more than one night in hospital (294 for two to three nights). 7/1735 had primary hemorrhage necessitating return to the operating room, all after tonsillectomy. 41/1735 (38 with major comorbidities) required peri-operative intensive care admission, mostly for respiratory support. Of these, 7 were admitted pre-operatively to intensive care, and 17 were planned post-operative transfers. Only 17/1735 required unanticipated post-operative admission to intensive care. Odds ratio analysis suggested a significantly higher chance of PICU admission in children with particular comorbidities (Down Syndrome, cardiac disease, obesity, cerebral palsy, craniofacial anomalies, mucopolysaccharidoses and hemoglobinopathy) when compared to children without comorbidities. Adenotonsillectomy was associated with a higher risk of PICU admission than adenoidectomy alone, but patient age less than 24 months was not associated with significantly higher rates of PICU admission. There were no peri-operative mortalities in this cohort. CONCLUSIONS The peri-operative course was largely uneventful for the majority of children undergoing surgery during this period, particularly given the high prevalence of sleep apnea and other risk factors in this cohort. Major complications were uncommon, with 2.4% of these selected, typically high risk cases requiring peri-operative intensive care admission. Importantly, only 1% of all admissions required unanticipated transfer to intensive care. This has informed changes in peri-operative management in this unit, with implications for other pediatric tertiary referral centres.

Laryngotracheal reconstruction and cricotracheal resection in children: Recent experience at Great Ormond Street Hospital

BACKGROUND Surgery for paediatric airway stenosis is constantly evolving. Surgery is the primary treatment modality via either an open or endoscopic approach. The objective of this study was to review the results of laryngotracheal reconstruction (LTR) and cricotracheal resection (CTR) procedures performed at Great Ormond Street Hospital over the past 10 years. METHODS All patients who underwent open airway reconstruction surgery from January 2000 to December 2010 were included in this study. Patients treated entirely endoscopically were excluded. The data was collected using the electronic operating theatre database and the discharge summary database. RESULTS Complete data was available for 199 patients who underwent open airway reconstruction from January 2000 to December 2010. The procedures included single stage LTR (57, 28.6%), two stage LTR (115, 57.7%), single-stage stomal reconstruction (14), single-stage CTR (8) and two-stage CTR (5). The diagnoses at the initial airway endoscopy were laryngeal web (22), subglottic stenosis (151), posterior glottic stenosis (9), suprastomal collapse (15), supraglottic stenosis (1) and tracheal stenosis (1). For those with subglottic stenosis, the stenosis was grade 1 in 1 patient, grade 2 in 26 patients, grade 3 in 117 patients and grade 4 in 6 patients. At the completion of intervention 175/199 (87.9%) patients reported improvement in their symptoms. Amongst the subglottic stenosis group, post LTR success was achieved in 100% with grade 1 stenosis, 92.3% with grade 2 stenosis, 88.1% in grade 3 stenosis and 83.3% in grade 4 stenosis. Of the two-stage LTR procedures, 100/115 (86.9%) had their tracheostomy removed and 15/115 (13.1%) have failed decannulation. Of the single-stage LTR group, 50/57 (87.7%) patients were better both on airway examination and symptomatically postoperatively. Of the single-stage stomal reconstruction group, 13/14 (92.8%) were better symptomatically and on airway examination. Patients who underwent single-stage CTR had a better airway on examination and were symptomatically improved in all cases (8/8). For the patients who underwent two stage CTR, the tracheostomy was removed in 3/5 (60%) and retained in 2/5 (40%). For the whole group, 15/199 (7.5%) patients underwent a revision LTR. On further analysis, revision LTR was required in 4/57 (7.1%) single-stage LTR, 9/115 (7.8%) two-stage LTR, 1/5 (20%) two-stage CTR and 1/8 (12.5%) single-stage CTR. In this study complications occurred in 13/199 (6.5%). CONCLUSIONS Subglottic stenosis in children needs to be approached on the basis of the nature and severity of stenosis and the individual patients general health. Good outcomes are achieved with both LTR and CTR. Good results are obtained both with single-stage and two-stage LTR, but restenosis remains a problem. An individual approach is required for treatment of paediatric airway stenosis to achieve good final outcomes. The overall success rate has increased only marginally in our institution over the last 20 years.

