Palatopharyngeal sling: a new technique in treatment of velopharyngeal insufficiency

OBJECTIVE: (a) Pharyngeal flap and sphincter pharyngoplasty are the procedures most frequently chosen by craniofacial surgeons for surgical management of velopharyngeal insufficiency. Both operations may be complicated by obstructive breathing and even sleep apnea. (b) The purpose of this study is to evaluate the efficacy of a palatopharyngeal sling in the treatment of velopharyngeal insufficiency in cases with weak palatal mobility and its effect on breathing. METHODS: Seventeen cases of post-palatoplasty velopharyngeal insufficiency were subjected to treatment by palatopharyngeal sling. This sling, created by elevation of bilateral myomucosal flaps formed of palatopharyngeus and superior constrictor muscles, passed through palatal split, sutured together and to a raw area on the oral surface of the soft palate. Pre- and post-operative evaluation was carried out by perceptual speech analysis and flexible nasopharyngoscopy. Polysomnography was used to assess the effect of the operation on breathing. RESULTS: Complete closure was achieved in 13 cases (76.5%) while 4 cases showed incomplete closure (2 of them showed improvement when compared with the pre-operative video). The overall improvement of speech and velopharyngeal closure was 88.2%. Polysomnography showed no obstructive breathing. CONCLUSIONS: Palatopharyngeal sling is a useful technique for correction of velopharyngeal insufficiency in cases with little palatal motion and it carries no risk of obstructive sleep apnea.     

Resolving feeding difficulties with early airway intervention in Pierre Robin Sequence

OBJECTIVES/HYPOTHESIS: To observe rates of gastrostomy tube (g-tube) placement in Pierre Robin Sequence (PRS) and to determine whether relieving airway obstruction solves feeding difficulties. STUDY DESIGN: All PRS referrals to a multidisciplinary cleft team for children at a tertiary pediatric hospital from January 1988 to June 2006 were retrospectively reviewed. METHODS: Patients were analyzed for occurrence of g-tube placement, neurologic disorders, and airway intervention including tracheotomy and mandibular distraction osteogenesis. RESULTS: Sixty-seven PRS patients were divided into two categories: 51 (76.1%) isolated PRS (iPRS) and 16 (23.9%) with additional disorders and syndromes (sPRS). Patients were then placed into two subgroups: those who received early airway intervention and those who received late or no airway intervention. Of the 51 iPRS children, 12 (23.5%) received early airway intervention, none of whom required a g-tube. There were 39 (76.5%) children who received late or no airway intervention, and 5 (12.8%) of these required g-tube placement. Of the 16 sPRS children, 8 (50%) received early airway intervention, and 7 (87.5%) of these still required a g-tube. Of the remaining 8 (50%) sPRS patients who received late or no airway intervention, 5 (62.5%) required a g-tube. CONCLUSION: In children with iPRS, feeding difficulties can be resolved with early airway intervention. Delaying airway intervention may necessitate feeding assistance because all of the iPRS children who required a g-tube fell into this category. The presence of additional disorders and syndromes further complicates treatment because most of the sPRS children required g-tubes regardless of airway intervention.     

Subglottic stenosis: another challenge for intubation and potential mechanism of airway obstruction in Pierre Robin Sequence

Objective

To determine the endotracheal tube (ETT) size and presence of subglottic narrowing in children less than 1 year old with Pierre Robin Sequence (PRS).

Materials and methods

We performed a retrospective review from 2005 to 2009 of infants with PRS who underwent diagnostic laryngoscopy (DL) and intubation.

Results

Fifteen children with a median age of 25 days were reviewed. All patients, except one, were born full term. Subglottic narrowing was visualized in 5 patients (33%). 73% of all patients required an ETT size smaller than what is recommended by intubation guidelines in the literature in regards to normative data based on age and weight.

Conclusion

Infants with PRS may have a higher incidence of subglottic stenosis and require a smaller ETT compared to the normal population. This pilot study warrants a larger prospective investigation to validate these findings.     

