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.

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.

The use of the Montgomery T-tube in difficult paediatric airways.

OBJECTIVE: We report our experience of using Montgomery T-tubes in children. METHOD: A retrospective review of medical records was performed. Data collected included particular clinical circumstances, the details of usage of the tube, and the eventual outcome. RESULTS: Between January 1999 and October 2003, our unit performed 293 tracheostomies, 76 laryngotracheal reconstructions and 31 other major airway procedures in children. 10 children have had a Montgomery T-tube inserted. Nine were boys. In eight cases, the T-tube was used because of severe granulations and subsequent fibrotic narrowing in the subglottis after laryngotracheal reconstruction surgery. These children had undergone between one and four major procedures prior to T-tube placement. In the other two cases, the T-tube was used to stent severe glottic and supraglottic stenosis (due to previous laser surgery for papillomas in one case and congenital ectodermal dysplasia in the other). At the time of T-tube placement the children were aged 2-18 years (median 8 years). The tube was initially fitted so as to protrude above the glottis in all cases. In one case, the T-tube was removed on the first post-operative day. One tube was removed after a week due to severe crusting. One tube blocked after 2 weeks. One child had re-stenosis in the supraglottis necessitating the placement of a T-tube with a longer upper limb. Two children have subsequently died from non-airway causes. Two children still have their T-tube in situ, one of whom is due to have it removed in the next few weeks. Six have been successfully decannulated and are well. CONCLUSIONS: The Montgomery T-tube provides a useful adjunct to the management of a small number of children with the most difficult airway problems. Its use can be problematic, however, and requires awareness of its specific complications. We have confined usage to complex stenoses where a reconstruction would be inappropriate, or (in one instance) to stent an unsupported larynx after revision reconstruction (tracheal resection).

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.

Chondrodysplasia punctata: case report and review of audiological and ENT features.

Chondrodysplasia punctata is a term referring to a clinically heterogeneous group of bone and cartilage dysplasias which cause characteristic epiphyseal stippling. The condition can involve the ear, nose and throat in diverse ways at many levels. We present a case of X-linked brachytelephalangic chondrodysplasia punctata, which illustrates the features of this condition particularly relevant to the audiological physician, otolaryngologist and neonatologist.

The role of the anterior cricoid split in facilitating extubation in infants.

OBJECTIVE: The use of the anterior cricoid split (ACS) procedure has facilitated the extubation of children with early subglottic stenosis who may otherwise have required a tracheostomy. Criteria have been established for the evaluation of patients suitable for ACS and adherence to these guidelines has increased the extubation rate and reduced mortality. The objective of the study was to assess the use of the procedure at our tertiary referral centre. METHOD: In this retrospective clinical study, 33 patients were identified over an 11-year period between 1993 and 2004 with subglottic narrowing at the level of the cricoid ring, and who subsequently underwent an ACS. Demographic data, duration of intubation, indication and number of reintubations, success rate and complications were noted. Patients included in the study were infants who repeatedly failed trials of extubation and those who presented with persistent stridor. RESULTS: Twenty-nine out of 33 (88%) children were successfully extubated as a result of the cricoid split procedure. Four children failed extubation after the cricoid split. Three required a tracheostomy and one child remained intubated for a prolonged period. CONCLUSION: The anterior cricoid split procedure has revolutionised the management of early subglottic stenosis in selected neonates with failed extubation. It is a safe operation, and if successful it avoids the formation of a tracheostomy.

Maturation sutures for the paediatric tracheostomy--an extra safety measure.

OBJECTIVE: Displacement of the tracheostomy tube in paediatric patients is a potentially fatal complication. We describe an extra safety measure which facilitates tube replacement. SETTING: Tertiary referral specialist paediatric centre. MATERIALS AND METHODS: The method involves the placement of sutures between the anterior tracheal wall and skin to hasten the formation of a mature stoma (maturation sutures). We also undertook a retrospective case note review on patients from an academic tertiary referral centre. Thirty-five notes were reviewed. The most common indication for tracheostomy was airway obstruction (65 per cent). Fourteen patients had early and 10 had late complications with three tube displacements occurring. No added complications due to the use of sutures were found. CONCLUSIONS: Our complication rates compare well with those in the literature, and we recommend considering the use of such a technique.

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.

Cricotracheal resection in children.

