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.

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.

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.

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.

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 patient's 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.

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.

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.

Parotid duct ligation for treatment of drooling in children with neurological impairment.

OBJECTIVE: Multiple surgical procedures have been advocated for the management of problematic drooling in neurologically impaired children. Parotid duct ligation is a quick and simple operation conducted via an intra-oral approach and usually performed simultaneously with other procedures. In this study, we aimed to evaluate the effectiveness of parotid duct ligation as a discrete procedure. METHODS: All children who underwent bilateral parotid duct ligation as the solitary operative intervention at that time, between February 2003 and September 2006, were included in the study. RESULTS: Ten children were studied. Surgery was successful in 80 per cent of cases. One patient (10 per cent) had a post-operative wound infection. CONCLUSIONS: Bilateral parotid duct ligation is an effective yet conservative operation for drooling in neurologically impaired children. It requires minimal surgical dissection and has a low morbidity rate. It should be considered as a potential first-line procedure in children who aspirate, and as a further surgical option in anterior droolers or those who continue to drool unacceptably following prior surgical intervention.

Drooling in oro-motor dyspraxia: is there a role for surgery?

OBJECTIVE: To report the successful use of a surgical approach for the treatment of drooling in a case of oro-motor dyspraxia. CASE REPORT: A seven-year-old girl with a diagnosis of oro-motor dyspraxia was referred for management of drooling; this was her only symptom. There were no focal neurological abnormalities. Conservative measures had not helped, and her parents were keen for definitive treatment. Bilateral submandibular duct transposition was performed with no complications. The patient's drooling improved immediately. CONCLUSION: There are currently no reports in the literature of surgery for persistent drooling in children with oro-motor dyspraxia who are otherwise neurologically and developmentally normal. The mainstay of treatment is speech and language therapy. We report the successful use of surgical techniques, usually reserved for children with cerebral palsy or severe neurological disorders, to treat drooling in an otherwise normal seven-year-old child with oro-motor dyspraxia.

Dysphonia--a rare early symptom of Ehlers--Danlos syndrome?

Ehlers-Danlos syndrome is a connective tissue disorder characterized by joint hypermobility, skin hyperextensibility and cutaneous fragility. It usually presents in young adults and is rarely diagnosed in children. Voice involvement in young children, to our knowledge, has not been reported in the literature. We present two cases with dysphonia from birth and an eventual diagnosis of Ehlers-Danlos syndrome. The syndrome and its relevance to voice pathology are discussed. We suggest that Ehlers-Danlos syndrome should be considered as an underlying diagnosis in atypical presentations of dysphonia in young children.

Chronic facial pain following injection of sodium tetradecyl sulphate into an intraparotid haemolymphangioma.

OBJECTIVE: We report a rare case of chronic facial pain following sclerotherapy for intraparotid haemolymphangioma, thereby highlighting an important clinical consideration when advising this treatment option as an alternative to surgery in the head and neck. METHOD: Case report, with a review of relevant literature. RESULTS: Sclerotherapy of lymphangiomata is well reported in the literature. Unusually, our young patient with an intraparotid haemolymphangioma experienced severe, chronic pain following intralesional injection of sodium tetradecyl sulphate, which required management by a specialist pain service. We discuss the technique of sclerotherapy for such lesions, and also discuss the potential side effects of two agents commonly used in our centre: OK 432 and sodium tetradecyl sulphate. CONCLUSION: Non-surgical treatments of lymphangiomata and venous vascular malformations are not without complication. Both patient and clinician should be aware of this, and of the other potential side effects of sclerotherapy, prior to its use in the head and neck.

Treatment of piriform fossa sinuses with monopolar diathermy.

INTRODUCTION: Embryological remnants of third or fourth branchial pouches are a rare but important cause of recurrent neck abscesses in children. They are characterised by an internal opening in the piriform fossa. Traditional management involves surgical excision of the entire tract. We present our experience with the use of monopolar diathermy applied to the internal sinus opening as a treatment modality for this condition. MATERIALS AND METHODS: A retrospective, case report review was performed. RESULTS: Four cases of piriform fossa sinus were treated with monopolar diathermy to the sinus opening via an endoscopic approach. The first three cases were treated in this way for recurrence, following external tract excision, while the fourth case had simultaneous excision of the tract and diathermy to the piriform fossa opening. There were no serious complications and no recurrence within a follow-up period ranging from nine to 27 months. DISCUSSION: Obliteration of the internal opening of these sinuses by endoscopic diathermy is a safe and effective management option for this condition, either as an alternative to or as an adjunct to external surgical excision of the tract.

