Fast and early mandibular osteogenetic distraction in a 24-day-old female newborn with Larsen syndrome

J Craniofac Surg. 2014 May;25(3):e304-7. doi: 10.1097/SCS.0000000000000407.

Abstract

Background: Larsen syndrome (LS) is a rare bone dysplasia characterized by multiple dislocations affecting large and small joints, progressive scoliosis, accessory and early ossifying carpal/tarsal bones, and characteristic craniofacial features.

Case presentation: A newborn with a clinical diagnosis of LS is presented. Shortly after birth, she had respiratory distress due to retrognathia. Such a life-threatening complication was resolved by mandibular distraction osteogenesis at 24 days of age.

Conclusion: Fast and early mandibular osteogenetic distraction could represent an optimal tool to avoid tracheostomy and to improve oral feeding in patients with rare conditions, such as LS.

Publication types

  • Case Reports

MeSH terms

  • Airway Obstruction / etiology
  • Airway Obstruction / surgery
  • Female
  • Humans
  • Infant, Newborn
  • Mandible / surgery*
  • Osteochondrodysplasias / complications
  • Osteochondrodysplasias / surgery
  • Osteogenesis, Distraction / methods*
  • Polysomnography / methods
  • Respiratory Distress Syndrome, Newborn / etiology
  • Respiratory Distress Syndrome, Newborn / surgery
  • Retrognathia / etiology
  • Retrognathia / surgery*

Supplementary concepts

  • Larsen Syndrome