Craniosynostosis Part I: Pathophysiology and Patient Evaluation

2020 ◽  
Author(s):  
Jonathan Y. Lee ◽  
Jesse A. Goldstein
Keyword(s):  
Intracranial Pressure ◽  
Apert Syndrome ◽  
Patient Evaluation ◽  
Crouzon Syndrome ◽  
Head Shape ◽  
Cranial Growth ◽  
Optimal Intervention ◽  
Intracranial Pressures ◽  
Team Setting ◽  
Multi Disciplinary Team

Craniosynostosis is the premature fusion and obliteration of one or more cranial sutures. As a result, cranial growth can only occur parallel to the involved suture(s) resulting in predictable head shape morphologies. More importantly, the restricted cranial growth can also have a deleterious effect on the growing brain with increased intracranial pressures leading to abnormal neurocognitive development, blindness, and death. Craniosynostosis can either be syndromic or non-syndromic; and therefore, patient evaluation should be performed in a multi-disciplinary team setting for comprehensive care. Physical exam focuses on head shape morphology, associated anomalies, and signs of intracranial pressure. Imaging with 3D CT is gold standard at confirming diagnosis and extent of suture involvement. Early diagnosis is key for planning optimal intervention. This review contains 14 figures, 4 tables and, 42 references. Keywords: craniosynostosis, virchow’s law, plagiocephaly, trigonocephaly, scaphocephaly, brachycephaly, Apert Syndrome, Crouzon Syndrome, Pfeiffer Syndrome, intracranial pressure

The effects of mass-induced intracranial pressures on arterial hypertension and survival in awake cats

1972 ◽  
Vol 37 (5) ◽  
pp. 514-527 ◽  
Author(s):  
Stanley J. Goodman ◽  
Donald P. Becker ◽  
John Seelig
Keyword(s):  
Intracranial Pressure ◽  
Systemic Hypertension ◽  
Content Type ◽  
Arterial Blood ◽  
Mass Size ◽  
Intracranial Pressures ◽  
Relationship Of ◽  
The Relationship ◽  
Awake Cats ◽  
Supratentorial Mass

✓ Intracranial pressures above and below the tentorium, arterial blood pressure, heart rate, and respiratory rate were recorded continuously before, during, and after expansion of a supratentorial mass in awake unsedated cats. In general, as the mass enlarged, the intracranial pressure rose; however, considerable variation was observed among animals with respect to specific mass size and associated intracranial pressures. There was considerable variation in the relationship of supratentorial pressure to infratentorial pressure. No animal survived that had sustained a mass-induced pressure exceeding 1100 mm H2O, and survival was shorter with greater pressures. Systemic hypertension occurred always and only when the infratentorial pressure exceeded 600 mm H2O, regardless of the magnitude of the associated supratentorial intracranial pressure. The methodological limitations of previous studies of mass-induced intracranial hypertension appear to have been substantially reduced by the technique described.

scholarly journals Lack of association of cranial lacunae with intracranial hypertension in children with Crouzon syndrome and Apert syndrome: a 3D morphometric quantitative analysis

2019 ◽  
Vol 35 (3) ◽  
pp. 501-507
Author(s):  
Ankita Mondal ◽  
Naiara Rodriguez-Florez ◽  
Justine O’Hara ◽  
Juling Ong ◽  
N. u. Owase Jeelani ◽  
...  
Keyword(s):  
Quantitative Analysis ◽  
Intracranial Hypertension ◽  
Apert Syndrome ◽  
Crouzon Syndrome ◽  
Hypertension In Children

Facial Growth in Patients with Apert and Crouzon Syndromes Compared to Normal Children

2012 ◽  
Vol 49 (2) ◽  
pp. 185-193 ◽  
Author(s):  
Jacobus H. Reitsma ◽  
Edwin M. Ongkosuwito ◽  
Peter H. Buschang ◽  
Birte Prahl-Andersen
Keyword(s):  
Growth Patterns ◽  
Apert Syndrome ◽  
Control Group ◽  
Crouzon Syndrome ◽  
Facial Growth ◽  
Normal Children ◽  
Control Subjects ◽  
Facial Height ◽  
Anterior Cranial Base ◽  
Lateral Cephalograms