The Great Ormond Street Hospital paediatric cochlear implant programme 1992-2004. A review of surgical complications.

The authors present a review of surgical complications following cochlear implantation at Great Ormond Street Hospital, since inception of the programme in 1992 until June 2004. Complications are defined as major (resulting in re-operation, explantation, re-implantation or which resulted in permanent serious morbidity or mortality) or minor (where the implant was not threatened). A comparison of surgical complication rates is made both with an earlier study in the same institution and also with other cochlear implantation centres worldwide. The decrease in surgical complication rates is discussed along with the difficulties inherent in cochlear implantation in the paediatric population with coexisting medical complaints.

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.

Subglottic haemangioma in children: experience with open surgical excision.

Subglottic haemangioma is a potentially life-threatening condition for which various treatment modalities are available. The objective of this study was to evaluate our results for open excision of subglottic haemangioma. The study assessed 18 patients who had been treated at a paediatric tertiary referral centre. Most of these patients (83.3 per cent) had undergone open surgical excision without post-operative tracheostomy and had been intubated for several days post-operatively (single-stage procedure). In most of these patients (66.7 per cent), an anterior cartilage graft had been used for reconstruction. The average follow up in this study was 25 months. All the patients in this series had achieved an adequate airway after the procedure. One patient had developed a recurrence of haemangioma in the trachea at a later date. The results of open surgical excision in this study were very encouraging. Seventeen out of 18 (94.4 per cent) patients had avoided tracheostomy or had been decannulated as a direct result of surgery. One of these 18 patients (5.6 per cent) had required a temporary post-operative tracheostomy for 13 months as the subglottis cleared; this was classed as a partial success. Our experience is that open excision is a highly successful 'one stop' treatment for subglottic haemangioma, which avoids prolonged use of steroids and multiple endoscopic procedures. No patient in this series developed subglottic stenosis, which can be a significant complication of laser application.

Absent/reduced glucose transporter-1 protein expression in infantile subglottic haemangiomas.

BACKGROUND: Positive immunohistochemical staining for glucose transporter-1 protein (GLUT1) is a characteristic of cutaneous infantile haemangiomas. OBJECTIVES: To examine GLUT1 expression in subglottic haemangiomas. METHODS: Review of clinical notes and biopsy tissue with immunostaining for GLUT1 in 14 patients with subglottic haemangiomas. RESULTS: GLUT1 immunostaining was negative in 11 cases, and focally positive in three. No subglottic haemangiomas demonstrated the intense diffuse positive GLUT1 staining seen in cutaneous infantile haemangiomas. Five patients had cutaneous as well as subglottic haemangiomas, one of whom had a GLUT1-negative subglottic haemangioma and a GLUT1-positive cutaneous haemangioma of the lip. CONCLUSIONS: Subglottic haemangiomas appear immunohistochemically different from cutaneous infantile haemangiomas, which may reflect differences in endothelial cell differentiation or underlying aetiology.

Cervico-thoracic teratoma masquerading as lymphatic malformation.

A case is presented of a nine-month-old male infant who presented acutely with an anterior neck and mediastinum mass compressing the trachea. Radiological assessment by ultrasound, magnetic resonance imaging and computed tomography suggested the nature of the mass to be a lymphatic malformation (cystic hygroma). The excised specimen was revealed as a mature teratoma. The inability to distinguish between lymphatic malformation and teratoma on multi-imaging modalities confers a risk of mismanaging these lesions with the use of sclerotherapy; surgical excision is the necessary treatment. The potential consequences are discussed.

The midfacial degloving approach to sinonasal tumours in children.

The midfacial degloving approach was originally described by Denker and Kahler in 1926 but has been little used in the paediatric population. The procedure allows access to benign and malignant lesions of the sinonasal region with the avoidance of an external scar. The advantages and application of this technique are presented in 9 paediatric patients, ranging from 3 months to 15 years of age with a mean follow-up of 7 months. Eight children had benign pathology. There were two juvenile angiofibromas, two nasal gliomas, one ossifying fibroma, one fibroma, one fibrous dysplasia and one benign myofibroblastic proliferation. One child had malignant disease in the form of recurrent embryonal rhabdomyosarcoma. All had excellent cosmetic results and no complications were encountered during follow-up.