Absence of the epiglottis in an infant with Pierre Robin sequence

Epiglottis anomaly associated with Pierre Robin sequence (PRS) is a rare occurrence. Most infants with PRS have presented life-threatening symptoms of respiratory distress and severe feeding problems that usually end with death. To the knowledge of the authors, this is the first reported case of epiglottis agenesis associated with PRS. The clinician must be aware of this unusual presentation in a PRS, and the presented case reveals the challenges in the treatment of the respiratory and feeding problems. This case is discussed with a review of the literature.     

Treatment of persistent post-adenoidectomy velopharyngeal insufficiency by sphincter pharyngoplasty

Objective

Persistent hypernasality after adenoidectomy is an infrequent problem in children with normal palate. However if it happened, it can render a child's speech unintelligible resulting in serious affection of social life. We aimed in this study to identify the causes of persistent post-adenoidectomy velopharyngeal insufficiency and to assess the efficacy of sphincter pharyngoplasty in the treatment of such problem.

Methods

This study was conducted on 18 patients complained of hypernasal speech following removal of their adenoids after variable periods of failed expected spontaneous improvement. Their hypernasality was rated as being mild, moderate and severe, all cases were subjected to conservative treatment in the form of speech therapy for 3 months to correct the problem, and patients that did not respond to speech therapy were subjected to surgical intervention in the form of sphincter pharyngoplasty. Velopharyngeal closure was assessed using flexible nasopharyngoscopy, while speech was assessed using auditory perceptual assessment and nasometry.

Results

Hypernasality was mild in 9 cases, moderate in 7 cases and severe in 2 cases. Flexible nasopharyngocopy showed occult submucous cleft in 5 cases, short palate in 2 cases, and deep nasopharynx in 3 cases. Speech improvement was achieved in 8 cases after completion of speech therapy program (all had mild hypernasality with no anatomical palatal defects). Ten patients that had palatal defects were subjected to sphincter pharyngoplasty, 8 of them showed complete recovery, while 2 cases with severe hypernasality showed partial improvement of their speech.

Conclusions

Persistent post-adenoidectomy velopharyngeal insufficiency may be due to anatomical abnormalities of the palate such as an occult submucous cleft, short palate or deep nasopharynx; such conditions may be overlooked during the preoperative preparation for adenoid removal. Speech therapy is an effective method in mild hypernasality especially if there is no anatomical abnormality, while surgical correction is usually needed in moderate and severe cases, and sphincter pharyngoplasty is a useful choice for those patients.     

Parapharyngeal neuroglial heterotopia in Pierre Robin sequence: MR imaging findings

Heterotopic neuroglial tissue is a rare lesion, occurring more frequently in the nasal cavities. Other rare locations are the orbit, the scalp, the palate, the pharynx, the parapharyngeal space and the lungs. They are usually detected occasionally because they are often asymptomatic, but sometimes they might present with dyspnoea, feeding difficulty, snorting and nasal flaring. Respiratory symptoms occur when heterotopic neuroglial tissue is located in the parapharyngeal space. We report a case of an infant affected by Pierre Robin sequence (PRS) who was admitted to our Institution for a worsening respiratory distress that was not explainable only by PRS.     

Teaching Furlow palatoplasty: The sticky note method

ObjectiveThe double-opposing Z-plasty (Furlow palatoplasty) procedure is a well-established method for palate repair in children. We propose a simple and easily accessible sticky note model to demonstrate the lengthening in palatal anatomy afforded by this technically challenging procedure.MethodsOur model involves creating a lengthened three-dimensional representation of the Z-plasty through making specified incisions and rearrangements of the palatal layers. The sticky note model was made a total of 20 times and length of the palate model pre and post Z-plasty was measured.ResultsThe average length of the palate pre-procedure was 72 mm. The average length of the palate post procedure was 78.9 mm, showing an increase of 6.9 mm (9.6%).ConclusionOur model provides an accurate and valuable educational tool that will aid in the visualization and understanding of the Furlow palatoplasty procedure.     

