B.E.J. Hartley

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.

Laryngotracheal reconstruction in congenital laryngeal webs and atresias

OBJECTIVE: To review the role of open laryngotracheal reconstruction (LTR) in congenital laryngeal webs and atresias. STUDY DESIGN AND SETTING: A retrospective chart review was undertaken in a specialist otorhinolaryngology unit in an academic tertiary referral paediatric hospital. Twenty-one patients with congenital laryngeal subglottic stenosis (SGS) underwent LTR between 1993 and 2003. Two groups were identified; one group had SGS alone (n = 6) and the other group had SGS associated with a laryngeal web (n = 15). Information recorded included presenting features, classification of lesion, surgery performed, and whether decannulation was achieved. RESULTS: All grades of stenosis (Myer-Cotton classification) and webs of type 2, 3, and 4 (Cohens classification) were recorded. Fifteen patients had a staged reconstruction and 6 patients had a single-stage LTR. Complications included 2 revision procedures, 3 tracheocutaneous fistulas, and 1 death (cause unrelated). All appropriate patients were successfully decannulated at between 3 and 18 months post-LTR (median, 5 months). CONCLUSION: This series illustrates the effective role of open LTR in both congenital SGS alone and that in association with glottic webbing.

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.

Surgical approach for congenital midline cervical cleft

This is a report of a case of a rare congenital midline cervical cleft and a description of the surgical approach. Congenital midline cervical cleft is a very rare developmental anomaly. It represents a failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Associated clinical features could include mandibular spurs, cleft mandible, microgenia, thyroglossal cyst, cleft lip or bronchogenic cysts. The authors present a case of a midline cervical cleft that was diagnosed and managed at an early age. They discuss the clinical presentation and embryological development of this rare condition. The operative findings, surgical excision and repair of the long vertical defect by Z-plasty are discussed in detail. The authors report a very satisfactory result following excision and Z-plasty closure of this rare congenital anomaly.

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.

Laser arytenoidectomy in children with bilateral vocal fold immobility.

Bilateral vocal fold immobility in children is a challenging problem because a balance between good airway and voice quality has to be achieved. Surgery to improve the airway is often postponed or avoided because of fear of losing the voice. In this study our results of laser arytenoidectomy in children are described. This was a retrospective case notes review at a tertiary level paediatric ENT department. The six patients in this case series ranged from nine to 16 years old at the time of laser arytenoidectomy. Post-operative airway and voice quality were assessed. All children in the series had an adequate post-operative airway. Four of these patients had tracheostomies pre-operatively and achieved decannulation. All six patients rated their post-operative voice as better than pre-operatively. This is principally due to increased loudness associated with increased airflow through the larynx, particularly after tracheostomy decannulation. It is recommended that special care should be taken not to disturb the anterior two thirds of the vocal fold during the surgery in order to achieve a good post-operative voice outcome.

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.

Dysphonia secondary to traumatic avulsion of the vocal fold in infants

OBJECTIVE Airway compromise due to paediatric intubation injuries is well documented; however, intubation injuries may also cause severe voice disorders. We report our experience and review the world literature on the voice effects of traumatic paediatric intubation. CASE SERIES We report five cases of children referred to Great Ormond Street Hospital for Children who suffered traumatic avulsion of the vocal fold at the time of, or secondary to, endotracheal intubation. All children had significant dysphonia and underwent specialist voice therapy. CONCLUSIONS The mechanisms of injury, risk factors and management of the condition are discussed. Children suffering traumatic intubation require follow up throughout childhood and beyond puberty as their vocal needs and abilities change. At the time of writing, none of the reported patients had yet undergone reconstructive or medialisation surgery. However, regular specialist voice therapy evaluation is recommended for such patients, with consideration of phonosurgical techniques including injection laryngoplasty or thyroplasty.

Advancement in post-meningitic lateral semicircular canal labyrinthitis ossificans

OBJECTIVE To assess whether lateral semicircular canal (LSCC) ossification is more advanced than that in the cochlear basal turn, in order to judge the value of the former as a predictor. METHODS Retrospective review of 33 paediatric patients from our cochlear implant programme, with profound sensorineural hearing loss after bacterial meningitis. Magnetic resonance imaging (MRI), computed tomography (CT) scans and operative findings were reviewed. RESULTS On CT, LSCC ossification scores were more advanced than those for the cochlear basal turn in 69.9 per cent of implanted ears. Forty-five per cent (15/33) of children had ossification at surgery. In predicting this, the sensitivity of CT LSCC ossification was 90 per cent and that of MRI LSCC ossification was 83.3 per cent. CONCLUSIONS The more advanced ossification found in the LSCC, compared with that in the cochlear basal turn, adds to previous findings of LSCC pathology predicting cochlear ossification. Surprisingly, CT of the LSCC appears to be no less valuable than MRI in pre-operative cochlear implant assessment of post-meningitic children.