RRPF
With our voices united, we can and will work to end the burden of this disease on patients and caregivers.
In 1991, after the diagnosis of Bill & Marlene Stern's two-year-old daughter, they resolved to learn as much about RRP as possible. The two scoured medical literature to identify institutions and individuals with expertise in RRP. As parents, they recognized the need to find other families fighting this rare, chronic, and possibly fatal disease. In the absence of a formalized organizations to unite RRP patients and researchers, the RRPF was founded. What saved their family was finding other parents obsessively listening for their infants' breathing, struggling to decide when to schedule surgery, walking down antiseptic hallways for surgery upon surgery, and helping their children navigate the sandbox with only a whisper.
What inspired Bill & Marlene was finding clinicans/researchers dedicating their lives to understanding and eradicating RRP. They were fortunate to have another RRP family join them in their journey as they worked to build the RRPF, Henry and Susan Woo, whose daughter was a RRP patient. Their daughter went on to become a MD who planned to focus on RRP, and sadly at the age of 31, Jennifer passed away due to complications arising from pulmonary RRP (RRPF President at the time of her passing).
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Anna Celeste Gibson, B.S., Mariah Small, M.D., Gresham Richter, M.D.
University of Arkansas for Medical Sciences, Arkansas Children’s Hospital
Introduction:
A papilloma is a benign tumor that is caused by human papillomavirus (HPV) commonly due to the strains 6 and 11. Children acquire these tumors intrapartum from an infected mother. HPV infects natural and metaplastic squamous mucosa which is the type of epithelium that lines the vocal folds. Tumors present as numerous, verrucous outgrowths from the mucosa and can become symptomatic due to mass effect. Common symptoms include hoarseness, dysphonia, aphonia and most concerning, respiratory distress.
A 7-year-old patient with dysphonia secondary to laryngeal papillomatosis also known as recurrent respiratory papillomatosis undergoes microlaryngoscopy and bronchoscopy with microdebridement, CO2 laser ablation, and cidofovir injections.
Methods:
The patient underwent spontaneous ventilation anesthesia and a dental guard was placed. The patient was positioned for microlaryngoscopy and the larynx was visualized and anesthetized with topical lidocaine. A zero-degree Hopkins rods was passed through the supraglottis, glottis and subglottis to document findings. There was supraglottic papillomatosis notably of the laryngeal surface of the left epiglottis, papillomatosis of the bilateral false vocal folds and papillomatosis of the bilateral true vocal folds with right more affected than left and anterior commissure involvement. The scope was then withdrawn and reintroduced to perform bronchoscopy. The scope was advanced through the trachea, carina and primary and secondary bronchi bilaterally. All were within normal limits. The Benjamin-Lindholm laryngoscope was passed into the vallecula and larynx and suspended in a normal fashion. The zero-degree Hopkins rod was used to visualize the larynx. 2 cc of 1% lidocaine with 1:100,000 epinephrine was injected into the bulk of the papillomas and then several biopsies were taken from this area. The microdebrider was used to debulk these areas. Protective eyewear was used by everyone in the operating room. The patient’s face was protected with water soaked towels and all oxygen sources were removed from the patient. The CO2 laser was set to 2 watts continuous and used to debulk the papillomas with eschar noted after each application. Care was taken to avoid injury to the deep elements of the true vocal folds. Any residual papillomas at the anterior vocal folds were then injected with 1 cc of cidofovir. All instrumentation was removed, the patient was extubated, awakened, and transferred to the recovery room.
Results:
The patient was discharged the same day without complications. He will follow up for revision microdebridement, CO2 laser ablation and cidofovir injections.
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Conclusion:
Microlaryngoscopy and bronchoscopy with microdebridement, CO2 laser ablation, and cidofovir injections is a successful solution for laryngeal papillomatosis and has been proven to eradicate the disease in many cases.
Watch the Full VideoAuthors
Adam Karkoutli1
Wayne Wortmann1
Rohan R. Walvekar, MD2
Nathan C. Grohmann, MD2
Author Affiliations
LSUHSC School of Medicine1
LSUHSC Department of Otolaryngology – Head and Neck Surgery2
Video Description
This video demonstrates the procedure for use of firberoptic flexible laryngoscopy. The preoperative steps and recommendations for use of flexible laryngoscopy are outlined. Followed by a visual demonstration of insertion of the laryngoscope along with outlining pertinent landmarks encountered during this procedure.
Watch the Full VideoThis video is an introduction to operative direct laryngoscopy and bronchoscopy (DLB) and will demonstrate 1) How to set up the equipment for a safe and comprehensive DLB and 2) How to assemble a rigid bronchoscope.Â
Authors: Alexander Moushey1; Taher Valika, MD2; Erik H. Waldman, MD3; Sarah E. Maurrasse, MD3
Voiceover: Vidal Maurrasse
1Yale School of Medicine, New Haven, CT
2Department of Surgery, Division of Pediatric Otolaryngology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine
3Department of Surgery, Section of Pediatric Otolaryngology, Yale School of Medicine, Yale New Haven Children’s Hospital
This video is an introduction to operative direct laryngoscopy and bronchoscopy (DLB) and demonstrates how to perform a safe and comprehensive exam in the operating room.
Authors: Alexander Moushey1; Taher Valika, MD2; Erik H. Waldman, MD3; Sarah E. Maurrasse, MD3
Voiceover: Vidal Maurrasse
1Yale School of Medicine, New Haven, CT
2Department of Surgery, Division of Pediatric Otolaryngology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine
3Department of Surgery, Section of Pediatric Otolaryngology, Yale School of Medicine, Yale New Haven Children’s Hospital
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