Evaluation of the efficacy of tongue-lip adhesion in Pierre Robin sequence

ObjectiveTongue-lip adhesion may be used to relieve obstructive sleep apnoea in infants with Pierre Robin sequence (PRS), but only a few studies have objectively evaluated its efficacy. The purpose of this study was to evaluate the results of tongue-lip adhesion by polysomnography.Materiel and methodsA single-centre retrospective study was conducted in infants with PRS treated by tongue-lip adhesion from 2004 to 2015, in whom at least laryngotracheal endoscopy and polysomnography were performed. The variables collected were the syndromic diagnosis, demographic data, respiratory management before tongue-lip adhesion, symptoms, and additional airway interventions. Obstructive sleep apnoea was classified into 3 groups according to severity. Polysomnography was performed one month after tongue-lip adhesion. Statistical analysis was performed with Wilcoxon signed-rank test with a limit of statistical significance of P < 0.005.ResultsThirty-seven subjects in whom tongue-lip adhesion was performed at a mean age of 45 days (8 to 210 days) were included. Thirty-one patients had isolated PRS and 6 patients had associated anomalies. All patients had confirmed severe obstructive sleep apnoea. All patients required respiratory support prior to surgery: 8 intubated patients, 15 patients with noninvasive ventilation and 14 patients with nasopharyngeal airways. Eight patients had bradycardia before tongue-lip adhesion. All parameters were improved on postoperative polysomnography: oxygen saturation, hypercapnia, apnoea-hypopnoea index, bradycardia (P < 0.005). Only 8 patients had persistent severe obstructive sleep apnoea and required tracheostomy (n = 5) or noninvasive ventilation (n = 3). No significant correlation was observed between treatment success and any predictive variables.ConclusionTongue-lip adhesion improved airway obstruction in all infants with PRS and resolved obstructive sleep apnoea in 29 patients. However, 8 patients required additional airway interventions.     

Trans-oral endoscopic cerclage pharyngoplasty for treatment of velopharyngeal insufficiency

Objectives

Velopharyngeal insufficiency (VPI) is a common problem after cleft palate repair, it leads to speech distortion with consequent affection of speech intelligibility. Many techniques have been used in the treatment of VPI with varying results and complications. The aim of this study was to evaluate the efficacy of trans-oral endoscopic cerclage pharyngoplasty in the treatment of VPI.

Methods

Eighteen patients with hypernasality after palatoplasty were subjected to trans-oral endoscopic cerclage pharyngoplasty. Pre and postoperative evaluation of velopharyngeal function were performed by using auditory perceptual assessment, nasometric assessment, and flexible nasopharyngoscopy.

Results

Significant postoperative improvement of speech parameters measured with auditory perceptual assessment were achieved, and the overall postoperative nasalance score was improved significantly for nasal and oral sentences. Also, flexible nasopharyngoscopy showed significant improvement of velopharyngeal closure. No marked postoperative complications were reported apart from throat pain and dysphagia that disappeared with time.

Conclusions

Trans-oral endoscopic cerclage pharyngoplasty is an effective method for the treatment of VPI.     

A clinical narrative review of mandibular distraction osteogenesis in neonates with Pierre Robin sequence

Introduction

Neonatal upper airway obstruction secondary to micrognathia can be managed with conservative or surgical interventions. Traditionally, severe upper airway obstruction was managed with a tracheostomy. Although tracheostomy may be life saving, it is associated with high rates of complications and can lead to developmental problems. More recently, mandibular distraction osteogenesis has been utilized to relieve micrognathia associated airway obstruction.

Methods

A clinical narrative review of the current literature was performed to evaluate the efficacy of mandibular distraction osteogenesis in neonates with Pierre Robin sequence.

