Complications of Therapies for Syndromic Craniosynostosis in Children

Surgical

Blood loss

  • Dural sinus tears: A tear of a dural sinus can lead to major blood loss with potential cardiac arrest and brain anoxia.
  • Risks of hypovolumia due to continuous small losses: Due to their small blood volume (75 ml/kg), continuous small losses can also lead to severe hypovolumia in babies. It is therefore important to have continuous communication with the anesthesia team and monitor blood loss closely as well as provide accurate replacement. If the child loses >1.5–2 times its blood volume, consideration should be given to the administration of platelets and fresh frozen plasma .
  • Postoperative blood loss: The critical period for blood loss does not end with the operation, and close monitoring with hematocrits and clotting profiles as well as the drains is required in recovery and high dependency unit .

CSF leakage

  • Dural tears: Any dural tears occurring during surgery need to be closed meticulously with sutures, a galeal flap, and fibrin glue. This applies particularly to patients where a fronto-facial advancement is planned. A persistent CSF rhinorrhea or leak in these patients might require a lumbar drain and/or open/endoscopic surgical repair. CSF leaks under vault reconstruction can lead to avascular necrosis of bone flaps, and any CSF collections must be avoided. Leakage of CSF through wounds or into the nasopharynx carries a risk of meningitis and osteomyelitis of the bone flaps.

Infection

  • Leading complication: In any craniofacial procedure infection is a risk, as it can lead to meningitis or osteomyelitis of bone flaps. Early high-dose antibiotic treatment is recommended if infection is a concern.
  • Risk increased with midface advancements: Midface advancement at the time of fronto-orbital advancement as well as wires extruding through the skin or into an intraparenchymal location further increase risk of infection.

Repeat surgeries

  • Multiple surgeries typical for syndromic craniosynostosis: In syndromic craniosynostosis most, if not all, children require staged and repeat procedures that have morbidity and mortality risks.
  • Varying rates of complications among syndromes: Proven genetic cases have a higher risk of additional craniofacial procedures and are associated with a higher frequency of complications. The risks differ for single gene and chromosomal disorders (53).

Death

  • Death due to hemorrhage and edema: All of the above complications can lead to a permanent injury to the brain, resulting in a major neurological deficit or death. The most common cause of death is venous sinus hemorrhage and generalized brain edema. In experienced hands the mortality rate should be less than 1%, with the greatest risk being in multisuture synostosis in infancy.

Other

  • Air embolism: If a major venous sinus is opened, a peri-operative air embolus can occur.
  • Sagittal sinus thrombosis: Sagittal sinus thrombosis due to manipulation or sinus kinking can occur if bone flaps are replaced too tightly.
  • Seizures: Seizures are a potential complication of a major craniofacial procedure and should be treated with anticonvulsants. If a postoperative seizure occurs, consideration is given for continuing anticonvulsants (the author’s preference is for a 6 month extension).
  • Severe conjunctival edema