Table of Contents  
Year : 2015  |  Volume : 8  |  Issue : 3  |  Page : 364-366  

Craniosynostosis surgery-anaesthetic challenges and implications

1 Department of Anaesthesiology and Intensive Care Medicine, Gian Sagar Medical College, Banur, Patiala, Punjab, India
2 Department of Neuroanaesthesia, Sanjay Gandhi Post Graduate Institute, Lucknow, Uttar Pradesh, India

Date of Web Publication15-May-2015

Correspondence Address:
Sukhminder Jit Singh Bajwa
House No. 27-A, Ratan Nagar, Tripuri, Patiala - 147 001, Punjab
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How to cite this article:
Bajwa SS, Haldar R. Craniosynostosis surgery-anaesthetic challenges and implications. Med J DY Patil Univ 2015;8:364-6

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Bajwa SS, Haldar R. Craniosynostosis surgery-anaesthetic challenges and implications. Med J DY Patil Univ [serial online] 2015 [cited 2023 Sep 30];8:364-6. Available from:

Craniofacial and neurosurgical procedures have always posed numerous challenges to the attending anesthesiologists. [1],[2],[3] Craniofacial anomalies are difficult to manage from surgical and anesthetic viewpoint. Craniosynostosis is a developmental defect characterized by premature fusion of one or more joints of the skull vault leading to deformity and restricted growth of brain. Surgical correction aims to remodel the skull bones employing techniques like strip craniectomy, spring assisted cranioplasty, bilateral craniectomy with or without fronto orbital advancement and posterior advancement and remodeling and the calvarial reconstruction. The association of this condition with different syndromes compounded by the various inherent intricacies of infant physiology makes it a demanding procedure in pediatric neuroanesthetic practice.

Craniosynostosis may occur in isolation (80%) or as a part of the syndrome (20%). Isolated craniosynostosis involves one suture, is not associated with any other abnormalities and has a nongenetic etiology (presumably intrauterine fetal head restriction).Conversely syndromic craniosynostosis are complex involving multiple sutures and have a genetic basis like mutations in the osteogenic process of the skull. [4] The different syndromes associated with craniosynostosis are - Muenke (commonest 30%), Apert, Crouzon, Pfeiffer and Saethre-Chotzen - each presenting with their characteristic appearances and defects.

Surgical correction is dictated by concerns relating to airway protection, eye protection and management of raised intracranial pressure (ICP). The timing of the surgery is contentious issues. Early surgery (3-6 months) provides the advantage of softer bones where remodeling is easier. Disadvantages consist of smaller blood volume in patients and need for repeat surgeries. In older children, bones become difficult to remodel, and loss of ossification properties necessitates bone grafting. Operating at 1-year confers the advantages of early surgery with reduced disadvantages. [5]

Anesthetic management of these cases requires meticulous preoperative assessment. A multidisciplinary evaluation is required along with the usual reviewing of medical history, medications, allergies and previous anesthetic history. Infants with asyndromic craniosynostosis are generally healthy infants, however in patients with syndromic craniosynostosis, the associated anomalies stipulate necessary precautions. History of obstructive sleep apnoea (50% prevalence in Apert, Crouzon and Pfeiffer syndromes) [6] and congenital heart diseases should be elicited. Preoperative laboratory tests include hematocrit, platelet count and coagulation studies. Availability of adequate volumes of typed and crossmatched blood products must be ensured beforehand.

Many young infants may not require any premedication. However that exhibiting separation anxiety may need premedications like oral midazolam. Its use should be restricted in patients with obstructive sleep apnea. Those with a potentially difficult airway might require an intravenous (i.v.) line, the placement of which should be aided with topical anaesthetics. At least two large bore i.v. catheters must be sited.

Standard monitoring in accordance with American Society of Anesthesiologist guidelines is needed. An invasive arterial line (for stringent blood pressure monitoring and frequent sampling) and a central venous catheter (anticipating massive blood loss or venous air embolism [VAE]) are required. Advanced monitoring aids like precordial Doppler and end-tidal capnography increase the detection of VAE and should be employed whenever feasible.

Induction of anesthesia can be inhalational or i.v. Difficulty in i.v. access and an anticipated difficult airway makes inhalational induction with spontaneous respiration preferable. This optimizes condition for i.v. access and also avoids the risk of sudden airway loss. Airway management difficulties are expected in these patients. In Apert syndrome midface hypoplasia and proptosis may be present. Choanal stenosis and small nares increase airflow resistance. These factors make mask ventilation difficult. [7],[8] Children with Apert syndrome may have fused cervical vertebrae requiring fiberoptic intubation. Previous surgeries altering the airway anatomy may also predispose to difficult intubation. Maneuvers such as oropharyngeal airway, nasopharyngeal airway or continuous positive airway pressure usually avert airway obstruction. Tube kinking or compression is avoided by using reinforced tubes or Ring-Adair-Elwyn tubes. Accidental dislodgement causing endobronchial intubation or extubation is circumvented by using enhanced adhesive tapes, suturing or circum-mandibular fixation of the tube. Balanced anesthesia is maintained using opioid, volatile agents and air/oxygen mixture.

