About the Author: Cody Dazen

My name is Cody Dazen, and I am currently a third year dental student at the University of Pennsylvania. A little bit about me- I am local to the area, originally from southern NJ. My family has since relocated to Old City Philadelphia. I attended college at the University of Pennsylvania, where I played varsity golf. My calling towards a career in dentistry stems from my mom, who is currently practicing as a general dentist in Mt. Holly NJ. In dental school, my love for problem solving, medicine, and surgery has grown in part thanks to my father, a veterinarian and brother, who is currently a fourth year at Robert Wood Johnson Medical School. Family, faith, and fun have always been very important values to me. As I continue my education, I personally hope to specialize as an Oral and Maxillofacial Surgeon. It is this interest that led me to become involved with the Philly Phaces. This organization has provided me with an invaluable opportunity to meet, help, and support such amazing craniofacial patients and families. The Philly Phaces community has taught me fundamental values that continue to shape me as a person and health care professional. I am thankful and blessed to be able to give back to the Philadelphia community of which I have been a part of for the past seven and a half years.

I am excited to be blogging along side several of my schoolmates! I plan to post insight into the pathophysiology and patient management of craniofacial differences the patients of Philly Phaces experience.

 

Pierre Robin Sequence

In the profession of dentistry, we are called on to help treat and manage problems surrounding the oral cavity, maxillofacial region and associated anatomy. Some patients may be more complex than others, presenting with a variety of craniofacial differences. It is important to understand these craniofacial differences in order to properly treat, manage, and know where/when to refer a patient for further assistance.

Pierre Robin sequence, also know as Pierre Robin syndrome or Pierre Robin malformation, is a rare congenital birth defect that affects the craniofacial region. It occurs due to abnormal development of the first and second pharyngeal arches. The first and second pharyngeal arches in the fetus develop into the cartilage, muscles, and nerves of the maxillofacial, cervical neck, and auditory regions. Patients with Pierre Robin sequence commonly experience micrognathia (underdeveloped jaws), leading to an enlarged and backward displacement of the tongue, cleft palate, and airway obstruction. Early warning signs include trouble breathing and feeding (mainly due to the tongue position, underdeveloped mandible and cleft palate), repeated ear infections, or the presence of natal teeth (teeth existing at birth). The syndrome is often diagnosed by physical examination at or around the time of birth.

Treatment of Pierre Robin sequence patients is often multifactorial and requires a team of specialists including oral and maxillofacial surgeons, craniofacial and plastic surgeons, ENTs, speech therapists, and dental specialists (general dentists, pediatric dentists, prosthodontists, and orthodontists). First priority is to achieve proper breathing. Sleeping on one’s stomach or side can help open a restricted oropharyngeal airway. If necessary, surgical intervention can include a nasopharyngeal airway, a nasal trumpet (a tube threaded through the nose into the upper airway), lower jaw expansion (if before 16 years of age), or a tracheotomy. Feeding difficulties are usually self-resolving within the first few years of life, however in extreme cases, there is risk of aspiration. A feeding tube may be necessary to allow for proper weight gain. Cleft palate repair is typically addressed at 1-2 years old. Children may require speech therapy later in life. Finally, due to micrognathia, dental development may be compromised. It is important that both dental and medical specialists monitor growth and development in these patients in order to provide treatment in a timely manner.

References

Johnson JM, Moonis G, Green GE, Carmody R, Burbank HN. Syndromes of the First and Second Branchial Arches, Part 2: Syndromes. American Journal of Neuroradiology. http://www.ajnr.org/content/32/2/230. Published February 1, 2011. Accessed December 31, 2020.

Le T, Bhushan V. First Aid for the USMLE Step 1 2019. New York, NY: McGrawHill Education; 2019.

Pierre Robin Sequence. Johns Hopkins Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases/pierre-robin-sequence. Accessed December 31, 2020.