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    KLEEBLATTSSCHADEL

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    Kleeblattschadel syndrome is a rare skull malformation caused by premature fusion of cranial sutures, giving the skull a cloverleaf shape. It can cause intellectual impairment and hydrocephalus. Diagnosis involves examining the head, radiographs, and CT scans. Treatment aims to correct deformities and open airways through early surgery.

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    KLEEBLATTSSCHADEL

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    klyebuenafe
    5 views17 pages
    Kleeblattschadel syndrome is a rare skull malformation caused by premature fusion of cranial sutures, giving the skull a cloverleaf shape. It can cause intellectual impairment and hydrocephalus. Diagnosis involves examining the head, radiographs, and CT scans. Treatment aims to correct deformities and open airways through early surgery.

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    Oral parhology

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    Kleeblattschadel syndrome is a rare skull malformation caused by premature fusion of cranial sutures, giving the skull a cloverleaf shape. It can cause intellectual impairment and hydrocephalus. Diagnosis involves examining the head, radiographs, and CT scans. Treatment aims to correct deformities and open airways through early surgery.

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    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    5 views17 pages

    KLEEBLATTSSCHADEL

    Uploaded by

    klyebuenafe
    Kleeblattschadel syndrome is a rare skull malformation caused by premature fusion of cranial sutures, giving the skull a cloverleaf shape. It can cause intellectual impairment and hydrocephalus. Diagnosis involves examining the head, radiographs, and CT scans. Treatment aims to correct deformities and open airways through early surgery.

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    KLEEBLATTSCHADEL

    SYNDROME
    BY BILBAO,BUENAFE,DE LOS SANTOS
    I.INTRODUCTION/DEFINITION
    What is Kleeblattschadel Syndrome?
    KLEEBLATTSCHADEL SYNDROME IS A VERY UNCOMMON SKULL
    MALFORMATION THAT OCCURS WHEN MANY SUTURES BETWEEN
    A BABY'S SKULL FUSE TOO EARLY.

    IT IS A TYPE OF CRANIOSYNOSTOSIS THAT MAKES THE


    SKULL PROTRUDE AND BROADENS THE FACE.

    IT IS A SEVERE DEFORMITY THAT GIVES THE SKULL A


    CLOVERLEAF SHAPE

    IT IS RARE WITH LESS THAN 130 CASE REPORTS GLOBALLY


    II. STATISTICAL DATA /EPIDEMIOLOGY

    ASPECT INFORMATION

    Prevalence Epidemiological data on kleeblattschädel is limited due to its rarity, but it is


    estimated to occur in about 3-6 0ut of 10,000 live births.

    Environmental:
    maternal smoking, teratogen exposure, intrauterine constraint, fetal
    Predisposing Factors
    positioning
    Genetic:
    mutations

    Genetic Causes Almost 20% of all cases; most inherited in autosomal dominant fashion;
    new mutations in 50% of cases

    Types Non-syndromic: 75% of cases


    Syndromic: 25% of cases
    III. ETIOPATHOLOGY
    Kleeblattschadel sydrome a type of Classification is based on the affected
    Craniosynostosis from premature fusion of cranial suture:
    sutures, disrupting normal skull growth. •sagittal
    Genetic mutations and environmental factors like
    •coronal
    maternal smoking and teratogen exposure •metopic
    contribute to its development. •lambdoid being primary classifications.

    Genetic causes account for about 20% of cases,


    Clinical identification typically occurs within
    with most being inherited autosomal dominantly or
    arising from new mutations.Non-syndromic the first year of life.
    craniosynostosis affects 75% of cases, while
    syndromic craniosynostosis affects 25% and is
    associated with specific genetic syndromes
    IV. CLINICAL FEATURES
    The skull has a trilobed appearance
    with high forehead, beaked nose, and
    depressed premaxillary region
    Intellectual Impairment and
    Hydrocephalus is common.
    Neonatal death is frequent.

    Other features involve marked


    proptosis, exophthalmos, corneal
    ulceration in the eyes and elbow
    ankylosis, dysostosis multiplex,
    syndactyly of toes and fingers,
    restricted joint mobility in the limbs.
    Abnormal vertebral size has
    occasionally been reported.
    IV. ORAL FEATURES
    Trilobal skull configuration with downward
    displacement of the ears, facial deformity
    with hypertelorism, nasal flattening, jaw
    abnormalities, skeletal abnormalities.
    Reports of optic atrophy, respiratory
    problems, disorders of speech and hearing.

    Anterior 3D volume rendering images showing multiple cranial deformities status post
    craniotomies for hydrocephalus, as well as severe skeletal and occlusal abnormalities.

    Blindness, obstructed nasolacrimal ducts,


    absent external auditory canals,
    macrostomia, macroglossia, oblique facial
    clefting, cleft lip-palate, bifid uvula, highly
    arched palate, natal teeth, and
    malocclusion.