OBJECTIVE: To review our experience with cricotracheal resection in a pediatric population. DESIGN: Prospective case review of a cohort of patients undergoing cricotracheal resection. SETTING: Tertiary care pediatric hospital. PATIENTS: Forty-four consecutive patients undergoing cricotracheal resection between January 1, 1993, and December 31, 1998. MAIN OUTCOME MEASURES: Decannulation rates. RESULTS: Thirty-eight (86%) of the 44 children are decannulated. The ultimate decannulation rate was independent of the presenting grade of subglottic stenosis. Fourteen children (100%) had a primary cricotracheal resection; all are decannulated. Twenty-one children had a salvage cricotracheal resection, and 19 (90%) are decannulated. Nine children had an extended cricotracheal resection, of whom 5 (56%) are decannulated. A primary cricotracheal resection was performed on a child on whom no previous open airway procedure had been performed. A salvage cricotracheal resection was performed on a child on whom previous open airway reconstruction had not resulted in an adequate airway. An extended cricotracheal resection was performed on a child on whom the cricotracheal resection was combined with a second procedure, either additional expansion cartilage grafting or an open arytenoid procedure. Most of these children had complex airway pathologic conditions. CONCLUSION: Cricotracheal resection complements standard laryngotracheal reconstruction techniques in a pediatric population.

Topical mitomycin application after laryngotracheal reconstruction: a randomized, double-blind, placebo-controlled trial.

OBJECTIVE: To explore the effect of mitomycin treatment on the pediatric airway following laryngotracheal reconstruction. DESIGN: Randomized, double-blind, placebo-controlled trial. PATIENTS: Children aged 2 to 17 years with subglottic or upper tracheal stenosis undergoing laryngotracheal reconstruction at a single, tertiary care, children's hospital. INTERVENTION: At the time of extubation or stent removal, the children underwent bronchoscopy and 0.4 mg/mL (2 mL of a 0.2-mg/mL solution of either mitomycin or an equal volume of isotonic sodium chloride was directly applied to the subglottic region for a single application of 2 minutes. These children then underwent interval endoscopy at 2 weeks, 6 weeks, and 3 months postoperatively for assessment of their airways. RESULTS: Granulation tissue was graded on a scale of 0 (none) to 4 (near-total or total occlusion). Videotapes of endoscopies were independently observed and graded by 3 pediatric otolaryngology fellows with a subsequent interobserver agreement of 91.6%. The results were then dichotomized to represent a single cohort in which further surgical intervention would be required and another separate cohort in which further surgery would not be required. At the 1-year mark, interim analysis was performed by a Data Safety and Monitoring Committee. At this time, 13 children had been randomized to the mitomycin-treated arm of the study and 11 children to the placebo-treated arm. A 2-tailed Fisher exact test revealed a value of 1.00. The Data Monitoring and Safety Committee advised that the trial should be stopped because the distributions between the 2 populations were almost identical. CONCLUSION: We cannot reject the null hypothesis that a single topical dose of mitomycin exerts an equal benefit as does isotonic sodium chloride when applied to the pediatric airway after laryngotracheal reconstruction.

Single-stage laryngotracheal reconstruction in children: a review of 200 cases.

OBJECTIVE: We reviewed our experience with pediatric single-stage laryngotracheal reconstruction (SSLTR) to identify factors that affect postoperative outcomes, including the need for reintubation and tracheostomy. STUDY DESIGN: Retrospective chart review was done. RESULTS: In total, 190 children underwent 200 SSLTRs; 29% were reintubated, and 15% required postoperative tracheostomy. Currently, 96% are decannulated. The use of anterior and posterior costal cartilage grafting, age less than 4 years, sedation for more than 48 hours, a leak pressure around the endotracheal tube at greater than 20 cm H2O, and moderate/severe tracheomalacia significantly increased the rate of reintubation. The duration of stenting did not affect outcomes. Children with anterior and posterior grafts and those with moderate or severe tracheomalacia were more likely to need a postoperative tracheostomy. CONCLUSION: SSLTR can be effective for the treatment of pediatric laryngotracheal stenosis. Diligent preoperative assessment of the patient and the patient's airway and close postoperative care are important to the success of this operation.

Acquired total (grade 4) subglottic stenosis in children.

Pediatric acquired total subglottic stenosis (SGS) is a challenging problem. The management of these patients has evolved at our institution over the past 25 years. We conducted a retrospective study to evaluate the surgical management and outcomes of children with grade 4 SGS. Fifty-six patients have presented with acquired grade 4 SGS since 1981. The causes included previous surgery (34), prolonged intubation (15), bums (1), and unknown causes (6). Of the 56 patients, 44 (79%) were decannulated; 120 total procedures were performed, and 39 patients (70%) required more than 1 procedure for decannulation. The decannulation rate has risen from 67% in the 1980s to 86% in the 1990s. Patients who underwent cricotracheal resection (CTR) had a higher decannulation rate than patients who underwent laryngotracheal reconstruction (LTR) with anterior and posterior costal cartilage grafting (CCG) (92% versus 81%), and were less likely to need additional open procedures to achieve decannulation (18% versus 46%). The decannulation rate for children with grade 4 SGS has improved because of advances in surgical technique. Currently, the principal operations used at our institution are 1) CTR and 2) LTR with anterior and posterior CCG. There was a trend toward a higher decannulation rate in patients who underwent CTR, and they were less likely to require further reconstructive surgery before decannulation.

