Expansion cranioplasty with jackscrew distracters for craniosynostosis and intracranial hypertension in transplanted osteopetrosis

Pediatr Neurosurg. 2007;43(2):102-6. doi: 10.1159/000098381.

Abstract

Background: An 11-month-old boy with autosomal recessive infantile osteopetrosis presented, 7 months after bone marrow transplantation, with normal ventricular size and life-threatening intracranial hypertension due to pansynostosis.

Methods: The cranial vault was expanded by using jackscrew distracters to upwardly advance the upper part of the calvarium.

Results: The procedure achieved a 15-mm upward expansion of the cranial vault over a 15-day period, and the volume of the cranial vault was increased by 139 ml. All clinical manifestations of intracranial hypertension resolved.

Conclusion: Cranial vault expansion with jackscrew distracters was successful in relieving intracranial hypertension in an infant with pancraniosynostosis complicating bone marrow transplanted malignant infantile osteopetrosis.

Publication types

  • Case Reports

MeSH terms

  • Cephalometry
  • Chromosome Aberrations
  • Craniosynostoses / diagnosis
  • Craniosynostoses / genetics
  • Craniosynostoses / surgery*
  • DNA Mutational Analysis
  • Follow-Up Studies
  • Genes, Recessive / genetics
  • Genetic Carrier Screening
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Infant, Newborn
  • Intracranial Hypertension / diagnosis
  • Intracranial Hypertension / surgery*
  • Magnetic Resonance Imaging
  • Male
  • Osteogenesis, Distraction / instrumentation*
  • Osteopetrosis / diagnosis
  • Osteopetrosis / genetics
  • Osteopetrosis / therapy*
  • Postoperative Complications / diagnosis
  • Postoperative Complications / surgery*
  • Reoperation
  • Tomography, X-Ray Computed
  • Vacuolar Proton-Translocating ATPases / genetics

Substances

  • TCIRG1 protein, human
  • Vacuolar Proton-Translocating ATPases