Background: Pancraniosynostosis is rare and presents with premature fusion of greater than three major cranial sutures. There are four main presentations of primary pancraniosynostosis: kleeblattschädel, difficult to classify complex cranial dysmorphologies, progressive cranial suture fusion in syndromic craniosynostosis, and normocephalic. Compared to the first three groups, patients in the latter group with normocephaly present much later in childhood with symptoms of elevated intracranial pressures (ICP) such as papilledema on funduscopic exam, headaches, personality changes, nausea, vomiting and developmental delay.
Objectives: The purpose of this study is to compare the clinical presentation and treatment course of patients with normocephalic pancraniosynostosis against those with kleeblattschädel, complex, and progressive types of pancraniosynostosis.
Methods: Retrospective chart review of patients who presented to the craniofacial surgery clinic at the Children’s Hospital of Philadelphia with primary pancraniosynostosis born between January 1, 2000 and February 1, 2009. Patients with secondary causes of pancraniosynostosis such as ventriculo-peritoneal shunting, metabolic disease, microcephaly, and hematologic disorders were excluded. The study group was subdivided into the following diagnostic groups: kleeblatschädel, complex, progressive, and normocephalic pancraniosynostosis. Data extracted include presenting symptoms, headshape, presence of clinical and radiologic evidence of elevated ICP. Radiologic studies were also reviewed for specification of locations of craniosynostosis. Surgical intervention(s) were noted and the clinical course of the patients after their operation(s) were followed. The between-group difference in the initial age of presentation and age of first cranial vault surgery were compared between the normocephalic pancraniosynostosis group and the remaining three groups using t test.
Results: There were 17 patients with primary pancraniosynostosis: 4 kleeblatschädel, 8 complex, 1 progressive, and 4 normocephalic. The average age at presentation and length of follow-up were 1.1 years and 3.8 years respectively. Compared to the other three groups of patients, the patients with normocephalic pancraniosynostosis presented at an average age of 3.5 years compared to 2 months (p=0.01). The age at first cranial vault remodeling surgery for the normocephalic pancraniosynostosis group was 4 years 8 months compared to 11 months in the other three groups (p=0.004). Table 1 summarizes the clinical and radiological findings of the four groups of patients.
Table:

Table 1: Clinical Course and Presence of Clinical Signs of Elevated ICP
Pancraniosynostosis Type	Kleeblattschädel (N=4)	Complex (N=8)	Progressive (N=1)	Normocephalic (N=4)
Age at Initial Presentation	1.5m (1d-6m)	0.7m (4d-2.5m)	16m	3y6m (3y-5y10m)*
Craniectomy at Infancy	3 (75%)	6 (75%)	-	-
First Cranial Vault Remodeling Procedure	8m	12m	18m	4y8m*
Papilledema	-	-	1 (100%)	3 (75%)
Headache	-	-	-	2 (50%)
Nausea/Vomiting	-	-	-	2 (50%)
Radiologic Thumbprinting	-	1 (14%)	1 (100%)	4 (100%)
Associated Syndromic Craniodysostosis	3/4 (75%)	5/8 (63%)	1 (100%)	1 (25%)
d=day(s); m=month(s); y=year(s); * p<0.05