eMedicine Specialties > Pediatrics: Surgery > Otolaryngology

Pierre Robin Malformation: Follow-up

Author: Marie M Tolarova, MD, PhD, DSc, Professor and Executive Director, UOP Craniofacial Team, Cleft Prevention Program, Department of Orthodontics, University of the Pacific School of Dentistry
Contributor Information and Disclosures

Updated: Mar 25, 2009

Follow-up

Further Inpatient Care

  • The major problem is airway compromise or obstruction. As mentioned previously in Etiology and pathogenesis, Robin sequence (RS) and Robin complexes cause different types of airway obstruction. In order to successfully manage this serious condition in a baby with Robin sequence, determine the accurate diagnosis as soon as possible.
  • The vast majority of infants with nonsyndromic Robin sequence and normal tongue size experience airway obstruction due to micrognathia of different degrees. If the baby is in the prone position, gravity pulls the tongue forward and keeps the airway open
  • Placement of a nasopharyngeal airway can help to avoid airway blockage. Consider it especially when hypotonia is also present (eg, deletion of chromosome band 22q11.2 syndrome), as well as in Robin complexes with neurological symptoms. Some cases require a tracheostomy to maintain an open airway in the baby.
  • In extensive studies dealing with airway problems in Robin sequence, Shprintzen demonstrated that different mechanisms of obstruction can occur within the same syndrome and noted that, in some patients, glossoptosis is frequently not the cause of the upper airway obstruction (see Treatment).26,4
  • In deformational Robin sequence, the mandible undergoes catch-up growth (see Media files 3-4), which starts after birth when intrauterine constraint disappears and thus eases airway and feeding problems.

    Patient from Media file 2 at age 4 years. The pro...

    Patient from Media file 2 at age 4 years. The profile is almost normal because of catch-up growth.

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    Patient from Media file 2 at age 4 years. The pro...

    Patient from Media file 2 at age 4 years. The profile is almost normal because of catch-up growth.


    Patient from Media file 2.

    Patient from Media file 2.

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    Patient from Media file 2.

    Patient from Media file 2.


    Usually, an improvement is observed after the first 3 months. Even with partial catch-up growth, a child's profile is almost normal at age 4-6 years without any treatment (see Media file 3). When, as in some patients, the mandible still lags behind, orthodontic treatment of malocclusion may be required (see Surgical Care).
  • In Robin sequence that is a part of a syndrome or in Robin complexes, initial problems during the neonatal period and early stages of life are similar to those in deformational Robin sequence.
  • A careful analysis of the type of airway obstruction is fundamental. Sher et al studied the mechanism of airway obstruction using flexible fiberoptic nasolaryngoscopy and developed a classification scheme based on four different processes.20 Identifying a type of airway obstruction and understanding its mechanism is essential for correct management and treatment.
  • One thing is common for patients with nondeformational Robin sequence: no catch-up growth of the mandible occurs. Because growth is altered in the mandible but may not be altered in other parts of the face, a dysmorphic feature may progress and become more prominent with age if not treated.
  • Although treatment in the beginning of an infant's life is similar for all patients with Robin sequences, management of airway obstruction may require a more invasive approach in the syndromic Robin sequence and in Robin complexes.
  • Smith and Senders (2006) reviewed 60 patients with Robin sequence.41 One third of patients who failed positional therapy were temporarily stabilized with a nasopharyngeal airway or endotracheal intubation. The remaining two thirds of patients required a surgical procedure. By age 3 years, most patients were successfully taking an oral diet.