Objectives

(1) To evaluate whether mandibular distraction osteogenesis can relieve the upper airway obstruction in micrognathic neonates and (2) to discuss and increase the awareness of various issues surrounding neonatal mandibular distraction procedures including preoperative workup, distraction protocols, and complications.

Results

Mandibular distraction osteogenesis can be a safe and effective intervention in neonates diagnosed with Pierre Robin sequence with severe micrognathia and airway obstruction. Interestingly, in patients with additional complex syndromes, the airway obstruction was not consistently alleviated.

Conclusion

When conservative measures fail, mandibular distraction osteogenesis should be considered to obviate the need for a tracheostomy in newborns with micrognathia associated upper airway obstruction.     

Partial adenoidectomy by suction diathermy in children with cleft palate, to avoid velopharyngeal insufficiency

Objectives

Adenoidectomy is indicated for the relief of paediatric nasal obstruction, sleep-disordered breathing and otitis media with effusion (OME). Velopharyngeal insufficiency (VPI) is a rare complication. The main risk factor is the presence of pre-existing velopharyngeal closure-impaired mechanisms, including submucosal or overt cleft palate. Despite possible benefits, adenoidectomy is frequently withheld in such children to avoid VPI. This study aims to demonstrate the efficacy and safety of partial adenoidectomy using suction diathermy in children who previously underwent overt cleft palate repair during infancy, to allow selective resection of tissue and symptom resolution without producing VPI.

Methods

Since 1994, 18 patients with previously corrected overt cleft palate have undergone partial adenoidectomy at this centre, for the treatment of nasal obstruction or sleep-disordered breathing, with or without OME. Three had existing VPI following their cleft correction surgery. Selective resection of the adenoid was performed transorally under indirect vision, using a malleable suction coagulator. This allowed exposure of the posterior choanae, leaving the remaining adenoid bulk intact.

Results

Patients were followed up at 4 weeks, and subsequently at regular intervals (total follow up 30-180 months, median 92 months), including perceptual speech assessment in all cases. All demonstrated symptomatic improvement with respect to the original indications for surgery. None developed worsening hypernasal speech or other features of VPI, and there were no cases of symptomatic adenoidal re-growth.

Conclusions

Partial adenoidectomy, employing a variety of methods, has been used successfully in children with submucosal cleft palate. This study demonstrates the safe and effective use of suction diathermy to enable partial adenoidectomy in children who have previously undergone surgical correction of overt cleft palate, allowing symptom resolution without producing VPI.     

Pierre Robin sequence: An institutional experience in the multidisciplinary management of airway, feeding and serous otitis media challenges

Objectives

To evaluate the course and prognosis of airway obstruction, feeding difficulties and hearing abnormalities in patients with Pierre Robin sequence (PRS).

Methods

A retrospective review was conducted, of 69 patients with PRS, attending between 1991 and 2010 at the Children's University Hospital in Dublin. Data regarding airway management, nutritional status and hearing difficulties was collected prospectively.

Results

Airway obstruction requiring intervention other than positional therapy was seen in 39% (27) patients. Fifty nine percent (16/27) of these patients, who failed positional therapy, were successfully managed with a nasopharyngeal airway. Following failed intervention with nasopharyngeal airways, two patients had airway maintenance achieved with a successful glossopexy procedure. One patient had an adequate airway achieved with nasal continuous positive airway pressure. Eight patients (12%) required a surgical tracheostomy. Of those who required a tracheostomy, six patients had isolated PRS, one patient had PRS in association with Stickler syndrome and one patient had Nager acro-facial dystosis. Duration of tracheostomy tube ranged from 10 to 19 months, mean 13 months.Seventy percent (48 patients) required supplementary feeding in the form of nasogastric (NG) or gastrostomy tube. Forty-four patients were successfully managed with a temporary NG tube. One patient required a prolonged NG tube, and three required a gastrostomy tube. Twenty-one (30%) patients were successfully managed with a specialised Haberman bottle. Twenty-four patients (35%) who had their airway managed successfully by positional therapy, still required supplemental feeding.Thirty-one patients (45%) demonstrated a conductive hearing loss at some stage, which affected their speech and language development. Twenty-four patients (35%) required tympanostomy tube insertion once, while 7 (10%) of patients required ventilation tube insertion twice or more.

