Pierre Robin Syndrome Life Expectancy- A rare Infantile Disease

Pierre Robin Syndrome Life Expectancy- A rare Infantile Disease

Pierre robin syndrome have first described as micrognathia in infants by Doctor Odilon Marc Lannelongue and doctor Maxime Ménard in year 1891. Then in year 1920, it have described by Doctor Pierre Robin with a triad of micrognathia, cleft palate and glossoptosis. After then, the disease called with this doctor’s name as Pierre Robin syndrome also. This disease have reported about in 1 of 10,000 births. Because of autosomal recessive inheritance, it is more common in individuals with consanguineous marriage. The most common genetic disorders are on chromosome 2 and 4 some gene deletions and on chromosome 3 and 7 some gene duplications.

Pierre Robin Syndrome symptoms:

  • Cleft palate is common in these patients and is one from the diagnosis triad also.
  • Very small chin structure that have called micrognatia also. This is the second finding from the triad for diagnosis. In micrognatia, the tongue pressures to eustachian tube. For this reason the patient may have repeated middle ear infections.
  • Large tongue if compared with chin. With it, the tongue have placed further back than normal. This finding have named glossoptosis and is the third sign from the diagnosis triad.
  • The chin may seen that is settled very back from throat. With it, this causes to difficult breathing problems.
  • Teeth may appear out when the baby is born. Because of small chin also.

Pierre Robin Syndrome treatment:

A multidisciplinary approach will necessary with plastic and reconstructive surgery, pediatric otolaryngology specialist, pediatric anesthesiologist, speech pathology, nutrition specialist and neonatology specialist. The most important treatment is to combat with respiratory problems and feeding difficulties. Prone or lateral positioning will solve often the airway obstruction. If the infant continues to desaturation, placement of a nasopharyngeal tube for bypass the site of upper airway may indicated. The pediatric anesthesiologist should done this procedure. With it, supportive airway may necessary only for 2 to 4 months. In babies under respiratory distress, feeding may difficult. Feeding via nasogastric tube may helpful for this patient also.

In some patient, surgical procedure that include tongue–lip adhesion, distraction osteogenesis and tracheostomy should planed. This procedure is the most common surgery that need for this patients.

Pierre Robin Syndrome lifespan:

After the infantile period the patients life expectancy will same as the healthy peoples. This disease does not affect the lifespan.


To read about Nager Syndrome click this link

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1 Comment

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