craniosynostosis

craniosynostosis

DEFINITION• Craniosynostosis is a congenital (present at

birth) defect that causes one or more sutures on a baby's head to close earlier than normal. Sutures are connections that separate each individual skull bones. The early closing of a suture leads to an abnormally shaped head.

• Craniosynostosis (from cranio, cranium; + syn, together; + ostosis relating to bone) is a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by ossification, thereby changing the growth pattern of the skull

Classification of craniosyntosis

FIRST CLASSIFICATION:Single suture craniosyntosisComplex suture craniosyntosis

• Single suture craniosyntosis: only one of the four sutures is permanently closed.

• Complex suture craniosyntosis: when two or more sutures are no longer open.

SECOND CLASSIFICATION:ScaphocephalyTrigonocephalyPlagiocephalyBrachycephaly pansyntostosis

SCAPHOCEPHALY

• Premature sagittal suture closure restricts growth in a perpendicular plane, thus the head will not grow sideways and remain narrow.

• When viewed from sideways the resulting shape of the head will look a bit like a boat.

scaphocephaly

TRIGONOCEPHALY

• is a result from the premature closure of the metopic suture.

• The fusion will result in narrow fore head

Trigonocephaly

PLAGIOCEPHALY

Anterior plagiocephalyPosterior plagiocephaly

ANTERIOR PLAGIOCEPHALY

The sagittal suture ‘divides’ the coronal suture in two halves; unilateral meaning that either the right side or the left side to the sagittal suture is fused

POSTERIOR PLAGIOCEPHALY

Unilateral lambdoid synostosis is also called posterior plagiocephaly, indicating that this gives, just like unilateral coronal synostosis, a ‘skew head’. The difference is that this time, the deformity mostly shows at the occiput.

BRACHYCEPHALY

• Brachycephaly, or a ‘short head’, is the result of a closure of both the coronal sutures.

brachycephaly

PANSYNOSTOSIS

• All the sutures are closed• Seen with primary microcephaly

Congenital Anomalies

Types CraniosynostosisTypes of Craniosynostosis :

A. Sagittal synostosisB. Coronal synostosisC. Lambdoid synostosisD. Metopic synostosisE. Pansynostosis

Congenital Anomalies

1. Sagittal synostosis:

Congenital Anomalies

2. Coronal synostosis:

Congenital Anomalies

2. Coronal synostosis:

Congenital Anomalies

3. Lambdoid synostosis:

SIGNS & SYMPTOMS

CROUZON SYNDROME•Wide set, bulging eyes, beaked nose & flat face

APERT SYNDROME•Fused figers or toes and flat midface

CROUZONODERMOSKELTAL SYNDROME•Wide set, bulging eyes, beaked nose, flat face, black velvety skin fold, spine abnormality, benign growth in jaw

JACKSON WEISS SYNDROME•Enlarged bent toes and flat mid face

Muenke syndrome•Coronal syntosis,skeletal abnormalities of the hands or feet, hearing loss.

Pfeiffier syndrome•Broad , short thumbs or big toes, webbed or vfused fingers or toes

Loeys dietz syndrome•Wide set eye, split uvula or cleft palate, anueurysms

Saethre chotzen syndrom•Loss frontal hair line, dropping eyelids, webbed finger or toes

Shprintzen gold berg syndrome•Bulging eys, flat face, hernias, long thin fingers & mental retardatio

CAUSESBiochemical factors- fetal head constraint

during pregnanacy.Environmental factors- maternal smoking,

exposure to drugs like valporic acid.Hormonal factors- high level of thyroid

hormone.Genetic factors- found in fibroblast growth

factor receptor 3 & twist genes.

CLINICAL MANIFESTATIONABSENCE OF NORMAL FEELING OF A SOFT SPOT FONTANELLE ON THE NEWBORNS SKULL

DISAPPEARANCE FONTANELLE EARLY

RAISED HARD RIDGE ALONG THE AFFECETD SUTURES

UNUSUAL HEAD SHAPES AND NO OR SLOW INCREASE IN THE HEAD SIZE AS THE BABY GROWS

DIAGNOSTIC FINDINGS

Medical history of mother during pregnancyRisk factors during pregnancyPhysical examinationRadiographic analysis

Tratment

• Time of surgery: perform surgery early in pregnancy between 6 to 12 months because the bone is still more malleable & can be remodelled.

SURGICAL MANAGEMENT

• FOR SCAPHOCEPHALY:ist step: craniectomyiind step- cranial vault remodelling

• TRIGONOCEPHALY :• A bone graft is placed in between the two

halves of the supraorbital bars , thereby increasing thewidth between orbits

• POSTERIOR PLAGIOCEPHALY:• Excision of the flattened occipital bone with

release of the fused sutrure.

Nursing management

• Preparation of child for surgery• Explanation of procedure • Preoperative teaching• Preparation of surgical part• If tracheostomy placed- tracheostomy care is

given.• Syringe may be used to feed the child slowly &

frequently