Perioperative management of patients undergoing craniosynostosis repair

Craniosynostosis is a type of craniofacial malformation because of premature fusion of cranial suture. The primary treatment is surgical correction which is usually done in stages over years to get best possible outcome. We did a retrospective analysis of five years from 1st August 2011 to 30th July 2016 in which we included twelve patients of craniosynostosis requiring corrective surgeries under general anaesthesia in our institution.


Introduction
Craniosynostosis is the premature fusion of one or more cranial sutures, resulting in an abnormal shape of head either from a primary defect of ossification (primary craniosynostosis) or as part of other syndromes (secondary craniosynostosis).It may be simple or single suture synostosis (only one suture) and complex or compound or multiple synostosis or craniosynostosis (more than one suture).Though craniosynostosis is a fairly prevalent disorder (1 per 2000 live births), syndromic craniosynostosis (SC, with other body deformities or systemic involvements) are relatively rare.The commonly associated syndromes include Crouzon, Apert and Pfeiffer, Saethre-Chotzen and Muenke syndromes with incidence of 1 in 25,000;1 in 100,000; 1 in 100,000; 1 in 25,000 to 50,000 and 1 in 10,000 live births respectively. 1he management of children with craniosynostosis is a multidisciplinary team approach.Providing anaesthesia for these patients presents unique challenges in terms of difficult airway, blood loss, prolonged anaesthesia in paediatrics, syndromespecific issues, metabolic disturbance and postoperative complications.

Case histories
We did a retrospective review of all the craniosynostosis cases done within five years (from 1st August 2011 to 30th July 2016) at a tertiary care center (Department of Anaesthesiology, S.M.S. Medical College, Jaipur, Rajasthan, India).We included twelve patients of craniosynostosis requiring corrective surgeries under general anaesthesia.Case specific presentation, examination findings and investigations in our patients are described in Table 1.

Discussion
Corrective surgeries involve craniofacial reconstruction to separate the fused bones which is mostly started in early childhood and performed in stages over years.Pre-anesthesia checkup (PAC) is undoubtedly an extremely important part of the anesthetic care for cranial vault surgery in patients with SC.Babies with craniosynostosis, particularly in case of fusion of multiple sutures or SC may develop increased intracranial pressure as their skulls don't expand enough to make room for growing brains which may lead to various manifestations including visual difficulties even blindness, nausea and vomiting, somnolence, headaches, seizures, brain damage and death in rare instances.We also transfused fresh frozen plasma and platelets for correction of microvascular bleeding in the setting of massive transfusion when coagulation tests could not be obtained in a timely manner.
During craniosynostosis surgery a large surface area is exposed to the atmosphere, which may result in excessive heat loss.We monitored core as well as peripheral temperature intraoperatively and normothermia was maintained by covering the body with cotton roll, using warm intravenous fluids and convective forced-air warming devices.Post-operative care should be provided in postoperative anaesthesia care unit or ICU because of lengthy surgery, unstable haemodynamics, elective ventilation and good nursing care.Out of the 12 cases described, 10 patients were discharged after successful recovery, but one patient (case 6, Table1) had arrhythmia and one patient (case 10, Table 1) developed seizures in the post-operative period.Appropriate antibiotics, anticonvulsant, good analgesics and maintenance of haematocrit level are the mainstays of postoperative care.

Conclusion
Management of craniosynostosis is challenging for an anaesthesiologist in view of paediatric patients with difficult airway, prolonged duration of surgery, high likelihood of significant blood loss, massive blood transfusions and post-operative complications.With proper planning, intraoperative vigilance, and postoperative care, patient outcome is satisfactory.

Table 1 :
Case-based presentation and management of patients