Cricophayrngeal Myotomy and Hypopharyngeal Diverticulotomy in the Pediatric Patient

Introduction:

Cricopharyngeal dysfunction (CPD) is a spectrum disorder encompassing multiple entities that ultimately result in dysphagia as a result of disruption of the normal anatomy or physiology of upper esophageal sphincter. It is a known and well described cause of dysphagia in adults, however, it’s role in pediatric dysphagia is less clear and limited to mostly small case series.1 Despite it’s relatively low prevalence, the complex pediatric otolaryngologist must be aware of this entity and it’s management. We discuss a complex case of CPD with an associated cricopharyngeal bar and pharyngeal diverticulum, as well as our successful endoscopic surgical approach highlighting the principles of CPD management in children.

Case Presentation:

We present a 21 month of female with a history of DiGeorge Syndrome and oropharyngeal dysphagia. Despite appropriate conservative measures including feeding therapy and diet thickening modification, as well as attempted Botox injection, the patient continued to demonstrate dysphagia. It was also noted on her swallow study that she had a posteriorly based pharyngeal diverticulum that potentially served as an aspiration reservoir. The decision was made to proceed with endoscopic cricopharyngeal division and diverticulum marsupialization.  

Technique:

With the patient intubated, a Lindholm laryngoscope was placed posteriorly into the hypopharynx, elevating the larynx and allowing visualization of the upper esophageal sphincter and isolation of the cricopharyngeal bar. A non- contact CO2 laser fiber at 2W continuous spray was then used to divide the cricopharyngeal bar layer by layer making sure to isolate the muscle and not create a pharyngotomy. Standard laser safety precautions were followed. Tension was maintained using a right-angle hook allowing for optimal laser division. This was continued until the entirety of the bar was divided. At this point, the posterior pharyngeal diverticulum was identified. Again, with the use of a right angle probe for traction and depth assessment, The anterior wall of the diverticulum was divided. This was continued until the diverticulum was fully marsupialized and in continuity with the posterior pharyngeal wall into the esophageal inlet.

Post operatively the patient was extubated and observed overnight in the hospital

Swallow study three weeks later demonstrated normalization of the flow of bolus through the UES as well as resolution of the previously seen diverticulum.

Conclusion:

Cricopharyngeal Dysfunction (CPD) is an uncommon but recognized cause of pediatric dysphagia with multiple treatment options of varying success. Endoscopic CO2 laser division is a viable and effective treatment option for this condition.

Pediatric Endoscopic Butterfly Inlay Tympanoplasty

Educational/Technical Point(s): Endoscopic butterfly inlay tympanoplasty is a reliable and useful technique in select pediatric patients. This technique prevents the need for flap elevation, intratympanic myringosclerosis excision that could result in significantly larger perforation, or malleus dissection which can result in permanent hearing reduction. The procedure has a shorter operative time than traditional techniques and excellent results in pediatric patients.

Introduction:

Butterfly inlay tympanoplasty is a more recently described but validated technique for repairing select tympanic membrane perforations.1 Following its validation in adult patients, small series have demonstrated its successful use in the pediatric population as well, including via endoscopic approach. 2 Despite these findings, the indications for when to use this repair technique remain nebulous. We discuss our institution’s approach to the use of this technique and factors that influence its implementation through a case presentation.

Case Presentation:

We present a 14-year-old female with a history of long standing anterior tympanic membrane perforation. She was seen in consultation at our quaternary children’s hospital with a remote history of ear tube placement, subsequent extrusion, and ongoing perforation. Audiometry revealed a moderate conductive hearing loss and large volume type B tympanogram. Examination demonstrated an ~30% anterior central clean dry perforation. Her perforation was anterior to the handle of the malleus and demonstrated a significant intratympanic myringosclerotic plaque adjacent to the perforation. Given the location, and adjacent plaque whose removal would have resulted in nearly the double the size of the perforation, endoscopic butterfly inlay technique was recommended.

Technique:

The patient was brought to the operating room and injected and prepped in standard fashion including injection of local anesthesia to the donor tragal site. The perforation was rimmed using a Rosen needle and the subsequent tissue removed with cup forceps. Following recipient site preparation, the perforation was measured using a standard right angle hook whose length is 3 mm demonstrating a 4 mm by 3 mm perforation.

Attention was turned to harvesting a tragal graft in standard fashion. Using a 5 mm dermal punch, a full thickness portion of the cartilage was obtained ex vivo and the residual cartilage was replaced into the donor site for any future needs and the wound closed in simple interrupted fashion. The cartilage was scored circumferentially with a 15 blade creating locking flanges for the graft. The graft was then placed via alligator. The graft was purposefully placed through the perforation into the middle ear cleft, and then retracted by its perichondrium into the perforation, essentially “locking” it into place. Additional flange adjustments were made using a Rosen needle to ensure the graft was seated, appropriately. The tympanic membrane was coated with bacitracin and the patient was awoken from anesthesia.

Standard post operative tympanoplasty care was recommended including dry ear precautions and avoidance of heavy physically exercise until her post operative follow up. At follow up, she demonstrated 100% graft take and resolution of her prior hearing loss with a mobile tympanic membrane.

Conclusion:

Endoscopic butterfly inlay tympanoplasty is a reliable and useful technique in select pediatric patients. This technique prevents the need for flap elevation, intratympanic myringosclerosis excision that could result in significantly larger perforation, or malleus dissection which can result in permanent hearing reduction. The procedure has a shorter operative time than traditional techniques and excellent results in pediatric patients.

Posterior Pharyngeal Flap for Large Gap Velopharyngeal Insufficiency

Velopharyngeal insufficiency (VPI) is a speech disorder characterized by inability for the palate (velum) to contact the posterior pharyngeal wall resulting in nasal air escape and subsequent speech abnormalities. All but the mildest cases are treated surgically, with technique chosen based on the closure pattern and gap size reserving the pharyngeal flap for the most severely affected patients. We present a 6-year old female with developmental delay and VPI with large (~60%) coronal pattern velopharyngeal gap subsequently deemed a candidate for posterior pharyngeal flap.

Following Dingman mouthgag placement, the posterior wall is inspected and palpated ensuring no carotid medialization. The flap is designed and marked as wide and long as possible to reduce tension. Local injection wis performed. Using an angled needle tip Bovie, the flap was then elevated in the plane the prevertebral fascia to the level of the nasopharynx. The donor site is closed with simple interrupted 4.0 chromic sutures. The palatal mucosa is divided in a T-shaped fashion, without violating palatal musculature. The flap is inset with horizontal mattress sutures using 4.0 chromic. The nasal ports are inspected frequently to ensure adequate nasal airway patency. The palatal mucosa is reapproximated and any residual donor site closed.  The patient is observed overnight, discharged home post-operative day 1, maintained on a soft diet for two weeks and abstains from speech therapy for 4 weeks to allow healing. Follow up demonstrated excellent healing well and VPI resolution on repeat speech sample.

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.

Submental Intubation

Presented is a case of submental intubation performed prior to maxillomandibular advancement for the treatment of obstructive sleep apnea. Submental intubation is a viable alternative to tracheostomy for cases in which nasal intubation is contraindicated (e.g. trauma), or uninterrupted access to the oral cavity is preferred. [1] Briefly, the technique consists of performing oral intubation, and then exteriorizing the endotracheal tube through a tract created from the floor of mouth to the submental triangle. At the end of the case, the tube can be passed into the oral cavity, returning to an oral intubation.

Surgeon: Raj C. Dedhia, MD, MSCR, Department of Otolaryngology, Emory University School of Medicine

Video Production: Clara Lee, MS4, Emory University School of Medicine

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