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Endoscopic Nd:Yag and Bleomycin Injection for the management of a Hypopharyngeal Venous Malformation

Venous malformations (VM) are congenital lesions, frequently affecting the head and neck, with poor respect for tissue planes. Established treatments include observation, sclerotherapy, laser, and surgical resection.1

Lesions affecting the upper airways present unique challenge due to frequent unresectability and difficult access/exposure for alternative standard treatments. We describe our approach of standard endoscopic airway techniques for the administration of advanced treatment modalities including simultaneous laser and sclerotherapy for an extensive airway VM.

Our patient is a 16-year-old female with an extensive multi-spatial VM with associated airway obstruction. The patient suffered from severe obstructive sleep apnea (OSA) and continuous positive airway pressure (CPAP) dependence as a result of airway compression. Direct laryngoscopy and bronchoscopy demonstrated extensive venous staining and large vascular channels of the hypopharynx. Lumenis Nd:Yag laser (Yokneam, Israel) via 550 micron fiber was passed under telescopic visualization. Treatment via previously described “polka dot” technique was performed (15W, 0.5 pulse duration) with immediate tissue response. The largest vascular channel was accessed via 25-gauge butterfly needle. Immediate return of blood following lesion puncture confirmed intralesional placement. Reconstituted bleomycin (1 U/kg; max dose = 15 U per treatment) was injected and hemostasis achieved with afrin pledgets. The patient was intubated overnight. She was extubated the next morning and advanced to a regular diet, discharging post-operative day two. Post-operative flexible laryngoscopy demonstrated significant improvement in the treatment areas, and follow up sleep study demonstrated sleep apnea resolution with liberation of her CPAP therapy.

Anterior cervical tracheoplasty using thyroid ala cartilage graft

Acquired tracheomalacia in the form of suprastomal collapse may occur as a complication of long-term tracheotomy dependence. Prolapse of the weakened suprastomal segment of trachea during inspiration may prevent safe decannulation. Management of such an issue may require a secondary surgical procedure such as anterior tracheoplasty.2 In 2001, Forte et al described the use of thyroid ala cartilage as a reliable cartilage source for anterior augmentation laryngotracheal reconstruction in neonates. This technique may yield a favorable result given similar thickness of the cartilages and use of a single incision operation for airway reconstruction.1 Here, we present a modification of the procedure described by Forte for anterior cervical tracheoplasty for the indication of suprastomal collapse preventing decannulation. The procedure begins with nasotracheal intubation and excision of tracheostomy tract and stoma. Strap muscles are then divided to expose the laryngotracheal cartilages. Cartilages are divided at the midline anteriorly, and the diseased segment of anterior trachea is discarded. The defect is measured, and if the size match is favorable, the superior thyroid alar cartilage is harvested. The resulting cartilage graft is slightly larger than the tracheal defect and is placed so that the perichondrium is facing into the airway lumen. Interrupted sutures of 4-0 vicryl are used to inset the graft in a submucosal fashion. Once the graft is secured with sutures, a Valsalva maneuver is performed after the cuff of the endotracheal tube is taken down to assure no leak. Strap muscles are reapproximated, a Penrose drain is placed, and the skin is closed. The child is kept intubated and sedated for 3 days before subsequent extubation in the intensive care unit. A bronchoscopy is performed at the 6-week postoperative interval to assure successful healing and to remove any persistent granulation tissue if present.

Pediatric Tracheostomy

Paediatric Tracheostomy

Position the child with chin extension appropriately
Drape the child as shown in the video
Mark the incision line
Use 15 number blade for skin incision
Remove the excessive subcutaneous fat tissue
Find the median raphe and strap muscles
Retract the strap muscles laterally
Identify the tracheal ring
Create the impression of tube for appropriate size incision
Place the stay sutures as shown in the video
incise the trachea with 11 number blade
Secure the maturation sutures
Insert the tracheostomy tube
Confirm the position and then inflate the cuff
Secure the ties and dressing at the end.

Microdebrider Assisted Lingual Tonsillectomy

Adrian Williamson, Michael Kubala MD, Adam Johnson MD PhD, Megan Gaffey MD, and Gresham Richter MD

The lingual tonsils are a collection of lymphoid tissue found on the base of the tongue. The lingual tonsils along with the adenoid, tubal tonsils, palatine tonsils make up Waldeyer’s tonsillar ring. Hypertrophy of the lingual tonsils contributes to obstructive sleep apnea and lingual tonsillectomy can alleviate this intermittent airway obstruction.1,2 Lingual tonsil hypertrophy can manifest more rarely with chronic infection or dysphagia. A lingual tonsil grading system has been purposed by Friedman et al 2015, which rates lingual tonsils between grade 0 and grade 4. Friedman et al define grade 0 as absent lingual tonsils and grade 4 lingual tonsils as lymphoid tissue covering the entire base of tongue and rising above the tip of the epiglottis in thickness.3

Lingual tonsillectomy has been approached by a variety of different surgical techniques including electrocautery, CO2 laser, cold ablation (coblation) and microdebridement.4-9 Transoral robotic surgery (TORS) has also been used to improve exposure of the tongue base to perform lingual tonsillectomy.10-13 At this time, there is not enough evidence to support that one of these techniques is superior.