Conclusion

Airway management in the majority of PRS can be successfully achieved by conservative methods. Even in the presence of an adequate airway, many patients will require supplemental feeding. Early audiological assessment is necessary as many patients will need tympanostomy tube placement to ensure adequate speech and language development.     

Primary aerodigestive presentations of Pierre Robin sequence/complex and predictive factors of airway type and management

Objective

To document the mode and age of primary aerodigestive presentation of Pierre Robin sequence/complex (PRS/C) children to the otolaryngologist and to explore predictive factors of upper airway type and management.

Methods

This is a retrospective cohort study conducted in a tertiary pediatric referral center. A prospective surgical database was searched for children who were diagnosed with PRS/C. Demographics, presenting complaint, secondary diagnoses, type of upper airway obstruction, secondary airway lesions, presence of cleft palate, and airway interventions were collected. Multiple linear regression analysis was performed to predict upper airway obstruction type and intervention.

Results

Seventy-seven potentially eligible patients were identified. Forty-six were included (20 females). Mean age at presentation was 20.4 ± 36.9 months (range 1–191.25 months). Twenty-three primarily presented with respiratory failure, 14 with sleep disordered breathing, and nine with swallowing dysfunction. Children with presentations other than respiratory failure were older (p = 0.004). Nineteen were syndromic. Overt cleft palate was more common in those presenting with respiratory failure (p = 0.01). The type of airway obstruction encountered and use of tracheostomy were positively predicted by the primary presenting feature of respiratory failure (p < 0.05) and male gender (p < 0.05).

Conclusion

A substantial number of PRS/C patients present later than the neonatal period with presentations other than respiratory failure. Both male gender and presentation with respiratory failure predicted a more severe airway obstruction type and the need for trachesotomy.     

Lateral pharyngeal flap with a double pedicle for repair of unilateral velopharyngeal insufficiency

ObjectivesConventional pharyngeal flap surgery, which closes the median portion of the velopharynx, has been performed for dysarthria patients with velopharyngeal insufficiency (VPI). However, for VPI due to unilateral pharyngeal paralysis, median closing disrupts pharyngeal contraction of the unaffected side and allows pharyngeal pressure to escape through the nose at the lateral portion of the affected side during speech and swallowing. The purpose of this study was to evaluate the effectiveness of lateral pharyngeal flap (LPF) surgery for unilateral VPI.MethodsSeven patients with unilateral VPI (five males and two females with an average age of 54 years) underwent LPF surgery combined with other transoral surgeries for dysphagia. The LPF surgical technique was as follows: after the laterocaudal-based pedicle flap of the soft palate and cranial-based pedicle flap of the posterior pharyngeal wall on the affected side were transorally elevated, each mucosal pedicle flap was sutured together. Functional oral intake scale (FOIS) scores and swallowing pressure before and after surgery were compared.ResultsUnilateral velopharyngeal closure preserved nasal breathing after LPF surgery in all patients. Rhinolalia aperta improved postoperatively in all patients except one. The mean FOIS scores were 2.3 preoperatively and 3.7, 5.3, and 5.9 at 2 weeks, 1 month, and 6 months postoperatively, respectively. The mean pressures significantly increased at the velopharynx (from 49 ± 30 mmHg to 92 ± 45 mmHg) and oropharynx (from 48 ± 18 mmHg to 66 ± 15 mmHg) six months after the surgery.ConclusionLPF surgery leaving the unaffected side intact can be an effective surgical procedure for patients with unilateral VPI.