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Craniosynostosis: Monobloc Distraction with Internal Device and Its Variant for Infants with Severe Syndromic

Giovanna Paternoster1, Samer Elie Haber1, Roman Hossein Khonsari2

  • 1Unité fonctionnelle de chirurgie craniofaciale, Service de Neurochirurgie Pédiatrique, Hôpital Necker - Enfants Malades, Assistance Publique - Hôpitaux de Paris, Centre de Référence Maladies Rares CRANIOST, Filière Maladies Rares TeteCou, Université Paris Descartes, ERN Cranio, 149 rue de Sèvres, 75015, Paris, France.

Clinics in Plastic Surgery
|May 30, 2021
PubMed

Insights

Distraction osteogenesis in frontofacial monobloc advancement enhances safety for infants with syndromic craniosynostosis. This technique effectively safeguards ophthalmic, neurological, and respiratory functions in young patients.

Area of Science:

  • Craniofacial Surgery
  • Pediatric Neurosurgery
  • Orthognathic Surgery

Background:

  • Syndromic craniosynostosis presents significant challenges in pediatric patients.
  • Frontofacial monobloc advancement is a critical surgical procedure for managing these complex conditions.
  • Distraction osteogenesis has been introduced to improve the safety and outcomes of frontofacial advancement.

Purpose of the Study:

  • To evaluate the safety and efficacy of frontofacial monobloc advancement using distraction osteogenesis in infants.
  • To assess the impact of this technique on ophthalmic, neurological, and respiratory functions.
  • To analyze outcomes in a cohort of patients, including very young infants.

Main Methods:

  • A retrospective analysis of 147 patients with syndromic craniosynostosis who underwent frontofacial monobloc advancement.
  • Utilized 4 internal distractors for the advancement procedure.
  • Specific attention was given to a subgroup of 25 patients aged 18 months or younger.

Main Results:

  • The procedure demonstrated increased safety with the use of distraction osteogenesis.
  • Ten patients with pre-existing tracheostomies showed positive outcomes, with 50% decannulated post-surgery.
  • Six patients required additional interventions, such as transfacial pins and external traction, highlighting the complexity of some cases.

Conclusions:

  • Very early frontofacial monobloc advancement with 4 internal distractors is a safe and effective treatment for severe syndromic craniosynostosis in infants.
  • This approach successfully protects vital ophthalmic, neurological, and respiratory functions.
  • The findings support the use of this technique in managing complex pediatric craniofacial abnormalities.

Keywords:
Craniofacial dysostosisDistraction osteogenesisExorbitismFaciocraniosynostosisFrontofacial monobloc advancementSleep apneaSyndromic craniosynostosisTracheostomy

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