Complications

  • Velopharyngeal dysfunction after palatoplasty is rather common. It is more common in patients with nonsyndromic Robin sequence, when the cleft is usually U-shaped, large, and wide, than in patients with syndromic Robin sequence.42

Patient Education

  • Children with Robin sequence also have difficulties with feeding. A cleft palate prevents production of the negative pressure necessary for sucking during breastfeeding. In addition, because of an abnormal jaw position, a baby with a small mandible usually has difficulties contracting its orbicularis oris muscle and squeezing the mother's nipple. In cleft palate, a wide communication between the oral and nasal cavities creates a risk of aspiration, nasal regurgitation, choking, and other feeding problems. Consultation with a feeding specialist is advised. In many cases, when carefully instructed, a mother is able to manage bottle feeding while her baby is in a semisitting position. Special cleft palate nipples and squeezing bottles are helpful (for more details, see Cleft Lip and Palate). In patients with severe problems, gavage feeding may be necessary in the beginning of the baby's life.
  • Verifying that the mother of the baby with Robin sequence is familiar with emergency techniques for the prevention of suffocation by food, such as the Heimlich maneuver, is important.

Miscellaneous

Medicolegal Pitfalls

  • The potential for legal issues in regard to complex congenital malformations always exists. Attention to feeding techniques, airway management, surgical plans, and hereditary implications for the infant and family are potential legal issues.
  • All neonates with significant Robin sequence (RS) are at risk from sudden death. The sudden infant death syndrome (SIDS) data show the risk of SIDS is increased when infants sleep in the prone position. Neonates with Robin sequence already have a compromised airway and additionally typically require prone positioning. Monitoring these neonates should be strongly considered.
  • Infants with Robin sequence deserve a multidisciplinary approach with a knowledgeable and experienced team in order to provide a comprehensive assessment, realistic plan of treatment, and follow-up. Engaging the family in the early stages of the evaluation, the ongoing medical investigations, issues regarding the child's care, and future planning generally leads to satisfaction, even in the most difficult of medical issues.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthors Craig W Senders, MD, and Alex M Espinoza, MD, to the original development and writing of this article.



More on Pierre Robin Malformation

Overview: Pierre Robin Malformation
Differential Diagnoses & Workup: Pierre Robin Malformation
Treatment & Medication: Pierre Robin Malformation
Follow-up: Pierre Robin Malformation
Multimedia: Pierre Robin Malformation
References
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Further Reading

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Keywords

Pierre Robin malformation, Pierre Robin syndrome, Robin sequence, Pierre Robin anomalad, Robin complexes, Pierre Robin malformation complex, syndromic Robin sequence, nonsyndromic Robin sequence, Velocardiofacial syndrome, Stickler syndrome, Stickler's syndrome, autosomal dominant Stickler syndrome, Treacher Collins syndrome, Nager syndrome, spondyloepiphyseal dysplasia congenita, spondyloepiphyseal dysplasia congenita, SED, cleft palate, CP, connective tissue dysplasia, 22q11.2 deletion syndrome, spondyloepiphyseal dysplasia congenita, respiratory distress, micrognathia, glossoptosis, oligohydramnios, treatment, diagnosis, orofacial cleft, hypoxia, cor pulmonale, failure to thrive, cerebral impairment

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Contributor Information and Disclosures

Author

Marie M Tolarova, MD, PhD, DSc, Professor and Executive Director, UOP Craniofacial Team, Cleft Prevention Program, Department of Orthodontics, University of the Pacific School of Dentistry
Marie M Tolarova, MD, PhD, DSc is a member of the following medical societies: American Cleft Palate/Craniofacial Association, American Society of Human Genetics, and International Association for Dental Research
Disclosure: Nothing to disclose.

Medical Editor

Orval Brown, MD, Director of Otolaryngology Clinic, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center at Dallas
Orval Brown, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Medical Association, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Alan D Murray, MD, Pediatric Otolaryngologist, ENT for Children; Full-Time Staff, Medical City Dallas Children's Hospital; Consulting Staff, Department of Otolaryngology, Medical Center of Lewisville, Children's Medical Center at Dallas, Cook Children's Medical Center; Full-Time Staff, Texas Pediatric Surgery Center, The Pediatric Surgery Center
Alan D Murray, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American College of Surgeons, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Glenn C Isaacson, MD, FACS, FAAP, Professor of Otolaryngology-Head and Neck Surgery and Pediatrics, Temple University School of Medicine
Glenn C Isaacson, MD, FACS, FAAP is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Bronchoesophagological Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Society of Pediatric Otolaryngology, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Covidien Honoraria Consulting

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