Short answer · Medically reviewed summary · Last updated: 2026-04-07
Pierre Robin Syndrome, often referred to as Pierre Robin sequence, is classified under the ICD-10 code Q87.0 (congenital malformation syndromes predominantly affecting facial appearance) and historically under the ICD-9 code 756.0 (anomalies of skull and face bones). Because it is a sequence of related structural anomalies rather than a single disease, it is frequently coded alongside its specific components, such as cleft palate (Q35.x). What is the clinical definition of Pierre Robin Syndrome? Pierre Robin Syndrome is not a single disease but a sequence of developmental anomalies characterized by the triad of micrognathia (a small, recessed lower jaw), glossoptosis (downward displacement of the tongue), and a U-shaped cleft palate.
ICD10 code of Pierre Robin Syndrome and ICD9 code
ICD-10 and ICD-9 codes for Pierre Robin Syndrome, with classification details for clinicians, coders and patients.
Pierre Robin Syndrome, often referred to as Pierre Robin sequence, is classified under the ICD-10 code Q87.0 (congenital malformation syndromes predominantly affecting facial appearance) and historically under the ICD-9 code 756.0 (anomalies of skull and face bones). Because it is a sequence of related structural anomalies rather than a single disease, it is frequently coded alongside its specific components, such as cleft palate (Q35.x).
What is the clinical definition of Pierre Robin Syndrome?
Pierre Robin Syndrome is not a single disease but a sequence of developmental anomalies characterized by the triad of micrognathia (a small, recessed lower jaw), glossoptosis (downward displacement of the tongue), and a U-shaped cleft palate. The clinical presentation of Pierre Robin Syndrome typically results in upper airway obstruction and feeding difficulties in newborns. Because the jaw is underdeveloped, the tongue lacks sufficient space, causing it to fall back into the pharynx, which creates the airway compromise. Understanding this sequence is vital for families, as 190 members of our DiseaseMaps.org community have shared their unique journeys in managing these early life challenges.
How is Pierre Robin Syndrome coded for medical billing?
Medical coding for Pierre Robin Syndrome can be complex due to its classification as a sequence. While Q87.0 is the primary ICD-10 code, clinicians often use a combination of codes to capture the full clinical picture:
- Q87.0: Congenital malformation syndromes predominantly affecting facial appearance (Primary code).
- Q35.9: Cleft palate, unspecified (Commonly associated).
- Q67.4: Other congenital deformities of skull, face, and jaw (Used to specify micrognathia).
- 756.0: Anomalies of skull and face bones (Historical ICD-9 code).
Is Pierre Robin Syndrome hereditary?
The etiology of Pierre Robin Syndrome is heterogeneous. In many cases, it occurs sporadically, meaning it is not inherited from the parents. However, it can also be a feature of a broader genetic condition, such as Stickler syndrome or velocardiofacial syndrome. A clinical geneticist is essential to determine if the Pierre Robin Syndrome diagnosis is isolated or part of a syndromic condition, which significantly impacts long-term management and genetic counseling for future pregnancies.
How is the airway managed in infants with Pierre Robin Syndrome?
The primary concern for clinicians treating Pierre Robin Syndrome is the maintenance of a patent airway. Management strategies vary based on severity:
- Prone positioning: Placing the infant on their stomach to allow gravity to pull the tongue forward.
- Nasopharyngeal airway (NPA): A soft tube inserted through the nose to keep the pharyngeal space open.
- Distraction osteogenesis: A surgical procedure to gradually lengthen the lower jaw.
- Glossopexy: A surgical procedure to attach the tongue to the lower lip to prevent it from obstructing the airway.
Next steps
- Consult a craniofacial team, including a pediatric otolaryngologist and a plastic surgeon, to create a personalized care plan.
- Request a formal genetic evaluation to determine if your child's Pierre Robin Syndrome is isolated or associated with a genetic syndrome.
- Join the DiseaseMaps.org community to connect with other families who have firsthand experience navigating the medical and emotional aspects of this condition.
- Work with a speech-language pathologist early on to address potential feeding and speech development delays.
Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Pierre Robin sequence.
- Orphanet: Pierre Robin syndrome (ORPHA:776).
- OMIM (Online Mendelian Inheritance in Man): Pierre Robin sequence (Entry #261800).
- American Cleft Palate-Craniofacial Association: Clinical standards for Pierre Robin sequence.