Pediatric fiberoptic endoscopic evaluation of swallowing.

Pediatric dysphagia is the presenting feature of many underlying diagnoses. Between July 1993 and July 1999, 643 fiberoptic endoscopic evaluations of swallowing (FEES) were performed on 568 patients. The median age of the population was 2.5 years (range, 3 days to 21 years). The principal medical and surgical diagnoses of the patients at the time of presentation to the FEES clinic were prospectively recorded: 36% of the patients presented with a diagnosis of structural abnormalities of the upper aerodigestive tract or airway; 26% with neurologic diagnoses; 12% with gastroenterological disorders; 8% with genetic syndromes; 7% with pulmonary dysfunction; 5% with prematurity; 3% with cardiovascular anomalies; and 2% with metabolic problems. The patients were classified according to the following feeding regimens: 9% normally fed; 38% orally fed with limitations; 13% orally fed, but with required supplemental tube feedings; and 40% prohibited from taking nutrition orally. The FEES enabled the following classification of feeding abnormalities: 15% had normal feeding; 56% exhibited behavioral abnormalities, including sensory-based feeding disorders; 15% exhibited structural abnormalities; 16% exhibited neurologic abnormalities; 1.5% exhibited metabolic abnormalities; and 0.5% exhibited cardiorespiratory abnormalities. The unique aspects of pediatric dysphagia are highlighted, and the role of FEES in the workup of this challenging aspect of pediatric otolaryngology is discussed.

Arytenoid prolapse as a consequence of cricotracheal resection in children.

Cricotracheal resection (CTR) is a technique introduced comparatively recently for treating severe laryngotracheal stenosis in children. The recognized complications of CTR include recurrent laryngeal nerve damage, anastomotic dehiscence, and restenosis. We describe a further complication of CTR, namely, prolapse of the arytenoid cartilage. The presentation may be late, with symptoms of shortness of breath on exertion and nocturnal stertor with a poor sleep pattern, or the prolapse may be an asymptomatic incidental finding. The diagnosis is performed with flexible nasopharyngoscopy with the patient unanesthetized, or with rigid endoscopy with the patient lightly anesthetized and spontaneously ventilating. The affected arytenoid cartilage is noted to prolapse anteriorly and medially with inspiration, partly obstructing the airway. If treatment is required, endoscopic laser partial arytenoidectomy is effective. In a series of 44 children who underwent CTR, 20 were noted to develop arytenoid prolapse after operation. Twelve were asymptomatic, and 8 required laser arytenoidectomy, 2 of whom now require continuous positive airway pressure for moderate supraglottic collapse.

Duration of stenting in single-stage laryngotracheal reconstruction with anterior costal cartilage grafts.

In single-stage laryngotracheal reconstruction (ss-LTR), the endotracheal tube is used as a stent. The optimal duration of stenting is not known. The stenting period requires a stay in the intensive care unit, as the patient is intubated. Sedation and, rarely, paralysis may be required. An analysis from a prospectively collected database was performed to investigate the effect of length of stenting on the outcome of ss-LTR. The outcomes used were reintubation rate and postoperative tracheostomy rate. Patients with anterior costal graft ss-LTR were selected, as they had undergone similar procedures and have similar stenosis types and grades. In 101 patients, the duration of stenting ranged from 2 to 14 days (mean, 7 days; SD, 2.6 days). No significant correlation was found between the number of days stented and the reintubation rate or the postoperative tracheostomy rate. Patients stented for longer than I week were an average of 15 months younger than those stented for less than 1 week; however, the stenosis grades for the two populations were equivalent. The differences in rates of reintubation (p = .68) and postoperative tracheostomy (p = .52) in these 2 groups were not significant. For patients undergoing ss-LTR with anterior costal cartilage grafts, no correlation was found between the number of days stented (intubated) and the reintubation rate or the postoperative tracheostomy rate.

Surgery for pediatric subglottic stenosis: disease-specific outcomes.

