Pierre Robin Malformation Follow-up

  • Author: Marie M Tolarova, MD, PhD, DSc; Chief Editor: Glenn C Isaacson, MD, FACS, FAAP   more...
 
Updated: Mar 25, 2009
 

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 the images below), 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 profPatient 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.

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. 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 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.

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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]

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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.

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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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

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.

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

References

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Three-week-old baby boy affected with nonsyndromic Robin sequence.
One-month-old baby affected with nonsyndromic Robin sequence.
Patient from Media file 2 at age 4 years. The profile is almost normal because of catch-up growth.
Patient from Media file 2.
U-shaped and V-shaped cleft palates.
Eight-year-old boy with Stickler syndrome. Note a flat, hypotonic face and small mandible. He also has a U-shaped, wide cleft palate (CP). His mandible does not show catch-up growth. He is a mouth-breather and snores. He is using his CP as an airway. A closing of the CP without preparation would compromise his airway passage. The authors recommend placement of an obturator (perhaps with a speech bulb) for a couple of hours a day at first and gradually increasing the time. After a few months, when the child will have changed his breathing pattern, the palate can be closed.
Eight-year-old boy with Stickler syndrome from the previous picture. Note a flat, hypotonic face and a small mandible. His mandible does not show catch-up growth.
Eight-year-old boy with Stickler syndrome from previous 2 pictures. Note a U-shaped, wide cleft palate.
Robin sequence in some recognized and unrecognized syndromes.
Robin sequence and complexes.
Robin sequences and complexes.
Child with Pierre Robin Sequence prior to distraction osteogenesis.
Distraction osteogenesis is completed, and the bone is consolidating.
 
 
 
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