craniofacial surgery dr milan knezevic
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CRANIOFACIALSURGERY
Calvarial Reconstruction for Syndromic and
Nonsyndromic Craniosynostosis
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Paul Tessier, Harold Gillies and Rene LeFort, for their remarkable work in the field of bony craniofacial surgery. Each one of them did it in his own way.
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The father of Craniofacial Surgery, Paul Tessier based his techniques on the principle that the bones must be repositioned or reconstructed before the soft tissue can be repaired.
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When this angel comes to the world his wonderful aspect is the nicest image we can feel…
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However, in early stages after birth, it must be checked out a state of cranial sutures and we know that they are….
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Craniosynostosisis a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses. This results in restricted skull and brain growth.
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Growing brain gives the shape of the skull.If all the sutures fuse early, the head remains abnormally small, which can cause intellectual disability or blindness, even death. If only one or some fuse early, the skull grows in other directions and becomes deformed. Surgery in the first two years keep these sutures open for longer time, thus minimizing the complications.
Cranial deformity is produced when the bones of the skull fuse too early
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NON SYNDROMIC CRANIOSYNOSTOSIS
SYNDROMIC CRANIOSYNOSTOSI
S
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1.PrimarySimpleNonsyndromic: sagittal, coronal, metopic, lambdoidCompoundNonsyndromic: bicoronalSyndromic: Crouzon’s disease, Apert’s syndrome, Pfeiffer’s disease, Saethre-Chotzen syndrome
2. SecondaryMetabolic disorders (e.g., hyperthyroidism)Malformations (e.g., holoprosencephaly, microcephaly, shunted hydrocephalus, encephalocele)Exposure of fetus (e.g., valproic acid, phenytonin)Mucopolysaccharidosis (e.g., Hurler’s syndrome, Morquio’s syndrome)
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Scaphocephaly : suturectomy and expanding osteotomies including
frontal bone remodelling
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Sccaphocephaly ctd.
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Occipital plagiocephaly: is remodeled by occipital bilateral craniotomy
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Positional molding: nowadays can be resolved by use of specially designed helmets
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Anterior unilateral plagiocephaly: Frontal bilateral remodelling with frontal-orbital “bandeau”
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Trigonocephaly: Frontal remodelling without fronto-orbital "bandeau"
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Brachycephaly ( flat head): standard bilateral fronto-orbital advancement with expanding
osteotomies
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Crouzon´s syndrome: an inherited disorder that is controlled by an autosomal dominant gene and that is characterized by malformation of the skull due to premature ossification and closure of the sutures and by widely spaced eyes, abnormal protrusion of the eyeballs, a beaked nose, and underdevelopment of the maxilla with (pseudo)protrusion of the mandible - called also craniofacial dysostosis.
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Crouzon´s Disease : standard bilateral fronto-orbital advancement with or without
fronto-orbital bandeau plus expanding fronto-parietal osteotomies
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Apert syndrome (acrocephalosyndactily):
A malformation syndrome characterized by a high short skull
underdevelopment of the midface, soft tissue and bony ("mitten glove")
fusion of fingers and toes, fusion of the neck vertebrae, and varying
degrees of developmental delay/mental retardation.
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Apert´s Syndrome (acrocephalosyndactily): standard bilateral fronto-orbital advancement plus expanding
fronto-parietal osteotomies and correction of syndactily
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From 2001 to 2008
142 patients operated
46 females 96 males
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Ages of treated patients
were from 6 months to 32
months with mean age at
the time of surgery of 12,7
months
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142 primary surgical procedures were performed
26 secondary surgical procedures needed to be done (as correction or as scheduled second phase surgery)
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77 cases of Scaphocephaly25 cases of Trigonocephaly16 cases of diverse craniofacial syndromes (6 Crouzon´s; 5 Aperts; 3 Pfeifers;1 Seathre-Chotzen and 1 clover-leaf skull)15 cases of Anterior Plagiocephaly9 cases of non syndromic multiple-suture synostosis
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Mortality rate : 1 case of Brachycephaly within 48 hours after the surgery
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Complications after first Surgery:
16 postoperative hyperthermias11 cases of infection7 subcutaneous hematomas7 cases of Dural tear2 cases of CSF leakage
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Complications after secondary surgical
procedure
23 cases of Dural tear
19 cases of CSF leakage
15 cases of infection
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Patient´s (Parent´s) Respond to Surgical Outcome:
119 defined them as GOOD
23 Conclude that they were BAD