Great Ormond Street Hospital treatment guidelines for use of propranolol in infantile isolated subglottic haemangioma

OBJECTIVE Treatment options for large subglottic haemangioma include steroids, laser ablation, open excision, tracheostomy and, more recently, propranolol. This article aims to present the Great Ormond Street Hospital guidelines for using propranolol to treat infantile isolated subglottic haemangioma by ENT surgeons. METHODS The vascular malformations multidisciplinary team at Great Ormond Street Hospital has developed guidelines for treating infantile haemangioma with propranolol. RESULTS The Great Ormond Street Hospital guidelines for propranolol treatment for infantile subglottic haemangioma include investigation, treatment and follow up. Propranolol is started at 1 mg/kg/day divided into three doses, increasing to 2 mg/kg/day one week later. On starting propranolol and when increasing the dose, the pulse rate and blood pressure must be checked every 30 minutes for the first 2 hours. Lesion response to treatment is assessed via serial endoscopy. CONCLUSION Recent reports of dramatic responses to oral propranolol in children with haemangioma and acute airway obstruction have led to increased use. We advocate caution, and have developed guidelines (including pre-treatment investigation and monitoring) to improve treatment safety. Propranolol may in time prove to be the best medical treatment for subglottic haemangioma, but at present is considered to be still under evaluation.

MRI brain abnormalities in cochlear implant candidates: How common and how important are they?

OBJECTIVE To investigate the incidence of abnormal findings on brain MRI in paediatric cochlear implantation candidates. METHODS Retrospective review of brain MRI scans of cochlear implant patients between 2000 and 2009 who underwent MRI brain as part of their pre-operative work-up. RESULTS MRI scans of 162 patients were reviewed (76 female patients and 86 male patients). The mean age at time of MRI scan was 3 years 8 months. Abnormalities were detected/ reported in 49 patients (30%). The total number of abnormalities detected was 51 (two patients had two separate abnormalities each). Of the abnormalities 82% could be related to known pre-existing conditions. 18% of the abnormalities were incidental/unexpected. Incidental/unexpected abnormalities were found in 9 patients (6%). Four of the patients with incidental abnormalities required referral and further investigations (2.5%). The most common abnormality detected was white matter changes (70%). All the white matter changes were related to pre-existing known medical conditions. CONCLUSION At our institution abnormalities detected by pre-operative brain MRI scans on cochlear implant candidates are common (30%). The majority of abnormalities are related to known pre-existing medical conditions. Incidental findings are rare (4%) and approximately half of them required further investigation or referral.

Nasal dermoids in children: a proposal for a new classification based on 103 cases at Great Ormond Street Hospital.

OBJECTIVES Nasal dermoids are rare developmental anomalies seen in children. This study reports the largest case series of 103 patients seen in a quaternary specialist unit over a 10-year period. We report the surgical and radiological findings and propose a new classification system, which clearly describes the extent of the lesions, thus allowing better surgical planning. METHODS A retrospective review of case notes was conducted. Data collection included demographics, initial presentation, site of lesion, pre-operative CT and MRI imaging, surgical procedure, intraoperative findings (including depth of lesion), complications and recurrence. Surgical findings were correlated with radiological findings. RESULTS A total of 103 patients were included in the study. The mean age at presentation was 29 months. 89% of children presented with a naso-glabellar or columellar lesion and 11% had a medial canthal lesion. All the patients underwent preoperative imaging and were treated with surgical excision. 58 children had superficial lesions, 45 had subcutaneous tracts extending to varying depths. Of these, 38 had intraosseous extension into the frontonasal bones, eight extended intracranially but remained extradural and two had intradural extension. There was good correlation between radiological and surgical findings. The superficial lesions were locally excised. The lesions with intraosseous tracts were removed via open rhinoplasty and the frontonasal bones drilled for access. Intracranial extension was approached either via a bicoronal flap and frontal craniotomy or the less invasive anterior small window craniotomy. CONCLUSIONS This report describes the largest published cases series of nasal dermoids. The cases demonstrate the presenting features and the variable extent of the lesions. The new proposed classification; superficial, intraosseous, intracranial extradural and intracranial intradural, allows precise surgical planning. In the presence of intracranial extension, the low morbidity technique of using a brow incision and small window anterior craniotomy avoids the more invasive and commonly used bicoronal flap and frontal craniotomy.

