CRANIOFACIALSURGERY

Calvarial Reconstruction for Syndromic and

Nonsyndromic Craniosynostosis

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.

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.

When this angel comes to the world his wonderful aspect is the nicest image we can feel…

However, in early stages after birth, it must be checked out a state of cranial sutures and we know that they are….

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.

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

NON SYNDROMIC CRANIOSYNOSTOSIS

SYNDROMIC CRANIOSYNOSTOSI

S

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)

Scaphocephaly : suturectomy and expanding osteotomies including

frontal bone remodelling

Sccaphocephaly ctd.

Occipital plagiocephaly: is remodeled by occipital bilateral craniotomy

Positional molding: nowadays can be resolved by use of specially designed helmets

Anterior unilateral plagiocephaly: Frontal bilateral remodelling with frontal-orbital “bandeau”

Trigonocephaly: Frontal remodelling without fronto-orbital "bandeau"

Brachycephaly ( flat head): standard bilateral fronto-orbital advancement with expanding

osteotomies

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.

Crouzon´s Disease : standard bilateral fronto-orbital advancement with or without

fronto-orbital bandeau plus expanding fronto-parietal osteotomies

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.

Apert´s Syndrome (acrocephalosyndactily): standard bilateral fronto-orbital advancement plus expanding

fronto-parietal osteotomies and correction of syndactily

From 2001 to 2008

142 patients operated

46 females 96 males

Ages of treated patients

were from 6 months to 32

months with mean age at

the time of surgery of 12,7

months

142 primary surgical procedures were performed

26 secondary surgical procedures needed to be done (as correction or as scheduled second phase surgery)

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

Mortality rate : 1 case of Brachycephaly within 48 hours after the surgery

Complications after first Surgery:

16 postoperative hyperthermias11 cases of infection7 subcutaneous hematomas7 cases of Dural tear2 cases of CSF leakage

Complications after secondary surgical

procedure

23 cases of Dural tear

19 cases of CSF leakage

15 cases of infection

Patient´s (Parent´s) Respond to Surgical Outcome:

119 defined them as GOOD

23 Conclude that they were BAD