Summary. Pierre Robin sequence (PRS) is characterized by a small lower jaw (micrognathia) and displacement of the tongue toward the back of the oral cavity. Pierre-Robin syndrome (or Pierre-Robin sequence) is characterised by triad of orofacial morphological anomalies consisting of retrognathism, glossoptosis and. Pierre Robin sequence (or syndrome) is a condition in which an infant has a smaller than normal lower jaw, a tongue that falls back in the throat, and difficulty breathing. It is present at birth.


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Support Groups The following resources can provide more information on Pierre Robin sequence: The National Craniofacial Association -- www. There pierre robin syndrome a high risk for problems if the child's airways are not kept from getting blocked.

Enlargement of the lower jaw brings the tongue forward, pierre robin syndrome it from obstructing the upper airway. The process of DO begins with preoperative assessment.

Pierre Robin Sequence - NORD (National Organization for Rare Disorders)

Doctors use three-dimensional imaging to identify pierre robin syndrome parts of the patient's facial pierre robin syndrome that need repositioning and determine the magnitude and direction of distraction.

They may then select the most appropriate distraction device or sometimes have custom devises fabricated. When possible, intraoral devices are used. DO surgery starts with an osteotomy surgical division or sectioning of bone followed by the distraction device being placed under the skin and across the osteotomy.

A few days later, the two ends of the bone are very gradually pulled apart through continual adjustments that are made to the device by the parents at home.

Pierre Robin Sequence

This gradual distraction leads to formation of new bone between the two ends. After the process is complete, the osteotomy is allowed to heal over a period of six pierre robin syndrome eight weeks.

A small second surgery is then performed to remove the device. In this theory, the pierre robin syndrome and regulatory organization of the rhombencephalus is related to a major problem of ontogenesis.

  • Pierre Robin Syndrome: Background, Etiology and Pathogenesis, Otolaryngologic Manifestations
  • Pierre Robin sequence: MedlinePlus Medical Encyclopedia
  • Pierre Robin syndrome
  • Etiology and Pathogenesis

Otolaryngologic Manifestations Micrognathia is reported in the majority of cases pierre robin syndrome It is characterized by retraction of the inferior dental arch mm behind the superior arch. The mandible has a small body, obtuse genial angle, and a posteriorly located condyle.

Pierre Robin Syndrome

The growth of the mandible catches up during the first year; however, mandibular hypoplasia resolves and the child attains a normal profile by approximately age pierre robin syndrome. The jaw index is defined as the alveolar overjet multiplied by the maxillary arch divided by the mandibular arch.

This index can be used to objectify mandibular growth.

The alveolar overjet is the distance between pierre robin syndrome most anterior points of the upper and lower alveolar arches. The maxillary arch is the measurement between the 2 tragi via the subnasal point, and the mandibular arch is the distance from the right to the left tragus passing through the pogonion.

The combination of micrognathia and glossoptosis may cause pierre robin syndrome respiratory and feeding difficulty in the newborn.

Pierre Robin syndrome - Wikipedia

Godbout et al published a study comparing anatomical cleft parameters between isolated cleft palate patients and Pierre Robin syndrome patients. They demonstrated a significant correlation between the width of the cleft of the soft palate and the severity of the respiratory symptoms and feeding difficulties pierre robin syndrome Pierre Robin syndrome patients.

Obstructive sleep apnea may also occur. Occasionally, it may present as a bifid or double pierre robin syndrome or as an occult submucous cleft. Temporal bone computerized planigraphs demonstrate inadequate pneumatization of the mastoid cavities in many patients with Pierre Robin sequence.

Pierre Robin sequence

Gruen et al studied 13 temporal bones by light microscopy and identified multiple architectural anomalies involving the entire ear, including abnormal auricles, and anomalies of the ossicles, including abnormal stapes footplates.

Anomalies of the inner ear included aplasia of the lateral pierre robin syndrome canals, a large vestibular aqueduct, and unusually large otoconia.


In the mastoid process there were islands of pierre robin syndrome in the expected position of Reichert's cartilage and dehiscence of the fallopian canal. Trained medical personnel may visualize characteristic features of PRS using ultrasound imaging.

If not pierre robin syndrome previously, craniofacial abnormalities are typically detected at birth on physical exam. Infants with severe airway obstruction may present with respiratory distress at birth, and may require medical intervention.

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