Cochlear implantation: An assessment of quality and readability of web-based information aimed at patients

Abstract Objectives Patients should have access to high-quality health information websites on which to base their decision-making. There are concerns regarding the accuracy and quality of some health websites. We aimed to objectively measure website quality related to cochlear implantation. Methods Selected patient-information websites were scored, depending on how highly they ranked on search engines and if they were ranked on more than one of the search engines used. The top 40 websites from three major search engines were analysed. The quality of each website was scored using the DISCERN tool and the readability was scored using the Flesch–Kincaid reading ease and the Gunning-Fog index. Results The average Flesch–Kincaid score was 49.7, giving an average reading age of a 15–17 years old, and the average Gunning-fog score was 13.1, which equals that of an 18 years old. Conclusion Internet-based information regarding cochlear implantation is of varied quality and is written above the expected reading level of an average person.

Surgical site infections in paediatric otolaryngology operative procedures

OBJECTIVE An assessment of the rate of surgical site infections associated with elective paediatric otolaryngology surgical procedures. METHODS Prospective data was collected for a 3-week period for all children undergoing surgery where either mucosa or skin was breached. The parents of the children were requested to complete a questionnaire at 30 days after the operation. RESULTS Data was collected on 80 consecutive cases. The majority of cases were admitted on the day of the procedure. The procedures included adenotonsillectomy (24), grommets (12), cochlear implantation (6), bone-anchored hearing aid (2), submandibular gland excision (1), branchial sinus excision (1), cystic hygroma excision (3), nasal glioma excision (1), microlaryngobronchoscopy (13), tracheostomy (3) and other procedures (14). Nearly half the cases had more than one operation done at the same time. 26/80 (32.5%) patients had a temporary or permanent implant inserted at the time of operation (grommet, bone-anchored hearing aid, cochlear implant). 25/80 (31%) operative fields were classed as clean and 55/80 (68.7%) as clean contaminated operations. The duration of the operation varied from 6 min to 142 min. Hospital antibiotic protocol was adhered to in 69/80 (86.3%) cases but not in 11/80 cases. In our series, 3/80 (3.7%) patients had an infection in the postoperative period. CONCLUSIONS Surgical site infections do occur at an appreciable rate in paediatric otolaryngology. With the potential for serious consequences, reduction in the risk of surgical site infections is important.

B1.2 Implementation of a screening tool and action plan for suspected vocal cord palsy

Background We review the implementation of a screening tool for suspected vocal cord palsy using ultrasound (US). This is used at our institution to evaluate children with changes to their voice or cry who are not under the primary care of otolaryngology services. Methods A retrospective review (1 st Jan 2015 – 1 st June 2017) of patients referred for US screening of vocal cord function at a single specialist paediatric centre. Results In thirty months, 36 children underwent US assessment of vocal cord function (aged 8 days – 16 years). It was possible to assess vocal cord mobility in all patients. Cases included 22 following cardiothoracic surgery. 21 of 36 had normal US and no subsequent endoscopy. 15 had abnormal function detected (11 left vocal cord palsy, 2 right, 2 bilateral). 12 of the 15 abnormal scans had subsequent endoscopic laryngeal assessment as per the screening protocol. 11 of 12 endoscopic assessments were concordant with US findings. One suspected left VC palsy was not evident at endoscopy. 3 cases lacked sufficient documentation of endoscopy to evaluate concordance. No cases with a negative US scan had a subsequent diagnosis of vocal palsy. Discussion Ultrasonography has been considered an adjunct for evaluation of vocal cord function. The largest cohort of patients in our screening process presented following cardiothoracic surgery. The implementation of this ultrasound screening protocol allowed 58% of cases to avoid endoscopic laryngeal evaluation with no false negatives. Concordance between positive US and endoscopic findings was high (92%). We suggest this is a reliable screening tool to identify vocal cord palsy and avoid unnecessary endoscopic assessment of the larynx.