S. Ifeacho

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.

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.

Surgical excision as primary treatment modality for extensive cervicofacial lymphatic malformations in children.

OBJECTIVE There has been much recent focus on sclerotherapy treatment of lymphatic malformations with OK432. Surgical treatment however can have a number of advantages, including complete curative excision. The aim of this study was to evaluate the results of surgical excision as the primary (first) treatment for this condition. This group includes a number of children with very extensive disease as well as some with smaller lesions. METHODS Prospectively collected database with additional information from medical records of children with cervicofacial lymphangiomas treated over 10 years at a tertiary paediatric referral centre. For this study only children who underwent surgical excision as the primary treatment modality were included. RESULTS Total of 118 children with lymphatic malformations were treated under the care of the senior author over a 10 year period. Of these 53 patients, who underwent surgical excision as the primary treatment modality for cervicofacial lymphatic malformations were included in this study. Forty-one patients who underwent sclerotherapy as the initial treatment were excluded. Also excluded were 6 patients who underwent thoracic surgery and 18 who were treated conservatively. The majority of the patients (41, 77.3%) underwent only a single surgical procedure (36 - surgical excision, 5 - laser excision). At the first follow up after the primary surgery, the result was complete resolution of symptoms in 29 patients, near complete resolution in 13 patients (together 79.3%) and partial response in 11 (19.7%) patients. Twenty-three patients with disease localised only to the neck, all (100%) had a complete or near complete resolution of the disease after the primary surgery. Complete/near complete response was achieved in 98% cases with macrocystic disease, regardless of the location. Minor complications occurred in 11.3% patients. No permanent nerve weaknesses occurred. CONCLUSION Cervicofacial lymphatic malformations in children should be managed in a multidisciplinary setting. Surgery remains a very important treatment modality. The majority of patients (80%) in this study had complete or near complete resolution with one surgical procedure. Isolated neck lesions have the best outcomes (100% resolution in this study). Patients with macrocystic disease, achieved complete or near complete resolution in 97% of cases, regardless of the location. Some children with extensive disease will need multiple treatments. Surgical excision as the primary treatment modality in selected cases is safe and reliable technique and has good aesthetic and functional outcomes in experienced hands.

Surgical technique for excision of first branchial cleft anomalies: how we do it

Dear Editor, First branchial cleft anomalies arise because of incomplete closure of the ectodermal first cleft. The defect can be a fistula or sinus depending on the degree of closure. These usually present with an infection. The external opening is usually in the pre-auricular region or cervical region (Fig. 1) above the hyoid bone. There can be an opening or swelling in the ear canal (Fig. 1). Various classifications have been proposed for these, but the most commonly used was described by Work. The only large series published in the literature was a multicentric study with four hospitals and multiple surgeons. Misdiagnosis and inadequate surgery are unfortunately often the norm. The underlying abnormality is a very significant sinus lined with squamous epithelium. Definitive surgery offers excellent outcomes, and this study describes clinical and surgical findings of a relatively large, single-centre, single-surgeon experience of this rare abnormality.

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.

Use of a post‐operative nasopharyngeal prong airway after adenotonsillectomy in children with obstructive sleep apnoea: how we do it

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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.

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.

The Effect of Midface Advancement Surgery on Obstructive Sleep Apnoea in Syndromic Craniosynostosis

Background: Children with syndromic craniosynostosis frequently suffer from obstructive sleep apnoea (OSA). The aim of the authors’ study was to investigate if midface advancement surgery for patients with SC improved the severity of OSA by examining the results of sleep studies before and after surgery. Methods: A retrospective comparison of the pre and postoperative sleep study data of children undergoing midface advancement surgery at Great Ormond Street Hospital between 2007 and 2016. Results: A total of 65 children underwent midface advancement surgery between 2007 and 2016 at Great Ormond Street Hospital and had recorded pre- and postoperative sleep studies. Thirteen patients were excluded from the analysis as their sleep study techniques before and after surgery were not comparable (e.g., different conditions with prong/continuous positive airway pressure use). Fifty-six percent of the patients were treated by monobloc surgery and the remainder with bipartition surgery. A greater proportion of patients had a normal OSA grading following midface advancement (42.3% postoperatively vs. 23.1% preoperatively, P = 0.059) although no statistically significant categorical changes in OSA grade were observed. Seventy-one percent of the patients had a decrease in Apnoea-Hypopnoea Index after surgery (21 patients 2011 onward). Similarly, there was no significant change in median oxygen desaturation index or in oxygen saturation nadir following surgery. Conclusion: The authors report one of the largest reviews of the effects of midface advancement surgery on sleep study parameters. Most patients showed improvements in Apnoea-Hypopnoea Index and OSA grading, although measures of oxygenation showed no consistent change.