To set the foundation to develop a disease-based, operation-specific model to predict the outcome of pediatric airway reconstruction surgery, we performed a retrospective database review of children operated on at a single, tertiary-care children's hospital. Over the 12-year period 1988 to 2000, a total of 1,296 airway reconstruction procedures were performed. Out of these, charts were identified for 199 children who underwent laryngotracheal reconstruction for a sole diagnosis of subglottic stenosis. Children were excluded from the study if their disorder included supraglottic, glottic, or upper tracheal disease. The main outcome measures were Myer-Cotton grade-specific decannulation and extubation rates, including both operation-specific and overall results. There were 101 children who underwent double-stage laryngotracheal reconstruction. The operation-specific decannulation rates for Myer-Cotton grades 2, 3, and 4 were 85% (18/21), 37% (23/61), and 50% (7/14) (chi2 analysis, p = .0007). The overall decannulation rates were 95% (20/21), 74% (45/61), and 86% (12/14) (chi2 analysis, p = .04). There were 98 children who underwent single-stage laryngotracheal reconstruction. The operation-specific extubation rates for Myer-Cotton grades 2, 3, and 4 were 82% (37/45), 79% (34/43), and 67% (2/3) (chi2 analysis, p = .63). The overall extubation rates were 100% (45/45), 86% (37/43), and 100% (3/3) (chi2 analysis, p = .03). Logistic regression analysis showed no effect of age (less than or greater than 2 years of age) on operation-specific or overall outcome parameters. We conclude that laryngotracheal reconstruction for pediatric subglottic stenosis remains a challenging set of procedures in which multiple operations may be required to achieve eventual extubation or decannulation. Children with Myer-Cotton grade 3 or 4 disease continue to represent a significant challenge, and refinements of techniques are being examined to address this subset of children. Disease-based, operation-specific outcome statistics are the first step in the development of a meaningful predictive model.

Endoscopic drainage of pediatric paranasal sinus mucoceles.

Paranasal sinus mucoceles are rare in children. The traditional treatment is to perform surgical drainage via an external incision. In adults endonasal surgical techniques are increasingly being used to treat mucoceles. A series of seven children is reported. All cases were successfully treated with endoscopic intranasal surgical drainage without complication or recurrence. When used by surgeons familiar with endoscopic sinus surgery this has proved a safe and successful procedure that avoids facial scarring. Despite previous reports suggesting cystic fibrosis is the major aetiological factor in pediatric mucoceles, no child in this series had been diagnosed as suffering from cystic fibrosis.

KTP laser treatment of suprastomal obstruction prior to decannulation in paediatric tracheostomy.

OBJECTIVE: Decannulation following tracheostomy in the paediatric patient is often complicated by the development of secondary suprastomal obstruction. We describe the technique of bronchoscopic KTP laser therapy in the management of such conditions, and have audited the results of this treatment with 12 children treated with this modality before attempted decannulation over the last 2 years at Great Ormond Street Hospital for Children (GOSH). METHOD: Via a retrospective record review. RESULTS: Eight (67%) were successfully decannulated, with four being unsuccessful. All children with less than 50% suprastomal obstruction were successfully decannulated following bronchoscopic KTP laser treatment. CONCLUSIONS: Bronchoscopic KTP laser therapy is a useful tool in the abolition of suprastomal obstruction prior to decannulation following paediatric tracheostomy. Children with greater than 50% obstruction are likely to require an open procedure.

Cricotracheal resection as a primary procedure for laryngotracheal stenosis in children.

OBJECTIVE: Cricotracheal resection (CTR) is being increasingly used in the treatment of children with severe laryngotracheal stenosis. In this institution the majority of children are treated with CTR as a salvage procedure having undergone previous unsuccessful laryngotracheal reconstruction (LTR). Selected children have undergone CTR as a primary procedure (without previous LTR). The objective of this study is to examine the outcome for children undergoing cricotracheal resection as a primary procedure for severe laryngotracheal stenosis. METHOD: analysis from prospectively collected database. RESULTS: 17 patients underwent CTR without previous LTR or anterior cricoid split between October 1994 and September 1998. All the patients had grade 3 or 4 stenosis. After a minimum of 1 year follow up 15 children are decannulated. Five children required further surgery to achieve this. Two children still have tracheostomies. Both had extended procedures. One included bilateral arytenoid abduction for bilateral vocal cord paralysis in a patient with quadraparesis following transverse myelitis. The other child, who suffered from multiple congenital anomalies, underwent a concurrent posterior cricoid cartilage graft. Nine patients had good voice post-operatively, five had acceptable voice and three had weak or no voice. CONCLUSION: the early experience for CTR in children as a primary procedure achieved an overall decannulation rate of 88% after 1 year follow up in children with severe laryngotracheal stenosis. Five children required further surgery to achieve this. The voice outcome was variable. CTR is an alternative primary procedure to LTR for severe laryngotracheal stenosis in children. The relative indications for these procedures are discussed.