Abstract
This chapter, provides an excellent review of a core pediatric surgical topic; craniosynostosis and cranial vault remodeling. The authors describe the pathophysiology of the four main cranial sutures and the characteristics associated with each of their premature closures. The syndromes associated with the synostoses are reviewed. The perioperative considerations for cranial vault reconstruction are presented.
A 12-month-old, 9.2 kg healthy child presents for an open cranial vault repair for sagittal synostosis. All preoperative laboratory studies (CBC, electrolyte panel, coagulation studies) are normal, with the exception of an elevated PTT (46.0, normal 22.0–36.0).
Physical examination, family history, and review of systems are unremarkable. There was no history of prior general anesthesia, and his surgical history was notable for an uneventful circumcision at birth. A repeat PTT was drawn the morning of surgery and remained elevated (41.0).
Diagnosis
What Is Craniosynostosis?
Craniosynostosis is a premature closure of one or more of the cranial sutures.
What Is the Incidence of Craniosynostosis in Children?
The incidence is 1:2000 births, males more commonly affected than females.
What Is the Natural Progression of Cranial Suture Closure?
The anterolateral fontanelle closes by approximately three months of age, the posterior fontanelle by three to six months, the anterior fontanelle by 9–18 months, posterolateral fontanelle by two years.
What Are the Four Cranial Sutures Involved in Craniosynostosis?
The four cranial sutures are sagittal, coronal, lamboid, and metopic. The premature closure of the sagittal suture is the most common simple craniosynostosis (50%) followed by coronal (20%) and metopic (10%) (Figure 40.1).
Figure 40.1 Pictorial representation of cranial sutures.
The growth of the skull is in the direction perpendicular to the fused suture, resulting in characteristic skull appearances.
Simple versus Complex Craniosynostosis
Simple Synostosis. Nonsyndromic or one suture prematurely closed, 80% of diagnoses.
Complex Synostosis. Syndromic or involving the premature closure of two or more sutures, 20% of diagnoses.
Which Syndromes Are Most Commonly Associated with Craniosynostosis?
Craniosynostosis can be a component of many named syndromes; however, the most common syndromes encountered are: Crouzon, Apert, Pfeiffer, Saethre-Chotzen, and Carpenter.
What Are the Common Clinical Characteristics of Apert Syndrome?
Apert syndrome is a spontaneously occurring autosomal dominant syndrome occurring in approximately 1:100,000–1:160,000 patients. A multitude of systems can be affected including:
Cranio-facial with coronal synostosis, proptosis, hypertelorism, midfacial hypoplasia, cleft palate, choanal or tracheal stenosis (Figure 40.2).
Respiratory with a 40–50% incidence of obstructive sleep apnea.
Cardiac with a 10% incidence of congenital heart disease, especially ventricular septal defect (VSD) and pulmonary stenosis.
Genitourinary with cryptorchidism and hydronephrosis.
Musculoskeletal with syndactyly.
Neurologic with developmental delay, and elevated intracranial pressures.
Figure 40.2 Child with Apert syndrome, hypertelorism, and midfacial hypoplasia.
What Are the Common Clinical Characteristics of Crouzon Syndrome?
Crouzon syndrome can be sporadically occurring or inherited. A multitude of systems can be affected including:
Craniofacial including cranial synostosis (most commonly coronal and lamboid), fontal bossing, midface hypoplasia, and airway obstruction leading to obstructive sleep apnea (Figure 40.3).
Optic atrophy occurs in roughly 20% of patients.
Neurologic: these patients can have mild developmental delay and increased intracranial pressure.
These patients can be difficult to mask ventilate due to their facial features.