The patients may be positioned in supine, prone or modified prone (sphinx) positions. Padding of pressure points and prevention of pressure over the orbits to avoid corneal abrasions, retinal ischaemia and optic nerve damage is required. Neck pressure is to be avoided which obstructs venous return and increase ICP. Ideally a neutral position without table tilt is suited as it balances venous bleeding control (head down position) versus prevention of VAE. [9] Proper care of eyes in the form of lubrication, taping or tarsorraphy should be done.

Intraoperatively management of the unavoidable blood loss presents the biggest challenge. Blood loss occurs from the subgaleal tissues and bony ends. Breeching the dural sinuses can have drastic effects. In cases with anticipated blood loss, transfusion should be started at incision and continued intraoperatively to avoid hemodynamic disturbances. The blood components for transfusion should be leucocyte depleted, seronegative for cytomegalovirus, stored for <2-3 weeks and transfused through screen filter (170-200 μm). [10] Strategies to conserve blood include preoperative autologous blood donation, isovolumic hemodilution, erythropoietin administration, intraoperative cell salvage, fibrinolytic therapy and induced hypotension.

These patients are vulnerable to develop hypothermia as significant heat loss occurs through the exposed head and blood loss. Moreover, the patients with Apert syndrome are known to have excessive sweating. Covering of the extremities raised operation theatre temperature, warm air blankets and fluid warmers should be employed throughout the procedure.

Venous air embolism is another potentially serious intraoperative complication with an incidence ranging up to 82.6%. [11] Rapid blood loss causing reduction in central venous pressure facilitates air entrainment with consequences like hypotension, cardiovascular collapse and paradoxical embolism in presence of patent foramen ovale.

Intraoperative airway complications like bronchospasm and wheezing are present to a greater degree than the normal population as complete or partial tracheal cartilage sleeve abnormalities lead to accumulation of secretions, decreased tracheal distensibility and increased injury during suctioning. Thus, suctioning is to be done carefully, and greater depth of anesthesia is to be maintained during airway stimulation. [12]

Postoperatively majority of the patients can be extubated. However in special circumstances like hemodynamic instability, prolonged surgery or difficult airway, elective postoperative ventilation can be considered. Serial hematocrit and coagulation studies are warranted continuously as oozing is common. I.v. opioids inadequate dosing forms the mainstay of pain management with caution to be exercised in patients with sleep apnea or difficult airway. Non-steroidal anti-inflammatory drugs's are avoided to prevent bleeding. Paracetamol can be used as a useful adjunct to opioids. [13]

To summarize, craniosynostosis is a complex condition presenting a challenge for anesthesiologist complicated by the young age of patients, associated syndromes, high incidences of intraoperative complication and demands strict intraoperative and postoperative care. With detailed planning and proper conduct, the outcome of such cases is usually excellent.

  References Top

Bajwa SJ, Haldar R. Endocrinological disorders affecting neurosurgical patients: An intensivists perspective. Indian J Endocrinol Metab 2014;18:778-83.  Back to cited text no. 1
Bajwa SS, Kulshrestha A. Craniofacial and maxillary anomalies: Anesthetic implications and management. J Sci Soc 2014;41:73-8.  Back to cited text no. 2
  Medknow Journal  
Bajwa SJ, Kaur J, Singh A, Kapoor V, Bindra GS, Ghai GS. Clinical and critical care concerns of cranio-facial trauma: A retrospective study in a tertiary care institute. Natl J Maxillofac Surg 2012;3:133-8.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
Morriss-Kay GM, Wilkie AO. Growth of the normal skull vault and its alteration in craniosynostosis: Insights from human genetics and experimental studies. J Anat 2005;207:637-53.  Back to cited text no. 4
Thomas K, Hughes C, Johnson D, Das S. Anesthesia for surgery related to craniosynostosis: A review. Part 1. Paediatr Anaesth 2012.  Back to cited text no. 5
Moore MH. Upper airway obstruction in the syndromal craniosynostoses. Br J Plast Surg 1993;46:355-62.  Back to cited text no. 6
Nargozian C. The airway in patients with craniofacial abnormalities. Paediatr Anaesth 2004;14:53-9.  Back to cited text no. 7
Cohen MM Jr. An etiologic and nosologic overview of craniosynostosis syndromes. Birth Defects Orig Artic Ser 1975;11:137-89.  Back to cited text no. 8
Faberowski LW, Black S, Mickle JP. Incidence of venous air embolism during craniectomy for craniosynostosis repair. Anesthesiology 2000;92:20-3.  Back to cited text no. 9
Gibson BE, Todd A, Roberts I, Pamphilon D, Rodeck C, Bolton-Maggs P, et al. Transfusion guidelines for neonates and older children. Br J Haematol 2004;124:433-53.  Back to cited text no. 10
Faberowski LW, Black S, Mickle JP. Incidence of venous air embolism during craniectomy for craniosynostosis repair. Anesthesiology 2000;92:20-3.  Back to cited text no. 11
Elwood T, Sarathy PV, Geiduschek JM, Ulma GA, Karl HW. Respiratory complications during anaesthesia in Apert syndrome. Paediatr Anaesth 2001;11:701-3.  Back to cited text no. 12
Koh JL, Gries H. Perioperative management of pediatric patients with craniosynostosis. Anesthesiol Clin 2007;25:465-81, viii.  Back to cited text no. 13


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