My Baby Was Diagnosed with Pierre Robin Sequence
Medically reviewed by Dr. Michael Okonkwo, MD, FAAP · Board-Certified Neonatologist
Content reviewed against published CHOP, NIH, Cincinnati Children's guidelines
Last reviewed:
If your baby has been diagnosed with or you suspect was diagnosed with pierre robin sequence, here is what the evidence says.
The short answer
Pierre Robin sequence (PRS) is a condition present at birth characterized by three features: a small lower jaw (micrognathia), a tongue that falls back in the mouth (glossoptosis), and often a U-shaped cleft palate. The small jaw causes the tongue to sit far back, which can obstruct the airway and make breathing and feeding difficult. While the diagnosis is frightening, the jaw typically grows significantly during the first 1-2 years of life, and most children's airway problems improve substantially. Treatment focuses on ensuring safe breathing and adequate nutrition.
Key takeaways
- Pierre Robin sequence (PRS) is a condition present at birth characterized by three features: a small lower jaw (micrognathia), a tongue that falls back in the mouth (glossoptosis), and often a U-shaped cleft palate. The small jaw causes the tongue to sit far back, which can obstruct the airway and make breathing and feeding difficult. While the diagnosis is frightening, the jaw typically grows significantly during the first 1-2 years of life, and most children's airway problems improve substantially. Treatment focuses on ensuring safe breathing and adequate nutrition.
- Usually normal when: Your baby has PRS and is breathing safely with positioning or minor interventions
- Call your doctor if: Your baby turns blue, stops breathing, or chokes during feeding — call 911. While waiting, place your baby on their stomach (prone position) to help open the airway
- Varies by age — see the age-by-age breakdown below
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What Parents Should Know
According to CHOP, NIH, Cincinnati Children's guidelines, pierre Robin sequence (PRS) is a condition present at birth characterized by three features: a small lower jaw (micrognathia), a tongue that falls back in the mouth (glossoptosis), and often a U-shaped cleft palate. The small jaw causes the tongue to sit far back, which can obstruct the airway and make breathing and feeding difficult. While the diagnosis is frightening, the jaw typically grows significantly during the first 1-2 years of life, and most children's airway problems improve substantially. Treatment focuses on ensuring safe breathing and adequate nutrition. At 0-3 months, the newborn period is often the most challenging. Your baby may have difficulty breathing, especially while lying on their back, and feeding can be very difficult due to the cleft palate and tongue position. Positioning strategies (prone or side-lying) often help the airway. Some babies need a nasopharyngeal airway (a small tube through the nose) or, in more severe cases, surgical intervention. Feeding may require specialized bottles (such as the Pigeon or SpecialNeeds feeder) and working with a feeding specialist. Weight gain is closely monitored. It is generally considered normal when your baby has PRS and is breathing safely with positioning or minor interventions. However, you should contact your pediatrician promptly if your baby turns blue, stops breathing, or chokes during feeding — call 911. While waiting, place your baby on their stomach (prone position) to help open the airway.
Normal vs. Concerning
By Age
What to expect by age
0-3 months
The newborn period is often the most challenging. Your baby may have difficulty breathing, especially while lying on their back, and feeding can be very difficult due to the cleft palate and tongue position. Positioning strategies (prone or side-lying) often help the airway. Some babies need a nasopharyngeal airway (a small tube through the nose) or, in more severe cases, surgical intervention. Feeding may require specialized bottles (such as the Pigeon or SpecialNeeds feeder) and working with a feeding specialist. Weight gain is closely monitored.
3-6 months
As the jaw begins to grow, many babies experience gradual improvement in airway symptoms. Feeding often becomes easier as well, though it remains more challenging than for typical babies. Your cleft team (a multidisciplinary group including a surgeon, speech pathologist, ENT, and others) will be coordinating your baby's care. Sleep studies may be performed to assess for obstructive sleep apnea.
6-12 months
Cleft palate repair surgery is typically performed between 9-14 months of age. By this time, the jaw has usually grown enough that airway issues have improved significantly. After palate repair, speech development will be monitored closely. Some children with PRS need additional jaw surgery (distraction osteogenesis) if the jaw growth is insufficient, but many do not.
