Craniofacial Syndromes

Craniofacial Syndromes Part II: Atypical Facial Clefts, Romberg Syndrome, Moebius Syndrome, Fibrous Dysplasia, and Neurofibromatosis

10.2310/ps.10120 ◽

2020 ◽

Author(s):

Francesca Saldanha ◽

Cory M. Resnick ◽

Carolyn R. Rogers-Vizena

Keyword(s):

Fibrous Dysplasia ◽

Treatment Plan ◽

Moebius Syndrome ◽

Hemifacial Atrophy ◽

Mccune Albright Syndrome ◽

Facial Clefts ◽

Progressive Hemifacial Atrophy ◽

Parry Romberg Syndrome ◽

Albright Syndrome ◽

Craniofacial Syndromes

This final article of the two-part craniofacial series continues to provide the embryologic and developmental foundations necessary to understand congenital craniofacial pathology. Clinical characteristics and molecular genetics needed to make an accurate diagnosis and formulate a treatment plan will be detailed for atypical craniofacial clefts, Moebius Syndrome, Fibrous Dysplasia, Progressive Hemifacial Atrophy (Parry-Romberg syndrome) and Neurofibromatosis. Details of the operations applied in treatment of these disorders are discussed in separate Scientific American: Plastic Surgery (SAPS) articles as referenced. This review contains 3 tables, and 10 figures, and 42 references. Keywords: craniofacial, Tessier cleft, atypical facial cleft, Romberg syndrome, Parry-Romberg syndrome, Moebius syndrome, fibrous dysplasia, McCune Albright syndrome, neurofibromatosis

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Craniofacial Syndromes and Malformations

Handbook of Pediatric Eye and Systemic Disease ◽

10.1007/0-387-27928-8_4 ◽

2007 ◽

pp. 146-226 ◽

Cited By ~ 1

Author(s):

Marilyn T. Miller ◽

Anna Newlin

Keyword(s):

Craniofacial Syndromes

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Elevated intracranial pressure with craniosynostosis: a multivariate model of age, syndromic status, and number of involved cranial sutures

Journal of Neurosurgery Pediatrics ◽

10.3171/2021.6.peds21162 ◽

2021 ◽

pp. 1-8

Author(s):

Christopher L. Kalmar ◽

Zachary D. Zapatero ◽

Mychajlo S. Kosyk ◽

Anna R. Carlson ◽

Scott P. Bartlett ◽

...

Keyword(s):

Intracranial Pressure ◽

Multivariate Model ◽

Cranial Sutures ◽

Actual Number ◽

Corrective Surgery ◽

Inclusion Criteria ◽

Significant Difference ◽

Single Suture ◽

Effect Of Age ◽

Craniofacial Syndromes

OBJECTIVE Children with multiple prematurely fused cranial sutures and those undergoing surgical correction later in life appear to experience worse neurocognitive outcomes, but it is unclear whether higher intracranial pressure (ICP) is implicated in this process. The purpose of this study was to elucidate the effect of age at intervention and number of involved cranial sutures on ICP, as well as to assess which cranial suture closure may be more associated with elevated ICP. METHODS The prospective craniofacial database at the authors’ institution was queried for patients undergoing initial corrective surgery for craniosynostosis in whom intraoperative measurement of ICP was obtained prior to craniectomy. Age, involved sutures, and syndromic status were analyzed in the context of measured ICP by using multiple linear regression. RESULTS Fifty patients met the inclusion criteria. Age at procedure (p = 0.028, β = +0.060 mm Hg/month) and multiple-suture involvement (p = 0.010, β = +4.175 mm Hg if multisuture) were both significantly implicated in elevated ICP. The actual number of major sutures involved was significantly correlated to ICP (p = 0.001; β = +1.687 mm Hg/suture). Among patients with single-suture involvement, there was an overall significant difference of median ICP across the suture types (p = 0.008), with metopic having the lowest (12.5 mm Hg) and sagittal having the highest (16.0 mm Hg). Patients with multiple-suture involvement had significantly higher ICP (p = 0.003; 18.5 mm Hg). Patients with craniofacial syndromes were 79.3 times more likely to have multiple-suture involvement (p < 0.001). Corrective surgery for craniosynostosis demonstrated significant intraoperative reduction of elevated ICP (all p < 0.050). CONCLUSIONS Syndromic status, older age at intervention for craniosynostosis, and multiple premature fusion of cranial sutures were associated with significantly higher ICP.

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Paediatric neuroscience care

Oxford Handbook of Neuroscience Nursing ◽

10.1093/med/9780198831570.003.0014 ◽

2020 ◽

pp. 467-492

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Neural Tube ◽

Neural Tube Defects ◽

Spinal Disorders ◽

Adult Services ◽

Central Nervous System Tumours ◽

Basic Understanding ◽

Craniofacial Syndromes

Paediatric neurology services care for children aged 0–16 years. Many of these children suffer genetic and complex neurological problems and frequently require life-long support. Hydrocephalus and central nervous system tumours are commonly presenting disorders; however, craniofacial syndromes, spinal disorders, spinal neural tube defects, epilepsy, and trauma including non-accidental injuries are frequently managed. Paediatric nurses specialize in the care of these children and support of their families but as they grow up and transition into adult services, it is the responsibility of general nurses to have a basic understanding of some of their conditions so they can maintain and continue their care.

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