Here, we describe the microdebrider assisted lingual tonsillectomy in an 8 year-old female with Down Syndrome. This patient was following in Arkansas Children’s Sleep Disorders Center and found to have persistent moderate obstructive sleep apnea despite previous adenoidectomy and palatine tonsillectomy. Unfortunately, she did not tolerate her continuous positive airway pressure (CPAP) device. The patient underwent polysomnography 2 months preoperatively which revealed an oxygen saturation nadir of 90%, an apnea-hypopnea index of 7.7, and an arousal index of 16.9. There was no evidence of central sleep apnea. The patient was referred to otolaryngology to evaluate for possible surgical management.

Given the severity of the patient’s symptoms and clinical appearance, a drug induced sleep state endoscopy with possible surgical intervention was planned. The drug induced sleep state endoscopy revealed grade IV lingual tonsil hypertrophy causing obstruction of the airway with collapse of the epiglottis to the posterior pharyngeal wall. A jaw thrust was found to relieve this displacement and airway obstruction. The turbinates and pharyngeal tonsils were not causing significant obstruction of the airway. At this time the decision was made to proceed with microdebrider assisted lingual tonsillectomy.

First, microlaryngoscopy and bronchoscopy were performed followed by orotracheal intubation using a Phillips 1 blade and a 0 degree Hopkins rod. Surgical exposure was achieved using suspension laryngoscopy with the Lindholm laryngoscope and the 0 degree Hopkins rod. 1% lidocaine with epinephrine is injected into the base of tongue for hemostatic control using a laryngeal needle under the guidance of the 0 degree Hopkins rod. 1. The 4 mm Tricut Sinus Microdebrider blade was set to 5000 RPM and inserted between the laryngoscope and the lips to resect the lingual tonsils. Oxymetazoline-soaked pledgets were used periodically during resection to maintain hemostasis and proper visualization. A subtotal lingual tonsillectomy was completed with preservation of the fascia overlying the musculature at the base of tongue.

She was extubated following surgery and there were no postoperative complications. Four months after postoperatively the patient followed up at Arkansas Children’s Sleep Disorders Center and was found to have notable clinical improvement especially with her daytime symptoms. A postoperative polysomnography was not performed given the patient’s clinical improvement.

Congenital Nasal Pyriform Aperture Stenosis (CNPAS): Sublabial Approach to Surgical Correction

Congenital nasal pyriform aperture stenosis (CNPAS) is defined as inadequate formation of the pyriform apertures forming the bony nasal openings resulting in respiratory distress and cyanosis soon after birth. Some clues such as worsening distress during feeding and improvement during crying may indicate a nasal cause of respiratory distress rather than distal airway etiology. Inability or difficulty passing a small tube through the nasal cavities may suggest CNPAS. The presenting clinical features of CNPAS can be similar to other obstructive nasal airway anomalies such as choanal atresia. Diagnosis is confirmed via CT scan with a total nasal aperture less than 11mm.

CNPAS may occur in isolation or it may be a sign of other developmental abnormalities such as holoprosencephaly, anterior pituitary abnormalities, or encephalocele. Some physical features of holoprosencephaly include closely spaced eyes, facial clefts, a single maxillary mega incisor, microcephaly, nasal malformations, and brain abnormalities (i.e. incomplete separation of the cerebral hemispheres, absent corpus callosum, and pituitary hormone deficiencies). It is important to rule out other associated abnormalities to ensure optimal treatment and intervention.

Conservative treatment of CNPAS includes humidification, nasal steroids, nasal decongestants and reflux control. Failure of conservative treatment defined by respiratory or feeding difficulty necessitates more aggressive intervention. The most definitive treatment for CNPAS is surgical intervention to enlarge the pyriform apertures.

Contributors:

Adam Johnson MD, PhD
Abby Nolder MD

Mandibular Distraction for Micrognathia in a Neonate

Introduction

Patients with Pierre-Robin Sequence (PRS) suffer from micrognathia, glossoptosis, and upper airway obstruction, which is sometimes associated with cleft palate and feeding issues. To overcome these symptoms in our full-term male neonate patient with PRS, mandibular distraction osteogenesis was performed.

