Craniostenosis

The Craniostenosis (Craniosynostosis or Craniofaciostenosi) is the premature fusion of one or more sutures that make up the time and the cranial base. This involves alterations in the growth of the skull, found in an abnormal morphology of the head.

Considering that the brain in early life has a remarkable development an impediment to its growth involves important issues. Simple craniosynostosis is defined as pathological situation in which only one suture is closed early, when they are involved in more complex sutures.

Simple craniosynostosis
  • Scaphocephaly (sagittal suture)
  • Trigonocephaly (furrow)
  • Brachicefalia (coronal suture)
  • Anterior plagiocephaly (suture emicoronale)
  • Posterior plagiocephaly (lambdoid suture)

The plagiocephaly may be true (from the closing of the suture) or false (plagiocephaly location). syndromic craniosynostosis

Syndromic craniosynostosis:
  • Apert syndrome
  • Crouzon syndrome
  • Pfeiffer syndrome
  • Saethre-Chotzen
  • Carpenter syndrome

Modern is crucial to address these malformations, given the complexity of the issues related to them, using a multidisciplinary approach. The protocol followed by our center provides:

  • Prenatal ultrasound diagnosis
  • First visit with a clinical diagnosis and grading of patient
  • Running TAC skull 3D characterization and diagnostic imaging
  • Ophthalmologic evaluation for the presence of any signs of intracranial hypertension
  • Visit to the child neuropsychiatrist for evaluation of psychomotor development and eventual implementation of MRI brain at his request
  • Planing therapeutic intervention with the decision of "urgent" if no signs of intracranial hypertension and possible cerebral damage or "elective"
  • Correction of deformity between 9 and 11 months (unless interventions "in urgent need")

In our center, in agreement with other authors (Posnick, Wolfe, etc.) and comforted by our study and the results obtained, we prefer to wait for 9 -11 months of life. In case of signs of increased intracranial pressure and / or compression of the optic nerve intervention is necessary in the same moment of diagnosis. .

In any case, the operation should be performed within the first year of life because, as demonstrated in the literature, the risk of developing mental retardation if not operated is 80%. .

The surgery, with the exception of scaphocephaly, provides a bilateral frontal craniotomy and removal bandeau fronto orbital that are reshaped to counter and repositioned through the use of resorbable plates and screws (SonicWeld Rx Martin). The use of such devices is of fundamental importance in pediatric age as the active growth of the cranial bones in the centrifugal direction determines the migration towards the inside of any device not resorbable. This observation has demonstrated clinically in cases undergoing reoperation.

  • Transfer in Intensive therapy with extubation in the 1st postoperative day, drain removal and transfer to the ward on day 2 post-operative.
  • Controls post-operative after a week and then at one, three, six, twelve months.
  • Eye check and at the child neuropsychiatrist.
  • 3D CT skull at 6 months for monitoring and evaluation system absorbable ossification after 2 years is no longer evident on CT control.
  • Remote controls in the years up to the end of development.

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