Objective To evaluate vertical and sagittal facial growth in children with Apert and Crouzon syndromes and compare it to the growth patterns of a nonsyndromic control group. Design Case-control study. Setting Department of Orthodontics, Children's Hospital Erasmus Medical Centre, Sophia, Rotterdam, The Netherlands. Patients, Participants Sixty-two patients (37 patients with Crouzon syndrome and 25 patients with Apert syndrome) born between 1971 and 2001 (age range 3.9 to 32 years) and 482 nonsyndromic children as a control group. Interventions Lateral cephalograms performed prior to any midfacial surgery of 62 patients and 482 nonsyndromic children were traced and horizontal and vertical measurements were digitized. Main Outcome Measures Cephalometric measurements of SNA, SNB, ANB, NSMe, and SN/palatal plane angles and lower facial height ratio. Results Horizontal measurements for the syndromic groups showed no change in SNA angle during growth. SNA angles were lower in patients with Apert syndrome compared to patients with Crouzon syndrome. The syndromic groups showed smaller values for ANB angles compared to the nonsyndromic group. Vertical measurements showed increased lower facial height ratios for the syndromic groups compared to control subjects. There was an increasing counterclockwise rotation of the palatal plane in relation to the anterior cranial base in syndromic patients. NSMe angles among the three groups were not significantly different. Conclusions Based on the growth differences identified, the sagittal and vertical jaw relationships differ in patients with Crouzon syndrome, patients with Apert syndrome, and control subjects. Syndromic patients show aggravation of midfacial underdevelopment and anterior rotation of the mandible.

Dental Maturation in Children with the Syndrome of Crouzon and Apert

2014 ◽  
Vol 51 (6) ◽  
pp. 639-644 ◽  
Author(s):  
Jacobus H. Reitsma ◽  
Inge H. Balk-Leurs ◽  
Edwin M. Ongkosuwito ◽  
Evert Wattel ◽  
Birte Prahl-Andersen
Keyword(s):  
Gender Differences ◽  
Cleft Palate ◽  
Apert Syndrome ◽  
Crouzon Syndrome ◽  
Tooth Formation ◽  
Dental Maturation ◽  
Panoramic Radiographs ◽  
And Gender ◽  
Logistic Functions ◽  
Over Time

Purpose Developing teeth are used to assess maturity and estimate age in a number of disciplines. The purpose of this investigation was to study the dental maturation in children with Crouzon or Apert syndrome compared with nonsyndromic controls. Patients and Methods Records of 40 children with Crouzon syndrome (18 boys and 22 girls, aged 4.0 to 17.9 years) and 28 children with Apert syndrome (10 boys and 18 girls, aged 3.9 to 15.1 years) were referred to the Department of Orthodontics, Cleft Palate Team and Craniofacial Team, Erasmus MC–Sophia. Data from syndromic children were compared with data from 451 nonsyndromic children (225 boys and 226 girls, aged 2.9 to 16.9 years). From panoramic radiographs, dental maturation was determined for patients with Crouzon and Apert syndromes and compared with data collected from control children. Logistic functions were constructed for dental maturation over time for syndromes and gender. Results Statistically significant gender differences in dental maturation scores were found for girls with Crouzon ( P < .05) and Apert syndrome ( P < .05). Patients with Apert syndrome demonstrated a significantly delayed dental maturation ( P < .05), while patients with Crouzon syndrome showed a nonsignificant delay. Conclusions Dental maturation in patients with Apert syndrome was more delayed than in patients with Crouzon syndrome. The delay of tooth formation in patients with Crouzon or Apert syndrome suggests a possible common genetic association.