Methods

The patient was intubated after airway endoscopy. A submandibular incision was carried down to the mandible. A distractor was modified to fit the osteotomy site that we marked, and its pin was pulled through an infrauricular incision. Screws secured the plates and the osteotomy was performed. The mandible was distracted 1.8 mm daily for twelve days.

Results

During distraction, the patient worked with speech therapy. Eventually, he adequately fed orally. He showed no further glossoptosis or obstruction after distraction was completed.

Conclusion

In our experience, mandibular distraction is a successful way to avoid a surgical airway and promote oral feeding in children with PRS and obstructive symptoms.

By: Ravi W Sun, BE

Surgeons:

Megan M Gaffey, MD

Adam B Johnson, MD, PhD

Larry D Hartzell, MD

Department of Otolaryngology – Head and Neck Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Arkansas Children’s Hospital, Little Rock, AR, USA

Recruited by: Gresham T Richter, MD

Superiorly Based Pharyngeal Flap for Velopharyngeal Dysfunction

Velopharyngeal dysfunction (VPD) refers to the improper control of airflow through the nasopharynx. The term VPD denotes the clinical finding of incomplete velopharyngeal closure. Other terms used to describe VPD include velopharyngeal insufficiency, inadequacy and incompetence. However, the use of VPD has gained popularity over these terms as they may be used to infer a specific etiology of impaired velopharyngeal closure.1

Control of airflow through the nasopharynx is dependent on the simultaneous elevation of the soft palate and constriction of the lateral and posterior pharyngeal walls. Disruptions of this mechanism caused by structural, muscular or neurologic pathology of the palate or pharyngeal walls can result in VPD. VPD can result in a hypernasal voice with compensatory misarticulations, nasal emissions and aberrant facial movements during speech.2

The assessment of velopharyngeal function is best preformed by a multispecialty team evaluation including speech-language pathologists, prosthodontists, otolaryngologists and plastic surgeons. The initial diagnosis of VPD is typically made with voice and resonance evaluation conducted by a speech-language pathologist. To better characterize the patient’s VPD, video nasopharyngeal endoscopy or speech videofluoroscopy can be used to visualize the velopharyngeal mechanism during speech.

VPD may first be managed with speech-language therapy and removable prostheses. For those who are good surgical candidates and do not fully respond to speech-language therapy, surgical intervention may be pursued.

Surgical management of VPD is most commonly accomplished by pharyngeal flap procedures or sphincter pharyngoplasty. In this video, a superiorly based pharyngeal flap with a uvular mucosal lining flap was preformed for VPD in a five-year-old patient with 22q11 Deletion Syndrome and aberrantly medial internal carotid arteries.

Neonatal Mandibular Distraction Osteogenesis with Multivector External Devices

Pierre Robin sequence (PRS) is a craniofacial malformation characterized by micrognathia and glossoptosis, with or without cleft palate. A subset of infants with PRS will suffer from airway obstruction severe enough to merit surgical intervention. Surgeries for PRS include tongue lip adhesion, tracheotomy, gastrostomy, and bilateral mandibular distraction osteogenesis. Distraction osteogenesis refers to a process in which a bone is lengthened after an initial osteotomy by means of separating the two resulting segments slowly over time. In the neonatal mandible, hardware used for distraction may be implanted beneath the skin or affixed externally. Each device has its advantages and disadvantages, however external devices are less expensive, do not typically require preoperative computed tomography scanning, may be adjusted easily throughout the distraction process, and are easily removed following consolidation, avoiding a second invasive procedure and lengthy anesthetic. This video presents the technique of neonatal mandibular distraction osteogenesis using multivector external distractors.

Endoscopic Grade 4 Subglottic Stenosis

We describe the management of a grade 4 subglottic stenosis, which was successfully performed endoscopically. This is the case of a 17 year-old female, tracheostomy dependent, with a complex history of failed open airway surgeries. Patient was referred to our center for a second opinion for decannulation. We found a grade 4 subglottic stenosis at her initial evaluation with a prolapsed anterior graft. Patient and family requested an endoscopic procedure, trying to avoid another open surgery. It was decided that an endoscopic procedure would be attempted. Patient was placed into suspension, and using alligator forceps, the stenotic area was probed until communication could be made with the distal tracheal. Using a series of balloon dilations and the microdebrider, a suprastomal stent could be endoscopically placed. Stent was removed 6 weeks later and showed a patent airway. Patient then underwent a series of 4 dilations and was successfully decannulated, just before graduating from college.

Endoscopic Repair of Type IIIB Posterior Laryngeal Cleft

We present a case of a type IIIB posterior laryngeal cleft treated successfully with endoscopic repair.

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