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    Craniosynostosis

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    Fathima Jansi Malapati
    Craniosynostosis is a birth defect where one or more skull sutures fuse prematurely, altering head shape. It can be classified as single or complex suture fusion. Types include scaphocephaly from sagittal fusion, trigonocephaly from metopic fusion, and brachycephaly from coronal fusion. Symptoms include abnormal head shape and lack of fontanelle movement. Causes may be genetic, biochemical or environmental. Treatment involves early surgical repair to reshape the skull.

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    Craniosynostosis

    Uploaded by

    Fathima Jansi Malapati
    450 views36 pages
    Craniosynostosis is a birth defect where one or more skull sutures fuse prematurely, altering head shape. It can be classified as single or complex suture fusion. Types include scaphocephaly from sagittal fusion, trigonocephaly from metopic fusion, and brachycephaly from coronal fusion. Symptoms include abnormal head shape and lack of fontanelle movement. Causes may be genetic, biochemical or environmental. Treatment involves early surgical repair to reshape the skull.

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    Craniosynostosis is a birth defect where one or more skull sutures fuse prematurely, altering head shape. It can be classified as single or complex suture fusion. Types include scaphocephaly from sagittal fusion, trigonocephaly from metopic fusion, and brachycephaly from coronal fusion. Symptoms include abnormal head shape and lack of fontanelle movement. Causes may be genetic, biochemical or environmental. Treatment involves early surgical repair to reshape the skull.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    450 views36 pages

    Craniosynostosis

    Uploaded by

    Fathima Jansi Malapati
    Craniosynostosis is a birth defect where one or more skull sutures fuse prematurely, altering head shape. It can be classified as single or complex suture fusion. Types include scaphocephaly from sagittal fusion, trigonocephaly from metopic fusion, and brachycephaly from coronal fusion. Symptoms include abnormal head shape and lack of fontanelle movement. Causes may be genetic, biochemical or environmental. Treatment involves early surgical repair to reshape the skull.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    of 36

    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 craniosyntosis Complex 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: Scaphocephaly Trigonocephaly Plagiocephaly Brachycephaly 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 plagiocephaly Posterior 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 Craniosynostosis
    Types of Craniosynostosis :

    A. Sagittal synostosis
    B. Coronal synostosis C. Lambdoid synostosis

    D. Metopic synostosis
    E. 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

    CAUSES
    Biochemical 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 MANIFESTATION
    ABSENCE 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 pregnancy Risk factors during pregnancy Physical examination Radiographic 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: craniectomy iind 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

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