    Para-sagittal and Lateral 3D volume rendering images displaying prominent apertognathia


    and deficiency of the mid-maxilla with rotation of the maxillary alveolus superiorly
    V. DIAGNOSIS
    DIAGNOSTIC AIDS TO HELP YOU IDENTIFY KLEEBLATTSCHADEL SYNDROME

    EXAMINING AND MEASURING THE


    HEAD

    RADIOGRAPHS

    DIAGNOSTIC IMAGING
    EXAMINING AND
    MEASURING THE HEAD
    RADIOGRAPHS
    DIAGNOSTIC IMAGING
    OR CT SCANS
    VI. TREATMENT MANAGEMENT
    The major goals of corrective surgery
    eliminate the pressure on the brain
    correct midfacial deformities
    open nasopharyngeal airways,
    SURGERY
    Surgery must be done as early as possible
    Minimally invasive surgery for infants
    younger than 3 months to open skull
    sutures for normal skull and brain
    growth
    Traditional surgery for infants 6 months
    or older to correct the skull’s shape
    VII. RELATION TO DENTISTRY
    The relation of clover leaf skull in dentistry is that
    patients with craniofacial anomalies require
    orthodontic services as a direct result of the
    medical condition and as an integral part of the
    rehabilitative process.
    Whether a deformity is congenital, acquired or
    developed, maxillofacial treatment offers life-
    altering physical, cosmetic and psychosocial
    benefits
    3D reconstruction of the child’s skull which could be used to operate on and successfully
    repair the deformity in Brazil.

    Craniofacial orthodontics uniquely combines


    various disciplines within the medical community,
    including orthodontics, dentistry, head and neck
    oncology, speech therapy, congenital
    malformation reconstruction, plastic surgery, etc.
    for optimal results for each patient.
    REFERENCES:
    • HTTPS://WWW.DEEPDYVE.COM/LP/SPRINGER-JOURNAL/CROUZON-SYNDROME-WITH-KLEEUBLATTSCH-DEL-
    SKULL-SATHGA0NP0?KEY=SPRINGER

    • HTTPS://WWW.NCBI.NLM.NIH.GOV/BOOKS/NBK544366/

    • HTTPS://WWW.CHILDRENSNATIONAL.ORG/GET-CARE/HEALTH-LIBRARY/CLOVERLEAF-DEFORMITY?
    FBCLID=IWAR3ETQ9RSTTAUU68NLC6QPWMZYCOCPCY8OYDA1Z0BAY9WH5YKC5B_OUQB9E

    • HTTPS://L.MESSENGER.COM/L.PHP?U=HTTPS%3A%2F%2FRADIOPAEDIA.ORG%2FARTICLES%2FCLOVERLEAF- S
    KULL-CRANIOSYNOSTOSIS&H=AT0-U9CSU5B7QTJHWN3RFVRG-ZKN1JAOLOCJ1TQYMIYXVDPTXPHVPSFKDKELJXJF6-
    RKUISJ4WDM8BTLV0UMHNT5JN4Y_OVP7MJJCZZ_PURPEBV4H6NVMRFDTRHESVMOSTNHHNMAKRDWU_RZJIH_GA

    (PDF) KLEEBLATTSCHÄDEL SKULL: A REVIEW OF ITS HISTORY, DIAGNOSIS, ASSOCIATIONS, AND TREATMENT
    (RESEARCHGATE.NET)

    HTTPS://WWW.SEMANTICSCHOLAR.ORG/PAPER/CLOVERLEAF-SKULL-SYNDROME-

    HTTPS://ACCESSANESTHESIOLOGY.MHMEDICAL.COM/CONTENT.ASPX?BOOKID=852&SECTIONID=49517406

    HTTPS://WWW.HINDAWI.COM/JOURNALS/IJD/2012/145749/
    QUIZ
    1. What is Kleeblattschadel Syndrome? 4. What percentage of Kleeblattschadel Syndrome
    a) A common skull malformation cases are attributed to genetic causes?
    b) A rare skull malformation resulting from premature a) 5%
    fusion of cranial sutures b) 20%
    c) A type of brain disorder c) 50%
    d) A genetic syndrome affecting limb development d) 75%

    2. What is the estimated prevalence of Kleeblattschadel 5. What are the primary types of craniosynostosis?
    Syndrome? a) Sagittal, coronal, metopic, and lambdoid
    a) 1 in 100 live births b) Occipital, temporal, frontal, and parietal
    b) 1 in 1,000 live births c) Central, peripheral, axial, and appendicular
    c) 3-6 out of 10,000 live births d) Anterior, posterior, lateral, and media
    d) 1 in 1,000,000 live births es

    3. Which of the following is NOT a predisposing factor for


    Kleeblattschadel Syndrome?
    a) Maternal smoking
    b) Teratogen exposure
    c) Fetal positioning
    d) Neonatal nutrition
    QUIZ
    1. What is Kleeblattschadel Syndrome? 4. What percentage of Kleeblattschadel Syndrome
    a) A common skull malformation cases are attributed to genetic causes?
    b) A rare skull malformation resulting from premature a) 5%
    fusion of cranial sutures b) 20%
    c) A type of brain disorder c) 50%
    d) A genetic syndrome affecting limb development d) 75%

    2. What is the estimated prevalence of Kleeblattschadel 5. What are the primary types of craniosynostosis?
    Syndrome? a) Sagittal, coronal, metopic, and lambdoid
    a) 1 in 100 live births b) Occipital, temporal, frontal, and parietal
    b) 1 in 1,000 live births c) Central, peripheral, axial, and appendicular
    c) 3-6 out of 10,000 live births d) Anterior, posterior, lateral, and media
    d) 1 in 1,000,000 live births es

    3. Which of the following is NOT a predisposing factor for


    Kleeblattschadel Syndrome?
    a) Maternal smoking
    b) Teratogen exposure
    c) Fetal positioning
    d) Neonatal nutrition

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