1 year+
Most children with isolated Pierre Robin sequence (not associated with another syndrome) do very well long-term. The jaw continues to grow and becomes less noticeably small. Speech therapy may be needed after palate repair. Ear infections and hearing issues are common with cleft palate, so regular audiology assessments are recommended. About 40% of PRS cases are associated with an underlying syndrome (such as Stickler syndrome), so genetic testing may be offered.
What to Tell Your Pediatrician
- Describe when you first noticed was diagnosed with pierre robin sequence and how it has changed over time.
- Note your baby's current age and which age-specific patterns you are seeing.
- Mention if your baby is having increasing difficulty with noisy breathing or seems to work hard to breathe.
- Mention if your baby is not gaining weight despite frequent feeding attempts.
- Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
- Bring a list of any questions or observations you want to discuss at the appointment.
What Should You Do?
When to take action
- Your baby has PRS and is breathing safely with positioning or minor interventions
- Your baby is gaining weight with specialized feeding techniques and bottles
- Your baby's breathing and feeding are gradually improving as their jaw grows
- Your baby is having increasing difficulty with noisy breathing or seems to work hard to breathe
- Your baby is not gaining weight despite frequent feeding attempts
- You are exhausted from the demands of specialized feeding and need additional support or resources
- You have questions about upcoming surgeries or your baby's long-term outlook
- Your baby turns blue, stops breathing, or chokes during feeding — call 911. While waiting, place your baby on their stomach (prone position) to help open the airway
- Your baby is unable to feed and seems dehydrated (no wet diapers for 6+ hours, dry mouth, sunken fontanelle) — seek immediate medical care
What You Can Do at Home
- Keep track of when you notice was diagnosed with pierre robin sequence — noting the time of day, duration, and any triggers can help your pediatrician.
- Remember that your baby has PRS and is breathing safely with positioning or minor interventions — this is generally within the range of normal.
- At 0-3 months, focus on observation rather than intervention unless your pediatrician advises otherwise.
- Follow any care instructions from your pediatrician. Keep a written log of symptoms to bring to appointments.
- While monitoring at home, seek immediate care if your baby turns blue, stops breathing, or chokes during feeding — call 911. While waiting, place your baby on their stomach (prone position) to help open the airway.
Related Conditions
My Baby Was Born with Blocked Nasal Passages (Choanal Atresia)
Choanal atresia is a condition present at birth where one or both nasal passages are blocked by bone or tissue. Since newborns are obligate nose-breathers (they breathe primarily through their nose), bilateral choanal atresia (both sides blocked) is a medical emergency that is usually detected immediately after birth. Unilateral choanal atresia (one side blocked) may not be diagnosed until later. Treatment is surgical, and outcomes are generally very good. About half of babies with choanal atresia have other associated conditions, so a thorough evaluation is important.
Could My Baby Have a Posterior Tongue Tie?
A posterior tongue tie is a restriction of the tissue under the tongue that is not easily visible and must be felt by an experienced provider. It can cause breastfeeding difficulties including a painful or shallow latch, clicking sounds during nursing, and poor weight gain. Diagnosis and treatment recommendations vary among healthcare providers, so getting an evaluation from a lactation consultant experienced with tongue ties is often a helpful first step.
Related Resources
Breathing Difficulty Triage
Assess breathing concerns and know when to seek emergency care.
Month-by-Month Development
Detailed monthly development guides from birth through 24 months.
When to Call the Doctor
General guide on when to call the pediatrician, visit urgent care, or go to the ER.
Frequently asked questions
Is was diagnosed with pierre robin sequence normal?
When should I call the doctor about was diagnosed with pierre robin sequence?
When is was diagnosed with pierre robin sequence normal?
What causes was diagnosed with pierre robin sequence?
What should I mention to my pediatrician about was diagnosed with pierre robin sequence?
Is was diagnosed with pierre robin sequence normal at 0-3 months?
Is was diagnosed with pierre robin sequence normal at 3-6 months?
Should I go to the ER for was diagnosed with pierre robin sequence?
Does was diagnosed with pierre robin sequence go away on its own?
References
- [1]Children's Hospital of Philadelphia. Pierre Robin Sequence. CHOP
- [2]MedlinePlus. Pierre Robin Sequence. NIH
- [3]Cincinnati Children's Hospital. Pierre Robin Sequence. Cincinnati Children's
Doctor Visit Checklist
Bring this checklist to your next pediatrician visit to discuss My Baby Was Diagnosed with Pierre Robin Sequence.
Things to mention
- Describe when you first noticed was diagnosed with pierre robin sequence and how it has changed over time.
