CRANIOSYNOSTOSIS is a congenital anomaly in which cranial sutures close prematurely and/or abnormally. Left uncorrected, craniosynostosis will lead to increased intracranial pressure and compression of the brain leading to seizures, developmental delay, and abnormal appearance. Therefore, surgical correction is recommended and delay of surgery to an older age is discouraged. Infants with craniosynostosis usually present for surgical repair between the ages of 4 and 9 months. Rarely, neonatal craniosynostosis, as in this patient, will be seen.

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Infants with craniosynostosis present challenges to the anesthesiologists in airway management, blood transfusion, and coagulation therapy.1,2Because of the abnormal head shape in these infants, positioning for intubation must be done carefully. For surgery done in first 4 months of life, the physiologic nadir of hemoglobin often coincides with the surgery, leading to increased need for intraoperative transfusion. In this case, the infant had surgery earlier than usual, increasing the risk of transfusion and need for postoperative intensive care.3The early timing of the surgery (patient was 5 weeks old and weighed 4 kg) was due to the evidence of impingement of brain growth seen in the image.

With premature closure of some of the cranial sutures, the growing brain and cranial contents bulge open other sutures and cause a misshapen head as can be seen with three-dimensional computed tomography scan (done at 3 days of life). Because of the prenatal and premature suture closures including the metopic and sagittal sutures, the growing brain in this confined skull causes forces resulting in enlarged squamousal sutures. In rare cases, the meninges are pushed into the skull, causing the lattice appearance on computed tomography scan.

1.
Ririe DG, David LR, Glazier SS, Smith TE, Argenta LC: Surgical advancement influences perioperative care: A comparison of two surgical techniques for sagittal craniosynostosis repair. Anesth Analg 2003; 97:699–703
2.
Duncan C, Richardson D, May P, Thiruchelvam J, Shong DC, Potter F, Grogan J, Caswell M: Reducing blood loss in synostosis surgery: The Liverpool experience. J Craniofac Surg 2008; 19:1424–30
3.
Meier PM, Goobie SM, DiNardo JA, Proctor MR, Zurakowski D, Soriano SG: Endoscopic strip craniectomy in early infancy: The initial five years of anesthesia experience. Anesth Analg 2011; 112:407–14