Neurochirurgie
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Patients with syndromic faciocraniosynostosis due to the mutation of the fibroblast growth factor receptor (FGFR) 2 gene present premature fusion of the coronal sutures and of the cranial base synchondrosis. Cerebrospinal fluid (CSF) circulation disorders and cerebellar tonsil prolapse are frequent findings in faciocraniosynostosis. ⋯ Different pathophysiological theories have been proposed, but none elucidated all the symptoms present in Apert, Crouzon and Pfeiffer syndromes. The first theory that addressed CSF circulation disruption was the constrictive theory (cephalocranial disproportion): cerebellum and brain stem are constricted by the small volume of the posterior fossa. The second theory proposed venous hyperpressure due to jugular foramens stenosis. The most recent theory proposed a pressure differential between CSF in the posterior fossa and in the vertebral canal, due to foramen magnum stenosis.
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The growth of the posterior fossa in syndromic craniostenosis was studied in many papers. However, few studies described the pathophysiological growth mechanisms in non-operated infants with fibroblast growth factor receptor (FGFR) type 2 mutation (Crouzon, Apert or Pfeiffer syndrome), although these are essential to understanding cranial vault expansion and hydrocephalus treatment in these syndromes. ⋯ Of the various techniques for measuring posterior fossa volume, direct slice-by-slice contouring is the most precise and sensitive. Posterior fossa growth follows a bi-phasic pattern due to opening of the petro-occipital, occipitomastoidal and spheno-occipital sutures. Some studies reported smaller posterior fossae in syndromic craniostenosis, whereas direct contouring studies reported no difference between normal and craniostenotic patients. In Crouzon syndrome, synchondrosis fusion occurs earlier than in normal subjects, and follows a precise pattern. This premature fusion in Crouzon syndrome leads to a stenotic foramen magnum and facial retrusion.
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Craniosysnostosis surgical corrections are routine procedures in the pediatric neurosurgical field. However, these procedures result in significant blood loss. Tranexamic acid (TXA) is an antifibrinolytic drug, which has demonstrated a significant reduction in perioperative blood loss in many pediatric surgical procedures such as cardiac surgery and scoliosis surgery. We conducted a systematic review to evaluate protocols of TXA use in pediatric craniosynostosis procedures and its effect on intraoperative blood loss and transfusions. ⋯ TXA significantly reduces blood loss and the need for transfusions in children undergoing craniosynostosis surgery. TXA administration should be a routine part of strategy to reduce blood loss and limit transfusions in these procedures.
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Review Case Reports
Transoral migration of the inferior end of a ventriculoperitoneal shunt: A case report with literature review.
Ventriculoperitoneal (VP) shunt surgery is the most widely used technique for the treatment of hydrocephalus. However, it can incur certain complications. Beside frequent complications (infection, obstruction), migration of the peritoneal catheter is a rare but dangerous complication. ⋯ Progression was favorable. To the best of our knowledge, only 7 cases of VP shunt transoral extrusion were reported, but many risk factors were identified. Bowel perforation is a serious complication of VP shunt surgery, sometimes leading to fatal outcome.