- Note your baby's current age and which age-specific patterns you are seeing.
- Mention if your baby is having increasing difficulty with noisy breathing or seems to work hard to breathe.
- Mention if your baby is not gaining weight despite frequent feeding attempts.
- Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
- Bring a list of any questions or observations you want to discuss at the appointment.
Observations to share
- Your baby is having increasing difficulty with noisy breathing or seems to work hard to breathe
- Your baby is not gaining weight despite frequent feeding attempts
- You are exhausted from the demands of specialized feeding and need additional support or resources
Urgent signs to report immediately
- Your baby turns blue, stops breathing, or chokes during feeding — call 911. While waiting, place your baby on their stomach (prone position) to help open the airway
- Your baby is unable to feed and seems dehydrated (no wet diapers for 6+ hours, dry mouth, sunken fontanelle) — seek immediate medical care
My notes
From ismybabyalright.com — free, evidence-based baby health guides
All content follows our editorial policy and is reviewed against published clinical guidelines.
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Related Resources
Bottom line
Most cases of was diagnosed with pierre robin sequence are normal. Talk to your pediatrician if your baby turns blue, stops breathing, or chokes during feeding — call 911. while waiting, place your baby on their stomach (prone position) to help open the airway.
Trust your instincts. If something feels wrong, reach out to your pediatrician. Worrying about your baby means you care — that is a good thing.
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Related Medical Concerns
My Baby Was Born with Blocked Nasal Passages (Choanal Atresia)
Choanal atresia is a condition present at birth where one or both nasal passages are blocked by bone or tissue. Since newborns are obligate nose-breathers (they breathe primarily through their nose), bilateral choanal atresia (both sides blocked) is a medical emergency that is usually detected immediately after birth. Unilateral choanal atresia (one side blocked) may not be diagnosed until later. Treatment is surgical, and outcomes are generally very good. About half of babies with choanal atresia have other associated conditions, so a thorough evaluation is important.
Could My Baby Have a Posterior Tongue Tie?
A posterior tongue tie is a restriction of the tissue under the tongue that is not easily visible and must be felt by an experienced provider. It can cause breastfeeding difficulties including a painful or shallow latch, clicking sounds during nursing, and poor weight gain. Diagnosis and treatment recommendations vary among healthcare providers, so getting an evaluation from a lactation consultant experienced with tongue ties is often a helpful first step.
My Baby's Head Shape Looks Abnormal
Many babies develop temporary head shape irregularities that are completely normal. A cone-shaped head from vaginal delivery reshapes within days. Mild positional flattening (plagiocephaly) from sleeping on the back is very common and usually improves with repositioning and tummy time. However, head shape changes involving ridges, a persistently bulging fontanelle, or rapid head growth changes should be evaluated to rule out craniosynostosis.
Achondroplasia (Dwarfism) in Babies
Achondroplasia is the most common form of short-limbed dwarfism, affecting about 1 in 15,000 to 40,000 births. It is caused by a mutation in the FGFR3 gene and is usually apparent at birth with characteristic features including short limbs, a larger head, and a prominent forehead. Intelligence is normal. With monitoring for specific complications and supportive care, children with achondroplasia lead full, active, and independent lives.
Adenoid Hypertrophy and Breathing
Adenoids are lymphoid tissue located behind the nose that help fight infection in young children. When adenoids become enlarged (adenoid hypertrophy), they can block the nasal airway, causing chronic mouth breathing, snoring, nasal speech, and sleep-disordered breathing. Enlarged adenoids are most common between ages 2-7 and are a leading cause of obstructive sleep apnea in young children. Treatment ranges from watchful waiting and nasal steroids to surgical removal (adenoidectomy) if breathing or sleep is significantly affected.
Adrenoleukodystrophy (ALD) in Babies
X-linked adrenoleukodystrophy (X-ALD) is a genetic disorder affecting about 1 in 17,000 newborns, caused by mutations in the ABCD1 gene on the X chromosome. It primarily affects boys and impairs the breakdown of very long-chain fatty acids (VLCFAs), which accumulate and damage the myelin sheath in the brain and the adrenal glands. The most severe form, cerebral ALD, typically affects boys between ages 4-10 with rapid neurological decline. Newborn screening now enables early detection, and hematopoietic stem cell transplant or gene therapy performed before significant brain involvement can be life-saving.