Apert syndrome results from localized mutations of FGFR2 and is allelic with Crouzon syndrome

1995 ◽  
Vol 9 (2) ◽  
pp. 165-172 ◽  
Author(s):  
Andrew O.M. Wilkie ◽  
Sarah F. Slaney ◽  
Michael Oldridge ◽  
Michael D. Poole ◽  
Geraldine J. Ashworth ◽  
...  
Keyword(s):  
Apert Syndrome ◽  
Crouzon Syndrome

Intracranial Pressure in Head-injured Patients with Various Intracranial Lesions Is Identical throughout the Supratentorial Intracranial Compartment

Neurosurgery ◽  
1987 ◽  
Vol 21 (5) ◽  
pp. 688-692 ◽  
Author(s):  
Masami Yano ◽  
Yukio Ikeda ◽  
Shiro Kobayashi ◽  
Toshibumi Otsuka
Keyword(s):  
Intracranial Pressure ◽  
Head Injured ◽  
Intracranial Lesions ◽  
Intracranial Pressures ◽  
The Right ◽  
Injured Patients

Abstract Subarachnoid pressure was measured by bifrontal catheterization to obtain concurrent comparative intracranial pressures (ICPs). These ICPs were compared in 15 head-injured patients who had differences in the pathological processes affecting the right and left hemispheres. ICPs in these patients ranged from -3 to 130 mm Hg, but no difference was found in the concurrent comparative ICPs. These findings suggest that the supratentorial space can generally be considered as one compartment regardless of the differences in types of intracranial lesions. (Neurosurgery 21:688-692, 1985)

scholarly journals Unilateral Coronal Craniosynostosis in an Apert-Like Patient

2018 ◽  
Vol 27 (1) ◽  
pp. 78-82
Author(s):  
Navid Pourtaheri ◽  
Derek Z. Wang ◽  
Robert P. Lesko ◽  
Christopher M. Bonfield ◽  
Peter Taub ◽  
...  
Keyword(s):  
Case Report ◽  
Computerized Tomography ◽  
Apert Syndrome ◽  
Coronal Suture ◽  
Anterior Fontanelle ◽  
Head Shape ◽  
The Third ◽  
Web Space ◽  
Hands And Feet ◽  
Surgical Release

Background and Significance: Apert syndrome is a congenital disorder of patients who typically present with bilateral coronal craniosynostosis and varying degrees of complex syndactyly of the hands and feet, among other features. We describe a unique presentation of a rare Apert-like patient with unilateral coronal craniosynostosis and complex syndactyly of the hands and feet. Case Report: A 2-year-old male patient presented to the craniofacial clinic with his mother due to a concerning head shape. The patient also had bilateral syndactyly of the hands and feet and underwent prior surgical release of the third web space. Computerized tomography of the head illustrated a small open anterior fontanelle, a left harlequin orbit, complete left coronal craniosynostosis, and a patent right coronal suture. The patient subsequently underwent fronto-orbital advancement for expansion of the cranial vault and correction of the asymmetric forehead and orbit. The procedure resulted in improvement of his deformity. Conclusion: This case illustrates a unique presentation of an acrocephalosyndactyly (ACS) syndrome with asymmetric, unilateral coronal craniosynostosis and complete complex syndactyly of the hands and feet that is most consistent with Apert syndrome. Although the majority of patients with ACS can be categorized into known syndromes, other more unusual presentations must still be considered. Such unique cases are exceedingly rare and only through additional reporting and review of unique phenotypes can new subtypes of common ACS syndromes be classified.

Eponyms and maxillofacial syndromes

2011 ◽  
pp. 263-266
Author(s):  
Luke Cascarini ◽  
Clare Schilling ◽  
Ben Gurney ◽  
Peter Brennan
Keyword(s):  
Intracranial Pressure ◽  
Apert Syndrome ◽  
Raised Intracranial Pressure ◽  
Genital Ulceration ◽  
Behçet Syndrome ◽  
Behcet Syndrome ◽  
Autoimmune Condition

Apert syndrome is a craniosynostosis plus syndactyly leading to lack of anterior growth of the midface, raised intracranial pressure, and blindness due subluxation and lack of corneal cover of the eye. Behçet syndrome is a multisystem autoimmune condition associated with HLA-B5. Patients have oral and genital ulceration and uveitis. Treatment is highly specialized; mainly immunosuppressant....

Raised Intracranial Pressure in Apert Syndrome

2008 ◽  
Vol 122 (4) ◽  
pp. 1162-1168 ◽  
Author(s):  
Damian D. Marucci ◽  
David J. Dunaway ◽  
Barry M. Jones ◽  
Richard D. Hayward
Keyword(s):  
Intracranial Pressure ◽  
Apert Syndrome ◽  
Raised Intracranial Pressure
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